ACPA Annual Meeting 2021 Abstracts

Amy Edmonson, Harvard Business School

Methods/Description: In the complex, fast-paced, uncertain world of health care delivery, teaming that unfolds as a dynamic learning process is more important than ever. This session will explain the concept of teaming, how it is different from teams, and what it takes to do it well. Everyone today knows that diverse experts must come together to deliver great care. But teamwork across roles doesn’t always go smoothly. I will describe why teaming thrives in a climate psychological safety—to ensure that everyone feels able to take the risks of speaking up and learning fast together—and depends on simple process skills.

(1) Augusta University—Section of Plastic Surgery, Augusta, GA, (2) Children’s Healthcare of Atlanta, GA, (3) Division of Plastic Surgery Phoenix Children’s Hospital, Barrow Cleft and Craniofacial Center, Phoenix, AZ, (4) Children’s Hospital Los Angeles, CA, (5) The Craniofacial Center, Dallas, TX

Background/Purpose: Educational Objectives: Cognitive: The attendees will understand what is ERAS and how to develop it. Psychomotor: The attendees will be able to organize and implement ERAS. Affective: The attendees will appreciate the importance and the value of AI and neural network in cleft and craniofacial care.

Methods/Description: There is a need to adopt Enhanced Recovery after Surgery, or ERAS, in cleft and craniofacial care. Developed in the 1990s, ERAS was the result of sustained and systematic approach to improve surgical outcomes and to reduce complications in colorectal and cardiothoracic surgery. Due to the tremendous success, both the AMA and ACS have accepted ERAS in recent years. However, to date, very few ERAS protocol or pathways exist in cleft and craniofacial care. The ASCFS established a presidential taskforce during its 2019 annual meeting in Tucson, Arizona, to address this. The proposed panel will provide the audience with information to bridge a critical gap in our knowledge and practice. The ASCFS taskforce has developed ERAS protocols for 5 commonly performed cleft and craniofacial procedures: lip repair, palatoplasty, alveolar bone graft, Le Fort I advancement, and FOAR. These ERAS protocols will be available to all attendees. As the second decade of this century closes, advances in AI, such as machine learning and neural network, have drastically transformed how we deliver health care in isolated areas. But the application will spread. The ability to mimic human cognition coupled with “Big Data,” NSQIP, HCUP, and NCHS, to name just a few, will propel cleft and craniofacial care in ways we cannot even imagine at present. It is time that we take the first step.

(1) The Children’s Hospital at Westmead, Sydney, New South Wales

Background/Purpose: Children with orofacial clefting conditions are at increased risk of middle ear pathology and hearing loss which requires a coordinated protocol of care and management. The aim of this study was to better understand and describe the profile of hearing loss in a group of children with orofacial clefting to better inform clinical recommendations for audiological surveillance, surgical management of ear pathology, and overall protocols of clinical care.

Methods/Description: A retrospective review of hearing outcome data was conducted for a group of 338 children, aged 5 years, with surgically repaired, nonsyndromic cleft lip and/or palate conditions, attending a tertiary referral hospital’s cleft palate team between 2001 and 2019. Digital audiogram data was obtained from the electronic medical record and matched to the patient’s cleft condition, syndromic status, cleft surgery history, including surgery for pressure-equalization (PE) tube insertion. Data were analyzed to inform current (and changing) clinical practices.

Results: Fifty-four percent (181/338) of patients obtained normal hearing sensitivity bilaterally by 5 years of age, with no difference in children with isolated cleft palate and those with cleft lip and palate. Of these patients with normal hearing sensitivity, 66% (120/181) required no PE tube insertion while the remaining 34% received between 1 and 4 sets of PE tubes. Of the 46% (157/338) of patients with abnormal hearing in at least one ear, only 39% received surgical intervention in the form of PE tubes, again receiving between 1 and 4 sets of tubes. The remaining 94 patients received no surgical intervention for their documented hearing loss.

Conclusions: This study has demonstrated that only half of the children (54%) with nonsyndromic cleft lip and/or palate conditions obtained normal hearing bilaterally by approximately 5 years of age. While it is important to note that two-thirds of these children required no surgical intervention to support this finding, one-third of children required some degree of surgical management in the form of PE tubes. It is concerning that 61% of children with abnormal hearing at 5 years of age had not received any surgical intervention for management of their documented hearing loss. Additionally, the 39% who did receive surgical management continued to present with hearing loss at this age. These results indicate the need to review the audiological management of children in this cohort and support the need for highly structured audiological surveillance to remain as an integral part of multidisciplinary cleft and craniofacial clinical care protocols.

(1) Osaka University Graduate School of Dentistry, Suita, Osaka, Japan (2) Fukuoka Dental College, Fukuoka, Japan

Background/Purpose: We focused on characterized expression of extracellular matrix during soft palate development, including Periostin (Postn) and Tenascin C (TnC). The immunohistochemical expression of Postn and TnC appeared to be wider and more clearly defined in posterior palatal mesenchyme before/after palatal fusion. However, there is little understanding regarding the role of the characteristic expression pattern of extracellular matrix protein through anterior–posterior palatal shelves.

Methods/Description: Using immunohistochemical analysis, the expression of Postn and TnC was examined in Wnt1-cre;Tgfbr2fl/fl, K14-cre;Tgfbr2fl/fl, and Tgfb3-/- mice. Quantitative real-time PCR was performed to investigate Postn and TnC expression in mouse cranial neural crest cell line (O9-1) and primary embryonic palatal mesenchymal cells (MEPM). Secreted protein in culture medium was also quantitated by ELISA.

Results: In immunohistological expression of Postn and TnC in TGF-β signaling deficient mice, Wnt1-cre;Tgfbr2fl/fl K14-cre;Tgfbr2fl/fl and Tgfb3-/-, Postn expression showed no changes in all categories of mice. Interestingly, TnC expression was diminished in K14-cre;Tgfbr2fl/fl and Tgfb3-/- mice exhibiting soft palate cleft, but not in Wnt1-cre;Tgfbr2fl/fl mice. These results indicate that TnC expression in soft palate mesenchyme during palatogenesis is regulated by TGF-β signaling of palatal epithelium. In O9-1, Fibronection, Postn, and TnC mRNA expressions were stimulated by TGF-β3, but not in MEPM. The O9-1 stimulated by TGF-β3 exhibited a fibroblast cell shape. ELISA confirmed that secreted TnC protein in cultured medium of O9-1 was also increased. Those responses were attenuated by the existence of inhibitor inter-cellular signaling of TGF-β, Smad, and p38.

Conclusions: These results indicate that Postn and TnC is an important extracellular matrix protein in soft palate development. It is suggested that TnC expression in palatal mesenchyme is paracrine regulated by TGF-β in the palatal epithelium, and plays a role in the differentiation of fibroblasts in immature palatal mesenchymal cells.

(1) Baylor College of Medicine/Texas Children’s Hospital, Houston, TX, (2) Smile Train, New York, NY

Background/Purpose: International cleft lip and palate repair surgeries often occur in settings where nurses have low professional status and inadequate training. However, skilled nursing supports optimal postoperative outcomes following cleft surgery. Although postoperative sentinel events occur at very low rates for patients with clefts, they are most frequently respiratory in origin and most likely to occur when nurses are the primary care givers. The provision of adequate postoperative monitoring and nurse-initiated interventions should complications arise is imperative. Recognizing that in resource-limited settings, nurses may lack the abilities and confidence to provide optimal care following cleft surgery, the international cleft charity Smile Train developed a training program to teach nurses the necessary skills to anticipate, recognize, intervene in, and reduce postoperative complications. Knowing that empowerment and high morale are drivers for successful nurse-led intervention, another goal of this program is to improve the (self-) perception of nursing capability. This presentation will outline nursing-related problems faced in postoperative cleft care in resource limited settings and present a program aimed to address these challenges.

Methods/Description: In 2011, Smile Train launched a 3-day, classroom-based program: “Nursing Care Saves Lives.” This presentation will highlight the program’s curriculum, which involves lectures, skill-building exercises, simulation, and group problem-solving centered around 4 pillars: patient assessment, postoperative nursing care, postoperative complications, and postoperative nursing interventions. Particular focus is given to early recognition of postoperative complications and essential nurse-initiated actions. Pre- and post-program tests are administered to identify knowledge gaps and assess improvement. Participating nurses are expected to develop individualized action plans to improve nursing care after the course. Additionally, nurses are instructed how to disseminate their new knowledge among their colleagues on return to their home hospitals. Since its inception, the Nursing Care Saves Lives course has been taught in 24 countries and directly trained over 1500 nurses. Nurses have consistently reported increases in knowledge and skills following the course. Through these improvements, this course has the potential to not only improve the postoperative care patients receive but also to increase nurses’ confidence and normalize nurse-led intervention in countries with historic stigmatization of the nurse’s role. Further measures of program success might include improvements in (1) the level of postoperative monitoring, (2) the number of nurse-initiated interventions, and (3) the rate of preventable respiratory complications following cleft surgery.

(1) Shriners Hospitals for Children—Chicago and University of Illinois at Chicago, IL, (2) Nationwide Children’s Hospital, Columbus, OH, (3) The Hospital for Sick Children, Toronto, Ontario, Canada (4) Lancaster Cleft Palate Clinic, Lancaster, PA, (5) Shriners Hospitals for Children, Chicago, IL, (6) University of Iowa Children’s Hospital, Iowa City, IA, (7) Children’s Medical Center, Dallas, TX

Background/Purpose: Children born with cleft lip and/or palate (CL/P) are vulnerable to social difficulties including bullying and social inhibition, likely due to speech and/or facial appearance differences. Negative social experiences can impact overall psychosocial functioning and may increase risks for anxiety, depression, and poor quality of life. However, few studies have examined children’s self-perceptions of social adjustment and stigma, while accounting for differences by sex or cleft type. To address these gaps, this study examined social adjustment and stigma in a large cohort of children with CL/P recruited from multiple centers in North America.

Methods/Description: As part of a larger study examining cleft care outcomes, 363 children ages 8 to 10 years old (50% male) with a confirmed diagnosis of CL/P (18% CP, 67% CLP, 15% CL) were consecutively recruited across 6 sites. Children completed the Pediatric Quality of Life Inventory (PedsQL) Social, Emotional, and School Subscales, PROMIS Stigma Scale, and the CLEFT-Q Social, Psychological, and School Subscales in conjunction with a clinic visit. Analyses examining differences in social adjustment and stigma by diagnosis and sex were completed using multivariate analysis of variance (MANOVA). Percentages of children having at-risk scores on the PedsQL and PROMIS Stigma Scale were determined using published cutoff points in the general population; comparisons to published norms of the CLEFT-Q were calculated.

Results: MANOVA results indicated that children with CP reported less stigma than children with CL and CLP with the difference reaching significance for CLP (P = .006). On the PedsQL Emotional Subscale, children with CL reported better functioning than children with CP and CLP with the difference reaching significance for children with CLP (P = .006). No significant differences by diagnosis or sex were found on the other PedsQL or CLEFT-Q subscales. On the PedsQL, 40% of the sample scored below the cutoff on the Emotional Subscale; 23% on the School Subscale, and 9% on the Social Subscale. On the PROMIS Stigma Scale, 9% fell in the at-risk range. For the CLEFT-Q subscales, 9% of the study children fell at least 1 SD below the mean on the Psychological Scale; 15% were below on the School Scale and 14% on the Social Scale.

Conclusions: This study observed few significant differences among children based on cleft diagnostic group or sex, although children with CLP appeared to be most vulnerable to stigmatization and emotional issues. Across the sample, risks for emotional, school, and social functioning were noted, with some children reporting lower scores on the CLEFT-Q compared to cleft population norms. These findings underscore the need for routine screening in these domains across diagnoses and specifically among youth with CLP and illustrate the benefits of using both generic and cleft-specific instruments for assessments.

(1) University of Iowa Children’s Hospital, Iowa City, IA, (2) The University of Iowa, Iowa City, IA

Background/Purpose: The first brain imaging study reporting subtle brain volume differences in adult males with iCL/P was published in 2000. Twenty years later, research has extended this work to young adults, children, and even (in 2 small studies) infants. Research has also found associations between these subtle volumetric differences and academic, behavioral, and cognitive functioning. The purpose of the current study is to utilize the largest database of structural MRI scans on children, adolescents, and young adults to evaluate longitudinal trajectories of global and regional brain growth in iCL/P.

Methods/Description: In all, 134 participants with iCL/P (33 iCLO, 34 iCPO, and 67 iCLP) and 144 unaffected controls, ages 7 to 27 years of age underwent cognitive assessment (Wechsler scales) and structural MRI scans between November 2002 and February 2013). Ninety-seven participants returned for a second visit and 17 had a third visit. A total of 392 evaluations were obtained (184 iCL/P and 208 unaffected), with mean age at evaluation of 15.2 (SD = 4.7) and 56% males. Mixed linear models evaluated the effect of sex, cleft, age, and the interaction of cleft and age on cognitive index scores as well as global and regional measures of gray and white matter.

Results: Cognitive index scores were in the average range for participants with iCL/P (FSIQ = 108 [15]) and high average for unaffected participants (FSIQ = 115 [16]). Verbal IQ – Performance IQ difference scores were low (< 4 points difference on average) across all subgroups except for boys with iCPO, where Verbal IQ was on average 16 points below Performance IQ. Significant main effects of group was found in cerebellar gray matter and the striatum; participants with iCL/P had lower volume in both regions. Significantly different growth trajectories were identified for participants with iCL/P in ICV, parietal white matter, temporal gray matter, and thalamus volumes. Subsequent analyses in regions with significant group and interaction effects did not find cleft type differences.

Conclusions: Globally, participants with iCL/P start with lower volumes in childhood and have steeper growth trajectories, resulting in some catch-up by young adulthood (early twenties). Growth trajectories for cerebral white and gray matter were different in parietal and temporal lobes. Volume in the cerebellum is consistently lower for participants with iCL/P. Cleft type differences were not identified in brain volumes but in cognitive data, boys with iCPO had a statistically and clinically significant difference between verbal and performance index scores (VIQ < PIQ). To best understand the etiology and potential effect of these differences, future research needs to enroll larger samples to evaluate sex and cleft type effects, utilize functional imaging measures, connect imaging data with standardized assessment, and extend the age range down to infancy. Replication of these studies has started within the past 10 years and should continue to ensure the validity of findings.

(1) The Children’s Hospital of Philadelphia, Philadelphia, PA

Background/Purpose: Previous literature has documented craniometric changes of the bony calvarium, increases in intracranial volume and the resolution of Chiari malformations from posterior vault distraction osteogenesis (PVDO). No studies have analyzed changes to the soft tissue envelope from PVDO. In this study, we aim to provide objective measurements of scalp thicknesses in patients undergoing PVDO, utilizing facial soft tissue thickness as a proxy to control for growth. We hypothesized that the soft tissues of the scalp are not made thinner by the distraction process either in the region of distraction or in neighboring areas.

Methods/Description: Subjects who underwent PVDO were identified by CPT billing codes and inclusion into the study required a craniosynostosis diagnosis and high resolution pre and postoperative CT scans within 100 days of PVDO. CT scans were analyzed on Materialize Mimics v21 (Materialise, Ghent, Belgium). In Mimics, bone and soft tissue maps by were created by software assisted manual segmentation. Six key craniometric landmarks—glabella, pogonion, zygion, vertex, euryon, and opisthocranium—were identified on the 3D bone masks with the head in the Frankfort horizontal and overlaid onto the soft tissue masks. The segmented masks and identified points were exported into 3matics software where soft tissue thickness “heat” maps were generated. Tissue thickness was measured 5 times for each craniometric point and the average of the 5 measurements was used for analysis. Pre and postoperative thicknesses were compared using appropriate statistics, and facial landmark thicknesses were utilized as controls for growth.

Results: Thirteen syndromic craniosynostosis patients with a median age at PVDO of 1.32 years (0.45, 3.82) met inclusion criteria. Nine patients were female (69.2%), 11 (84.6%) were Caucasian, 2 patients (15.4%) had bicoronal craniosynostosis, while the remaining 11 (84.6%) had multisuture disease. Percent change in thickness at pogonion and glabella were used to create a correction factor to account for growth (Point*(1-.062)) and applied to the remaining points. Thicknesses for the pre and post PVDO cohorts did not differ significantly at glabella, pogonion, opisthocranium, vertex, and zygion. The corrected median thickness at euryon was significantly increased in our cohort post-PVDO (2.67 mm [2.49, 4.02] vs 5.26 mm [3.83, 7.82], P = .002), but this was shown to be due to postoperative edema as subgroup analysis only including patients who had a postoperative scan greater than one day after PVDO demonstrated no significant difference (preoperatively 3.79 mm [2.2, 4.14]; postoperatively 4.03 mm [3.00, 5.18]; P = .138).

Conclusions: This is the first study to quantify changes in soft tissue thicknesses pre- and post-operatively in patients undergoing PVDO, demonstrating maintenance of scalp thickness in all measured regions of interest. This data is important, as a healthy soft tissue envelope is paramount when performing future cranial and midface expansion.

(1) Nationwide Children’s Hospital, Columbus, OH

Background/Purpose: Health care providers involved in multidisciplinary 22q deletion syndrome clinics are often referred patients or contacted by parents of children with chromosomal microarray abnormalities involving the 22q11.2 region that are not the typical recurrent deletions associated with 22q11.2 deletion syndrome. These providers should be familiar with the basic principles of microarray interpretation, especially in the context of copy number variants (CNV) in the 22q11.2 region. Nationwide Children’s Hospital (NCH) performs ∼1500 chromosomal microarrays per year. Review of NCH laboratory and institutional experience will help to characterize atypical deletions and identify any recurrent challenges or trends.

Methods/Description: Methods: Two laboratory genetic counselors reviewed all postnatal chromosomal microarrays (n = 25,667) performed in the NCH cytogenetics laboratory dating from 2009 (when oligonucleotide platform was introduced) to the present. Any microarray with a deletion involving the 22q11.2 band met the inclusion criteria. A microarray result was determined to be an atypical deletion if it involved the 22q11.2 band but was not either the common 3 Mb or 1.5 Mb recurrent deletion that include TBX1 and HIRA. For atypical deletion patients, genotype and phenotype characteristics were recorded. Results: 136 microarray results meeting inclusion criteria were identified. Hundred were typical 22q deletions representing 22q11.2 deletion syndrome; 36 were atypical deletions. Genotype and phenotype information were available for 32 of these 36 patients. Of the microarray findings, 8 were likely pathogenic (LP), 18 variants of unknown significance (VUS), and 8 likely benign copy number variation (LBCNV). Two patients had 2 separate CNVs both involving the 22q11.2 region. Where inheritance could be determined, 1 of 1 LP was de novo, 5 of 6 VUS were inherited, and 3 of 3 LBCNV were inherited. Mean size of CNVs were as follows: LP 1310 ± 857 kb, VUS 55.9 ± 20.4 kb, and LBCNV 30.8 ± 4.6 kb. Five out of 8 LP results were the same ∼700 kb deletion spanning from breakpoints LCR22-B to LCR22-D. A 29.73 kb deletion that includes the 3′ portion of the putative gene c22orf25 was identified in 12 patients and categorized as a VUS 7 times and LBCNV 5 times. A 37.58 deletion that includes the 3′ end of TANGO2 and the 5′ end of DGCR8 was identified in 4 patients and categorized as VUS twice and LBCNV twice. Conclusions: Deletions involving the 22q11.2 region are found frequently on clinical chromosomal microarray analysis. In our single institutional experience, a typical recurrent deletion was found approximately 3 times more commonly than an atypical deletion. Although less common than typical deletions, a few recurrent findings in this study provide insight into the ongoing challenge of the clinical interpretation of atypical 22q deletions.

(1) University of Central Florida, Orlando, FL, (2) East Carolina University, Greenville, NC, (3) Nationwide Children’s Hospital, Columbus, OH

Background/Purpose: Dysmorphology in velopharyngeal structures has been shown to have significant negative implications on speech among individuals with 22q11.2 deletion syndrome (22q). Velopharyngeal dysfunction associated with 22q has a complex etiology. MRI investigations so far in the 22q population have reported variations in velopharyngeal structures among individuals with 22q, including a short, thin, asymmetric levator muscle with an increased angle of origin and a significantly shorter origin-to-origin distance. However, the majority of these studies utilized sedation, limiting the information to static data. As such, no studies to date have assessed the internal velar musculature during speech activity in children with 22q. The purpose of this study was to evaluate variations of the velopharyngeal mechanism during rest and sustained phoneme production in children with 22q, using an innovative, unsedated MRI protocol.

Methods/Description: Ten children with 22q and 10 children with normal velopharyngeal anatomy (ages 4-12 years) participated in the study. All participants were native English speakers and were imaged in the supine position using a Siemens 3 tesla MRI scanner. Images were collected during rest and sustained phoneme production. There was no use of sedation for the MRI exams. Paired t tests were used to assess variations between rest and sustained phoneme production conditions. Independent samples t tests were used to examine differences between experimental (22q) and control (children with normal anatomy) groups.

Results: Magnetic resonance imaging data were successfully obtained using the child-friendly scanning protocol. Quantitative and qualitative differences of the levator muscle and associated velopharyngeal structures were noted across both tasks and between the two groups. The levator muscle contracted differently across different phonetic contexts. Levator muscle length and angle of origin were reduced on the sustained phonation (P < .05). Across all subjects, the length of the levator muscle reduced going from rest to /i/ production. However, preliminary results suggest that the change was less evident in the 22q group compared to the control group, which may due to hypoplasia of the levator muscle. For levator angle of origin, the angle was reduced going from rest to /i/ production across both groups, however, the change was more evident in the 22q group compared to the control group.

Conclusions: Results from this study suggest that individuals with 22q present with unique velopharyngeal muscle variations that may contribute to the high rate of velopharyngeal dysfunction associated with this syndrome. This is the first study to demonstrate the successful utilization of MRI in obtaining functional 3D imaging data on velopharyngeal structures in children as young as 4 years with 22q, without the use of sedation.

(1) University of Pittsburgh, School of Dental Medicine, Orthodontic Residency Program, Pittsburgh, PA, (2) University of Pittsburgh School of Medicine, Pittsburgh, PA, (3) Case Western Reserve University School of Medicine, Cleveland, OH, (4) Children’s Hospital of Pittsburgh, Pittsburgh, PA

Background/Purpose: Maxillary hypoplasia in patients with cleft lip and palate may be intrinsic or related to growth restriction from surgical intervention. Protraction headgear (PHG) is used in early to late mixed dentition to treat maxillary deficiency. There is little literature discussing the long-term prognosis of PHG in these patients and whether PHG correlates with a decreased incidence of orthognathic surgery (OS). The aim of this study is to assess cephalometric parameters and the need for OS and the relationship with compliance in PHG therapy.

Methods/Description: Forty-three patients with nonsyndromic cleft lip and palate and history of lip and palate repair were treated with PHG and orthodontic treatment. PHG lasted about a year with 170-g elastics worn daily for at least 12 hours. Cephalometric measurements at initial (T1), post-PHG (T2), and presurgical or postorthodontic treatment (T3) of at least age 15 for females and 17 for males and the presence of OS were compared. Chi-square and 2-sample t tests were used.

Results: In all, 77% (33) of patients reported compliance with therapy. Of those compliant, 55% (18) had OS and 45% (15) did not. There was no significant association between PHG compliance and OS (P = .42). Inter-group comparisons show that there was no difference at T1. In the compliant group at T2, U1-SN proclined 12° (P = .04), interincisal angle increased 56° (P = .02), and overjet increased 5 mm more (P < .01) compared to the noncompliant group. Upper incisors remained 9° more proclined at T3 (P = .02). Patients who had OS, the upper lip was 3 mm closer to E-plane (P = .02) and nasolabial angle 13° smaller (P = .01) at T1. At T2, the lower lip to E-plane was 2 mm more (P < .01) for the OS group than the non-OS group. At T3, the OS group had ANB 4° (P < .01), convexity 8° (P < .01), Wits 6 mm (P < .01), and overjet 10 mm (P < .01) less than non-OS group; SNB 3° (P = .02) and lower lip protruded 5 mm more (P < .01). Intra-group comparisons show compliant patients gained 7° U1-SN and 7 mm overjet versus 3° and 1 mm in the noncompliant group from T1-T2. Overjet reduced 4 mm in the compliant group from T2-T3. The OS group gained 5° U1-SN and 5 mm overjet versus 7° and 7 mm in the non-OS group from T1-T2. From T2-T3, the OS group lost 7 mm overjet and U1-SN proclined 15° while the non-OS group gained 1 mm overjet and 14° U1-SN.

Conclusions: Patients compliant with PHG showed no difference in the need for OS compared with those that were not. Compliance with PHG proclines upper incisors and creates positive overjet, however, overjet does not remain. This indicates that PHG compliance did not avoid OS and though it may create positive overjet, it is not stable.

(1) Children’s Mercy Hospital, Kansas City, MO, (2) Children’s Hospital of Philadelphia, PA, (3) Emory University, Atlanta, GA, (4) UC Davis, Sacramento, CA, (5) Children’s Minnesota, Minneapolis, MN

Background/Purpose: The most common types of surgery for velopharyngeal dysfunction (VPD) are pharyngeal flap, sphincter pharyngoplasty, Furlow palatoplasty, and more recently buccal myomucosal flap. These surgeries either add length to the soft palate, add bulk to the posterior pharyngeal wall, or physically block space in the back of the nose to prevent air escape with speech. Common to all these surgeries is the risk of airway obstruction and subsequent obstructive sleep apnea (OSA). Currently, there is no consensus on necessary airway workup and management either before or after VPD surgery, leading to a myriad of different practice patterns. While OSA is a common side effect of these procedures (pharyngoplasty more so than palatal lengthening), there are limited data on the treatment options and outcomes of these treatments for children with OSA following speech surgery. The aim of this panel discussion will be to engage providers on different approaches to airway management before and after speech surgery, to assess advantages and disadvantages of these approaches, and explore options for management of OSA when present postoperatively.

Methods/Description: Case-based presentations will be used to guide the panel discussion. Complex cases will be presented and discussed that explore various aspects of airway management for children undergoing surgery for VPD as well as highlight the current variability that exists between providers on this topic. Continued dialog among the panelists and the audience using a case-based discussion will address the following topics: (1) How do you screen patients for obstructive airway symptoms preoperatively? (2) What are specific considerations for airway management in patients with 22q11.2 deletion syndrome or Robin sequence? (3) Does information from nasopharyngoscopy/video fluoroscopy help to assist you in airway management for patients considering VPD surgery? (4) Do you obtain preoperative or postoperative sleep studies for patients undergoing VPD surgery? (5) Which patients benefit from staged adenotonsillectomy prior to VPD surgery? (6) What precautions during VPD surgery do you take to hopefully prevent the development of OSA? Lastly, a case-based discussion will allow panelists to discuss and debate management options for patients who have developed OSA following VPD surgery. Complex/advanced OSA management will be discussed including drug-induced sleep endoscopy and its utility in surgical decision-making. We will encourage audience participation at this time, taking questions and hearing opinions of those in the audience.

(1) Al Jalila Children’s Hospital, Dubai, United Arab Emirates

Background/Purpose: Primary surgical correction of the cleft nasal deformity is challenging. The cleft side nasal lining and cartilage is slumped and tethered laterally to the pyriform base. Current primary nose correction techniques are unsatisfactory in the long term, with a significant rate of relapse. The author has observed that nasal lining is tight and deficient, with clear differences between the normal, unilateral, and bilateral cleft nose deformity. The aim of this study is to describe the anatomical relationships of the nasal lining to the cartilage in a cleft nasal deformity, with a view to comprehensive long-term surgical correction.

Methods/Description: The two dimensional topography of the anterior nasal airway lining was observed and mapped in a normal patient, patient with unilateral cleft lip, and bilateral cleft lip. Nasal endoscopy was used to define the nasal lining sub units and vestibule where critical differences were observed. The vestibular nasal subunit was noted to be distorted and deficient in the patient with unilateral and bilateral cleft lip. This subunit had a triple structural overlap (Lateral crus, vestibule, and alar units). Pre and post-operative anthropometry measurements (nasal width, nasal projection, nostril width, and nostril height) were obtained, and repeated at 3 and 6 months follow-up.

Results: Nasal lining subunit based corrective strategy was executed in 2 patients with unilateral and bilateral cleft lip. The lateral crural tethering was released and differential repositioning of the cartilage/lining complex performed. The difference in domal height between the cleft and noncleft sides was translated into a superior and medial advancement of the cartilage/lining composite subunit. The vestibule sub unit defect was resurfaced with a vermilion full thickness graft, taken at the time of primary cleft lip repair. Primary septal relocation was performed and no percutaneous cartilage sutures or nasal tip dissection was done. Complete nasal correction was seen in the unilateral and bilateral cleft lip patient. Columella height increased in the patient with bilateral cleft lip. At 3 months and 6 months follow-up, nasal anthropometry parameters are maintained and no nasal stenosis or relapse of deformity noted.

Conclusions: A novel nasal cartilage/lining subunit classification is proposed and forms the basis for a surgical strategy addressing comprehensive correction of the unilateral and bilateral cleft nasal deformity. The unilateral and bilateral cleft nasal correction is stable at 6 months.

(1) University of California San Diego, CA, (2) University of California San Francisco, CA

Background/Purpose: Giant cell granuloma (GCG) is a rare fibro-osseous benign lesion which is seldom observed arising in the nasomaxillary region. The authors report a unique case of a rapidly growing GCG of the nasomaxillary region of a 9-year-old male that rapidly recurred following initial resection and was resistant to medical therapy.

Methods/Description: Repeat resection of the tumor was performed, and genetic analysis of the tumor was performed. The defect of the naso-orbito-ethmoid region was reconstructed primarily, using split calvarial bone grafts and a medpor orbital floor implant. Stents were placed to reconstruct the nasolacrimal duct. There is no evidence of recurrence at 5 months postoperatively.

Results: Genetic analysis of the tumor using the UCSF500 Cancer Gene Panel test revealed the presence of mutations in smoothened (SMO) and phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha (PIK3CA). Sirolimus treatment was initiated prior to the repeat resection, but failed to stop tumor progression.

Conclusions: GCGs are locally destructive lesions that can grow rapidly and can recur aggressively following initial resection. The mutations reported here, have not been previously described in GCG, and further studies are warranted to determine whether the sonic hedgehog and mTOR signaling pathways are a potential therapeutic targets or biomarkers for tumor aggressiveness and prognosis in GCG.

(1) University of Virginia, Charlottesville, VA, (2) East Carolina University, Greenville, NC, (3) Consultant, Charlottesville, VA

Background/Purpose: Technological advances are transforming the research process, particularly in data acquisition and data analyses. Difficulties with data standardization and data acquisition often arise when coordinating multisite research studies. SpeechSampler is a program developed for Windows-based systems to standardize the methods for obtaining speech samples of subjects for any given research study. The purpose of this project was to develop a platform to facilitate standardized data collection across research sites. This platform has been developed to facilitate multisite studies requiring data acquisition for speech, resonance, oral structures, and MRI data. We present this platform as an open-source tool for craniofacial researchers engaging in multisite studies.

Methods/Description: The core functionality of the program was written in Powershell. The program allows researchers to record individual participant results, through a menu-based system, for consistent data acquisition across participants and sites. Categorical data are collected for participant demographics and oral exam structures. For speech sample data acquisition, the program displays visual and audio stimuli to the participant and automatically initiates speech sample recordings. The current program utilizes the CAPS-A-AM stimuli to collect data at the sentence and conversational level. Audio and video stimuli were recorded by an individual with a neutral dialect and stimuli were embedded into the program for consistency of stimuli presentation across research sites. Standardized naming conventions are utilized and automatically applied to all data collected. Participant results are saved on the user’s desktop and automatically organized and named according to Site, Subject, and the timestamp of file creation. All recorded video files are consistent in duration and content to achieve consistency for review and analysis of speech samples. A cumulative results spreadsheet is appended to each subject with the results of the data collection. Individual site results are then able to be aggregated and shared with the approved cloud/server storage system for easy, centralized analysis by the research team. The program additionally includes an MRI training module to condition participants for research MRI scans.

Results: A standardized, computer-based application was developed for Windows-based computers. Average data acquisition time, per subject, is estimated at 15 minutes. The tool has currently been disseminated to 3 research sites for further implementation, development, and testing. Advantages of using a standardized data collection tool include improved data quality, consistency, and reduction in data acquisition time.

Conclusions: This tool is a promising development for multisite MRI-based research for the collection and analysis of demographic data and speech/resonance data. Preliminary results show promise for the use of this tool to facilitate a standardized process for data collection across research sites.

(1) Yemen Global Smiles, Sanaa, Yemen (2) Mohammed, Muscat, Oman

Background/Purpose: Minimal blood loss in palatoplasty can be achieved with adrenaline saline hydro dissection, and a good knowledge of key anatomical structures as well as the stubborn areas of static resistance, where dissection is difficult because of fixed tissue planes. With the advent of minimal incision palatoplasty, button-hole, and medial-only incision procedures, the amount of blood loss is significantly reduced in most palate operations. The 2-stage palate repair also leads to less blood loss and is a popular concept among cleft groups worldwide following the initial promotion by Swedish cleft groups at Gothenburg. The learning curve is reasonable and techniques can be added, as one gains more experience with cleft palate surgeries.

Methods/Description: The samples we have chosen for this study from our humanitarian smile mission experience show different examples of how minimal blood loss palatoplasty was performed in our missions over the years (courtesy Yemen Global Smiles). Most of the patients were latecomers in palatoplasty (after 3 years of age). For this, we have chosen a relatively bloodless method using 1: 500 000 saline adrenaline hydro dissection by mixing 1 mg adrenaline in 500 mL saline. The distension allows for an easy approximation of flaps. Using fine angled Potts scissors, the levators are dissected from their insertion on the palatal aponeurosis/bony hard palate and repositioned in a transverse position from their abnormal sagittal orientation; the uvular is pared, and two-third retro positioned. The complete (larger Veau 2, to 4) palate is closed in 2 stages—stage one includes soft palate closure with levator retro positioning; the stage 2 minimal incision palatoplasty is done after a year or year and a half. Stage 2 hard palate closure is often easier because of the spontaneous narrowing of the palate after stage one. A hemi flap, hinge palate repair is done. In the second stage of palatoplasty, we have identified some areas of ‘Résistance Statique (RS), (static’ resistance) and suggest ways to solve the problems by releasing the tight tissue planes with fine dissection. Static resistance areas are found in the following zones—near posterior spine area, neurovascular bundle, the medial part of hard palate flap with nasal mucosa, palatal aponeurosis.

Results: The procedures using hydro dissection cause minimal bleeding, and operating time is shortened significantly in Veau 1 and smaller Veau 2 cleft palates. There was no incidence of postoperative bleed, hypoxemia, or airway obstructive problems. Identifying the zones of static resistance enabled us to perform better repairs. The main advantages were ease of closure without tension on the flaps, short operating time, less anesthesia time, and minimal blood loss.

Conclusions: Blood loss in palatoplasty can be minimal, especially in Veau 1, 2 cleft palate if minimal incisions are used. Identifying key areas in palatoplasty helps the cleft surgeon to perform the surgeries with ease and avoid intraoperative complications.

(1) NYU College of Dentistry, Department of Pediatric Dentistry, New York, NY

Background/Purpose: Individuals with a craniofacial condition experience significant disparities in the quality of health care that they receive. To address these disparities and improve the training of future pediatric dentists, the Department of Pediatric Dentistry of NYU College of Dentistry developed and implemented a craniofacial curriculum in the postgraduate training program in Pediatric Dentistry through clinical and didactic components. Though the PG students had been rotating through the referring Craniofacial Team, the supporting pediatric dental care was not consistently provided in a systematic way. To implement the craniofacial curriculum, clinical and didactic components were developed. This included a lecture series highlighting embryology, diagnosis, and all aspects of care for patients and clinical component of a dedicated monthly clinic day. Patient visits were consolidated on these days to improve the skills of the PGs in treating patients with craniofacial conditions and streamline the care coordination with the referring craniofacial team and other health providers. Emphasis was placed on educating the PGs on all aspect of multidisciplinary team care. Our aim was the establishment of a dental home for patients with Craniofacial conditions as early as possible, to provide the optimal oral health care.

Methods/Description: In 2014, a monthly craniofacial clinic day was established at NYU’s Department of Pediatric Dentistry to provide specially trained faculty and continuity of care. PG students treated patients in this highly stimulating environment. Due to the success and growth of the clinical component, the didactic component was augmented in 2018 with a seminar series highlighting all aspects of care required for patients with craniofacial conditions. This series included basic sciences, clinical treatment, psychosocial components, and case presentations. The knowledge the PGs acquired throughout the 2-year training program was assessed biyearly. Patient and family satisfaction was queried yearly.

Results: Without a robust didactic component, PG students were missing some of the knowledge and skills needed to manage the oral health care for patients with craniofacial conditions in a multidisciplinary approach. Assessment showed that care coordination and comprehensive treatment for the patients has improved. Patient and family satisfaction has also grown annually.

Conclusions: There is a lack of sufficient training about craniofacial conditions in pediatric dentistry postgraduate training programs. This can present a challenge for graduates when trying to provide optimal care to these patients because of a lack of knowledge and preparedness about overall multidisciplinary care. Our program demonstrated that combining a strong didactic curriculum with an extensive clinical experience is crucial to improving the skills of future pediatric dentists and to improving the oral health care of patients with craniofacial conditions.

(1) Global Smile Foundation, Norwood, MA, (2) Tufts University School of Medicine, Boston, MA, (3) University of Arkansas for Medical Sciences, Little Rock, AR, (4) Hansjörg Wyss Department of Plastic Surgery, NYU Langone Medical Center, New York, NY, (5) The University of Maryland Medical Center/Shock Trauma Center, Baltimore, MD, (6) New York University Langone Medical Center, New York, NY

Background/Purpose: Global Smile Foundation (GSF) is a not for profit foundation whose founders and volunteers have been providing cleft care to underserved communities around the world for 33 years. In 2012, GSF incorporated nasoalveolar molding (NAM) into its treatment model in Guayaquil, Ecuador. We present an evaluation of nasolabial aesthetic outcomes and scarring in patients treated with NAM prior to primary cleft lip repair versus patients who were not in similar outreach settings.

Methods/Description: The Cleft Aesthetic Rating Scale (CARS) used frontal photographs taken at least 1-year post primary cleft lip repair to assess the nose: tip, nostrils (symmetry, size, flaring) and upper lip (vermillion symmetry and continuity and length of the philtrum), ranging from 1 (very good) to 5 (very poor). Photos were standardized to reveal only the nasolabial area and excluded any time points after any additional surgical revision to the nasolabial area. Each NAM time point was matched to a control time point based on their age at primary cleft lip repair (maximum of 3 months) and their time postoperative from primary cleft lift repair (maximum of 6 months). All included NAM and control patients had Unilateral Cleft Lip and Palate, and were from Ecuador. Patients with congenital syndromes other than cleft lip and palate (CLCP) affecting facial appearance were excluded. Twelve independent raters including 3 surgeons, 3 orthodontist, 3 pediatric dentists, and 3 medical students rated the photographs. As a modification to CARS, raters were also asked to assess scar quality utilizing a previously developed scar subtype scoring system.

Results: Of the 192 patients treated with NAM in Guayaquil since 2012, 96 patients had long-term follow up, and 27 patients with 34 photographic time points qualified for inclusion, 15 (55.56%) male, 12 (44.44%) female. The average time post primary cleft lip repair was 2.19 ± 1.65 months (range: 0.98-7.37 months). Matched to the patients who had received NAM were 31 control patients with 34 photographic time points, 26 (83.87%) male, 5 (16.13%) female with an average time post primary cleft lip repair of 2.19 ± 1.63 months (range: 0.93-6.98 months). The average rating of nasal aesthetics was significantly lower in patients who had received NAM compared to nasal controls (2.60 ± 1.05 vs 2.82 ± 1.12; P < .01). The average rating for lip aesthetics showed similar differences between patients who had received NAM and matched controls (2.23 ± 0.96 vs 2.56 ± 1.07; P < .01). Similarly, the average rating of scar quality was significantly lower among patients who had received NAM (1.82 ± 0.93 vs 2.03 ± 0.89; (P < .01).

Conclusions: Using the CARS and a modified scar rating scale, patients who had received NAM were found to have superior nose and lip aesthetic outcomes in comparison to non-NAM controls. This suggests that NAM is not only feasible in an outreach setting, but it is also associated with improved lip, nose, and scar outcomes.

(1) Phoenix Children’s Hospital, Phoenix, AZ, (2) Division of Plastic Surgery Phoenix Childrens Hospital, Barrow Cleft and Craniofacial Center, Phoenix, AZ

Background/Purpose: Hospital- or team-based patient registries use clinical data to help providers understand the clinical services their team is providing, help conduct quality assurance and quality improvement activities, and enable the team’s clinical research endeavors. Entering data into these registries, however, can be time-intensive. In this presentation, we describe the successful integration of a cleft lip and palate registry into a hospital’s electronic health record (EHR). We discuss the benefits of this approach and the positive downstream effects.

Methods/Description: Registry development followed the process outlined by the Agency for Healthcare Research and Quality (AHRQ). Cleft team providers convened to establish the registry’s purpose. The team then defined the target population, core data elements, and outcomes of interest. Each specialty drafted their initial data collection forms on paper. Forms were iteratively revised during a pilot testing period. Finalized forms were then integrated into the Sunrise™ EHR (Allscripts) by hospital IT staff. The integration was achieved by creation of a cleft-specific registry note with structured data elements. Data were extracted electronically from the EHR into a data warehouse. A Power BI™ (Microsoft) reporting dashboard was created to visualize the collected data. Bidirection integration was achieved, whereby individual patient entries in the reporting dashboard led directly to the patient’s electronic health record.

Results: Team providers established the registry’s purposes as identifying all patients under active treatment for cleft lip and/or palate, tracking the number and types of cleft procedures performed, and monitoring fistula incidence after palate repair. Data collection forms consisted of a cleft classification and medical history form completed at the initial team visit and an operative procedure form completed following each surgery. In addition, structured data elements were added to the existing clinical note template to record occurrence of readmission, return to operating room, and palatal fistula. The reporting dashboard has subsequently been used to conduct annual quality assurance audits of palatoplasty outcomes and to facilitate screening and enrollment for a prospective clinical study.

Conclusions: Data collection for hospital-specific patient registries can be integrated into an institutional EHR. Electronic capture coupled with automated reporting facilitates identification of patients under active treatment, monitoring of postoperative complications, and collection of data for prospective clinical studies.

(1) Global Smile Foundation, Norwood, MA, (2) East Carolina University, Greenville, NC, (3) European Cleft Organisation (ECO), Rijswijk, Netherlands, (4) Smile Train, New York, NY, (5) Operation Smile, Virginia Beach, VA

Background/Purpose: The World Cleft Coalition (WCC) is an alliance of international non-governmental organizations (NGO’s) that work in the area of cleft lip and palate and engage in long-term local capacity-building partnerships. The WCC was initiated at the 13th International Congress of Cleft Lip and Palate and Related Craniofacial Anomalies in Chennai, India in 2017. The objective set forth by Congress delegates at the end of the first NGO day “NGO’s: Think, Treat, Teach,” was to encourage international collaboration in the development of an agreed set of globally recognized minimum core practice and best practice guidelines for safe and comprehensive cleft care. In addition to creating these guidelines, it was the collaborative process between the founding NGO’s that was the unexpected strength and success of this initiative. It is an example of diverse organizations collaborating in the enhancement of cleft care.

Methods/Description: Through collaboration among participating organizations (American Cleft Palate Craniofacial Association, European Cleft Organization, Global Smile Foundation, Operation Smile, Smile Train, and Transforming Faces) and consultations with outside constituents and partnerships, the WCC created an “International Treatment Program Standards.” From Feb 2017 to Present (Aug 2019), the team has met on monthly/bimonthly basis via video conferencing. This exchange was built on sharing experiences between the founding NGO’s on what is of primary importance for ensuring the delivery of high quality cleft care in outreach settings. The group was able to present the core standards at the European Cleft and Craniofacial Equality Conference in Nis, Serbia, September 2018. This was followed by the official launch of the Program Standards at the 76th Annual ACPA Conference in Arizona in April 2019. This was followed by a presentation at the European Cleft and Craniofacial Meeting in Utrecht, Netherland, in June 2019.

Results: The goal was to compile standards for safe, comprehensive, and sustainable cleft care. The starting point was a review of existing guidelines (internal and external) to create globally recognized international treatment program standards. Focus was kept on ethical, safe, accessible, and patient-centered care, not on technique and timing. Attention for wording these guidelines was focused on being inclusive and encouraging while avoiding mandatory and prescriptive language. These national and international presentations also allowed significant feedback from various participants and organizations. Statements of official support, versus endorsement, are being considered. A website was constructed to enhance awareness and dissemination of the proposed standards. Recent acceptance for publication in the Cleft-Palate Craniofacial Journal.

Conclusions: This is an example of diverse organizations collaborating in the enhancement of cleft care globally.

(1) University of California San Francisco, San Francisco, CA

Background/Purpose: Craniofacial care is administered by a coordinated interdisciplinary group of providers who typically work in close proximity. However, social distancing restrictions imposed during the COVID-19 pandemic have disrupted this care model. Accordingly, the UCSF Craniofacial Center adopted telehealth for most appointments. This study sought to characterize our transition to a hybrid model of care and to assess the impact of telehealth on craniofacial patient satisfaction and clinic volume.

Methods/Description: Standardized institutional patient experience surveys from March through May 2020 were compared with the year prior using χ ² tests. All English- and Spanish-speaking patient families who had video appointments during this period received a telehealth satisfaction survey that was administered electronically or over the phone.

Results: All patients, excluding those with time-sensitive diagnostic questions or wound care needs, underwent video visits with their providers. Clinicians each were assigned a clinic room in which they saw all telehealth and in-person patients. During the first 10 weeks of the pandemic, the UCSF Craniofacial Center saw an average of 40 patients across 81 visits each day, compared to 58 patients across 138 visits the year prior. Of all, 88% of visits occurred over telehealth, whereas none occurred prior to COVID-19; 87% of patients were satisfied with their video visits, and 93% felt comfortable using the telehealth technology. Furthermore, 87% of patients agreed that video visits saved time (median 3.0 hours, IQR 0.5-4.3), and 68% saved money. However, 47% preferred in-person appointments, with the desire for a complete physical exam cited as the most common reason. Before and after our telehealth transition, similar proportions of patients trusted their provider (P = .39), found that their provider explained things well (P = .36), and would recommend their provider to a friend (P = .13). Fewer telehealth patients agreed that their pain was fully addressed (62% vs. 82%, P = .002) and felt that they had enough input in their care (66% vs. 79%, P = .04).

Conclusions: Telehealth is an effective tool in delivering interdisciplinary craniofacial care, associated with time and money savings. However, patients continue to prefer in-person visits in many cases, and the true safety and efficacy of this care methodology remains unknown. To address some of these challenges, providers should receive specific training on virtual communication to facilitate patient input into their care.

(1) Cook Children’s Medical Center, Fort Worth, TX, (2) Smile Train, Flemington, NJ, (3) Smile Train, New York, NY, (4) Fundación Clínica Noel, Medellín, Antioquia

Background/Purpose: The physical effects of clefting can obscure the psychosocial aspects of the disease. With this in mind, the idea for a unique mental health booklet arose. “9 Reasons to Smile: Family Fun Activity Booklet” was developed when Smile Train international programs suspended all cleft treatment for patients at the height of the COVID-19 pandemic. Patients could not receive cleft surgery and other nonsurgical treatments, such as nutritional support, orthodontic care, dental care, psychosocial care, and speech therapy, to ensure long-term, successful rehabilitation. Suspension of cleft treatment poses significant challenges for children and families. Recognizing these challenges, Smile Train developed this booklet to raise awareness for psychosocial health as it is not readily accessible or prioritized around the world. The booklet includes art projects on self-love, engaging games to help children explore their emotions, their relationships, and their self-confidence.

Methods/Description: The booklet was developed with support from Fundación Clínica Noel, a Smile Train Partner in Colombia. It was first launched in English, French, and Spanish during the World Health Organization Walk the Talk Virtual Event on May 17, 2020. A global launch for the booklet in 10 languages took place on June 17, 2020. To further increase awareness for psychosocial health, Smile Train hosted a live, virtual celebration of “9 Reasons to Smile” on July 21st and Smile Train Brazil and Mexico participated in their own local celebrations. The event convened representatives from the global health and global cleft communities to discuss art, health, clefts, and the importance of maintaining self-esteem and positivity through adversity.

Results: The booklet is now available in 15+ languages and is shared through WhatsApp messaging platforms worldwide. The global virtual celebration had over 4000 views on Facebook and YouTube, and its reach continues to grow. Smile Train has received thousands of photos and stories from families and partners sharing their experiences using the booklet and its tremendous impact on their lives. The overwhelming response confirms the demand for programs and resources that focus on cleft patients and their psychosocial health needs.

Conclusions: There is a need for supporting psychosocial services in global cleft care which is particularly evident during the COVID-19 pandemic. Access to these services is not always prioritized in many countries around the world and the enthusiasm for the “9 Reasons to Smile” booklet indicates the positive impact such resources have on mental health. Smile Train is committed to raising awareness on the importance of psychosocial health in global cleft care and will continue to support and scale initiatives for cleft patients and their families around the globe.

(1) UT Southwestern Medical Center, Dallas, TX, (2) UT Southwestern School of Medicine, Dallas, TX, (3) Analytical Imaging and Modeling Center, Children’s Health, Dallas, TX

Background/Purpose: Facial dynamics is an important element of human communication and is therefore a useful quantitative descriptor for many craniofacial abnormalities. It may be used perioperatively for characterization of a condition and determination of outcomes. Conventional 2D and 3D photographs may be used but are limited static representations at a single point in time. 4D imaging is a newer modality and has proven to be useful in analyzing dynamic facial movement, but this technology is expensive, cumbersome, and not universally available. Recent innovations in machine learning facilitated the further utilization of standard 2D videos in a capacity similar to 4D technology. As a potential alternative, we propose a convenient method to systematically landmark and track dynamic facial movements utilizing standard 2D videos, using fully automated computer vision algorithms.

Methods/Description: After obtaining IRB approval, 2D videos of patients between 6 and 18 years old were obtained using a 2D camera. The subjects were asked to perform various facial expressions such as smiling, lip pursing, and cheek puffing. Fifty facial features were automatically identified and landmarked on the first frame, then were subsequently automatically tracked throughout the video using a computer vision Kanade-Lucas-Tomasi (KLT) feature tracking algorithm. Tracking of facial features was accomplished by calculating the sum of squared intensity differences between a selected feature on a frame and the same feature on subsequent images. Quantitative measurements such as tissue displacement and facial asymmetry were obtained.

Results: The fully automated computer vision program accurately landmarked and tracked the digitized landmarks throughout the 2D videos. This strategy was able to derive applicable measures of facial animation from different facial expressions including asymmetry and displacement values, as well as computation of an estimated 3D head pose.

Conclusions: This automated technique was able to successfully obtain excellent objective dynamic measures of complex aesthetics and functional facial characteristics from 2D videos, which are ubiquitous and can be obtained on any video camera. These findings suggest clinical relevance and utility in outcome determination of craniofacial anomalies effecting facial movements.

(1) Texas Tech University, Lubbock, TX

Background/Purpose: Cleft clinic encounters multiple cleft patients along various parts of timeline of cleft care. As such, understanding how to evaluate a cleft care patient can often be challenging to a student or resident in Cleft Clinic. Purpose of this investigation was to study the utility of virtual clinic with images to assist students and residents in identifying and interpreting common and important findings in cleft clinic.

Methods/Description: Virtual computer based cleft modules were created emulating cleft patient care from birth to final procedures (rhinoplasty for our modules). Interactive quizzes were given along the module to assess ability to correctly identify salient Cleft Clinic findings including but not limited to vermillion insufficiency, hypertrophic scar, palatal fistula, extruded pressure equalizing tubes, maxillo-manidubular insufficiency. Subjective reports were then given by students and junior residents who took module of how helpful they felt the module was in preparing for cleft clinic.

Results: Confidence scores were compared prior to cleft clinic module and after taking cleft clinic module. Finding specific data was gathered to figure out possible weaknesses in physical exam assessment. Student t test was used to compare pre and post test results indicating significant improvement in confidence with understanding cleft care and identifying cleft key findings during Cleft Clinic.

Conclusions: Given improved confidence of students and residents with Cleft Clinical module, we support the use of such modules for students/residents who may be assisting in clinic to better understand how to evaluate and assess patients in Cleft Clinic.

(1) Cincinnati Children’s Hospital, Cincinnati, OH

Background/Purpose: Patients presenting with head-shape changes phenotypical for craniosynostosis may have incomplete fusion of the involved sutures. Surgical literature is lacking in appropriate management strategies for these patients. We sought to evaluate our experience with a novel treatment strategy; suturectomy of only the fused portion followed by helmeting in patients presenting with incomplete suture synostosis and significant skull deformity.

Methods/Description: Patients presenting to the senior author with a diagnosis of craniosynostosis with incomplete suture synostosis who underwent operative intervention between 2018 and 2020 were included for evaluation. The clinical diagnosis of craniosynostosis was confirmed by computed tomography (CT). Patients were selected for partial suturectomy if the patent portion of the suture had a normal appearance (suture gap distance less than 1 SD different from other fully patent reference sutures). All patients were treated by craniectomy of the involved portion of the synostosed suture. Intraoperative ultrasound (US) was used to reassess the degree of fusion at the time of surgery and to define the region of fusion for incision planning. A 2 to 3 cm strip craniectomy was performed under direct visualization through a single small access incision. Postoperative helmeting was utilized with laser scans for helmet manufacture and adjustment. Demographic and perioperative data were collected. Laser scan data collected included the cranial index (CI) and cranial vault asymmetry (CVA; the difference between 2 diagonal measurements (frontozygomaticus to opposite eurion).

Results: Patient demographics include 3 males and 1 female with an average age of 2.6 months (range 1.1-3.9 months) at presentation. All patients were diagnosed with incomplete sagittal synostosis, with one patient also diagnosed with an incomplete left lambdoid synostosis. All patients underwent surgical excision of the involved portions at an average age of 3.2 months (range 2.0-4.1 months) without intraoperative or 30-day postoperative complication. All patients were compliant with postoperative helmeting. Average age at last follow-up was 13.8 months (range 2.9-13.7 months) with an average follow-up duration of 10.7 months (range 0.5-19.6 months). Final laser scan evaluations were available for 3 patients (including the patient with the lambdoid synostosis) and showed improvement of the CI from an average of 71.3 (range 70-73) to 84.3 (range 82-86). The CVA improved from an average of 9.67 mm (range 2 mm-22 mm) to 1.67 mm (range 1 mm-2 mm).

Conclusions: Minimally invasive direct excision of the involved portion of fused cranial sutures followed by helmet therapy for phenotypical craniosynostosis is a safe and effective treatment strategy. This technique is suitable for very young patients and offers similar outcomes to complete suturectomy. Further studies are required to see if this approach reduces the deformity severity for patients requiring vault remodeling later in life.

(1) Pedo Planet Children Dental Centres, Chennai, Porur, India, (2) Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India (3) College of Medicine University of Lagos, Nigeria, (4) University of Dundee, Dundee, United Kingdom, (5) University of Casablanca, Morocco, Morocco, (6) Peking University, Beijing, China, (7) Foundation for Cleft Lip and Palate Children, Santiago, Chile, (8) FDI World Dental Federation, Geneva, Switzerland, (9) Smile Train, New York, NY

Background/Purpose: Clefts of the lip and/or palate (CL/P) represent the most common congenital craniofacial anomalies. The global prevalence is estimated at 1 in 700 live births. Clinical practice guidelines (CPGs) exist to present recommendations and policies aimed at optimizing oral health of children and adolescents with CL/P. The aim of this review was to identify and assess the scope, quality, adequacy, and consistency of CPGs related to oral health in children and adolescents with CL/P along with reporting any differences and shortcomings.

Methods/Description: A systematic review of the literature of CPGs following PRISMA (Preferred Reporting Items for Systematic Reviews) guidelines was conducted. Assessment of selected CPGs was performed by 4 appraisers using Appraisal of Guidelines for Research & Evaluation (AGREE II) methodological quality instrument. The scores were calculated for each of the 6 AGREE II domains. To measure the reliability between appraisers an intraclass correlation coefficient (ICC) was calculated. Tabulation and analyses were performed using Microsoft Excel, version 15 and SPSS Statistics.

Results: Only 7 CPGs fulfilled criteria out of which 4 were from the American Cleft Palate Craniofacial Association (ACPA), one each from American Academy of Paediatrics (AAP) and Academy of Breastfeeding Medicine (ABM) and the American Academy of Pediatric Dentistry (AAPD). The lowest overall mean scores were in the rigour of development domain (mean 29.58%, SD 17.11) indicating low quality for reported guideline methodology. The domain with the best score was clarity of presentation (mean 73.80%, SD 7.87) as most guidelines clearly highlighted recommendations for treatment. The mean scores for “Scope and Purpose,” “Stakeholder Involvement,” “Applicability” and “Editorial Independence” were 69.63% (SD 11.54), 46.81% (SD 18.39), 35.17% (SD 12.36), 32.14% (SD 24.47), respectively. While using the 70% quality threshold for each domain as per the AGREE II assessment manual, 5 guidelines had one or more domains with more than 70% scores. Two guidelines (AAPD, ACPA [neonatal]) did not score 70% in any of the 6 domains.

Conclusions: Our results reveal significant limitations of the existing guidelines, and minimal or no recommendations with regard to dental caries prevention in children with CL/P. Hence there is a need for a more objective and specific guideline pertaining to oral health of children with CL/P. The unique situation of guidelines on oral health for CL/P individuals involves multiple stakeholders, oral health professionals (OHPs), nonoral health care professionals (nOHPs), parents and caregivers.

(1) Long Island Plastic Surgical Group, Garden City, NY, (2) Childrens Healthcare of Atlanta, Atlanta, GA

Background/Purpose: The cleft nasal deformity is one of the more challenging aspects of cleft care, made so because of the complex intrinsic and extrinsic forces that act to not only create the deformity but also to maintain it. Inherently the cleft nose is marked by a deficiency and deformation of local tissues, which is even more so pronounced in the multioperated nose. A number of surgical techniques and approaches have been developed and utilized for correction of the cleft nasal deformity which utilize both local and regional autogenous tissues. However, in situations where there is a paucity of both local and regional tissues, as might be the case in a multioperated cleft nose, the reconstructive effort becomes complicated. The iliac chondro-osseous graft is an ideal graft for predictable framework correction in patients who lack or have pathologic local tissue to work with.

Methods/Description: We will present our technique for harvesting and utilizing split chondro-osseous iliac crest grafts for restoring nasal projection and support. Pre and postoperative evaluation and images will be presented to highlight the utility and fidelity of this technique. A discussion of outcomes including possible complications will also be presented.

Results: This descriptive examination of our technique will demonstrate the feasibility and utility of utilizing iliac chondro-osseous grafts for nasal augmentation in the multioperated cleft nose.

Conclusions: The objectives of this presentation are to highlight the utility of iliac chondro-osseous grafts for cleft nasal reconstruction and present our technique. Particular attention to its effect in patients who lack local tissue for cleft nasal correction will be discussed.

(1) Chang Gung Memorial Hospital, Taipei, Taiwan

Background/Purpose: Vermillion asymmetry is a common problem after primary unilateral cheiloplasty. Even though the vermillion is adjusted to equal height intraoperatively, the vermillion height has been observed to be progressively thicken during long-term follow-up. The purpose of this study were to determine the vermilion symmetry between the cleft and noncleft sides of unilateral cleft lip patients during the early and late postoperative periods and also to compare vermilion height differences between the complete and incomplete groups.

Methods/Description: In this retrospective longitudinal study, 57 complete and 38 incomplete unilateral cleft lip patients were evaluated. All the patients operated during year 2010 to 2014 with complete postoperative follow-ups during the immediate (within 1 month) (T1), 9 months to 1 ½ year (T2), and more than 4 years (T3) postoperative periods were included in the study. Frontal view photographs were retrieved and the vermilion height of the cleft and noncleft sides were measured. The Cleft Lip Component Symmetry Index (CLCSI) was used to determine the symmetry of the cleft and noncleft sides and then was analyzed.

Results: From the 95 patients that were studied, vermillion height was excessive at the cleft side all throughout the 3 time periods, with a significant increase from T1 to T2 of 5.675% for the incomplete and 6.516% for the complete group. Between T1 and T3, the incomplete group CLCSI mean for vermillion height significantly increased to 3.826%, whereas no significant increase was found in the complete group. No significant increase was found between T2 and T3 for both groups.

Conclusions: Even with efforts to obtain a symmetric vermilion height during primary cheiloplasty, vermillion height excess was noted for the incomplete and complete cleft lip, especially with the former group. It may be recommended that intraoperative vermilion heights be noted to provide a better basis for the subsequent measurements. Long-term follow-up of these patients is still warranted for proper counselling of parents.

(1) Johns Hopkins University School of Medicine, Baltimore, MD

Background/Purpose: Despite the advancement of techniques for primary palatoplasty and an increasing focus on levator repositioning, velopharyngeal insufficiency may persist in 10% to 20%of patients with a history of cleft palate. While some patients may avoid the need for corrective speech surgery in earlier childhood, the need for maxillary advancement to correct Class III malocclusion in patients with cleft lip and/or palate may worsen velopharyngeal disproportion and secondarily create the need for surgical intervention. Palatal lengthening via double-opposing buccal flaps has been well described for secondary velopharyngeal insufficiency and may minimize the sequelae of obstructive sleep apnea commonly associated with pharyngoplasty. In older patients, however, buccal flap surgery may be complicated by a risk of biting trauma to the pedicles in the setting of fully erupted permanent molar teeth.

Methods/Description: In 3 patients with velopharyngeal insufficiency requiring secondary surgical correction after maxillary advancement, double-opposing buccal flap palatal lengthening was proposed. A novel orthodontic appliance was fabricated for the mandibular arch to open the bite posteriorly. It consisted of acrylic bite blocks on the occlusal surfaces of the mandibular first and second premolars and a metal framework banded to the permanent mandibular first molars. The device was cemented in place within 7 days prior to the buccal flap surgery for patient accommodation. The degree of clearance in the retromolar region was checked and, if not deemed sufficiently adequate, additional acrylic was added chairside to the occlusal surfaces of the bite blocks. The device was removed 3 weeks after the buccal flap surgery, at which time pedicle division was deemed safe.

Results: The device was well tolerated by all 3 patients. No difficulties with intubation were experienced in the operating room at the time of the first surgery. All patients healed without fistulae. One patient experienced oral side distal flap tip loss that healed secondarily. Another experienced mild relapse of the mandibular anterior dentition secondary to not wearing a retainer. An active Essix retainer was fabricated for the mandibular anterior region and corrected the relapse within the 3-week postoperative period. Biting trauma to the pedicles appeared to have been prevented in all patients. Speech assessments are ongoing.

Conclusions: The double-opposing buccal flap technique for palatal lengthening can allow significant velar retropositioning and is an advantageous method for patients in need of secondary surgical correction after maxillary advancement. We have developed a reliable, commercializable appliance to prevent biting trauma to the pedicles in older patients undergoing such surgery. Future work will focus on optimizing acrylic block height predictions and adaptations to minimize mandibular anterior dental relapse.

(1) Children’s Hospital of Richmond at Virginia Commonwealth University, Richmond, VA, (2) Children’s Hospital New Orleans, New Orleans, LA, (3) Wyss Department of Plastic Surgery, NYU Langone Health, New York, NY, (4) Stanford Children’s Health, CA

Background/Purpose: In the setting of COVID-19, the commitment to provide multidisciplinary care for children with craniofacial diagnoses has created multiple challenges for families, children, and health care providers. Team coordinators have been particularly tasked with managing a constantly changing rule book in complying with institutional guidelines and public health recommendations requiring consideration of use/availability of PPE, technology limitations and a significant increase in family requests for resources and reassurance. This panel will present an overview by 4 highly experienced team coordinators and examine the challenges of providing team care for children and families in a safe, comprehensive yet efficient manner.

Methods/Description: Exploring both virtual and in person formats, areas to be addressed include patient compliance, the growing waitlists for team visits, the need of families for resources and support, and the increased undocumented time required to provide counseling and reassurance. Strategies will be shared which have been developed to manage urgent patient/family needs including newborn feeding, VPI assessment, wound care, and COVID-19 specific questions.

(1) Children’s Hospital of Philadelphia, PA, (2) Children’s Medical Center, Dallas, TX, (3) Johns Hopkins All Children’s Hospital, St. Petersburg, FL, (4) Texas Children’s Hospital, Houston, TX, (5) Children’s Hospital Los Angeles, Los Angeles, CA, (6) Shriners Hospitals for Children Chicago, Chicago, IL

Background/Purpose: The COVID-19 pandemic has had a profound impact on the psychosocial experiences and service delivery for children with cleft and craniofacial conditions and their families. Many centers were unable to provide in-person multidisciplinary care for weeks to months, operating rooms were shut down to all but essential surgeries, and schools were rapidly transitioned to online instruction. These changes, designed to promote a public health response to an unprecedented pandemic, contributed to challenges in implementation of educational and therapeutic support services. Patients and their families, as well as clinical providers, have worked to balance risk abatement, quality of life, and medical/surgical needs in a rapidly changing and often inconsistent global health landscape.

Methods/Description: A nationally representative panel of psychologists will share their multidisciplinary teams’ experiences managing the impact of COVID-19 and associated interruptions in treatment, the effects on patients’ psychosocial and academic functioning, and strategies that have been effective in addressing clinical needs in the context of a global health crisis. Implications of facial coverings and virtual interaction for youth with facial and speech differences will be explored. The experiences of patients and teams in the context of COVID-19 will be explored relative to our understanding of multisystemic psychosocial functioning, including but not limited to health related quality of life, social and emotional functioning, educational outcomes, and resiliency factors.

(1) Baylor College of Medicine/Texas Children’s Hospital, Houston, TX, (2) Smile Train, New York, NY, (3) Cook Children’s Medical Center, Fort Worth, TX

Background/Purpose: International cleft initiatives require a mechanism for assessing whether a surgeon is performing successful operations. When determining whether a cleft lip repair outcome is acceptable, the severity of a patient’s cleft at initial presentation must be considered. At present, there is not a widely agreed upon scale for grading the preoperative severity or postoperative surgical outcome for patients with unilateral and/or bilateral cleft lip. This study presents the Hubli grading system, which uses 5 categories to generate separate pre- and postoperative ratings, and then combines these ratings to produce one scaled measure of overall cleft repair acceptability. The aim of this study is to validate the Hubli grading system as a reliable and reproducible system for characterizing both unilateral and bilateral cleft lip repair outcomes.

Methods/Description: Nine craniofacial surgeons from a variety of countries independently evaluated 2489 patients undergoing both unilateral and bilateral cleft lip repair by Smile Train partner surgeons from April 2004 to December 2018. Preoperative severity and postoperative surgical result were assessed, and these numbers were then multiplied to determine whether a surgical outcome was acceptable based on initial cleft severity. Intra-rater and inter-rater reliability were calculated as percentages of agreement.

Results: Intra-rater and inter-rater acceptability scores had an agreement of 94.50% and 87.04%, respectively. These results indicate that using the Hubli grading system, raters are able to reliably and reproducibly grade the acceptability of a cleft lip repair based on initial presentation of cleft severity and postsurgical result. Notably, 94% of surgical outcomes were rated as “acceptable,” suggesting that the vast majority of cleft lip repairs performed by Smile Train partner surgeons have a successful surgical outcome.

Conclusions: This study validates the use of the Hubli grading system to evaluate the acceptability of both unilateral and bilateral cleft lip repair surgeries. To date, this study is the largest of its kind to evaluate cleft lip severity and repair outcomes, and the novel Hubli grading system is the only scale to account for the acceptability of surgical outcomes based on initial cleft severity. This system is best designed to identify surgeons with suboptimal outcomes in order to implement timely, individualized training on a global scale.

(1) University Of Illinois at Chicago, Chicago, IL

Background/Purpose: While a large amount of literature has been dedicated to technical aspects of cleft lip repair, a much more limited amount has described perioperative care of these patients. As of yet, no finite set of guidelines exists to inform surgeons on evidence-based perioperative management of cleft lip repair. There is clearly a wide range of clinical protocols after cleft lip repair, as evidenced by conflicting survey results regarding incision closure, prophylactic antibiotics, arm restraints, length of hospital stay, and postoperative feeding protocols. This study seeks to describe the current state of research on 5 aspects of perioperative management of cleft lip repair: Non-absorbable sutures, absorbable sutures, or tissue adhesives for final layer skin closure, the use of arm restraints, the use of prophylactic antibiotics, outpatient surgery versus hospital admission, and feeding restrictions immediately after lip repair. Through a systematic review and limited meta-analysis, we aim to describe the gaps in the current knowledge and provide a set of working guidelines for perioperative cleft lip surgery.

Methods/Description: PubMed was used to search MEDLINE on February 23, 2020 with inclusion criteria including papers published between 1970 and 2020, available in English, with human subjects, with a topic regarding perioperative management of cleft lip repair. Only comparative studies were included. Articles were initially screened by title and abstract. Articles that passed screening underwent a full-text review. A secondary search was performed of articles cited within these articles. Risk of bias was assessed by the senior author using the Cochrane Risk of Bias Tools for randomized and nonrandomized studies.

Results: The initial search returned 3103 articles. After screening, 42 articles were selected for full-text review. A secondary search resulted in an additional 12 articles screened and 7 of these were included for full text review. 23 articles met appropriate inclusion criteria and were included in the systematic review. Only studies of outpatient versus inpatient management and postoperative feeding restrictions had similar enough outcome reporting to allow meta-analysis.

Conclusions: After review we have reached the following conclusions: (1) Skin Closure: There is no evidence for a clearly superior or inferior closure material. (2) Arm Restraints: Existing literature does not support the use of arm restraints postoperatively after cleft lip surgery. (3) Prophylactic Antibiotics: Literature recommends against the practice of preoperative oronasal culture swabs to guide antibiotic treatment. Non-cleft head and neck surgical data does not support the prolonged use of postoperative antibiotics. (4) Hospital Admission: With careful patient selection, outpatient cleft lip repair appears to be safe. (5) Postoperative Feeding: Existing literature supports the practice of allowing breast or bottle feeding immediately after repair.

(1) McMaster University, Ancaster, Ontario, Canada, (2) University of Toronto, Toronto, Ontario, Canada (3) McMaster University, Hamilton, Ontario, Canada, (4) Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada, (5) Great Ormond Street Hospital For Children NHS Trust, London, United Kingdom

Background/Purpose: Acquired and congenital facial differences are conditions which can have an important impact on appearance, facial function, and health-related quality of life (HR-QOL). The outcomes most important to children and young adults with facial differences undergoing treatment are not well-defined. Measurement of treatment outcomes from the patient’s perspective is necessary to ensure goals of treatment are met. We aimed to develop a conceptual framework for a patient-reported outcome measure (PROM) for children with facial differences by developing a comprehensive understanding of the concepts important to them.

Methods/Description: A total of 72 in-depth qualitative interviews were conducted in Canada and the United Kingdom. Participants were aged 8 to 29 with a visible or functional facial difference. The qualitative methodology of interpretive description was followed. Interviews were audio-recorded, transcribed verbatim, and coded. The data were analyzed to develop a conceptual framework to inform the development of a PROM.

Results: Four domains were identified to form a conceptual framework: facial appearance, facial function, HR-QOL (psychological, social, school), and adverse effects of treatment. Participants described changes in these areas across their treatment journey. The analysis also revealed common themes related to HR-QOL across participants with diverse facial conditions and ages, suggesting a common lived experience for children and young adults with facial differences.

Conclusions: The conceptual framework developed in our study was used to create scales forming the FACE-Q Craniofacial Module, which was recently validated in an international field test consisting of patients 8 to 29 years of age. It is important to understand the impact of having a facial difference in children and young adults provide high quality patient-centered care and measure outcomes which matter most to this population.

(1) University of Pittsburgh Medical Center, Pittsburgh, PA, (2) Department of Plastic Surgery, University of Pittsburgh Medical Center, Pittsburgh, PA, (3) Children’s Hospital of Pittsburgh, Pittsburgh, PA

Background/Purpose: Infantile Hemangiomas (IH) are the most common vascular tumor in children. IH has many different morphologies and a characteristic lifecycle of appearance, growth, quiescence, and involution. While the rate of growth and involution between varying morphologies has been explored, the relationship between morphology and time of appearance remains unknown.

Methods/Description: A retrospective cohort study was conducted. All patients diagnosed with IH at a major children’s hospital multidisciplinary vascular anomalies center from 2009 to 2015 were reviewed. Data collected included date of birth, sex, hemangioma depth (superficial, mixed, or deep), and timing of appearance.

Results: A total of 473 children met inclusion criteria. By pooled t test, superficial IH was found to have the shortest time to hemangioma appearance (superficial = 11.3 ± 1.4 days, mixed = 24.5 ± 4.8 days, deep = 59.1 ± 5.9 days, P < .0001). By odds ratio comparison with Fisher exact test, patients with superficial IH had 2.7 higher odds of having their IH appear earlier than those with deep IH. Patients with mixed IH had 1.9 higher odds of having their IH appear earlier than those with deep IH (HR 1.9; 95%CI 1.5-2.5). However, when measure with LogRank comparisons, other factors did not contribute to timing of presentation of IH when controlled for by depth.

Conclusions: This study is the first to demonstrate a clear relationship between IH morphology and the timing of IH appearance, with longer time to appearance correlated with increasing lesion depth. These findings underscore the importance of delineating the different IH morphological subtypes when studying incidence, natural life, pathogenesis, and treatment.

(1) Cooper University Hosptial, Camden, NJ, (2) Cooper University Hospital, Moorestown, NJ, (3) Cooper University Health Care, Camden, NJ, (4) Cooper Hospital University Medical Center, Camden, NJ, (5) Cooper Medical School of Rowan University, Camden, NJ

Background/Purpose: A validated sleep screen (PSQ) used to identify cleft lip and/or palate children at risk for sleep-disordered breathing (SDB) is routinely deployed by our interdisciplinary cleft-craniofacial program. An increased incidence of SDB initially identified by PSQ was previously reported by our institution. Screening scores less than or equal to 8 were considered positive. The purpose of the current study was to first evaluate contributing patient factors to elevated sleep scores. Secondly, we analyzed the change in subsequent PSQ scores following surgical interventions aimed at alleviating the SDB.

Methods/Description: A retrospective review of the medical records of all patients over the age of 3 years evaluated over a 4-year period in a single cleft-craniofacial program. Patients with syndromes were excluded. The following points of information were recorded: Pediatric Sleep Questionnaire (PSQ) scores, diagnosis, Veau classification, surgical history, dental occlusion, facial skeletal alignment, nasal airway patency, BMI, documented history of ADD or ADHD, polysomnography results, nighttime CPAP use, adenotonsillectomy, or septorhinoplasty. Results were analyzed using a univariate logistic regression to identify risk factors for positive PSQs. Treatment modalities employed were measured by reduction in the PSQ.

Results: N = 239 patient charts were reviewed, 45/239 patients had positive PSQs (8 or >8). Class III dental occlusion was noted in 24 of the 45 patients (Odds Ratio [OR] = 2.65; P = .02). A history of ADD and or ADHD was noted in 20 of the 45 patients (OR = 5.90, P < .001). No other variables were found to be significantly different from those with negative PSQs. Thirty-three children of the 45 with positive PSQs had polysomnogram results (73%); 29/33 had polysomnogram results indicating disordered breathing (88%). Seventeen children received one or more forms of treatment for OSA, with treatment choice dictated by presumed etiology. Adenotonsillectomy was performed for 12 children and was found to have an average reduction in follow-up PSQ of 5.3 (P = .003).

Conclusions: Routine screening for OSA reveals a high number of patients at risk, and subsequent polysomnography supports the diagnosis in a high proportion of these patients as found in our previously reported study. This investigation found patients with SDB are more likely to have class III occlusion, and have a documented history of ADD and or ADHD. Only a small subset of our study population underwent surgical treatment for OSA which demonstrated an improvement in PSQ.

(1) UC Irvine Medical Center, Orange, CA, (2) UC Riverside Medical Center, Riverside, CA

Background/Purpose: Non-syndromic cleft lip and/or palate (NSCLP) is the most common congenital craniofacial anomaly. Early recognition of any associated developmental delay is critical to counseling families and developing individualized treatment plans. Here we sought to identify developmental delays associated with NSCLP in a large population of children in order to begin identifying etiology and improve multidisciplinary management.

Methods/Description: This is an IRB-approved, single-center retrospective analysis of all patients with a diagnosis of cleft lip and/or cleft palate between 5 and 21 years of age. Demographic and clinical variables were collected from this patient population as well as from children comprising the 2018 National Survey of Children’s Health (NSCH) database.

Results: All children with an identified or suspected genetic syndrome were excluded (160 in our cohort and 1383 in the NSCH database). Subsequently, 619 children in our cohort and 29 147 in the NSCH database were identified with NSCLP and included in our analysis. The mean birth weight among NSCLP children was lower than that in the national cohort (108.5 ± 24.8 oz vs 117.8 ± 19.1 oz; P < .0001). Nearly one-fourth (25.8%) of children with NSCLP were admitted to the NICU at birth. Compared to the national cohort, children with isolated cleft palate had significantly higher rates of intellectual disability (3.2% vs 0.5%, P < .00001), speech delay (70.8% vs 7.1%, P < .00001), global developmental delay (15.7% vs 5.8%, P < .00001), cerebral palsy (2.2% vs 0.3%, P < .00001), and hearing loss (25.9% vs 1.0%, P < .00001). Rates of learning disability (7.0% vs 5.9%, P = .529), behavioral delay (7.6% vs 11.4%, P = .1038), ADD/ADHD (2.7% vs 2.3%, P = .7032), autism (4.3% vs 5.5%, P = .5005), and vision loss (1.6% vs 1.2%, P = .5764) were comparable between those with isolated cleft palate and the national cohort. Children with cleft lip (with or without cleft palate) had significantly higher rates of ADD/ADHD compared to the normative national cohort: isolated cleft lip (7.7% vs 2.3%, P = .0092), unilateral cleft lip and palate (4.6% vs 2.3%, P = .0088), bilateral cleft lip and palate (5.9% vs 2.3%, P = .0153).

Conclusions: Our study demonstrates, for the first time, higher rates of various developmental delays in children with NSCLP compared to the general pediatric population. This includes increased rates of intellectual disability, global delay, and cerebral palsy in children with non-syndromic isolated cleft palate and increased ADD/ADHD in children with cleft lip (with or without cleft palate). The association of NSCLP diagnoses with developmental delays highlights the importance of proper risk assessment of patients, appropriate family counseling, and multidisciplinary team management.

(1) Baylor Scott & White Medical Center, Temple, TX

Background/Purpose: The opioid crisis has increased attention on the issues of opioid misuse and associated harm throughout the medical community. There is no current consensus regarding the role of opioids in the management of perioperative pain in children undergoing cleft lip and/or palate repair. The goal of the present study is to evaluate opioid prescribing patterns in members of the American Cleft Palate-Craniofacial Association (ACPA).

Methods/Description: An institutional review board exempt, anonymous 20 question online-based REDCap survey was distributed to surgeon members of the ACPA. Data were collected from December 2019 until January 2020 and included surgeon factors (years of experience, geographical region, operative techniques, timing of surgery), patient length of stay, and pain regimen (use of narcotics as inpatient vs outpatient, duration of narcotics, narcotic type, and use of nonopioid analgesics). Responses were strictly voluntary. Sample characteristics were analyzed using descriptive statistics (SAS 9.4).

Results: Survey respondents who perform cleft lip repair typically operate on patients between 3 to 6 months of age (86%), admit their patients postoperatively (82%), and discharge them on the first postoperative day (72%). Comparatively, those that perform cleft palate repair, operate between the ages of 10 and 12 months (62%), almost always admit the patients (99%), and typically discharge them on the first postoperative day (78%). Opioids were more frequently prescribed after palatoplasty than after cleft lip repair, both for inpatients (66% vs 49%) and at discharge (38% vs 22%), and mostly for a duration of 1 to 3 days in both cases (81.5% vs 81.2%). All respondents who reported changing their prescribed narcotic regimen (34.4% changed dose, 32.8% changed duration) after cleft lip repair decreased the amount prescribed. Similarly, respondents who changed the dose (32.2%) and duration (42.5%) of narcotics after palatoplasty over the course of their practice, mostly reported decreases (>96%). Nonopioid analgesics after cleft surgery were reportedly prescribed by 92% of cleft surgeons, with acetaminophen being most commonly prescribed medication (86%).

Conclusions: Cleft surgeons who have changed their prescribing patterns typically decreased the dose and duration of opioids prescribed after both cleft lip and palate surgery. Most patients are prescribed opioids while inpatient, and only a minority are prescribed opioids upon discharge. While there is no consensus the current method for pain control is typically a multimodal approach that can include intraoperative nerve block and adjunct medications (acetaminophen and NSAIDs).

(1) Department of Oral and Maxillofacial Surgery, College of Medicine, University of Lagos, Lagos, Nigeria, (2) Department of Oral Pathology, Radiology and Medicine, College of Dentistry, University of Iowa, Iowa City, IA, USA, (3) Iowa Institute for Oral Health Research, University of Iowa, Iowa City, IA, (4) Surgical Department, School of Medicine, College of Health Sciences, Addis Ababa University, Addis Ababa, Ethiopia, (5) Kwame Nkurumah University of Science and Technology, Kumasi, Ghana, (6) KwaZulu-Natal University, KwaZulu-Natal, Durban, South Africa, (7) University of PortHarcourt, PortHarcourt, Rivers, Nigeria, (8) University of Ferrara, Ferrara, Italy

Background/Purpose: Concurrent presentation of cancer and congenital malformations has been reported from several population-based case-control studies. Many associations have been made between oral clefting and cancers, though some of these results are conflicting. Some small studies have reported an increased risk of cancer among first-degree relatives of cleft cases and vice versa, and also an excess risk of cancers of the breast, lung, and brain tumors among those with oral clefts. This study aimed to determine if the genetic mutations found in some related cancers also play a role in orofacial cleft.

Methods/Description: This study was a case-control and case-triads study in which cases were subjects clinically diagnosed with nonsyndromic cleft lip and/or palate (CL ± P) while controls were subjects without CL ± P. This study was part of an ongoing study where over 13 800 saliva samples from Africa were obtained for our cleft project (African Craniofacial Anomalies Research Network). DNA extraction was carried out at the Butali laboratory at the University of Iowa. We did a database search to identify the common GWAS significant SNPs (markers) that have been reported to be associated with cancers that sometimes occur concurrently with congenital malformations. A total of 12 markers were used in this study. The assay plate and samples were prepared and sent to the Iowa Institute of Human Genetics (IIHG) for Fluidigm. Data obtained from the Fluidigm run were analyzed using the Fluidigm SNP Genotyping Analysis software. The genotypes at different loci were called, and a test of association was done. Case-parent triads analysis was done using Family-based association test (FBAT) using PLINK. We also conducted Case–control analysis using PLINK, and P = .05 was set for nominal significance.

Results: The SNPs rs10941679 (5p12) and rs55658222 (8q24.21), which are known GWAS significant SNPs for breast cancer and lung cancer respectively, showed suggestive evidence for an association with nonsyndromic cleft lip and/or cleft palate (P = .046, OR = 0.806 and P = .022, OR = 1.392, respectively). Rs34775372 (4p16.2) associated with colorectal and breast carcinoma, along with rs55658222 (8q24.21) and rs72728755 (8q24.21), both associated with lung cancer, showed an association with cleft lip only (P = .013, OR = 1.443, P = .0002, OR = 2.078 and P = .0005, OR = 1.98, respectively), while the same SNPs and rs11117758 (1q41) associated with breast carcinoma showed a significant association with cleft palate only (P = .002, OR = 0.393).

Conclusions: The results from this study represent an important starting point for the understanding of the concurrent presentation of some cancers in orofacial cleft, and cancer risks in cleft patients. Further research on the identified risk loci and replication of this study in other cohorts is advised.

(1) Division of Plastic and Reconstructive Surgery, University of Southern California, Los Angeles, CA, (2) Division of General Surgery, University of Southern California, Los Angeles, CA, (3) Southern California Permanente Medical Group, Los Angeles, CA, (4) Albert Einstein College of Medicine, Bronx, NY

Background/Purpose: Computerized tomography (CT) of the head is considered the gold standard for diagnosing craniosynostosis (CS). At most craniofacial centers, the preoperative workup for suspected CS involves a head CT scan. Recent literature has demonstrated the ability to accurately diagnose CS without preoperative imaging. The purpose of this study is to investigate the benefits of preoperative CT scans in the diagnosis and treatment of nonsyndromic single-suture CS (NSSCS).

Methods/Description: A retrospective review was performed for all children who underwent surgery for NSSCS at our institution between 2007 and 2018. Our preoperative assessment of NSSCS is based on clinical examination, with imaging studies reserved only for cases where a diagnosis cannot be made clinically (Group 1). However, some patients already have imaging studies performed prior to presentation (Group 2). The objective data we collected included: age at presentation to craniofacial team, age at surgery, preoperative clinical diagnosis, any preoperative imaging and if before or after initial craniofacial visit, coexisting radiographic findings, and the correlation of the intraoperative diagnosis. Descriptive statistics were calculated for the scale data with averages and standard deviations. ANOVAs were run for comparisons between the age of initial craniofacial visits and the age at surgery.

Results: A total of 138 patients were included in this study with a median age of 3.1 months at initial craniofacial visit and 7 months at time of surgery. Of these, 27 (19.6%) patients had imaging obtained before the first craniofacial visit (Group 2): head CT in 6 and X-ray in 21 patients. The remaining 111 (80.4%) patients had no imaging studies obtained before their first craniofacial visit (Group 1). Based on initial clinical assessment in group 1, single-suture synostosis was diagnosed in 102 (92%) patients, whereas in 9 (8%) the diagnosis was not clear. Of the patients with unclear diagnosis, one was successfully diagnosed clinically at a planned follow up exam, and the remaining 8 (7%) patients were diagnosed using imaging studies (3 CT scans and 5 X-rays). In all patients, the preoperative diagnosis was confirmed to be accurate during intraoperative assessment. No radiographic abnormalities nor findings that changed operative plans were found in either group. There were no differences in the age of initial craniofacial visit (ANOVA; F = 0.604, P = .4) and surgery (ANOVA, F = 0.391, P = .5) between the groups.

Conclusions: Our results suggest that head CT scanning is not necessary for diagnosis by experienced craniofacial surgeons in 93% of NSSCS cases. Imaging obtained before referral to our craniofacial clinic provided no additional benefits in identifying coexisting conditions, age at referral to craniofacial clinic, or age at surgery. Thus, we challenge the utility of routine head CT scan in the evaluation of craniosynostosis and recommend use only by an experienced craniofacial team and only when clinical diagnosis is uncertain.

(1) Division of Plastic and Reconstructive Surgery, Northwell Health, Great Neck, NY

Background/Purpose: The use of allogeneic cartilage is gaining popularity in facial reconstruction due to its affordability, safety, lack of donor site morbidity, and structural capabilities. Although it is well described in adult rhinoplasty, there remains a paucity of information regarding its use in the pediatric population. In an attempt to spare children from increased operative time, pain, donor site morbidity, and psychosocial stress, we have expanded the use of cadaveric cartilage grafting for pediatric cleft nasal deformity and microtia type 1 repair.

Methods/Description: Pediatric patients who necessitated nasal reconstruction and ear reconstruction with cadaveric cartilage grafting (MTF Biologics; Edison, New Jersey) between 2014 and 2020 were included. During secondary rhinoplasty and microtia type 1 repair, segments of cadaveric cartilage were carved and prepared on the operating room back table. The cut segments were allowed to warp on the table prior to implantation and were cut into patient-specific carved segments to enhance the structural integrity of the external ear, alae, or columella. Patients were scheduled for postoperative follow-up to evaluate for resorption, infection, and rejection of grafts.

Results: Twenty-eight patients were included, 24 patients who underwent cleft rhinoplasty, and 4 patients who underwent grafting and otoplasty for type 1 microtia. Ages of patients varied from 5 to 17 years old and range of follow up varied between 1 to 6 years with a mean of follow up time of 3.2 years. Subjectively, grafts were cosmetically and structurally successful in all patients with no incidence of infection, extrusion, or need for reoperation.

Conclusions: The several cases described in this study inspire confidence that cadaveric cartilage acts as viable grafts for pediatric nose and ear reconstruction. Parents were pleased to spare their child from donor site morbidities while still achieving similar aesthetic and functional outcomes as autologous grafting. No cases of infection, extrusion, or rejection of the cadaveric grafts were noted, but longer follow-up times and a larger sample size are necessary to better understand potential long-term complications in pediatric patients as this is a growing population.

(1) Plastic & Reconstructive Surgery, Chang Gung Memorial Hospital, Taipei, Taiwan, (2) Sobrapar Hospital, Campinas, Sao Paulo, Brazil, (3) Craniofacial Research Center, Chang Gung Memorial Hospital, Taoyuan, Taiwan, (4) Chang Gung Memorial Hospital, Taoyuan, Taiwan

Background/Purpose: Oral cleft is the most common congenital craniofacial malformation in the newborn. Among those cleft patients, maxillary constriction is often noted during the long-term care. Inherited growth deficiencies and scar formation after palatoplasty are generally considered to be the main reasons for the maxillary constriction. Traditionally, Surgicel has been used to cover the lateral relaxing surfaces during palatoplasty. Nowadays, buccal fat pad (BFP), as many other arising autogenous fat grafting, has also started to be used as the covering material. This study aimed to find out whether Surgicel or BFP chosen as the covering material during palatoplasty will result in less constriction in transverse palatal development in cleft patients.

Methods/Description: Seventy-eight enrolled patients were divided into 3 groups. Surgicel group (n = 27) and BFP group (n = 20) consisted of complete unilateral cleft lip and palate (UCLP) patients who received palatoplasty using Surgicel or BFP, respectively, as the covering material at around 9 months old; CL group (n = 24) consisted of cleft lip (CL) only patients, who didn’t receive palatoplasty. All patients underwent the same treatment protocol for their cleft lip or/and cleft palate by the same craniofacial team in the same medical center, Chang Gung Memorial Hospital, Taiwan. Cone beam computerized tomography (CBCT) scans taken right before alveolar bone graft (ABG) surgery at around 9 years old were compared using 2 linear measurements and 1 square measurement representing the transverse development of posterior palate. Those measurements include U6T-MSP, the distance between the tips of the distolingual cusp of U6 (U6 T) to Midsagittal plane (MSP), U6J-MSP, the distance between the medial dentoalveolar junction of U6 (U6 J) to MSP, and Area, a fictitious square area calculated by U6J-MSP multiplying the vertical distance between U6 J and greater palatine foramen (GPF).

Results: In UCLP patients, all 3 summed-up measurements of both sides were significantly wider or larger in BFP group than in Surgicel group (Summed-up U6T-MSP: Surgicel group = 44.09 ± 4.50, BFP group = 47.50 ± 4.05, P = .003; Summed-up U6J-MSP: Surgicel group = 33.75 ± 3.53, BFP group = 35.65 ± 3.19, P = .025; Area: Surgicel group = 404.13 ± 72.19, BFP group = 437.41 ± 68.50, P = .048). Comparing BFP group and CL group additionally, we even found that BFP group was also significantly wider than CL group in all 3 summed-up measurements.

Conclusions: The cleft patients receiving BFP as covering materials for lateral relaxing raw surfaces during palatoplasty have less constriction in transverse palatal development than the cleft patients receiving Surgicel as covering materials.

(1) University of Bristol, Bristol, United Kingdom

Background/Purpose: Research focusing on the wider implications of being born with a cleft suggests that cleft is associated with poor psychosocial outcomes and behavioral difficulties in children. Speech and language difficulties, poor self-image, and negative social experiences, compounded by the stress of chronic medical treatment can increase the risk of developing behavioral problems in childhood. The aim of this study was to summarize behavioral outcomes in 5-year-old children born with cleft lip and/or palate from the Cleft Collective Cohort Study and compare outcomes to controls from general population cohort samples.

Methods/Description: In this study we employed a cross-sectional design to describe patterns of behavioral outcomes, as reported by mothers from the Cleft Collective Cohort Study, in the Strengths and Difficulties Questionnaire (SDQ). The Cleft Collective Cohort Study is a longitudinal cohort of children born with cleft lip and/or palate in the United Kingdom with questionnaire information on surgery, early development, speech, dental health, psychology, and well-being of parents and children as well as genetic data and clinical records. The study cohort was defined as (n = 340) children born with cleft lip and/or palate (mean age = 5.6 years), whose mothers returned questionnaires. We used estimates from 3 general population cohorts as comparators.

Results: Children from the Cleft Collective 5-Year-Old Cohort experience greater behavioral problems, as represented by elevated mean total difficulties scores, than do 5-year-old children from the Millennium Cohort Study (MCS), the primary comparator group used in this study (study cohort (CC): M = 9.08, SD = 6.45; MCS: M = 6.7, SD = 11.99; p = 2.03 × 10-10). Of the four SDQ subscales contributing to the total difficulties scores, the hyperactivity subscale represented the area where the study cohort showed the greatest departures from general population estimates.

Conclusions: We summarized behavioral outcomes in a cleft population, reporting that an estimated 14.15% of 5-year-old children with cleft lip and/or palate experience behavioral difficulties. Though there is evidence to suggest elevated levels of behavioral problems, particularly hyperactivity-related problems in boys born with cleft lip and/or palate, further investigation is required into the differences that may exist between cleft lip and/or palate patients and the general population.

(1) University of Wisconsin School of Medicine and Public Health, Madison, WI, (2) University of California San Diego, San Diego, CA, (3) Rady Children’s Hospital, San Diego, CA

Background/Purpose: Background: Alveolar bone grafting is utilized to manage alveolar clefts in patients with cleft lip and palate. However, the timing of bone grafting is variable with conflicting evidence supporting the use of primary alveolar bone grafting (PABG) in clinical practice. Primary Aim: To provide a qualitative systematic review analysis of long-term outcomes after PABG.

Methods/Description: Materials and Methods: A qualitative systematic review was performed following the Cochrane Handbook and reported using Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Summative findings were evaluated using Confidence in the Evidence from Reviews of Qualitative research (GRADE-CERQual) to assess the quality of evidence supporting the findings.

Results: After removing duplication 2182 publications were identified, and 2131 were excluded after screening through titles and abstracts. Inclusion criteria for this study included patients who underwent PABG at 24 months of age or younger and a minimum of 5 year follow-up. Thirty two publications met the inclusion criteria and were included for qualitative analysis. Primary outcome measures included cephalometric analysis, bone graft survival, occlusal analysis, hypomineralization, tooth eruption, radiograph analysis, and arch relationships. Four assessment themes were characterized from the systematic review: (1) Bone graft survival, (2) Craniofacial skeletal relationships, (3) Occlusion and arch forms and 4. Recommendations for utilizing PABG in practice.

Conclusions: The reported systematic review provides evidence that performing PABG leads to poor long-term outcomes related to bone-graft survival and maxillary growth restriction despite some reported positive outcomes.

(1) Case Western Reserve University School of Medicine, Cleveland, OH, (2) University Hospitals/Case Western Reserve University, Cleveland, OH

Background/Purpose: Patients with cleft lip and palate often require multiple maxillofacial and orthognathic surgeries throughout their management. However, the overall health care resource requirement and utilization of these procedures in this patient population remain poorly understood. The purpose of this study was to examine national trends in patient utilization and hospitalization charges associated with oral and maxillofacial surgeries for cleft lip and palate patients over multiple years.

Methods/Description: The Healthcare Cost and Utilization Project National Inpatient Sample database was analyzed from January 2007 to December 2014. All patients who were diagnosed with cleft lip or palate who underwent oral and maxillofacial procedures were included. Variables of interest included demographic data, hospital characteristics, hospitalization data, and total hospital charges. All charges were inflated to May 2019 value with the consumer price index. Univariate and generalized linear models were used to examine associations between various factors of interest and the final adjusted hospitalization charge as well as the change in these factors over the multiple years included.

Results: In all, 62,471 patients were diagnosed cleft lip or palate during hospitalization between January 2007 and December 2014. Of these patients, 1292 (2.1%) received an oral and maxillofacial surgery, including osteoplasty of maxilla (879, 68.0%), genioplasty (30, 2.3%), and other facial bone repair and reconstruction (183, 14.2%) and were included in the study. Regression analysis showed that total procedure volume did not change significantly over the years examined (P = .199). Higher procedure volume (>10 cases/year) was associated with large bedsize hospitals (P < .001), nonprofit private hospitals (P < .001), and urban teaching hospitals (P < .001). Only one observed case of maxillofacial surgery in the database occurred at a rural hospital. The mean total hospital charge for these patients was $61 157.47 (interquartile range: $33 099.62 – $72 180.56). Hospitalization charges also increased significantly in this time period (P = .042), in conjunction with an average hospital length of stay of 2.54 days that did not change significantly during the study period (P = .693).

Conclusions: Oral and maxillofacial procedures for patients with cleft lip and palate appear to be utilized mainly at larger, urban teaching and nonprofit private hospitals and very rarely at rural hospitals. While hospital length of stay and procedure volume appears be stable year to year within the study period, hospitalization charges associated with the procedures rose significantly throughout. Further studies are necessary to examine other factors that could be contributing to the increasing hospitalization charges.

(1) University of Kansas Medical Center, Kansas City, KS

Background/Purpose: The metopic craniosynostosis is often the most difficult to evaluate and treat as its phenotypes ranges broadly on a severity spectrum. Currently, there is no universally accepted algorithm for cases that definitively require surgical management especially those who present after 12 months of age. This study was designed to explore the role of standardized developmental assessment in the surgical decision making for infants who present late (after 12 months) with metopic craniosynostosis.

Methods/Description: A prospective, nonrandomized study was performed. Infants who presented after 12 months of age with single-suture, nonsyndromic metopic craniosynostosis were included. The Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III) was used to assess cognitive, language, and motor development after initial presentation. Comparisons were made between infants who underwent surgical repair and those whose parents elected observation.

Results: Ten infants, ages 12 to 26 months (mean = 19 mo), with late presenting metopic craniosynostosis were enrolled in the study. There were 6 males and 4 females. Five infants underwent surgical repair and 5 infants had parents who elected observation (one infant underwent surgery after a prolonged observation period). Upon initial presentation, 5 of the 10 infants tested as cognitively delayed. Of those delayed, the average score was 70.8 (<85 is considered delay for Bayley-III). All infants in the surgical group improved in their cognitive scores postoperatively. For the nonsurgical group, no such trend was observed from the cognitive assessment. For both the surgical and nonsurgical groups overall, there was improvement in the language assessments (mean Δ = 8, 6.3). The surgical group had an average increase of 16 in motor assessment, whereas the nonsurgical group scored worse on later assessments in motor (mean Δ = −2).

Conclusions: The results of this study demonstrate the benefit of standardized developmental assessments in the surgical decision-making for infants with metopic craniosynostosis. Neurocognitive assessments should be considered as a potential addition into the diagnostic work-up for infants presenting with metopic craniosynostosis especially for infants with moderate severity.

(1) Hospital Dr. Luis Calvo Mackenna, Santiago, Chile, (2) Fundacion Gantz, Pudahuel, Chile, (3) Alfredo Gantz Mann Foundation, Santiago, Región Metropolitana, Chile (4) Alemana Clinic, Santiago, Chile

Background/Purpose: Speech language disorders and velopharyngeal insufficiency are common in children with cleft lip palate (CLP) in developing countries. The continuous training of Speech and Language Therapists (SLT) is fundamental for an adequate evaluation and treatment of these alterations in interdisciplinary CLP teams. The purpose of this study was to describe the working context and learning process in Latin America and Central America therapists, remotely trained by an interdisciplinary team in CLP.

Methods/Description: A team of 10 professionals with more than 30 years of experience in the interdisciplinary rehabilitation of CLP (SLT and plastic surgeons from the Fundación Gantz Hospital, Santiago de Chile) in collaboration with Smile Train, managed and designed a training of 12 theoretical-practical sessions in a synchronic teleeducation modality, reinforced weekly with asynchronic self-employment (reading, homework, and direct activities with their patients), plus a 3-month mentory assincronic after class.

Results: Forty-seven Latin American SLT from Argentina, Bolivia, Colombia, Peru, Ecuador, Mexico, Guatemala, Republica Dominicana, and Cuba completed a total of 114 chronological hours of training in evaluation and treatment of patients with CLP. In the questionnaire of labor situation, resources and participation in the team, 76.6% of the participants reported working in an interdisciplinary FLP team. According to the evaluation, SLT report orofacial myofunctional assessment (78.3%), speech (88.3%), velopharyngeal insufficiency (56.7%), and language (76.7%), using only 19.5% of standardized tests. Twenty-five percent of the centers carry out instrumental evaluation of velopharyngeal insufficiency by videofluoroscopy (23.4%) and nasopharyngoscopy (18.1%). Regarding treatment, 56.8% of SLT report that their opinion is considered in surgical decisions or treatment of their patients; 90.2% perform speech treatment and 82.4% language treatment. Finally, the average performance of the initial and final evaluation demonstrated the progression of learning in all participants.

Conclusions: Speech Online Training, organized by Gantz and Smile Train Foundation, handed over according to the work characterization, evaluation skills and treatment to Latin American and Central American therapists to care patients with CLP in interdisciplinary equipment.

(1) Southern Connecticut State University, New Haven, CT, (2) University of Arkansas for Medical Sciences, Little Rock, AR, (3) Global Smile Foundation, Norwood, MA, (4) Hansjörg Wyss Department of Plastic Surgery, NYU Langone Medical Center, New York, NY, (5) Louisiana State University Health Sciences Center, New Orleans, LA, (6) Medical University of South Carolina, Charleston, SC

Background/Purpose: Management of velopharyngeal dysfunction (VPD) is particularly challenging in global outreach settings. While nasendoscopy is a valuable tool for the surgeon in industrialized cleft care settings, there is limited access to the necessary equipment in international outreach settings. To evaluate the role of nasendoscopy in surgical decision-making, a prospective study was conducted to compare surgical treatment recommendations with and without nasendoscopy data. The aim was to compare surgical recommendations for individual VPD cases with and without nasendoscopy data in mission-based care.

Methods/Description: A 2-year prospective cohort study comparing surgical recommendations with and without nasendoscopy data was performed. Participants consisted of 5 Craniofacial surgeons participating on an international cleft lip/palate team mission. Each surgeon screened between 7 and 17 patients (n = 52 patients) for velopharyngeal dysfunction (VPD) during a day long screening and triage session prior to a surgical mission in Guayaquil, Ecuador. Each surgeon rated the patient’s speech and resonance according to their own guidelines based upon a speech sample and clinical examination. They then made surgical recommendations based upon their examination. All patients who were identified as having VPD were then evaluated by the team speech pathologist who conducted nasendoscopy evaluations on a portable KayPentax system with video archiving. Each study was then deidentified and presented to the evaluating surgeons who made surgical decisions based upon the nasendoscopy video. Pre and post nasendoscopy results for each patient were analyzed using descriptive statistics.

Results: Our analysis indicates that, surgeons in an outreach setting modified their treatment recommendations for VPD an average of 63.2% of the time when presented with nasendoscopy information. Across the 5 surgeons in this study, the range of recommendation agreement between pre and post nasendoscopy ranged from 33% to 40%. Mean agreement among raters was 36.8% (SD = 4.49). The most common surgical decision conversion was from a posterior pharyngeal flap to a sphincter palatoplasty (N = 11, 21%).

Conclusions: The results of this study suggest the need for flexible nasendoscopy to be an integral component of the cleft palate surgical decision-making process in outreach settings. We conclude that incorporating nasendoscopy information would improve the delivery of comprehensive cleft care internationally.

(1) McGovern Medical School at The University of Texas Health Science Center at Houston, Houston, TX

Background/Purpose: Postpartum depression (PPD) affects 11% of women in the US and has been shown to negatively impact mother-infant interactions essential for cognitive and behavioral development. Maternal depression rates in cleft lip/palate remain unclear. Prenatal ultrasound technology has allowed for the prenatal diagnosis of most cleft lip and/or palate. Although this technology is available, its impact is hindered by lack of prenatal care due to myriad reasons and limited ability to visualize isolated cleft palates. We proposed to screen mothers of infants with cleft lip and/or palate for PPD, determine whether rates of PPD differed when the diagnosis was made prenatally, and examine the impact of prenatal therapeutic interventions on PPD rates.

Methods/Description: A retrospective survey was created to screen mothers of children with cleft lip and/or palate. The survey incorporated the Edinburgh Postnatal Depression Scale, the most commonly used and widely validated PPD screening tool. Additional demographic information was also included. Parents were contacted anonymously via established social media websites independently run by the parents of children with cleft lip and/or palate. Inclusion criteria consisted of any mother who had a prenatal or post-partum diagnosis of cleft lip and/or palate and completed the survey.

Results: We received 73 complete survey responses. Of these, 32% of mothers scored 10 or more points on the Edinburgh Postnatal Depression Scale—a high-risk marker of PPD. Prenatal diagnosis of cleft lip and/or palate was made in 63% of mothers. Mothers with a prenatal diagnosis had lower EPDS scores as compared to their postnatal diagnosis counterparts (8.25 vs 9.52). Mothers with a prenatal diagnosis had higher rates of participation in social media led support groups (92% vs 56%). Of social media support group participants, mothers with prenatal diagnosis had lower EPDS scores as compared to their postnatal diagnosis counterparts (8.36 vs 10).

Conclusions: Mothers of children with cleft lip and/or palate have higher rates of PPD than the general population. Mothers with a prenatal diagnosis have lower EPDS scores, possibly a result of having additional time to process the diagnosis and better prepare for the care of their future child. Mothers with prenatal diagnosis who participate in social media led support groups have lower EPDS scores than their postnatal diagnosis counterparts, suggesting that social media support may normalize the diagnosis and provide healthy coping strategies. Additional prospective studies will clarify the effect of postnatal intervention on PPD rates in mothers with prenatal diagnosis of cleft lip and/or palate.

(1) Children’s Minnesota, Minneapolis, MN, (2) University of Utah, Salt Lake City, UT, (3) New York University, New York City, NY, (4) Tufts Medical Center, Boston, MA

Background/Purpose: As the airway experts in children, Pediatric Otolaryngologists treat multiple levels of airway obstruction including stenosis within the nasal cavities, midface hypoplasia, and micrognathia. Numerous techniques exist for treatment of airway obstruction, but goals are to relieve the airway obstruction, make feeding more efficient, and improve quality of life. Distraction osteogenesis is a technique that generates new bone formation, allowing for rapid growth and correction of the underlying craniofacial skeleton. This technique has been adapted for use in a variety of craniofacial abnormalities to improve upper airway obstruction. This panel will include in-depth discussion the variety of distraction techniques to treat obstruction at different airway levels. The panel will discuss airway management that might be necessary with the following procedures: mandibular distraction, infant surgically assisted rapid palatal expansion (iSARPE), maxillary distraction, and craniosynastosis, including emergency airway management, indications for surgery, work-up that should be completed, and controversies in management.

Methods/Description: This panel hopes to achieve the following objectives: (1) Review different techniques that might be incorporated in airway management in both an emergency and surgical situation for complex craniofacial patients. (2) Understand the different sites of upper airway obstruction that can be successfully treated with distraction osteogenesis, including mandibular distraction, infant surgically assisted rapid palatal expansion (iSARPE), midface/LeFort distraction and craniosynastosis. (3) Appreciate the indications for distraction osteogenesis, including controversies on patient selection and recognize ways an Otolaryngologist-Head and Neck surgeon is involved in management.

(1) Phoenix Children’s Hospital, Phoenix, AZ, (2) Seattle Children’s Hospital, Seattle, WA, (3) Children’s Hospital of Philadelphia, PA, (4) Nationwide Children’s Hospital, Columbus, OH, (5) Barrow Cleft and Craniofacial Center, Phoenix, AZ

Background/Purpose: Surgical management of velopharyngeal insufficiency (VPI) in patients with 22q11.2 deletion syndrome must take into account the unique characteristics of this patient population. While most surgeons are familiar with management of VPI in patients with submucous cleft palate, many patients with 22q11.2 deletion syndrome and VPI will have no overt signs of a palatal anomaly. Speech and language delays are highly prevalent in this population, limiting the extent of speech assessment and potentially hindering postoperative improvements in intelligibility. Further, many of these patients will have anxiety, ADHD, and or developmental delay which can limit compliance with preoperative velopharyngeal imaging. On top of all this, a significant proportion of these patients have persistent VPI symptoms after their first VPI surgery; deciding when to offer secondary VPI surgery and what procedure to offer can be even less clear than primary surgical decision making.

Methods/Description: This panel will address the complex nature of evaluation and treatment of VPI in patients with 22q11.2DS through case-based discussion by 4 surgeon panelists with distinct treatment approaches. Speech-language pathologists will present complex patients and the panelists will propose and defend their surgical management plan. Attendees will see contrasting approaches to management of patients with submucous cleft palate, occult submucous cleft palate, persistent VPI after primary pharyngeal flap, and patients with multiple prior VPI procedures. Be prepared for a lively exchange of ideas and an opportunity to learn what we do and do not know about managing VPI in patients with 22q11.2 deletion syndrome.

(1) University of Calgary, Calgary, Alberta, Canada, (2) Alberta Children’s Hospital, Calgary, Alberta, Canada

Background/Purpose: Cleft palate repair is a painful procedure and achieving adequate postoperative analgesia is challenging. The optimal perioperative management approach for patients undergoing cleft palate repair is unknown. Comprehensive clinical care pathways can improve postoperative pain control and decrease length of hospital stay. We instituted a clinical care pathway for patients undergoing cleft palate repair and evaluated its impact on specific clinical outcomes.

Methods/Description: A novel pathway was created through an iterative process, combining literature review with expert opinion and discussions with institutional stakeholders. The pathway focused on multimodal analgesia throughout the perioperative course, including intraoperative suprazygomatic bilateral maxillary nerve blocks and scheduled acetaminophen and ibuprofen postoperatively. Medical records of 40 consecutive patients undergoing primary cleft palate repair prior to pathway implementation (January 2016-October 2018) were retrospectively reviewed as controls; 40 patients treated with the pathway were prospectively reviewed. Primary outcomes were: (1) length of hospital stay; (2) cumulative opiate consumption; and (3) oral intake postoperatively. This work received ethics approval from the Conjoint Health Research Ethics Board (CHREB) at the University of Calgary.

Results: There were no differences in baseline demographic data between the treatment and control groups. The patients following the pathway had shorter mean length of stay (31 vs 57 hours, P < .001); decreased cumulative morphine consumption (75 vs 727 µg/kg, P < .001); shorter time to initiate oral intake (10 vs 22 hours, P < 0.01), and greater volume of oral intake in first 24 hours postoperatively (446 mL vs 171 mL, P < .001). There were no differences in length of total anesthesia time or total surgical time between the control and treatment groups. There were no differences in complication rates between the 2 groups. Data analysis was conducted using SPSS version 24.0.

Conclusions: Implementation of a perioperative clinical care pathway for cleft palate repair is safe, feasible, and associated with reduced length of stay, reduced opioid consumption, and improved oral intake postoperatively. Consequently, this pathway is now the standard of care at our institution and will form the basis for the development of further protocols for other surgical procedures. These findings could also provide a foundation for other pediatric centers to institute a similar clinical care pathway.

(1) Washington University in St. Louis, Saint Louis, MO, (2) Washington University in St. Louis School of Medicine, St. Louis, MO

Background/Purpose: Two surgical methods for treating single suture craniosynostosis (SCC) are open cranial vault remodeling and endoscope-assisted craniectomy with the use of a postoperative molding helmet. Choosing a repair can be difficult for families given the benefits and drawbacks of the 2 options. To allow families to choose the surgical treatment based on outcomes important to them, we developed and piloted a decision aid to support families and clinicians in making a patient-centered choice of SSC repair.

Methods/Description: We conducted a pilot study within a single academic children’s hospital between August 2019 and March 2020 to develop a decision aid to support surgical treatment for SSC. Risks and benefits of treatment options were collected from the literature and distilled by the study team which included a craniofacial plastic surgeon and a pediatric neurosurgeon, plastic surgery research fellows, an expert in decision aid development, a clinical nurse practitioner, and graphic design experts. Families of patients who were under 4 months of age, carried a diagnosis of SSC, and were candidates for both repairs were recruited to provide content suggestions and feedback. Socioeconomic status (SES) of families was measured by the Hollingshead Four-Factor index. Development steps included reviewing decision aid prototypes, interviewing families and clinicians, and directly observing clinic encounters.

Results: The 16 participating families had high SES (median 41.3; IQR: 32.0-51.1, indicating similar social strata as business owners and professionals). Distinct booklets were created to enable focused discussion of treatment options for the 3 major types of SSC (sagittal, metopic, and unicoronal). Using before and after photos, illustrations, and plain language, each booklet covered the basics of diagnosis and repair. After 3 major revisions, stakeholders found the pamphlets acceptable for clinical use. The approved booklets included frequently asked questions and a section to identify the factors crucial to each family. Families found the decision aid to be a concise and useful tool. The head-to-head comparison of techniques enabled families to compare and contrast both procedures and provided clinicians the opportunity to identify issues most important to them. Blood loss was the most frequently cited concern, followed by the need for postoperative helmeting. Other concerns cited by families included length of hospital stay, surgery duration, discomfort, and presence of postoperative scar.

Conclusions: We developed a customizable decision aid for the treatment of SSC. This tool lays the foundation for shared decision making by assessing family preferences and providing clear, concise, and credible information regarding surgical treatment. By using this decision aid, clinicians can focus on family goals, priorities, preferences, and values. Future studies can evaluate this decision aid in a randomized multicenter trial to test its efficacy.

(1) University of Pennsylvania, Philadelphia, PA, (2) Vietnam National Children’s Hospital, Hanoi, Vietnam, (3) University of Texas Health Science Center at Houston, Houston, TX

Background/Purpose: Current disease surveillance in Vietnam relies on underdeveloped electronic medical record (EMR) systems which are insufficient for timely standardized data collection about craniofacial and cleft pathologies. Concurrently, the World Bank reports Vietnamese mobile cellular subscription rates (per 100 people) of 128, some of the highest in the world. Herein, we discuss the development of an SMS-based surveillance system based in Hanoi, Vietnam, and its utility in contrast to an EMR-based approach to craniofacial epidemiology study.

Methods/Description: Vietnam National Children’s Hospital (VNCH) is a pediatric tertiary care center comprised of 13 clinical departments and 1300 beds, serving approximately 30 provinces and 12 million children. The Department of Plastic and Reconstructive Surgery is comprised of 4 surgeons, all who possessed SMS-enabled mobile phones prior to project introduction and were trained in data entry over the course of 1 week. The SMS database was designed using FrontlineSMS, a 2-way mobile gateway software application with automated messaging capabilities. Inbound messages can be downloaded as comma-delimited .xslx files; outbound messages prompting data entry are programmed based on participant input. VCNH has served as the pediatric pilot site for the introduction of Electronic Health Records (EHRs) in Vietnam since 2007. Individual demographic data for patients with cleft and craniofacial pathologies seen by the department was obtained for the years 2010 through 2019.

Results: Our retrospective search identified 2501 cleft and craniofacial case encounters between 2010 and 2019. There was a marked increase in cases logged in the VNCH database, from 11 in 2010 to 542 in the first 7 months of 2019. Birth prevalence of CLP was calculated using demographic data for patients born in 2017 and the crude birth rate over the same period as obtained from the General Statistics Office of Viet Nam. This yielded a contact prevalence of 5.57 in 10 000 live births (1 in 1795). Birth prevalence of patients from Ha Noi was 7.40 in 10 000 live births (1 in 1350). Lastly, correcting for health care access disparities for patients from rural provinces yielded an estimated corrected birth prevalence for CLP of 9.77 in 10 000 live births (1 in 1020). Six rural provinces >150 km from Hanoi were found to be significantly (P < .01) under-served surgically by the VNCH (Ha Giang, Ha Tinh, Lai Chau, Quang Binh, Son La, and Thanh Hoa). A significantly (P < .01) higher rate of Pierre Robin in Lang Son province and facial clefts in Ha Tinh province were identified. The prospective data collection system received 7 patient data sets over 4 months, resulting in a capture rate of 2.9%.

Conclusions: This feasibility study offers valuable insight into the true cleft and craniofacial prevalence in Vietnam and alternate methods to study in low and middle income countries (LMICs). Additionally, study represents a pathway forward for equitable and sustainable partnership between LMICs and more developed nations.

(1) Dublin Cleft Centre, Dublin, Ireland, (2) Nationwide Children’s Hospital, Columbus, OH, (3) Great Ormond Street Hospital NHS Trust, London, United Kingdom, (4) Royal College of Surgeons in Ireland, Dublin, Ireland, (5) Seattle Children’s Hospital, Seattle, WA, (6) unaffiliated, London, London

Background/Purpose: Velopharyngeal dysfunction (VPD) is present in up to 40% of patients following cleft palate repair. A patient with VPD, unable to easily produce oral consonants, may develop compensatory articulation, hypernasality, and nasal airflow. The overall result is decreased intelligibility of speech and social impairment. There are several surgical approaches for the management of VPD that can be broadly described as palatal, pharyngeal, and palatopharyngeal. However, standard treatment protocols for VPD have not been well defined. In a systematic review, the authors of this paper found mainly retrospective case series describing results using diverse parameters, particularly with regard to perceptual speech assessment. The COS-VPD Initiative is an international collaboration, which was established to address the need for a core outcome set (COS) to reduce both outcome reporting bias and heterogeneity across studies of interventions for VPD. That core outcome set is now being reported.

Methods/Description: The study was carried out according to the guidance of the Core Outcome Measures in Effectiveness Trials (COMET) initiative. An international steering group was formed and a long list of outcomes was derived from the systematic review. A 2-round Delphi process was carried out among international cleft clinical teams and their patients and caregivers to achieve consensus on the outcomes to be included in the COS. Participants rated outcomes on a Likert scale from 1 to 9, with 1 labelled “not important at all” and 9 labelled “critically important.” Consensus criteria specified a priori stated that any outcome with a rating of 7 to 9 by 75% or more of the participants and 1 to 3 by 25% or fewer was to be included in the COS. The Delphi survey was administered online using Research Electronic Data Capture (REDCap) software.

Results: A list of 67 outcomes was derived from the systematic review. Thirty-five participants from 13 countries completed both rounds of the Delphi process. Forty outcomes met the inclusion criteria. These outcomes were grouped in to 5 domains: patient reported outcomes, speech, surgical care, airway, and anatomical outcomes. In rank order, the top 10 outcomes were as follows: Presence of hypernasality; Passive cleft speech characteristics; Patient-reported acceptability of speech; Patient-reported intelligibility/understandability of speech; Severity of hypernasality; Obstructive sleep apnea; Patient-reported quality of life related to speech; Presence of audible nasal emission; Presence of compensatory misarticulation; Further operations performed for speech. The full core outcome set will be presented and discussed in detail.

Conclusions: Reporting of future studies in accordance with this COS will allow meaningful collation of results across different etiologies, surgical protocols, and institutions. It is hoped that this will facilitate more informed decision-making for surgeons and better preoperative counselling for patients/families about anticipated outcomes of their VPD surgery.

(1) Yale University School of Medicine, New haven, CT, (2) Mayo Clinic Florida, Jacksonville, FL, (3) Department of Plastic and Reconstructive Surgery, Yale University School of Medicine, New Haven, CT, (4) Department of Craniofacial Surgery, Hospital for Rehabilitation of Craniofacial Anomalies, University, Bauru, Sao Paulo, Brazil (5) Department of Plastic Surgery, University of Sao Paulo, Sao Paulo, Brazil

Background/Purpose: The severity of obstructive respiratory difficulty varies among affected Crouzon syndrome patients. This study aims to investigate the correlation between the restricted airway volume in Crouzon syndrome with the associated type of cranial vault suture synosotsis.

Methods/Description: CT scans of 68 unoperated Crouzon Syndrome patients and 89 controls were subgrouped as: type I. Bilateral coronal synostosis type II. Sagittal synostosis type III. Pansynostosis IV. Perpendicular combinations of synostoses. CT scans were measured using Mimics software.

Results: Type I, bicoronal synostosis, Crouzon had a 42% (P = .002) restricted nasal airway, while the pharyngeal airway volume was not significantly reduced. Type II, sagittal synostosis, associated Crouzon syndrome, grew normal segmental airway volumes. Type III, pansynostosis, developed simultaneously reduced nasal and pharyngeal airway volumes, by 38% (P = .034) and 51% (P = .014), respectively in infancy. However, the nasal airway volume achieved normal volume by 2 years of age without any intervention, yet the pharyngeal airway remained significantly less than normal by 42% (P = .013) unto 6 years of age. Type IV, perpendicular suture synostoses, Crouzon syndrome developed a reduced nasal airway volume (32%, P = .048) and a nonsignificant restricted pharyngeal airway of 18% (P = .325).

Conclusions: Airway compromise in Crouzon syndrome is variable, when associated with different craniosynostosis fusion patterns. Type II sagittal synostosis Crouzon grew a normal nasopharyngeal airway volume. Type I bicoronal synostosis and type IV perpendicular synostoses had significantly restricted nasal airways, and a tendency toward reduced pharyngeal volume. Type III pansynostosis infants had the worst restriction of both airways, although some improvement develops with age.

(1) University of Western Ontario, London, Ontario, Canada (2) Thames Valley Children’s Centre, London, Ontario

Background/Purpose: A primary goal of cleft palate repair is normal speech, and velopharyngeal insufficiency (VPI) is a significant outcome following cleft palate repair that affects normal speech development. The purpose of this study was to assess the variables associated with VPI as an outcome following cleft palate repair.

Methods/Description: Data was prospectively collected for all nonsyndromic patients with a cleft palate undergoing primary cleft palate repair by a single surgeon between 2002 and 2016. The following outcomes were recorded: incidence of VPI, and the rates of secondary surgery for VPI after failing speech therapy. Outcomes were correlated with the following patient and surgical parameters: preoperative palatal width and length, changes in palatal length, palate classification, and the type of surgical repair used.

Results: Two hundred twenty-nine patients (113 male and 116 female) were identified. Mean age at primary palate surgery was 12.2 months. Mean cleft width was 9.70 mm (4-18 mm) and mean palate width was 29.7 mm (23-40 mm). Cleft lip and palate was associated with greater mean cleft and palate width (P = .03, P < .01) compared to cleft palate only. Of the patients with both preoperative and postoperative palatal length measurements, 28 and 75 had Furlow and von Langenbeck repair, respectively. Average percentage of soft palate lengthening following surgery for the Furlow group was 26.2% and for Von Langenbeck was 28.3% (P = .46). Out of 201 patients who were old enough to be tested for VPI, 26 of these patients required secondary surgery for VPI (12.9%) and 25 had VPI that required speech therapy only (12.4%). Patients with VPI had significantly larger preoperative cleft widths (P = .04) and longer postoperative palate lengths (P = .01) compared to patients with no VPI, but no difference in preoperative palate length (P = .79), preoperative palate width (P = .14), or overall percent palatal lengthening (P = .90). Risk of developing VPI was not associated with type of palatoplasty (P = .10), age of palatoplasty (P = .31), or whether patients had an isolated cleft palate (P = .62). No factors were found to predict whether patients with VPI would require surgery. Statistical tests included χ ² tests and independent sample t tests with a significant P value defined as <.05.

Conclusions: In this study looking at a single surgeon’s cohort of 229 patients, cleft palate repair resulted in 25.4% of patients having VPI. In these patients, risk of developing VPI was associated with preoperative cleft width and post-operative palate length.

(1) East Carolina University, Greenville, NC, (2) Children’s Healthcare of Atlanta, Atlanta, GA

Background/Purpose: Treatment of maxillary hypoplasia with osteotomy is required in 25% to 40% of patients with a history of cleft lip with or without cleft palate (McComb et al., 2011). Advancement of the maxilla can increase the size of the velopharyngeal (VP) port resulting in incomplete VP closure and VP insufficiency, which is characterized by hypernasality (O’Gara & Wilson, 2007; Okushi et al., 2011). The effect of maxillary advancement on speech and VP function in the population with repaired cleft lip and palate remains controversial. Some studies conclude that there is no adverse effect on the speech while others report significant speech deterioration. The assessment of resonance and VP functioning relies on perceptual assessment by an experienced speech language pathologist, followed by objective instrumental measures to support perceptual observations. The Nasometer has been widely used as a clinical tool to quantify the level of nasality in speakers with a history of VP dysfunction. To our knowledge, only one study with 10 subjects has utilized both instrumental and perceptual methods to report changes in VP function following Lefort I maxillary advancement (Schultz et al., 2019). The purpose of this study is to quantify the changes in resonance following Lefort I maxillary advancement and distraction using perceptual ratings and Nasometry.

Methods/Description: Forty-five (38 males, 7 females) individuals between 7 and 22 years of age (M = 14.16 years, SD = 3.03). Pre- and postoperative speech assessment include perceptual ratings of articulation, resonance, and VP function. Nasalance scores were calculated using the Nasometer from 3 speech samples (sustained nasal sound, nasal sentence, oral sentence). Paired-samples t test and independent t tests were used to analyze between-group data.

Results: There was a significant (P < .05) increase in nasalance scores for all speech samples following maxillary advancement and distraction. There is a significant difference (P < .05) in perceptual ratings of VP function. Of those who presented with hypernasal resonance preoperatively, 36% became more hypernasal while 45% remained hypernasal postoperatively. Of those who presented with normal resonance preoperatively, 65% developed hypernasal resonance postoperatively and 35% maintained normal resonance. Results show a significant increase in nasalance scores for individuals without previous speech surgery (P = .039) and for individuals undergoing distraction (P = .005).

Conclusions: Pre- and post-operative speech assessments should include perceptual and instrumental assessment. Providers should incorporate patient counseling for those at risk for developing VPI postoperatively. Maxillary advancement and distraction can result in approximately 11% to 15% increase in nasalance scores, and deterioration in VP function. Individuals with previous speech surgery may demonstrate less deterioration in VP function following maxillary advancement and distraction, compared to those without previous speech surgery.

(1) Children’s Hospital of Philadelphia, Philadelphia, PA

Background/Purpose: The goal of this study was to describe the 10-year evolution of our surgical technique and institutional perioperative outcomes using posterior vault distraction osteogenesis (PVDO) in patients with syndromic and multisuture craniosynostosis.

Methods/Description: A retrospective review of patients who underwent PVDO for treatment of syndromic and multisuture craniosynostosis at a single institution over a 10-year period (2009 through 2019) was completed. Demographic data, perioperative outcomes, distraction patterns, and complications were analyzed. Outcomes for patients in the first 5 years of the experience (“early cohort”) were compared to those of the latter 5 years (“late cohort”).

Results: Of all, 110 patients underwent a total of 118 PVDO procedures. Syndromic patients represented 83.6% of the cohort (n = 92) and were significantly younger than nonsyndromic patients at the time of first PVDO (median 14.13 [6.63, 40.14] vs 42.71 [15.18, 59.61] months, P = .014). Median length of follow-up was 2.7 years (0.9, 5.00). Mean distraction distance in the anterior–posterior direction was 30.84 (SD = 7.39). Overall, 20 patients (18.18%) had 27 complications, related to either the distractor devices (4 patients), surgical site infection, device exposure, CSF leak with or without meningitis, VPS infection, or a combination (16 patients), with 11 requiring unanticipated reoperation, all of whom successfully completed PVDO. Compared to the early cohort, PVDO in the late cohort had faster median operative times (144.00 min [123.00, 189.00] vs 161.00 min [138.00, 199.00], P = .038), lower estimated blood loss as a percent of blood volume (28.45 [20.58, 45.32] vs 49.95 [31.05, 95.76], P < .001), and lower blood replacement as percent of blood volume (39.45 [23.80, 59.05] vs 56.25 [37.75, 110.10], P = .009). PVDO significantly delayed age to first BFOAR and age to first frontal surgery (P < .001).

Conclusions: Our 10-year experience with PVDO demonstrates continued overall safety and efficacy with improved perioperative outcomes over time. While it has become our first line of expansion in syndromic craniosynostosis, shortcomings such as need for device removal, infectious concerns due to transcutaneous hardware, and potential for CSF leak merit additional attention by the craniofacial community.

(1) Emory University, Atlanta, GA, (2) Emory University, Georgia Institute of Technology, Atlanta, GA

Background/Purpose: Craniofacial bone loss or maxillary bone disease (MBD) is a complex clinical problem with limited regenerative solutions. Currently, BMP2 is used as a bone-regenerative therapy in adults, but in pediatric cases of bone loss, it is not FDA-approved due to concerns of life-threatening inflammation and cancer. Development of a bone-regenerative therapy for pediatric cases of MBD will transform our ability to avoid the BMP2-associated inflammation and the morbidity associated with current autologous bone grafting techniques. Our research focuses on identifying alternative treatments for MBD, one of which is JAGGED1 (JAG1). We previously recapitulated the MBD phenotype in Wnt1-cre;Jagged1f/f mice and demonstrated deficient maxillary osteo- and vascular-genesis. We identified a novel noncanonical JAG1-NOTCH1 signaling target, JAK2, that is essential to induce differentiation of cranial neural crest cells (CNC) (osteoblast precursors) during intramembranous ossification for maxillary development.

Methods/Description: We incorporated JAG1-Fc-Beads complex (5 μM and 10 μM) into 4% PEG-MAL Hydrogels and delivered these treatments cranial critical sized (4 mm) defects in C57BL/6 mice as 3 separate doses (Initial dose, Week 4, Week 8). After 12 weeks, we harvested skulls and quantified differences in bone deposition between groups by MicroCT analysis. In addition, we investigated targets of JAG1-NOTCH noncanonical signaling in O9-1 cells, a CNC cell line. O9-1 cells were stimulated with recombinant JAG1-Fc bound to beads (5 μM) ± DAPT (15 μM), an inhibitor of the NOTCH canonical pathway for 12 hours and lysates were subjected to RNA sequencing using Illumina’s NEXTSeq system followed by gene set variation analysis. All data are presented as mean ± SD and subjected to ANOVA and Tukey’s post-test (P < .05) or FDR adjusted q values < .05.

Results: JAG1 delivery using synthetic hydrogels containing CNC cells into critical-sized calvarial defects in mice provided robust bone-regeneration. JAG1 upregulated expression of multiple NOTCH canonical pathway genes (Hes1), bone chemokines (Cxcl1), which were downregulated in the presence of DAPT. JAG1 induced a regulator of cytoskeletal organization and cell migration (Rhou), signaling targets (STAT5), promoters of early osteoblast cell proliferation (Prl2c2, Smurf1 and Esrra), and inhibitors of osteoclasts (Id1) which remain elevated with DAPT treatment, suggesting that JAG1 induces osteoblast proliferation through these noncanonical genes. Pathway analysis of revealed significant JAG1+DAPT-induced upregulation of multiple noncanonical pathways, including the cell cycle, tubulin pathway, regulators of Runx2 initiation, and phosphorylation of STAT5 pathway.

Conclusions: In total, our data show that JAG1 upregulates multiple pathways involved in osteogenesis, independent of the NOTCH canonical pathway. Moreover, our findings suggest that JAG1 delivery using a synthetic hydrogel is a bone-regenerative approach with powerful translational potential.

(1) Children’s Hospital of Philadelphia, Philadelphia, PA

Background/Purpose: Congenital oropharyngeal masses are rare (reported incidence of 1 in 20,000-200,000 live births) but can cause life-threatening airway compromise, significant patient morbidity, and impact quality of life. The purpose of this study was to describe the authors’ experience treating prenatally diagnosed oropharyngeal masses in a novel, multidisciplinary collaboration and to perform an outcomes analysis of neonates with prenatally identified oropharyngeal masses, identifying risk factors associated with less favorable outcomes.

Methods/Description: Our institutional programmatic fetal imaging database was queried to identify parents who underwent fetal imaging for an oropharyngeal mass between 1/2008 and 1/2019. Three analyses were conducted: Demographic/Mass Characteristics, Short-Term Outcomes, Long-Term Outcomes.

Results: Sixty-two cases met all inclusion criteria. The majority of masses were lymphatic malformations (n = 27, 47.4%), followed by teratomas (n = 22, 38.6%). The median mass volume from all available patient imaging (n = 57) was 60.54 cm ³ (range 1.73-742.5 cm ³ ). Thirteen pregnancies were interrupted and 6 infants expired. Fourteen (56%) of the surviving patients were delivered by EXIT procedure and all went to the NICU after birth with median stay of 36 days (range 3-215 days). All fetuses who did not survive had neck involvement on prenatal imaging. Mass involvement of the neck (P < .001), upper thorax (P = .002), orbit (P = .007), or ear (P = .044) was associated with increased mortality. There was no significant association between mass size and fetal outcome (χ ² (43) = 43.0, P = .47). Seventeen patients had over 1 year of follow-up (mean 5.3 ± 2.4 years). These patients underwent general anesthesia a total of 92 times (mean 5.4 ± 4.3) and had a total of 23 surgeries for mass resection and reconstruction (mean 1.6 ± 1.8). The great majority of patients required an artificial airway at birth, feeding support, and speech/swallow therapy.

Conclusions: Our intensive multidisciplinary approach is associated with a lower mortality rate than previously documented. While larger mass size in itself is not associated with increased mortality, mass involvement of the neck, upper thorax, orbit, and ear is associated with increased mortality. Perhaps due to the available delivery, breathing, and feeding interventions, neither airway compression/deviation/displacement, smaller stomach size, nor polyhydramnios on prenatal imaging is associated with increased neonatal demise. All neonates in this series required NICU level care. The great majority of patients with prenatally diagnosed oropharyngeal masses require an artificial airway and feeding support in the immediate postnatal period followed by speech/swallow therapy and auditory testing and interventions in the first 5 years of life. This is the largest series of patients with prenatally diagnosed oropharyngeal masses and can be used to guide counseling for parents in the prenatal period.

(1) Children’s Mercy Hospital-Kansas City, Kansas City, MO

Background/Purpose: A significant overlap exists between craniofacial disorders and sleep disorders breathing/obstructive sleep apnea and children. The goal of this session is to fill the knowledge gap in understanding sleep-related breathing disorders in children with complex craniofacial malformations. This session will highlight recent advances in the diagnosis and management in a succinct and interactive case-based format with a focus on obstructive sleep apnea across the lifespan from neonates with Robin sequence to teenagers with facial deformities. Attendees will also learn about state of the art obstructive sleep apnea management including surgical and nonsurgical options for children.

Methods/Description: This session will focus on filling the knowledge gap in understanding sleep related sequelae of common craniofacial disorders seen in pediatric practice that overlap with pediatric sleep disordered breathing (SDB). The majority of medical professionals do not undergo formal or structural training in sleep medicine during their early careers. Therefore, this proposal bridge the gap by (1) creating awareness of the learning gap pertaining to pediatric SDB and (2) sharing information to improve skills to eliminate the gap. Using common pediatric craniofacial disorders as a template, a case-based interactive format will be used for instruction. As a result of attending the session, participants will be better able to integrate diagnosis and management of SDB in the care of their patients.

(1) University of Arkansas for Medical Sciences, Little Rock, AR, (2) University of Arkansas for Medical Sciences College of Medicine, Little Rock, AR, (3) Arkansas Children’s Hospital, Little Rock, AR

Background/Purpose: Velopharyngeal insufficiency (VPI) is a common speech disorder in patients with a history of cleft palate (CP) or 22q11.2 deletion syndrome. Pharyngeal flap (PF) and sphincter pharyngoplasty (SP) are 2 common surgeries to treat this disorder by decreasing unwanted nasal air emission and hypernasal resonance. Because otologic issues in patients with CP may be more frequent after surgery for VPI, we examined whether Eustachian tube dysfunction (ETD) was related to either type of surgery.

Methods/Description: We retrospectively analyzed the difference in risk of ETD by both surgical group (PF vs SP) and by proxies for postoperative nasal obstruction. These proxies included postoperative resonance measures and development of obstructive sleep apnea (OSA). Our patient population included pediatric patients who had undergone either primary PF or SP from 2006 to 2017. Demographics, pre- and postoperative resonance assessment (including perceptual speech assessment [PSA], nasometry, and pressure-flow testing [PFT]), postoperative complications, otologic history, type of palatal cleft (CP vs submucous CP vs no CP), and incidence of OSA were obtained from a chart review. Outcomes for 201 PF and 24 SP patients were analyzed. Both groups had similar preoperative measures, except the PF group had higher hypernasality by PSA. Postoperatively, the PF group demonstrated lower hypernasal resonance by nasometry and PSA. No differences were found between PF and SP groups with regard to ETD. Proxies for postoperative nasal obstruction (resonance and OSA) were not predictive of postoperative ETD. However, degree of CP was found to be a risk factor for ETD. There was no significant difference in the effects of PF and SP on ETD in this study. Neither lower range hypernasality nor incidence of OSA had any negative impact on ETD. Degree of CP was the only significant risk factor for ETD that this study identified.

(1) University of California, Irvine, Orange, CA, (2) Children’s Hospital Orange County, Orange, CA

Background/Purpose: Polysomnography is vital in evaluating neonatal airway obstruction. Although many institutions use sleep data to select patients for mandibular distraction osteogenesis (MDO), no “normal” published references exist for this age. We present normative polysomnography data for newborns age 0 to 1 month. We also compare this normative reference to pre- and postoperative data of infants of this age undergoing MDO.

Methods/Description: Following IRB approval, normative subjects were recruited from our NICU to undergo nap polysomnography. Included were infants without airway obstruction, gestational age 37 to 42 weeks, and age less than 28 days. Data included apnea–hypopnea indices, pulse oximetry, CO2, EEG, and ECG. One blinded sleep physician read all studies. Sleep data for newborns undergoing MDO were collected prospectively (2016-18). All data were collected and analyzed using REDCap and SPSS software.

Results: Twenty-two neonates without airway obstruction provided normative sleep data; median age at polysomnography was 5 days and median sleep time was 181.5 minutes. Median total apnea–hypopnea (AHI), obstructive apnea–hypopnea (OAHI), and central apnea indices (CAI) were 6.9, 4.9, and 0.7 events/hour. The median O2 nadir was 91%. Polysomnography was done on 13 neonates with airway obstruction before and after MDO. Median age at preoperative study was 7 days and median sleep time was 333.0 minutes. Median AHI was 38.3, OAHI was 37.0, and CAI was 1.9. Median O2 nadir was 83%. Prior to undergoing MDO, neonates with airway obstruction had significantly worse AHI, OAHI, and O2 nadir than normative counterparts (P < .001). There was no significant difference in CAI. Postsurgical sleep data were collected after activation phase of MDO; median sleep time was 343.0 minutes. In this group, median AHI was 6.1, OAHI was 4.0, and CAI was 1.3. Median O2 nadir was 92.5%. Paired t tests demonstrated significant improvements in OAHI, AHI, and oxygen saturation nadir after MDO (P < .001). When comparing the normative group to neonates who underwent MDO, there was no significant difference in oxygenation or any apnea hypopnea index.

Conclusions: In children, OAHI > 1 is considered abnormal; this norm has been extrapolated to neonates. Our findings demonstrate “normal” neonates have more obstructive and central apneic events than previously appreciated, with a median of 5.0 obstructive and 7.6 total events per hour. Furthermore, newborns without airway obstruction still exhibit a wide range of “normal” OAHI values (1.6-35.7). Newborns with airway obstruction had significantly worse OAHI/AHI and O2 saturation nadir than their nonobstructed counterparts and exhibited improvement in normative levels following MDO. Each center with a multidisciplinary MDO team should consider collecting normative neonatal sleep data to reflect their regional population, enabling calibration of existing patient selection algorithms, and informing important discussions with anxious parents.

(1) Nationwide Children’s Hospital, Columbus, OH, (2) University of Utah, Salt Lake City, UT

Background/Purpose: Both congenital hearing loss and persistent transient hearing loss associated with chronic middle ear disease are prevalent in children with craniofacial anomalies. Consequently, a strong audiologic protocol is recommended to identify, monitor, and treat hearing loss and to reduce developmental consequences (ACPA, 2018). The purpose of this presentation is to provide an update to a quality improvement (QI) project aimed at improving adherence to an evidence-based audiologic management protocol for children with cleft palate and craniofacial anomalies.

Methods/Description: A multidisciplinary QI project team was assembled at a single large volume Cleft Lip and Palate/Craniofacial Center within a pediatric hospital setting. The QI team included an audiologist, speech/language pathologist, pediatric otolaryngologist, and nursing staff from the cleft team. Baseline data were collected through retrospective chart review of children provided care through our clinic between 2012 and 2019, ages 0-6 with cleft palate, and other craniofacial anomalies to document audiologic and ENT follow-up for children (N = 205, 53% male). Within this cohort, 56% were children with cleft palate in isolation (CP) while 44% were children with cleft lip and palate (CL & P). Newborn hearing screening results, audiologic evaluations through age 5, associated ENT treatment for middle ear disease, and missed opportunities for assessment were recorded. Noncompliance with guidelines was assessed using a cause-effect analysis and attributed to either patient-related reasons (ie, parent refusal, patient untestable, loss to follow-up) or provider-related reasons (ie, unaware of need for testing, deferred testing). Several interventions were implemented between 2016 and 2019 to improve adherence to recommended care guidelines, including increased audiology coverage of clinics, formulation of an evidence-based audiologic protocol, weekly triaging of patient schedules, provider education, and facilitation of communication among providers.

Results: Baseline analysis revealed 55% of patients received audiologic care commensurate with ACPA parameters. Barriers to audiologic protocol adherence were attributed to patient/parent related noncompliance (19%), provider-related noncompliance (69%), or a combination of both patient- and provider-related reasons (12%). With the introduction of several interventions aimed at protocol formulation, provider knowledge, and clinic logistics, adherence increased to a mean of 85% in 2018 and 2019. Additional education materials targeting parent/family education have been developed to aid continued improvement.

Conclusions: Implementation of an evidence-based audiologic care protocol, consistent with ACPA parameters of care, is feasible in a high-volume multidisciplinary clinic if strategies to improve access to care, educate team members and families, and enhance communication among team providers are implemented.

(1) UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, (2) University of Pittsburgh, Pittsburgh, PA, (3) University of Pittsburgh School of Medicine, Pittsburgh, PA

Background/Purpose: To evaluate the impact of breast milk feeding (BMF) on otologic outcomes in the first 3 years of life in children with and without cleft palate (CP). We hypothesized that children in both groups who received breast milk would receive their first set of tubes at older ages and be less likely to experience effusion prior to tubes.

Methods/Description: In a previous study, 102 biological mothers of children with CP and 102 mothers of children with intact palate seen at a tertiary care children’s hospital completed a survey with questions regarding whether breast milk was provided and duration of breast milk provision as well as demographic information including household composition and maternal education level. Children were excluded if they had a history of receiving thickened liquids prior to 3 months of age. Otologic outcomes, including tympanostomy tube history and the result of audiologic examinations, were obtained from review of the electronic medical record. In the subset of patients for whom access to primary care provider (PCP) records were available, appointments with middle ear effusion during the first year of life were recorded. The impact of BMF on these outcomes was evaluated separately in the CP and control groups using logistic regression, Wilcoxon rank-sum, and log-rank tests.

Results: Similar proportions of children in the CP and control groups were female (44.1% and 43.1%, respectively). Median age at data collection was also similar (48.9 months, range 21.7-78.9 months, CP and 46.9 months, range 20.6-78.5 months, control). In both groups, 79 (77.5%) mothers initiated BMF. Median duration of BMF was 2 months (range 1 day-18 months) in children with CP and 3 months (range 1 day-26 months) in children with intact palates. Ninety-four (92.2%) of those with CP and 42 (41.2%) of those with intact palates received tympanostomy tubes. Effusion was observed at first tube placement in 94.5% of children with CP. Although not significant, in children with CP, serous effusion, rather than mucoid or purulent, was more common in children who received breast milk compared with those who did not (OR: 3.83, 95% CI: 0.811-18.1, P = .090). PCP appointments with effusion during the first year of life, age at first tube placement, presence of effusion at first tube placement, indication for tubes, absent otoacoustic emissions, elevated auditory brainstem response thresholds, and flat or negative tympanograms prior to first tubes were not significantly different between patients with and without BMF in either group. Comparing the subset of patients who received breast milk for at least 6 months with those who received no breast milk also did not reveal any significant differences in these otologic and audiologic outcome measures.

Conclusions: Although this study was likely underpowered, BMF could reduce the prevalence of mucoid or purulent effusion in children with CP. This has positive implications since serous effusions are more likely to clear and be of shorter duration than mucoid.

(1) Children’s Hospital of Philadelphia, PA, (2) University of Pittsburgh Medical Center, PA

Background/Purpose: Submandibular gland pathologies requiring excision present with varying comorbidities and effects on surrounding tissue architecture, and we hypothesize that this spectrum of indications confers different risks of adverse events. The purpose of this study is to elucidate the perioperative profile of submandibular gland excision in children across an assortment of surgical indications.

Methods/Description: The ACS NSQIP-Peds dataset was queried for submandibular gland excision performed from 2012 through 2017. Indications were subclassified based on ICD-9 and ICD-10 codes. Complications, readmissions, and reoperations were analyzed with appropriate statistics.

Results: Submandibular gland excision in children (n = 304) were mostly performed for ptyalism (56.9%), followed by inflammatory conditions (20.7%), benign neoplasms (10.9%), mucoceles (10.2%), and malignant neoplasms (1.3%). Overall related adverse events were 7.6% (n = 23 of 304). Patients requiring submandibular gland excision for ptyalism were significantly younger (9.2 years vs 12.4 years) and underwent significantly longer procedures (123 min vs 109 min) likely due to extra time to perform duct ligation and bilateral gland excision in these cases. Ptyalism was associated with significantly higher related adverse events (P = .010, 11.0% vs 3.1%), related readmission (P = .013, 9.2% vs 2.3%), and medical complications (P = .013, 4.6% vs 0.0%), which included a significantly higher risk of pneumonia (P = .050, 2.9% vs 0.0%). Mucoceles on the other hand were associated with significantly higher rates of surgical complications (P = .008, 6.5% vs 0.7%), which included a significantly higher risk for superficial surgical site infections (P = .003, 3.2% vs 0.0%). Increased risk for medical complications with ptyalism is likely related to these children having baseline pulmonary disease secondary to uncontrolled salivary secretion with consequent aspiration. Our data set supports that children with ptyalism have significantly higher rates of overall respiratory comorbidities (P < .001, 53.8% vs 12.2%), including chronic lung disease (P < .001, 23.7% vs 1.5%), supplemental oxygen support (P < .001, 15.6% vs 1.5%), tracheostomy (P < .001, 21.4% vs 2.3%), and ventilator dependence (P < .001, 13.9% vs 0.8%). Much of this aspiration could also be secondary to neurocognitive impairment with consequent lack of oral competence, which is supported by these patients having significantly higher rates of developmental delay (P < .001, 87.9% vs 9.9%) and neuromuscular disorders (P < .001, 42.8% vs 2.3%). Patients undergoing submandibular excision for benign (P all ≥ .082) or malignant (P all ≥ .565) neoplasms did not have significantly higher rates of any indexed postoperative adverse event.

Conclusions: Patients requiring submandibular gland excision for ptyalism represent a unique cohort than those requiring excision for other indications, with significantly higher burden of preoperative risk factors, intraoperative durations, and postoperative adverse events.

(1) The Warren Alpert Medical School of Brown University, Providence, RI

Background/Purpose: Children with craniofacial anomalies are at higher risk for developing obstructive sleep apnea (OSA). In patients with cleft palate, upper airway obstruction is likely multifactorial and related to underlying craniofacial differences as well as anatomical changes following cleft palate repair and other pharyngeal procedures. Previous studies have suggested that 15%-32% of patients with cleft palate have OSA, with the percentage potentially higher in syndromic patients. This study aims to assess the prevalence of OSA in patients with palatal clefts in a single institution and determine if there are predisposing factors.

Methods/Description: A retrospective chart review was conducted of patients aged 0 to 18 years with cleft palate with or without cleft lip under the care of a multidisciplinary Craniofacial Team at a tertiary children’s hospital from January 2010 to August 2020. Demographics, clinical records, operative reports, and polysomnography (PSG) data were analyzed. Palatal clefts were classified as submucous or by Veau classification (I-IV). Descriptive statistics and Pearson χ ² tests were performed to evaluate the relationships between OSA (diagnosed clinically or with PSG) and syndromic status, Veau classification/cleft type, age at primary palatal repair, and repair technique.

Results: Of all, 214 children (53% male, mean age: 7.98 yrs) met inclusion criteria. Children were predominately White (52%) and of Hispanic/Latino ethnicity (29%). A slight majority had isolated cleft palate (52%); 27/214 (12.6%) patients had at least one PSG. Overall prevalence of OSA was 11.2% (24/214); 13% of patients underwent tonsillectomy and/or adenoidectomy; 29% of children had a syndromic diagnosis, including Pierre Robin Sequence (44%), Stickler Syndrome (17%), and 22q11.2 Deletion Syndrome (10%). Syndromic patients were significantly more likely to have a documented sleep study (26%) (P = .0002). Syndromic patients were more likely to be diagnosed with OSA (21.6%) than nonsyndromic patients (9.4%), with the data approaching statistical significance (P = .053). The most common repair techniques were Furlow palatoplasty, Radical Intravelar Veloplasty (IVV), and Overlapping IVV. There was no significant relationship between Veau classification/cleft type (P = .926), presence of cleft lip (P = .849), age at time of primary palatal repair (P = .533) or palatal repair technique (P = .890), and the diagnosis of OSA.

Conclusions: Patients with cleft palate are at increased risk for OSA relative to the general population, although the risk may be lower than has previously been reported. In this study, syndromic patients were more likely to develop OSA, with data approaching statistical significance. No other predictive factors were identified. These findings, while limited due to the retrospective nature and size of the study, improve understanding of the development of OSA in children with palatal clefts and may inform anticipatory guidance for clinicians and parents.

(1) Emory University Hospital, Atlanta, GA, (2) Long Island Plastic Surgical Group, Garden City, NY, (3) Children’s Healthcare of Atlanta at Scottish Rite, Atlanta, GA, (4) Childrens Healthcare of Atlanta, Atlanta, GA

Background/Purpose: The mainstay of analgesia in orthognathic interventions for maxillary hypoplasia is perioperative opioids; however, the side effect profile is broad with the potential for well-described deleterious effects. The suprazygomatic maxillary nerve block has been previously shown to be effective in decreasing pain associated with palatal surgery. To date, there have been no studies detailing the use of maxillary nerve blocks as an adjunctive pain control measure during correction of maxillary hypoplasia. Consequently, we sought to evaluate the efficacy of intraoperative, ultrasound-guided bilateral suprazygomatic maxillary nerve blockade in decreasing postoperative narcotic consumption in patients undergoing Le Fort I level surgical orthognathic correction of cleft-related maxillary hypoplasia.

Methods/Description: Methods: Between January and December 2019, patients underdoing suprazygomatic maxillary nerve blockade for orthognathic correction of maxillary hypoplasia via either Le Fort I advancement or distraction were prospectively collected and compared to controls. Patient demographics, narcotic use (represented as morphine milligram equivalents per kg; MME/kg), self-reported pain scales, operative times, length of stay (LOS), and complication rates were compared.

Results: Over the 12-month interval, 40 patients met inclusion criteria (n = 19 Block; n = 21 Control). Mean ages were 15.6 and 15.9 years, respectively. The block group demonstrated a significant reduction in postoperative narcotic requirements on POD1 and POD2 when compared to controls (POD1: 0.020 mg/kg vs 0.066 mg/kg, P < .005; POD2: 0.030 mg/kg vs 0.080 mg/kg, P < .016), with a trend toward significance thereafter. Corroboratively, self-reported pain scores in the first 24 hours were significantly decreased in the block compared to control groups with a trend toward significance thereafter (POD1: 1.13 vs 2.72, P < .001; POD2: 1.72 vs 2.56, P < .08; POD3: 1.21 vs 2.07, P < .06). LOS was decreased by an average of 1 day in the block group, operative times were unchanged, and neither group evidenced perioperative complication or return to service within 30 days.

Conclusion: Administration of bilateral suprazygomatic maxillary nerve blocks in patients undergoing Le Fort I maxillary osteotomy for correction of cleft-related maxillary deficiency demonstrated a significant reduction in postoperative narcotic requirements, self-reported pain scales, and LOS without increased complications, suggesting its utility as a safe and effective analgesic adjunct in this patient population.

(1) University of Illinois at Chicago, Chicago, IL, (2) University of Illinois College of Medicine, Chicago, IL

Background/Purpose: Alveolar bone grafting (ABG) supports eruption of the canine tooth, allows orthodontic movements, and stabilizes the alveolar arch. While many single surgeon series have been published on ABG technique, there have been few comparative studies, and no high-quality randomized studies, thus limiting data-driven decisions in ABG. We hypothesized the lack of evidence to guide practice causes significant differences to exist between surgeons with regards to the preoperative workup, timing of surgery, materials used for bone graft, surgical techniques, perioperative management, and postoperative evaluation. The purpose of this study is to report areas of agreement and disagreement in the clinical practice of ABG in order to guide future research on the topic.

Methods/Description: A deidentified online survey was sent to 791 surgeon members of the American Cleft Palate-Craniofacial Association via REDCap. Data collected included current alveolar bone graft surgical techniques in addition to pre- and post-operative protocols. In addition to raw survey results, the data were analyzed to compare practices between plastic surgeons and maxillofacial surgeons, as well as between craniofacial fellowship trained and nonfellowship trained surgeons.

Results: The survey was sent to 791 subjects and there were 177 responses (22% response rate). Plastic surgeons (PS) comprised 102 (58%) responses and 73 (41%) responses were from oral and maxillofacial surgeons (OMFS). The majority of respondents (72%) were practicing in the United States. Many were craniofacial fellowship trained (66.5%). There were many aspects of ABG agreed on by a large majority of surgeons. Most surgeons perform grafting between ages 6 to 10 (82%), do not stage soft and hard tissue closure (84%), use iliac crest for primary grafting (87%), and use iliac crest for secondary grafting (74%). Surgeons were more evenly split on several aspects: sulcular (63%) versus Abyholm (32%) incisions, cancellous bone (55%) versus cortical chip with cancellous bone (36%), and obtaining postoperative imaging at 3 to 5 months (33%), or 6 to 12 months (47%). There was little agreement on perioperative treatment protocols. There were multiple significant differences between specialties, including greater use of cone beam CT scan among OMFS, more consideration for lateral incisor presence among OMFS, more consideration for noniliac crest materials in secondary grafting by OMFS, and more trochar-based iliac crest harvest among PS, as well as others. There were also significant differences in practice between fellowship-trained and nonfellowship trained surgeons, as well as between more experienced and less experienced surgeons.

Conclusions: There is significant variability in the practice of alveolar bone grafting. More research should be performed to determine if there is any superiority of any of these technical or perioperative variations.

(1) University of Pennsylvania, Philadelphia, PA, (2) Children’s Hospital of Philadelphia, Philadelphia, PA

Background/Purpose: Secondary alveolar bone grafting (SABG) of autologous bone from the iliac crest is a widely accepted modality for repair of residual alveolar cleft in patients with mixed dentition with previously repaired complete cleft lip and palate. There remains debate regarding surgical timing and pre-, peri-, and post-operative management of these cases. Herein we describe our institution’s experience with and outcomes of alveolar repair in patients with a history of repaired unilateral complete (UCLP) and bilateral complete (BCLP) cleft lip and palate.

Methods/Description: This is a retrospective cohort study of patients with a history of repaired UCLP and BCLP who underwent maxillary SABG between January 1, 2012, and September 9, 2019, and had at least 6 months of follow-up in the cleft team outpatient clinic. Exclusion criteria included syndromic and immune-deficient patients. Medical records of all patients were reviewed for demographic information, pre-, peri-, and post-operative course, and imaging; discrepancies in the clinical note and imaging record were resolved by reevaluation by our orthodontic team. 3DCBCT was used to assess postoperative graft take at the 6-month postoperative visit. The orthodontic cleft team classes take as excellent, adequate, none, or forming a continuous labial bridge. “Adequate” is defined as clinically sufficient for dental implant, while “excellent” is defined as take beyond the alveolus to the hard palate.

Results: One-hundred and fifteen patients with UCLP and 40 patients with BCLP met our inclusion criteria. Average patient age (SD) at operation was 8.13 (±1.26). On univariate analysis, age at operation >9 years old, history of oronasal fistula (ONF), history of cleft lip or palate revision, and history of international adoption were all found to significantly increase likelihood of graft failure (P < .05). On multivariate analysis, age >9 years old was found to be the single most significant predictor of graft failure (P < .05). Patients with at least adequate take at either the single cleft site or bilateral cleft sites were found to be significantly (P < .05) younger than patients with take classified as none in at least one cleft site (mean age 7.99 vs 8.32 years, respectively) on t test. There was no significant difference in graft outcomes between patients with unilateral or bilateral cleft lip and palate, and no one variable was found to significantly correlate to increased complication rates. Our practice graft success rate overall was 86.2%, with a complication rate of 7.7%. Failure rate was higher in patients with BCLP (20.0%) than UCLP (11.2%).

Conclusions: SABG is an integral part of the cleft lip and palate surgical treatment series; this study identified several outcome predictors for both graft failure and adverse events, most significant of which was age at operation. While the mixed dentition phase often extends to 12 years of age, it is recommended to perform bone grafting before 9 years of age to optimize outcomes.

(1) Boston Children’s Hospital, Boston, MA

Background/Purpose: Historically success rates for alveolar bone grafting have been determined using 2-dimensional periapical radiographs taken > 6 months postoperatively. However, 2-dimensional analyses with dental radiographs overvalue the osseous bridge because of superimposition and distortion and it is not possible to assess bone volume in the horizontal plane. Cone beam CT (CBCT) scans provide 3-dimensional imaging and accurately display the bony anatomy that can be used to determine success. The purpose of this study was to evaluate the success of alveolar bone grafting using a 3-dimensional analysis in a large cohort of patients treated by one surgeon.

Methods/Description: Retrospective case series of patients with cleft lip/palate (CL/P) who had iliac crest bone graft by one surgeon and had CBCT scans taken > 6 months after the graft. Vertical height and labiopalatal (horizontal) thickness at the cervical, middle, and apical root levels of the adjacent teeth were scored from 1 to 4 (1: < 50%; 2: 50%-75%; 3: >75%; 4: 100%) and the nasal floor height compared to the unaffected side in patients with unilateral CL/P. Scores of 3 and 4 were considered good/excellent and the graft was deemed successful if there was a score of 3 or 4 at every level in the vertical and horizontal planes. CBCT scans were assessed by 2 independent raters; inter- and intra-rater reliability was good to excellent (ICC > .85). Descriptive statistics were summarized.

Results: In all, 887 patients had alveolar bone grafting by one surgeon between 2005 and 2020. There were 600 alveolar cleft sites (59% male/41% female), 340 unilateral and 130 bilateral with adequate CBCT scans taken a mean of 14 + 8.1 months after grafting. Mean age at grafting was 10.2 years + 2.7 years. Alveolar bone grafting (scores of 3 or 4 at all levels) was successful in 94% of patients.

Conclusions: Three-dimensional assessment of 600 alveolar bone grafts demonstrate good to excellent outcomes in 94% of patients. Future studies will assess predictor variables associated with bone graft outcomes.

(1) Yale University School of Medicine, Department of Plastic and Reconstructive Surgery, New Haven, CT

Background/Purpose: Black and Hispanic/Latino patients in the United States often experience poorer health outcomes in comparison to White patients. We aimed to assess the impact of race on quality measures following orthognathic surgery.

Methods/Description: There were 8809 pediatric and young adult orthognathic surgeries (age < 21) isolated from the Kids Inpatient Database from 2000 to 2012. Procedures were grouped into cohorts based on the preoperative diagnosis: apnea, malocclusion, or congenital anomaly. T-tests and χ ² analyses were employed to compare complications, length of stay (LOS), and costs among Black, Hispanic, Asian/Pacific Islander, and Other patients in comparison to White patients. Multivariable regression was performed to identify associations between sociodemographic variables and the primary outcomes. Post hoc χ ² analyses were performed to compare proportions of patients of a given race/ethnicity across the 3 surgical cohorts.

Results: Compared to White patients, complication rates were increased among Hispanic patients (2.1% vs 1.3%, P = .037) and Other patients treated for apnea (8.7% vs. 0.83%, P = .002). Hospital LOS was increased in both Black (3.3 vs. 2.1 days, P < .001) and Hispanic (2.9 days, P < .001) patients. Costs were higher than Whites ($35,633.47) among Hispanic ($48,029.15, P < .001), Black ($47,034.41, P < .001), and Asian/Pacific-Islander ($44,192.49, P < .001) patients. White patients comprised a larger proportion of the malocclusion group (77.8%) than apnea (66.9%, P < .001) or congenital anomaly (59.1%, P < .001), while the opposite was true for Black, Hispanic, and Asian/Pacific-Islander patients.

Conclusions: Non-white patients experienced increased complications, LOS, and costs following orthognathic surgery. Further studies are needed to better understand the causes of disparity and their clinical manifestations.

(1) Johns Hopkins All Children’s Hospital, Harrisburg, PA, (2) University of South Florida, Tampa, FL

Background/Purpose: The purpose of this study is to examine the association of steroid use during orthognathic surgery and postoperative outcomes including major complications, 90-day all-cause readmission, and postoperative length of stay.

Methods/Description: A retrospective review was implemented utilizing the Pediatric Health Information System (PHIS) database from 2004 to 2014 was undertaken. Steroid exposure was defined as having been billed for the generic drug code for Dexamethasone (154035) at any time for up to 7 days from the surgery date. Unadjusted and adjusted random-intercept logistic regression models were utilized to assess the association between steroid exposure and these outcomes.

Results: The sample included 5194 patients, 54% of patients were exposed to steroids, with 20.16% exposed only on the day of surgery, 27.76% on the day of surgery and after, and 6.22% after the day of surgery. In models adjusting for age, sex, race, procedure, and hospital variation, the odds-ratio of steroid exposure was 3.40 (95% CI = 2.93-3.96) for an increased length of stay, 1.04 (95% CI = 0.8-1.35) for major complications and 1.19 (95% CI = 0.95-1.50) for 90-day all-cause readmission.

Conclusions: The administration of steroids in patients undergoing orthognathic surgery is significantly associated with increased odds of length of stay. This may be due to a large set of patients receiving steroids during orthognathic procedure are less healthy than those not selected to receive steroids, and thereby require an increased length of stay. The limitations of large, administrative databases do not allow determination of this, but future prospective study is warranted.

(1) Yale University School of Medicine, New Haven, CT, (2) University of Connecticut, Orthodontics and Yale School of Medicine, Plastic and Reconstructive Surgery, Farmington/New Haven, CT, (3) Johns Hopkins School of Medicine, Baltimore, MD, (4) Yale University School of Medicine, Department of Plastic and Reconstructive Surgery, New Haven, CT

Background/Purpose: Recent studies have shown that orthognathic surgery can improve age and personality perception among a subset of patients with dentofacial deformities. This study aims to expand upon these studies by assessing the role of patient profile on perceived changes following surgery.

Methods/Description: The pre- and post-operative 3D images of 65 patients operated on by a single surgeon were separated and randomly distributed across 6 online surveys. Fifteen plastic surgery trained and 8 untrained respondents predicted the age of the patient and rated several personality attributes of the patient on a Likert scale. Patients were then categorized into subgroups based on facial profile: convex, concave, or straight. Both paired and unpaired t tests were used to measure differences pre- and post-operatively and across facial subgroups. Multivariate regression analysis and post hoc receptor operator curve (ROC) analyses were utilized to adjust for and quantify the influence of mediating demographic factors.

Results: A decrease in perceived age following orthognathic surgery was seen overall (−1.58 years, P < .01) and in all 3 profile subgroups: concave (−1.38 years, P = .04), straight (−1.29 years, P = .02), and convex (−2.0 years, P ≤ .01). After controlling for differences in patient age and demographics in subgroups, there were no significant differences in age change based on facial profile. Rather, older age at the time of surgery was independently associated with greater perceived age changes (P = .04); older patients (>25.3 years, determined by ROC) experienced greater net decreases in perceived age in comparison to younger patients (−2.5 vs −1.2 years, P < .01). Improvements were seen in overall attractiveness (P < .01) and in each of the 5 tested patient personality characteristics following surgery (P < .01). These differences were not significantly associated with different patient sociodemographics or facial profile.

Conclusions: Our data add to the growing base of evidence that orthognathic surgery improves both age and personality perception among patients with various forms of dentofacial dysharmony. The more modest decreases in perceived age among patients receiving orthognathic surgery in comparison to other facial procedures may due to the young age of the patient population, particularly among those with more severe (ie, concave) deformities. Clinically significant decreases in perceived age are more likely to be gained by patients receiving orthognathic surgery at an older age.

(1) University of Toronto, Toronto, Ontario, Canada, (2) Three Rivers Oral and Facial Surgery, Portland, OR, (3) Faculty of Dentistry, University of Toronto, Toronto, Ontario, Canada

Background/Purpose: In recent years, numerous materials have been proposed for use in reconstructing patients with a cleft maxillary alveolus, challenging the gold standard of using iliac crest bone grafts. Given the mixed evidence for use of these materials, the objective of this systematic review is to compare the success rates of the various materials used for secondary cleft alveolar bone grafting during the mixed dentition phase.

Methods/Description: A comprehensive search of prospective studies evaluating two or more materials was applied to the following electronic databases: MEDLINE, EMBASE, Cochrane Library, Scopus, and Google ScholarTM. To identify grey literature, abstracts from select conferences as well as references of included studies were searched. No limitations were placed on language. The search and data extraction were conducted in duplicate. Outcomes were combined via a multivariable network meta-analysis with random effects, adopting a Bayesian approach. Additionally, surface under the cumulative ranking (SUCRA) values, used to determine the probability of each treatment being the best, were calculated.

Results: From an initial yield of 2280 studies, 18 were included in this review. Evaluating percent bone fill of the defect following 3 to 12 months of follow-up, it was suggested that platelet-rich fibrin in combination with an iliac crest bone graft, hydroxyapatite in combination with collagen, or iliac crest graft alone were the most effective means of reconstruction (9 studies and 181 patients; 74.1% [95% CI 59.3-90.7], 64.6% [95% CI 49.1-80.7], 59.9% [95% CI 52.8-66.7], respectively). When evaluating bone volume gained at 6 to 12 months, iliac crest graft alone was suggested to be superior to other methods (10 studies and 192 patients; 431 mL [95% CI 402-458], compared to 342 mL [95% CI 303-383] and 186 mL [95% CI 132-240] for hydroxyapatite in combination with collagen and platelet-rich fibrin in combination with an iliac crest, respectively).

Conclusions: Results of this study suggest the superiority of iliac crest with or without platelet-rich fibrin or hydroxyapatite in combination with collagen for the reconstruction of cleft alveolar defects. Given the high risks of bias and low sample sizes of all the included studies, further high-quality studies are necessary to make definitive conclusions regarding the effectiveness of these grafting materials.

(1) Yale University School of Medicine, New Haven, CT, (2) Yale New Haven Hospital, New Haven, CT, (3) Yale University, New Haven, CT, (4) Yale University School of Medicine, Department of Plastic and Reconstructive Surgery, New Haven, CT

Background/Purpose: Postoperative nausea and vomiting (PONV) is a major clinical end-point for directing enhanced recovery after surgery (ERAS) protocols in facial plastic surgery. Orthognathic surgery has historically been associated with a high incidence of PONV. This study aimed to assess the incidence of PONV in a large number of patients undergoing orthognathic surgery at a single institution and to analyze contributing factors.

Methods/Description: A retrospective cohort study was performed including all patients who went orthognathic surgery at our institution from 2011 to 2018 with 12 months minimum clinical follow-up. Multiple patient characteristics and surgical details were recorded for each patient. The incidence of PONV was determined by documented emesis or administration of as needed anti-emetic for nausea. Receiver operating characteristic (ROC) curves were constructed to determine threshold amounts of narcotics administered perioperatively, postoperatively, and overall that corresponded with PONV. Relative risk (RR) were calculated for all groups with AUC > 0.70. Regression analysis was used to evaluate the relationship between number of postoperative antiemetic PRN doses and MEQs. Patients were stratified by gender, age, and BMI. Statistical analysis was performed using student t tests, Fischer exact tests, and one-way ANOVA (P < .05 was significant).

Results: Five hundred patients were included; mean age was 22.7 years (range: 13-60); 54% were female; 91.8% underwent Lefort-I (21.1% with segmentation), 84.5% underwent mandibular split, and 84.2% underwent genioplasty; the mean number of these 3 procedures performed was 2.6. Average procedure length was 4.6 ± 1.3 hours and average length of stay was 2.23 ± 1.36 days. Within their hospital stay, 67.2% of patients experienced PONV and 27.5% experienced emesis. There was no statistically significant difference between cleft history, total number or individual type of osteotomies performed, receipt of longer-acting local anesthetics, or procedure length. Threshold values for narcotics administered postoperatively leading to PONV in various demographic subgroups were 10.6 mg MEQ (sensitivity 0.92, specificity 0.75, RR = 3.68) for BMI < 18.5, 13.4 mg MEQ (sensitivity 0.93 and specificity 0.75 RR = 3.73) for BMI > 35, 10.83 mg MEQ (sensitivity 0.875, specificity 0.83, RR = 3.21) for patients in the oldest age group (46-60 years).

Conclusions: Orthognathic surgery can dramatically improve dentofacial harmony and aesthetics. Unfortunately, there is a high incidence of PONV appears that is independent of surgical technique. Instead, efforts to reduce PONV in orthognathic surgery should focus on limiting use of narcotics to 10 mg MEQ postoperatively, particularly in patients at outside of a normal weight range or age greater than 45 years in order to help optimize ERAS protocols.

(1) University of Illinois at Chicago, Chicago, IL, (2) Northwestern University Feinberg School of Medicine, Chicago, IL, (3) University Of Illinois at Chicago/Shriners Hospitals for Children, Chicago, IL

Background/Purpose: Alveolar bone grafting is a critical part of the comprehensive dental rehabilitation of patients with orofacial clefting, and by far the most common material for grafting is autologous bone. Across literature, the success of this procedure seems to vary widely. We sought to review existing published information on outcome of autologous alveolar cleft bone grafting, and the clinical and radiological methods used for assessment; analyze the range of success and complication rates reported; identify the employed grafting techniques, perioperative protocols, and their influence on success rates.

Methods/Description: A systematic review was carried out adhering to guidelines set in the PRISMA Statement. A National Library of Medicine search was performed for studies on alveolar bone grafting in cleft patients. Studies on nonhuman subjects, published in languages other than English and employing nonautologous graft material were excluded. Studies of primary bone grafting were excluded. Studies with nonautologous materials were included in the study so long as they had an autologous bone comparison group—however only the autologous groups were included in analysis. Titles and abstracts were evaluated by 2 independent researchers. The full-text of these articles were reviewed and the studies with objective data on rates of success, failure, reoperation, or complications were included. These were further classified using the American Society of Plastic Surgeons Level of Evidence Rating scale and evaluated for Risk of Bias or quality using the Cochrane ROB tool, Cochrane ROBINS-1, and the modified or standard Newcastle-Ottawa Scale, as appropriate.

Results: The initial search delivered 682 abstracts, of which 205 studies were selected for full-text review. Of these, 105 met inclusion criteria. Study population size ranged from 6 to 340 patients. Of all, 101 studies obtained postoperative imaging studies, mostly dental imaging. Eleven studies defined graft success through primarily clinical criteria (10%), 7 used a combination of clinical and radiographic criteria (6%), and 79 primarily used radiographic criteria to define success rates (75%). The clinical and radiographic criteria used for success varied widely among studies, with the most common method being the Bergland (Oslo) Scale (40%). Success rates also varied widely, from 33% to 100%. Formal meta-analysis is ongoing to determine if there are predictors of graft success across the included studies. Targets of evaluation include source and type of bone grafting, patient age, and timing of grafting with respect to canine development.

Conclusions: Although a large amount of literature has been published on cleft alveolar bone grafting, it is difficult to interpret the factors that contribute to success at face value. Meta-analysis provides a mechanism to understand the effect of variables on success rates across multiple studies.

(1) Children’s Hospital of Philadelphia, PA, (2) University of Pittsburgh Medical Center, PA

Background/Purpose: The purpose of this study was to utilize a multicenter data set to elucidate the perioperative profile of orthognathic surgery in pediatric patients.

Methods/Description: Retrospective cohort study was conducted of LeFort I (LF1) and bilateral sagittal split osteotomies (BSSO) performed at 60 hospitals using the ACS NSQIP-Peds data set. Perioperative characteristics were analyzed.

Results: During the study interval, 281 pediatric patients underwent orthognathic surgery, including 63.3% (n = 178) LF1, 20.3% (n = 57) BSSO, and 16.4% (n = 46) double jaw procedures. Age demonstrated a bimodal distribution with the majority undergoing orthognathic surgery as teenagers (83.3%, n = 234). Patients below age 5 years requiring orthognathic surgery were more likely to have cardiac (P < .001, 30.0% vs 4.6%), respiratory (P < .001, 75.0% vs 15.3%), or overall comorbidities (P < .001, 90.0% vs 29.1%), and congenital malformations (P = .043, 65.0% vs 41.8%), but were not more likely to have chromosomal abnormalities (P = .782). Age was inversely correlated to length of stay (P < .001) with patients less than 5 years being hospitalized significantly longer (P < .001, 12 days vs 2 days). There was no difference in length of stay between single- and double jaw procedures (P = .963). Overall, 25.3% of orthognathic procedures were performed outpatient. Bimaxillary procedures (P = .037) and children with respiratory comorbidities (P = .006) or younger that 5 years (P = .030) were more likely to be admitted as inpatients. Overall adverse event rate after orthognathic surgery was 7.8% (n = 22), including medical complications (1.1%), surgical complications (3.6%), related readmissions (0.7%), and related reoperations (3.9%). Patients undergoing double jaw procedures were more likely to experience medical complications (P = .018, 4.3% vs 0.4%), but not surgical complications (P = .154). Younger patients were more likely to experience adverse events (P = .006), related readmission (P = .028), and surgical complications (P = .027). Most common complications were surgical site infections (1.1%), and there were no nerve injuries in any cohort. Patients less than 5 years (P < .001) and undergoing BSSO only (P = .045) were more likely to experience surgical site infections, which were associated with oxygen support (P < .001), pulmonary abnormality (P < .001), CNS abnormality (P = .003), and presence of any comorbidity (P = .014). Children less than 5 years were significantly more likely to experience adverse events (P < .001, 30.0% vs 6.1%), including medical and surgical complications (P = .014, 15.0% vs 3.4%), related reoperations (P < .001, 20.0% vs 2.7%), and related readmissions (P < .001, 10.0% vs 0.0%).

Conclusions: Pediatric orthognathic surgery demonstrates a bimodal age distribution, clustering in infancy and the late teen years. Orthognathic surgery in children below 5 years was associated with significantly increased likelihood of adverse events, including infection, readmission, and surgical complications. Teenagers undergoing double jaw procedures were more likely to have medical complications.

(1) Marquette University, Milwaukee, WI, (2) Cleft and Craniofacial Center, Shriners Hospital for Children, Chicago, IL, (3) Orthodontics and Dentofacial Orthopedics, Forbes Orthodontics, West Dundee, IL, (4) School of Dentsitry, Marquette University, Milwaukee, WI

Background/Purpose: Patients with cleft lip and palate (CL/P) often require correction of various forms of malocclusion, especially a Class III skeletal malocclusion due to maxillary deficiency. A Le Fort I osteotomy with advancement of the maxilla not only improves occlusion and facial balance but also has the potential of enlarging the airway of the patient. However, to best knowledge of the authors, there is no study utilizing cone beam computed tomography (CBCT) to analyze the changes in upper airway volume after maxillary advancement in patients with CL/P. In this study, we aimed to characterize the effects of single jaw (maxillary) advancement to the airway in patients with unilateral CL/P.

Methods/Description: In this retrospective study, we used previously acquired Cone-Beam Computed Tomography (CBCT) images of 30 patients (13 males and 17 females, 17-20 years old) with unilateral cleft lip/palate. The skeletal changes (ie, SNA, SNB, ANB, PP-SN, Occl-SN angles; and N-A vertical and Point “A” and “B” distances to the true vertical line) associated with maxillary advancement were measured on the CBCT images before and after surgery. To evaluate the airway, preoperative (T1) and postoperative (T2) images were analyzed using Dolphin 3D to quantify the volume, sagittal area, and minimal cross-sectional area (MCA) of the retropalatal and retroglossal airways.

Results: Retropalatal (RPA), retroglossal (RGA), and total airway (TA) volumes increased significantly from T1 to T2 (P = .019, P = .019, P = .002). RGA and TA sagittal area increased significantly (P = .020, P = .016). For minimal cross-sectional area (MCA), only the RPA increased significantly with P = .002. All the cephalometric changes were statistically significant between T1 and T2 with the exception of SNB. The greatest cephalometric changes were to SNA, ANB, and the distance from A-TVL (true vertical line). Data were expressed as mean ± SD. For each of the variables, paired t test was used to statistically compare the measurements between T1 and T2, with a P value of .05 being considered significant.

Conclusions: Based on the results from cephalometric measurements, maxillary advancement single jaw surgery significantly moves the maxilla forward in correcting midface deficiencies in patients with unilateral cleft lip/palate. This type of surgery also produces statistically significant increases in the retropalatal (volumetric and MCA), retroglossal (volumetric and sagittal), and total (volumetric and sagittal) airways based on data from CBCT imaging.

(1) The Children’s Hospital of Philadelphia, PA, (2) The University of Pennsylvania, Philadelphia, PA

Background/Purpose: Aesthetic perceptions of faces affect aspects of everyday life including likeability, employment, and compensation, with those deemed most attractive reaping the benefits. Recent evidence points to negative biases against individuals with facial anomalies. Reconstructive surgery improved personality inferences made about patients undergoing orthographic surgery. However, this effect seems limited as patients with repaired cleft lip and palate are still viewed less favorably than their noncleft peers. Craniofacial reconstructions were designed to normalize anomalous faces, but the regions of such faces that “catch” the eyes of observers and influence their perceptions are, to date, unknown. This study will characterize the areas of the face that capture lay people’s visual attention by comparing gaze preference pre- and post-reconstruction in a cohort of hemifacial macrosomia patients. The results may direct surgeons’ priorities toward attentional “hot” spots, with repairs reducing appearance-related disease burden.

Methods/Description: Images of 17 hemifacial microsomia patients pre- and post-reconstructive surgery were collected and 4 areas of interest were defined on the anomalous side of each face: cheek and ear, forehead and orbit, mandible and chin, and nose and lips. At the study visit, two runs of an eye-tracking task were acquired using the Tobi Pro Nano eye-tracking system. Participants saw 34 images per run (68 total; 17 in normal orientation, 17 flipped to account for left gaze bias) for 5 seconds each and visual fixations were recorded. Next, participants completed measures of implicit and explicit biases and rated each face on attractiveness, trustworthiness, contentedness, dominance, anxiousness. The data were submitted to linear mixed modeling.

Results: Five subjects piloted the eye-tracking task, registering a total of 8109 fixations (mean: 1st run: 818.6; 2nd run: 803.2). Of those fixations, 253 (prereconstruction: 109 [1.34%]; postreconstruction: 144 [1.78%]) were in the cheek and ear (mean duration: prereconstruction: 296 ms; postreconstruction: 294 ms), and 51 (prereconstruction: 21 [0.26%]; postreconstruction: 30 [0.37%]) were in the forehead and orbit (mean duration: prereconstruction: 436 ms; postreconstruction: 475 ms). No fixations were recorded in either mandible/chin or nose/lips on the anomalous side. Subjects spent less time looking at the anomalous side (234 s vs 981 s). A power analysis, using the pilot data, indicated N = 60 participants will provide adequate statistical power.

Conclusions: We demonstrate the feasibility of assessing visual attention using eye tracking to characterize gaze preference “hot” spots for faces with and without anomalies and provide preliminary descriptive results. Volunteers looked less often, and for less time, at the anomalous side of the face compared with the nonanomalous side. Future work will focus on full enrollment and expansion to other facial anomalies.

(1) Global Smile Foundation, Norwood, MA, (2) Amsterdam UMC, location AMC, Amsterdam, Netherlands, (3) Hansjörg Wyss Department of Plastic Surgery, NYU Langone Health, New York, NY, (4) Medical University of South Carolina, Charleston, SC, (5) NYU Langone Health, New York, NY, (6) The University of Maryland Medical Center/Shock Trauma Center, Baltimore, MD

Background/Purpose: The annual incidence of congenital clefts of the lip and/or palate (CLPs) in Low- and Middle-Income Countries (LMICs) is estimated to be around 250 000 per year. Repair of CLPs is recommended within the first year of life, due to potential future complications. Barriers to cleft care have resulted in countless number of patients with CLPs reaching adulthood without undergoing surgery, which has made cleft surgery an attractive target for humanitarian initiatives in LMICs. As part of those initiatives, we organized Simulation-Based Comprehensive Cleft Care Workshops (CCCWs) in 2018 and 2019. In this study, we analyze the perceptions of participants and faculty members in these workshops regarding the most important barriers and interventions to comprehensive cleft care in LMICs.

Methods/Description: The total number of attendees in the two workshops was 313: 273 participants (87.2%) and 40 (12.7%) faculty members, representing a total of 44 countries. Data was collected from both groups at the end of the workshops. Each group noted what they believed was the most significant barrier to comprehensive cleft care delivery in LMICs, and what they perceived was the most important intervention to deliver comprehensive cleft care in those countries. We also investigated whether the 2 groups’ perceptions were concordant or discordant. Data analyses were performed using the Statistical Package for the Social Sciences (SPSS, version. 23.0, IBM Corp). Chi-squared testing was used to compare categorical variables and perform our stratified analysis comparing participants’ and faculty’s responses.

Results: The biggest perceived barrier facing the delivery of comprehensive cleft care in LMICs was financial (35.0%), followed by the absence of multidisciplinary cleft teams (30.8%), poor training (16.1%), the absence of cleft centers (9.8%), patient travel (4.2%), and the absence of awareness regarding congenital cleft lip and/or palate (4.2%). The most important intervention to deliver comprehensive cleft care in LMICs was creating multidisciplinary cleft teams (32.2%), followed by providing cleft training (22.6%), financial support (20.5%), creating cleft centers (17.1%), and raising awareness regarding congenital cleft lip and/or palate (7.5%). No statistically significant difference between participants’ and faculty members’ perceptions regarding the biggest barriers (P = .46) and most important interventions (P = .38) to deliver comprehensive cleft care in LMICs.

Conclusions: Factors preventing access to comprehensive cleft care are multifaceted, ranging from logistical to financial challenges, and are most palpable in LMICs. This study acknowledges the significance of a team-based approach to cleft care, and its potential to collectively overcome barriers to care. The Simulation-Based CCCW is by itself an intervention that aims to address some perceived barriers. Continued workshop evaluation and quality assurance will be important for program success and sustainability.

(1) Yale School of Medicine, New Haven, CT, (2) University of Connecticut, Orthodontics and Yale School of Medicine, Plastic and Reconstructive Surgery, Farmington/New Haven, CT, (3) Yale University, New Haven, CT, (4) Johns Hopkins School of Medicine, Baltimore, MD, (5) Yale University School of Medicine, Department of Plastic and Reconstructive Surgery, New Haven, CT

Background/Purpose: Children with cleft lip and/or palate (CLP) often undergo longitudinal, multidisciplinary care requiring numerous medical visits. Few studies have examined geographic barriers to provision of care in the CLP population in the United States. This study evaluated the spatial availability and accessibility of certified cleft providers across the country.

Methods/Description: All certified CLP teams in the United States were identified from official ACPA listings. A geographic catchment area within a 1 hour travel radius of each center was mapped using the TravelTime software, which uses distance matrix programming to approximate mean driving travel times from any location. The number of children located within each center catchment area was estimated at a per-county level using data from the National Kids Count Data Center.

Results: Two-hundred and sixty cleft palate centers were included in our analysis. As many as 19.2 million children under 18 (26.8% of children in the US) did not, per study estimates, live within 1 hour travel distance to one of these certified cleft teams. One-hour access to care was highest in the Northeast (84.6% of children within 1 hour) followed by the West (75.6%), Midwest (74.3%), and South (66.5%). The majority of patients in the Northeast had access to 2 or more centers (61.9%), a number higher than the West (52.3%), Midwest (44.4%), or South (33.4%). The number of the overall children-per-center was higher in the West (394,776 per center) and South (309,537) than in the Northeast (215,553) or Midwest (212,206).

Conclusions: Over 25% (19.2 million children) may lack easy access to cleft care teams in the United States. Future efforts (ie, telehealth medicine) should aim to mitigate geographic barriers in order to improve CLP outcomes.

(1) Amsterdam UMC, location VUmc, Amsterdam, Netherlands, (2) Department of Pediatrics, University of Alberta, Stollery Children’s Hospital, Alberta, Canada, (3) Department of Plastic and Reconstructive Surgery University Medical Centre Utrecht, Wilhelmina Child, Utrecht, Noord Holland, Netherlands, (4) Department of Neonatology Tuebingen University Hospital, Tuebingen, Germany, (5) Department General Pediatrics Necker University Hospital, Necker-Enfants Malades Hospital, Paris, France, (6) Department Pediatric Intensive Care Erasmus MC – Sophia Children’s Hospital, Rotterdam, Zuid-Holland, Netherlands, (7) Boston Children’s Hospital, Boston, MA, (8) Hospital for Rehabilitation of Craniofacial Anomalies, University of Sao Paulo, Bauru, Sao Paulo, Brazil, (9) University of North Carolina at Chapel Hill, Chapel Hill, NC, (10) Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada, (11) Department of Pediatrics University Medical Centre Amsterdam, location AMC, Emma Children’s Hospital, Amsterdam, Noord Holland, Netherlands, (12) New York University Langone Health, New York, NY, (13) Seattle Children’s Hospital, Seattle, WA

Background/Purpose: Identifying optimal treatment for infants with Robin sequence is challenging due to substantial variability in presentation in this population at risk for upper airway obstruction upper airway obstruction. Objective assessments of upper airway obstruction and airway treatments are not standardized. A systematic review of measures from oximetry, polysomnography and blood gas was conducted as an initial step toward building evidence to guide clinical decision-making in Robin sequence.

Methods/Description: A literature search was performed in the Pubmed and Embase databases (1990-2020) according to the PRISMA guidelines. Utilizing consensus among reviewers, articles reporting on Robin sequence, and the treatment of upper airway obstruction were included if the following objective tests were studied: oximetry, polysomnography, and blood gas. Risk of bias was assessed for individual studies and summarized narratively across all studies. Study quality was appraised by the methodological index for nonrandomized studies (MINORS, range 0-24).

Results: Of all, 1123 studies were identified and data extracted independently by multiple observers among 91 included studies. The mean MINORS score was 7.1 (range: 3-14). The prevalence of measures and metrics of upper airway obstruction were summarized. Polysomnography was used most frequently (76%), followed by oximetry (20%) and blood gas values (11%). The position of the infant was reported in 35%, including the supine position most frequently, and monitoring time in 42%, including overnight recordings in more than half. Of 71 studies that evaluated UAO interventions, the majority used PSG (90%), of which 61% did not specify polysomnography technique. Reported polysomnography metrics included oxygen saturation (61%), apnea-hypopnea index (52%), carbon dioxide levels (31%), obstructive apnea-hypopnea index (27%), and oxygen desaturation index (16%). Only 42 studies reported indications for upper airway obstruction intervention, with oximetry and polysomnography thresholds used equally (both 40%). In total, 34 distinct indications for treatment were identified.

Conclusions: This systematic review demonstrates a lack of standardized use, interpretation and reporting of upper airway obstruction measures for treatment indications and evaluations. A wide variation was observed in the use, interpretation, and reporting of these values. An international, multidisciplinary consensus protocol is needed to guide pediatricians on the optimal assessment of upper airway obstruction in Robin sequence.

(1) Johns Hopkins All Children’s Hospital, St. Petersburg, FL, (2) Johns Hopkins All Children’s Hospital, Harrisburg, PA (3) The Ohio State University, Columbus, OH

Background/Purpose: Oronasal fistulae following palatoplasty may affect patients’ quality of life by impacting their ability to eat, speak, and maintain oral hygiene. We aimed to quantify the impact of previous oronasal fistula repair on patients’ quality of life using patient-reported outcome psychometric tools.

Methods/Description: A cross-sectional study of 7 to 19-year-old patients with CP ± L was completed. Patients who had a cleft team clinic between September 2018 and August 2019 were recruited. Participants were divided into 2 groups (no fistula, prior fistula repair). Differences in the individual CLEFT-Q and COHIP-SF 19 Oral Health scores between the 2 groups were evaluated using a multivariate analysis controlling for Veau classification and syndromic diagnosis.

Results: Sixty patients with a history of cleft palate were included. Forty-two (70%) patients had an associated cleft lip. Thirty-two patients had no history of fistula (53.3%) and 28 patients had undergone a fistula repair (46.7%). CLEFT-Q Dental, Jaw and Speech Function were all higher in patients without a history of a fistula repair, however, none of these differences were statistically significant. The COHIP-SF 19 Oral Health score demonstrated a significantly lower score in the fistula group, indicating poorer oral health (P = .05).

Conclusions: One would expect that successful repair of a fistula would result in improved function and patient satisfaction, but the consistent trend toward lower CLEFT-Q scores and significantly increased COHIP-SF 19 Oral Health scores in our study group suggests that residual effects linger and that the morbidity of a fistula may not be completely treated with a secondary correction.

(1) University of Pittsburgh School of Medicine, Pittsburgh, PA, (2) University of Pittsburgh, Pittsburgh, PA, (3) Children’s Hospital of Pittsburgh, Pittsburgh, PA

Background/Purpose: Pierre-Robin Sequence (PRS) is characterized by micrognathia and glossoptosis and can result in upper airway obstruction. Prenatal diagnosis of PRS facilitates delivery team preparation for an airway emergency. Routine 20-week ultrasounds screen facial features and can characterize mandibular morphology and the maxillomandibular relationship. This study aimed to evaluate 20-week fetal ultrasounds to determine if specific facial measurements could predict PRS diagnosis and disease severity.

Methods/Description: A retrospective case control study of 40 patients with PRS and 40 gender-matched controls (24 male, 16 female in each cohort) between January 2014 and May 2019 was performed. Respiratory and surgical interventions were recorded and scored for severity. Mid-sagittal profile images of the ultrasounds were reviewed and measured for 3 parameters to assess micrognathia: facial nasomental angle (FNMA), facial-maxillary angle (FMA), and alveolar overjet. FMA <66° and FNMA <136° signify micrognathia. A one-tailed t test was calculated and the mean ± standard deviation was reported.

Results: Patients with PRS demonstrated significantly smaller FNMA compared to the control group (129.6 ± 9 vs.137.9 ± 2.8, respectively; P < .001). FMA was significantly smaller in the PRS group compared to the control group (64.1 ± 9.3 vs. 75.3 ± 6.5; P < .001). The PRS group also demonstrated significantly larger overjet compared to the control group (3.7 ± 1.3 vs. 2.3 ± 0.8, respectively, P < .001). In the PRS group, 53% had an abnormally acute FMA compared to 3% in the control group. For FNMA, 82% in the PRS group demonstrated micrognathia compared to 18% in the control group. In the PRS cases, 38% did not require external respiratory support (n = 15), 43% needed supplemental oxygen by nasal cannula or CPAP (n = 17), and 20% were intubated (n = 8). As respiratory support needs increased, median FMA decreased and alveolar overjet increased. In the control group, 93% required no external respiratory support (n = 37) and 7% needed supplemental oxygen by CPAP (n = 3). In the PRS cases, 45% received mandibular distraction or tongue-lip adhesion (n = 18), 15% underwent supraglottoplasty only (n = 6), 8% required tracheostomy (n = 3), and 32% did not require surgical intervention (n = 13). Surgical patients tended to have smaller FNMAs and greater overjet compared to nonsurgical patients; median FNMA was 127° versus 132°, and median overjet was 2.8 versus 4.15 mm, respectively, however this was not significant. None of the control patients underwent surgical intervention.

Conclusions: Mandibular features on 20-week anatomy ultrasound can be measured to predict PRS prenatally and prepare for respiratory intervention at delivery to minimize hypoxia at birth. Alveolar overjet, previously not described in prenatal ultrasound literature but routinely assessed on neonatal clinical evaluation, is measurable and has utility in prenatal diagnosis, as do FMA and FNMA.

(1) Universidad El Bosque, Bogotá, Colombia

Background/Purpose: Folic acid (FA) is the synthetic form of folate, obtained through diet or supplements. Periconceptional administration of FA supplements has been found to reduce neural tube defects; however, its effect on cleft lip and palate (CLP) prevention and its mechanism of action in osteoblastic differentiation and bone formation is not clear. Previous animals studies have reported possible FA ways of action that converge on the activation of transforming growth factor β (TGFβ). Bone morphogenetic proteins (BMP) are members of TGFβ superfamily and its signaling Smad1/5/8 and p38 is essential for palatogenesis, therefore, the aim of this investigation was to determine changes in expression and phosphorylation of p38 and Smad1/5/8 proteins induced by treatment with different doses of FA.

Methods/Description: Isolated and characterized human mesenchymal dental pulp cells (hDPSC) from 2 independent patients were treated with different FA concentrations: 0 mM (control), 0.8 mM and 1.6 mM and osteoinduction medium (OIM) during 7 and 21 days. Changes in Smad1/5/8 and p38 expression and phosphorylation were evaluated using Western Blot (WB) technique and quantified by densitometry using Labimage 1D Software (Kapelan Bio-Imaging Solutions, Germany). Experiments were done by duplicate. This research was approved by the institutional ethics committee.

Results: Inmunodetection of proteins of interest and densitometry analysis revealed p38 over fosforilation in both FA groups and OIM group compared to control at 7 days of treatment, reducing its expression at 21 days. Smad1/5/8 fosforilation were detected in every group, with great intensity in FA 0,8 mM at 7 days and in OIM at 21 days of treatment.

Conclusions: WB allowed to determine that FA treatment induced early activation and phosphorylation of p38, nevertheless Smad1/5/8 pathway was also stimulated with less intensity.

(1) Marquette University, Milwaukee, WI, (2) Cleft and Craniofacial Center, Shriners Hospital for Children, Chicago, IL, (3) Orthodontics and Dentofacial Orthopedics, Forbes Orthodontics, West Dundee, IL, (4) School of Dentsitry, Marquette University, Milwaukee, WI

Background/Purpose: The purpose of this study was to explore the anatomical considerations of children with unilateral cleft lip and palate (UCLP) for the purpose of placing orthodontic miniplates for maxillary protraction.

Methods/Description: Cone beam computed tomography (CBCT) images of 41 patients with UCLP (18 females and 23 males with a mean age of 9.8) and 36 (19 females and 17 males with a mean age of 9.9) age-matched controls were assessed in this retrospective study. Multiple linear measurements were taken to evaluate the bone thickness of the infrazygomatic crest region (IZCR), buccal alveolar bone, and inferior portion of the zygoma. In addition, the width of 10 craniofacial and circummaxillary sutures were measured in the coronal, axial, and sagittal plane. Furthermore, the maturation level of the zygomaticomaxillary sutures (ZMS) were identified. Lastly, the volume of the maxillary sinuses was calculated. Statistical comparisons were made for each of the variables between the control and UCLP groups.

Results: There were no statistically significant differences of age and gender distributions between the groups. The greatest average bone thickness was found in the zygoma region in both groups, ranging from about 7 to 9 mm. The mean Infrazygomatic crest thickness did not exceed 3 mm in patients with UCLP. Analysis of the maxillary sinus revealed no significant differences between the 2 groups. The mean suture width of the right pterygomaxillary, left ZMS, and internasal sutures were larger in control group. The mean suture width of the right and left frontomaxillary, intermaxillary, left nasomaxillary, and midpalatal sutures were larger in UCLP group. All patients were either at Stage A or Stage B of the maturation level of the ZMS and Stage B made up the majority in both groups.

Conclusions: Patients with UCLP have sufficient bone thickness to accommodate miniscrews for fixation of miniplates in the zygoma but may not have enough in the infrazygomatic crest. The maxillary sinus volumes were similar between UCLP and the control group, but there were some significant differences in suture width between the control and UCLP group.

(1) University of California Irvine, CA, (2) UC Irvine Department of Computer Science, Irvine, CA, (3) Stanford University, Department of Computer Science, Stanford, CA, (4) Stanford University, Department of Electrical Engineering, Stanford, CA, (5) UC Irvine School of Medicine, Orange, CA, (6) Global Smile Foundation, Norwood, MA, (7) UC Irvine Department of Plastic Surgery, Orange, CA

Background/Purpose: Cleft lip and nasal repair restores oral function and strives to achieve normal nasolabial aesthetics. Anthropometry of the lip and nose guides operative design, yet mastering cleft operative markings and surgical sequencing can be challenging. To accelerate this learning curve, we developed an artificial intelligence (AI) based platform that uses a novel machine learning algorithm to reliably detect cleft nasolabial anthropometry.

Methods/Description: We utilized High-Resolution Net (HRNet), a recent family of deep learning models that has achieved state of the art results in many computer-vision tasks, including facial landmark detection. HRNet follows the current trend in computer vision of stacking multiple convolutional layers, but differs in one key area. Whereas previous models generally downsample the dimensionality of the input at each layer, HRNet performs this downsampling in parallel with a series of convolutional layers that preserves dimensionality and allows for intermediate representations with higher dimensionality while simultaneously extracting lower dimension features. To adapt the facial landmark detection HRNet for our task, we employed transfer learning, a technique in machine learning to transfer knowledge gained from a source task to a target task. Transfer learning reduces training time, increases accuracy on target task, and reduces required training examples in the target task. Here, a craniofacial plastic surgeon manually marked the key anthropometric landmarks of 460 two-dimensional photographs of infants and children with unilateral cleft lip. These images are compared against the detected markings assigned by our algorithm. For model evaluation, we calculated error using the Normalized Mean Error (NME), an evaluation metric in facial landmark detection.

Results: After training on our data set, we obtained NMEs for each anthropometric point. All values were between 0.2929 (rala) and 0.05544 (rcphi). In comparison, NMEs for state-of-the-art facial recognition data sets ranges between 0.0385 (300 W) to 0.0460 (WFLW). Our training data set is ∼1% the size of these benchmarks, illustrating ability to leverage relatively small quantities of data to achieve surprisingly accurate cleft lip/nose markings.

Conclusions: In the present study, we developed a deep learning model that accurately identifies the nasolabial anthropometry of a unilateral cleft lip deformity and uses this information to mark a unilateral cleft lip/nose repair on a preoperative photograph. Using light-based 3 dimensional surface projection technology developed by our team, we plan to harness our cleft facial recognition algorithm to project cleft repair markings onto the 3 dimensional surface anatomy of nasolabial clefts. Combining this AI-based platform with augmented reality (AR) based platforms optimizes remote guidance and facilitates knowledge and skill transfer. Such technology can impact and accelerate both domestic surgical teaching and overseas cleft outreach.

(1) New York University Medical Center, New York, NY, (2) University of Wisconsin, Madison, WI, (3) New York University Langone Health, New York, NY

Background/Purpose: For the past 25 years computer-based simulation of cleft lip repair has been an elusive goal. To date, interactive 3D models have allowed students to make preoperative incision markings. Animation generated “blend shapes” allow premodeled surgical animations to be “played back” in 3D video game format. Neither of these efforts allow the student to actually perform his/her own lip repair. This article presents what we believe to be the first cleft lip simulator to allow the student to prospectively do complete cleft lip/nose corrections. Historical procedures and proposed new ones can be performed. A deeper level of understanding can be obtained using this cognitive experiential approach without injuring a real patient.

Methods/Description: A 3D solid model of a complete unilateral cleft lip/nose has been prepared based on a laser scan of an actual patient. Procedures are performed using a surgical “toolkit” consisting of (1) skin hooks, (2) skin/mucosa scalpel, (3) skin and periosteal undermine tool, (4) deep cut tool for cutting through muscle, fat, and cartilage, and (5) a suture tool. First order biologically accurate physics are provided by modeling the solids as a half million tetrahedra. Projective dynamics are used to implement biphasic tissue behavior in which at low strain Hooke’s law elasticity is provided, but as strain exceeds 14% the tissue becomes much stiffer to model the commonly observed “the flap won’t reach” phenomenon. Collision is modeled between the teeth and bone of the maxilla and the undersurface of the lip using a Schur complement approach. Near real-time performance is provided on a laptop computer in 3D video game format. A “history” files may be recorded of the sequence of actions performed by the surgeon.

Results: A demonstration of the model and the surgical toolset will be performed in real time. A full cleft lip/nose repair using the simulator will be demonstrated using its history feature.

Conclusions: A first order biophysically accurate simulator of cleft lip and nose repair has been developed. It permits the student to cognitively explore different methods of repair and store his/her surgical sequence. This simulator has application in experiential education, proficiency testing, and the design of new surgical procedures.

(1) Yale University School of Medicine, Department of Plastic and Reconstructive Surgery, New Haven, CT, (2) University of Connecticut, Orthodontics and Yale School of Medicine, Plastic and Reconstructive Surgery, Farmington/New Haven, CT

Background/Purpose: Secondary surgeries are important for the long-term cosmetic and functional outcomes following cleft lip and/or palate repair. The current study sought to analyze the impact of race and ethnicity on the utilization of these surgeries in the United States.

Methods/Description: Primary cleft lip and palate repairs, cleft palate revisions, and secondary rhinoplasty surgeries performed from 2013 to 2018 were identified from the Pediatric National Surgical Quality Improvement Program (NSQIP) database. The proportion of patients of a specified ethnicity/race were compared across surgical categories using χ ² analyses. Weighted proportions (WP) are described to compare differences in proportions across subgroups.

Results: In all, 17 457 cleft procedures performed in the study timeframe were identified: 5429 primary cleft lip repairs 1700 cleft rhinoplasties, 7534 primary palatoplasties, and 2794 cleft palate revisions. In comparison to primary cleft lip repair, cleft rhinoplasty was decreased among White (WP = 0.94, P = .001) and Black (WP = 0.69, P < .001) patients and increased among Asian (WP = 2.0, P < .001) and Hispanic (WP = 1.16, P = .002) patients. In comparison to primary palate repair patients, palate revision was decreased among Black (WP = 0.67, P < .001) and Hawaiian/Pacific Islander (WP = 0.48, P = .02) patients and increased among Asian (WP = 1.74, P < .001) patients. In comparison to national demographic percentages, white (WP = 1.4) and Asian (WP = 1.6) children were overrepresented across all subgroups, whereas black (WP = 0.58) and Hispanic (WP = 0.51) children were significantly underrepresented.

Conclusions: Our findings show considerable differences in the proportion of patients receiving secondary cleft surgeries based on their race and/or ethnicity. In comparison to primary cleft procedures, Black patients constituted a much smaller proportion of both cleft rhinoplasty and revision surgeries, whereas Asian patients made up a much larger proportion. These findings could suggest disparity in the utilization of secondary cleft surgeries which may be related to, among other factors, sociocultural beliefs, insurance policies, and access to and quality of longitudinal cleft care.

(1) Children’s Hospital of Pittsburgh, PA, (2) University of Pittsburgh, PA, (3) Department of Plastic Surgery, University of Pittsburgh, Medical Center, Pittsburgh, PA, (4) Department of Plastic Surgery, University of Pittsburgh, School of Medicine, Pittsburgh, PA, (5) University of Pittsburgh, School of Medicine, Pittsburgh, PA

Background/Purpose: Surgical management of complications following primary pharyngoplasty is a complex problem faced by the cleft surgeon. While revision pharyngoplasty may be necessary in those with persistent velopharyngeal insufficiency (VPI) and other complications, little is known regarding the indications for and outcomes following these procedures. The purpose of this study is to describe the authors’ experience with management and outcomes in revision pharyngoplasty.

Methods/Description: A single-center retrospective review was performed of all patients undergoing revision pharyngoplasty between 2003 and 2016. Demographic data and Pittsburgh Weighted Speech Scores, diagnoses, comorbidities, and complications were tabulated. Two-tailed Student t test was used and a P ≤ .05 was considered statistically significant.

Results: Twenty-nine patients met inclusion criteria (4 patients with velopharyngeal dysfunction, 5 submucous cleft palates, and 19 cleft palates; 17 male, 12 female; mean age at primary pharyngoplasty 5.7 ± 2.5 years). Of all, 75.9% had a history of palatoplasty prior to primary pharyngoplasty (n = 22); of those, 2 had revision palatoplasty prior to pharyngoplasty. Primary pharyngeal procedures included 24 posterior pharyngeal flaps (PPF), 3 palatoplasties with PPF (2 revision, 1 primary palatoplasty), and 2 sphincter pharyngoplasties (SP). Complications associated with the index pharyngoplasty included wound dehiscence (n = 10, 34.5%), obstructive sleep apnea (OSA; n = 5, 17.2%), and oronasal fistula formation (n = 3, 10.3%); persistent VPI was noted in 14 patients (48.3%). The mean speech score pre- and post-primary pharyngoplasty was 19.8 ± 7.5 and 14.2 ± 10.5, respectively (P = .23). Revision procedures included 22 PPF and 7 SP. Postoperative complications included OSA (n = 8, 27.6%) and wound dehiscence (n = 6, 20.7%); persistent VPI was noted in 6 patients (20.7%). The mean speech score after secondary pharyngoplasty was 6.95 ± 7.8 (prerevision procedure vs postrevision procedure, P = .04). A subset of patients (n = 15) required further intervention that included 4 pharyngeal flap take-downs, 4 conversion Furlow palatoplasties, and 7 tertiary pharyngoplasties (3 PPF, 4 SP) followed by 1 flap takedown and 2 conversion Furlow palatoplasties. One patient experienced mild persistent VPI (13 years after tertiary SP), there were no other reported complications. The pre- and post-operative speech score for this cohort was 10.7 ± 9.3 and 3.5 ± 1.8, respectively (P = .02).

Conclusions: The present study describes the authors’ experience with revision pharyngoplasty in patients with cleft palates, submucous cleft palates, and VPI. Complication rates may be as high as 27.6%. Indications for revision procedures include persistent VPI, OSA, and surgical site complications (ie, oronasal fistulae) and significant improvement in speech outcomes can be expected. Revision pharyngoplasty, pharyngeal flap take-down, and conversion Furlow palatoplasties remain options for management of this challenging patient population.

(1) Johns Hopkins All Children’s Hospital, Harrisburg, PA, (2) University of South Florida, Morsani College of Medicine, Tampa, FL, (3) Johns Hopkins All Children’s Hospital, St. Petersburg, FL, (4) The Ohio State University, Columbus, OH

Background/Purpose: The goal of a synchronous bilateral cleft lip/nasal correction is to achieve normal dimensions and growth of the nasolabial subunits. Mulliken demonstrated that to achieve normal adult proportions, these nasolabial subunits must be over or under corrected based on their future growth trajectory. The proportions utilized in his prescribed operation were derived from normative anthropometric data published by Farkas et al. This data represents normative anthropometric measurements of Caucasian children. We aimed to assess if infants’ nasolabial anthropometry exhibited significant ethnic variabilities, which would motivate variations in surgical correction. Specifically, we sought to investigate whether a long columella is a Caucasian feature; therefore, accepting a short columella and/or delayed columella lengthening as a suitable strategy for reconstruction in ethnic patients.

Methods/Description: Thirty-three infants without craniofacial pathology (10 African American [AA]; 7 Hispanic [H]; and 16 Caucasians [C]), ages 3 to 8 months, presenting to the Johns Hopkins All Children’s general pediatric clinic were recruited. Four separate 3D photographs (2 submental views and 2 frontal views) were taken using the Vectra H1 handheld camera (Canfield Imaging). Each single stereoimage was used to construct a separate 3D facial surface. Eighteen linear facial distances were measured using Mirror 3D analysis (Canfield Imaging Systems). Analysis of variance (ANOVA) coefficients with the Bonferroni/Dun post hoc comparisons, at alpha = .05, was used to measure significant differences between ethnic groups. Pearson correlation was used to determine intra and interrater reliability. All statistical analyses were carried out using SPSS version 21.0 (IBM Corp, Armonk, New York), with statistical significance set at P < .05.

Results: Significant differences were seen between ethnic groups in nasal width (sbal-sbal [C-AA; P = .02]; ac-ac [C-AA; P = 0.00; H-AA; P = .04] and al-al [C-AA; P = .00 H-AA; P = .001]), as well as labial length (sn-ls [C-AA; P = .041]; sn-sto [C-AA; P = .005] Cphs-Cphi L [C-AA; P = .013]; Cphs-Cphi R [C-AA; P = .015]). African American infants exhibited wider noses and longer lips, while Hispanic infants represented intermediate values between Caucasian and African American infants. Nasal projection (sn-prn) (P = .974) and columella length (sn-c) (P = .99) did not differ significantly between groups. Correlation coefficients for both intra and interrater reliability were good to excellent and were significantly correlated for all 18 measures.

Conclusions: Significant differences were noted between nasolabial anthropometry for African American and Caucasian infants. Nasal width and lip length were greater in African American patients, indicating that slightly wider intra-alar distances may be tolerated in ethnic patients. No difference was noted in nasal projection or columella length, indicating columellar lengthening should be performed with the primary labial repair and not deferred.

(1) Arkansas Children’s Hospital, Little Rock, AR, (2) University of Arkansas for Medical Sciences, Little Rock, AR

Background/Purpose: Velopharyngeal insufficiency (VPI) is a common speech disorder in patients with a history of cleft palate (CP) or 22q11.2 deletion syndrome. Superiorly-based pharyngeal flap (PF) is a common procedure for reducing nasal air emission during speech. One disadvantage of the procedure is that the inferior surface of the flap is not mucosalized, and heals by secondary intention, which may create unstable long-term results. We report the differences in outcomes of the pharyngeal flap procedure at our institution when using uvular lining versus without uvular lining.

Methods/Description: This study evaluated outcomes retrospectively for all children <18 years of age with a history of pharyngeal flap performed between 2006 and 2017. Based on operative records, patients were categorized into either the uvular lining (UL+) or no uvular lining (UL−) group. Demographic characteristics were extracted, as well. Outcomes included complications, revision surgery (further categorized as airway-expanding or airway-contracting revision surgeries), and incidence of polysomnogram-confirmed obstructive sleep apnea. UL+ and UL− groups were compared via χ ² tests for the outcomes above.

Results: Of all, 199 patients with pharyngeal flap were analyzed, 150 with uvular lining and 49 without uvular lining. There were no differences regarding preoperative baseline characteristics including attending surgeon, age, type of cleft (including presence of cleft lip, cleft palate, submucous cleft palate) and developmental delay. There was no statistically significant difference in incidence of OSA (10/49 vs 21/150, P = .363) or airspace expanding revision surgeries (4/49 vs 7/150, P = .470). With uvular lining, there was a significant reduction in patients requiring revision surgeries (14/49 vs 12/150, P < .001), overall number of revisions (21/49 vs 27/150, P < .001), airspace contracting revisions (9/49 vs 9/150, P = .018) and patients with complications (21/49 vs 31/150, P = .004).

Conclusions: The small additional step of using the uvula to line the raw undersurface of the pharyngeal flap demonstrates significant improvement in the risk of undergoing revision surgery in this nonrandomized retrospective study. We advocate for its use in pharyngeal flap surgery for velopharyngeal insufficiency.

(1) Division of Plastic and Reconstructive Surgery, Stanford University, Palo Alto, CA, (2) Division of Plastic Surgery, University of Washington, Seattle, WA

Background/Purpose: As health care spending within the United States continues to grow, payers have attempted to curb spending through higher cost sharing for patients. For families attempting to balance their financial obligations with their children’s surgical needs, high cost sharing could place families in difficult situations deciding between life-altering surgery and bankruptcy. We aim to investigate trends in patient cost sharing and provider payments for cleft lip and palate repair.

Methods/Description: The IBM MarketScan national commercial claims databases were queried to extract patients younger than age 18 years who underwent primary or secondary cleft lip and/or palate repair from 2007 to 2016 based on CPT code. Financial variables recorded included gross payments to the provider (facility and/or physician), net payment as reported by the carrier, coordination of benefits and other savings, and the beneficiary contribution, which consisted of patients’ coinsurance, copay, and deductible payments. Linear regression was used to evaluate trends in payments over time. Poisson regression was additionally utilized to trend the proportion of patients with a non-zero beneficiary contribution. All financial values were adjusted to 2016 dollars per the consumer price index to account for inflation.

Results: The sample included 6268 cleft lip and 9118 cleft palate repair episodes. Total provider payments increased significantly from 2007 to 2016 for patients undergoing cleft lip (median $2527.33 vs $5116.30; P = .008) and palate ($1766.13 vs $3511.70; P < .001) repair. Beneficiary contribution additionally increased significantly over the study period for both cleft lip ($155.75 vs $193.31; P < .001) and palate ($124.37 vs $183.22; P < .001) repair, driven by an increase in deductibles (P < .002). For patients undergoing cleft palate repair, the proportion with a non-zero beneficiary contribution increased by 1.6% per year (P = .002). Higher provider payments and beneficiary contributions were found in the Northeast (P < .001) and South (P < .011), respectively, for both cleft lip and palate repair.

Conclusions: United States national data demonstrate that for commercially insured patients with cleft lip and/or palate, there has been a trend toward higher patient cost sharing that is most pronounced in the South. This suggests that patients are bearing an increased cost burden at the same time that provider payments are accelerating. Additional studies are needed to understand the impact of increased cost sharing on parents’ decision to pursue cleft lip and/or palate repair for their children.

(1) University of Pittsburgh Medical Center, Pittsburgh, PA, (2) NYU School of Medicine, New York, NY, (3) University of California, Los Angeles, CA, (4) UCLA Medical Center, Los Angeles, CA, (5) University of California, Irvine, CA, (6) UCLA, Los Angeles, CA

Background/Purpose: Orofacial clefting is a common congenital craniofacial anomaly that often necessitates multiple procedures to address functional and aesthetic concerns. Developing effective strategies to manage perioperative pain while limiting opioid use remains a focus of cleft care. The present study’s purpose is to systematically review perioperative pain control strategies for cleft lip and palate repair.

Methods/Description: A systematic review and meta-analysis was performed in line with PRISMA guidelines. Online databases were reviewed using MeSH and generic terms. Primary outcomes included pain scale scores and time to analgesia failure. Cohen’s d normalized effect size permitted comparison between studies and a fixed-effects model was used for analysis. I ² and Q-statistic P values were calculated for each comparison to assess heterogeneity. Methodological quality assessment was performed using the Jadad instrument and assessed via weighted Cohen’s Kappa analysis for inter-rater reliability.

Results: Twenty-three studies met inclusion criteria for systematic review; 8 of 23 studies provided sufficient data for meta-analytic comparison (inter-rater reliability: 0.85, indicating substantial agreement). All studies included for meta-analysis evaluated the efficacy of intraoperative nerve blocks on postoperative pain management. Meta-analysis included a total of 475 treatment and control patients; no significant differences in age or sex were noted. Meta-analysis of cleft lip studies demonstrated significantly improved pain control with infraorbital nerve block versus control via pain scale scores (P < .0001) and time to analgesia failure (P < .0001). Measurement of effect size over time demonstrated superior pain relief with local anesthetic versus placebo up to 12 hours postoperatively. Palatoplasty studies showed significantly improved time to analgesia failure (P < .005), but not pain scores, with maxillary nerve blocks. Multiple studies demonstrated a decrease in opioid use associated with intraoperative nerve blocks.

Conclusions: The present systematic review and meta-analysis of randomized controlled studies demonstrates that intraoperative nerve blocks for cleft lip and palate surgery provide effective pain control as evidenced by improved pain scores and time to analgesia failure. Opioid-sparing effects were appreciated in multiple studies. Intraoperative nerve blocks should be considered in all cases of cleft lip and palate repair to improve to postoperative pain management.

(1) Boston Children’s Hospital, Boston, MA, (2) Shriners Hospital for Children, Los Angeles, CA, (3) Boston Children’s Hospital Simulator Program, Boston, MA

Background/Purpose: Demonstrating competence before independent practice is increasingly important for graduating residents and surgeons participating in cleft-related missions. High fidelity simulation allows the operator to use real instruments to perform varied maneuvers requiring both judgment and skill. Thus, it may be uniquely advantageous for evaluating abstract procedures like cleft lip repair. This study tests the hypothesis that a high fidelity cleft lip simulator can discriminate surgical performance between training levels, demonstrating its utility for assessing competence.

Methods/Description: Twenty-one participants performed cleft lip repair on a high fidelity simulator. Multiple participants were recruited from each training level with the supposition that if simulation possessed the construct validity to assess performance, there would be progressive improvement from one training level cohort to the next. Procedures were assisted by the same surgeon. No guidance or coaching was offered. Procedural videos were blindly rated using the modified Objective Structured Assessment of Technical Skills (OSATS) and the Unilateral Cleft Lip Repair Competency Assessment Tool (UCLR). UCLR sub-scores were individually evaluated. The influence of training level and cumulative prior experience on score was estimated using Pearson r.

Results: Training levels included Integrated PGY 3 (n = 4), Integrated PGY 4/Independent PGY 6 (n = 4), Integrated PGY 5/Independent PGY 7 (n = 4), Integrated PGY 6/Independent PGY 8 (n = 5), and craniofacial fellow (n = 4). Cumulative prior experience ranged from 0 to 45 cleft lip repairs (median = 5), with the majority (n = 16) having participated in 8 or fewer. Training level correlated with both OSATS (r = 0.444, P = .044) and UCLR (r = 0.579, P = .006) scores whereas cumulative prior experience did not correlate with either OSATS (r = −0.004, P = .988) or UCLR (r = 0.325, P = .151). Looking granularly at UCLR sub-score, marking the repair (r = 0.368, P = .101) did not correlate with training level whereas performance (entailing key elements such as repairing the orbicularis oris muscle) correlated strongly with training level (r = 0.660, P = .001).

Conclusions: High fidelity simulation has the construct validity to evaluate performance for cleft lip repair, as evidenced by correlation between training level and objective performance. Of interest was the finding that marking the repair did not correlate with training level while performing the repair strongly did. This indicates simply being able to mark a cleft lip repair is not an accurate assessment of competence. For these reasons, high fidelity simulation has value for evaluating trainees as well as for screening skill in the context of humanitarian missions. We were surprised cumulative prior experience did not correlate with performance, but this may be related to the greatly skewed participant experience and deserves further study.

(1) West China Hospital of Stomatology, Sichuan University, Chengdu, Sichuan, (2) Department of Cleft Lip and Palate Surgery, West China Stomatological Hospital, Sichuan University, Chengdu, China

Background/Purpose: The aim of this study was to present a modified technique for cleft palate repair and evaluate its efficacy with particular attention to the incidence of postop operative fistula formation.

Methods/Description: We performed a modified technique, Sommerlad-Furlow (SF), which combined the repositioning of the levator veli palatini muscles as described by Sommerlad with the double opposing Z-plasty of Furlow to lengthen the soft palate. Of all, 1283 patients underwent either an SF, a Sommerlad, or a Furlow procedure between 2009 and 2018, and the incidence of fistula formation was analyzed.

Results: Our analysis revealed that the Sommerlad technique was associated with a higher fistula rate in both unilateral and bilateral cleft lip and palate (B/UCLP) patients. In addition to not requiring relaxing incisions, SF advantages included (1) the lowest fistula rate in the 2 to 4 year old and the >4 year old age groups, (2) a consistently lower fistula rate compared to the Sommerlad technique, (3) the patients had stable postoperative velopharyngeal function was 95% respectively, (4) good growth results have been showed with this technique, and (5) the lowest fistula rate in patients with both hard and soft palate clefts without cleft lip (HSCP). The differences between the 3 technique did not reach statistical significance in B/UCLP and in patients with soft palate only (SCP) or submucosal cleft palate (SMCP).

Conclusions: The combination Sommerlad-Furlow (SF) technique demonstrated advantages compared to either technique alone. The avoidance of relaxing incisions will theoretically also benefit future maxillary growth.

(1) Spectrum Health Medical Group, Grand Rapids, MI

Background/Purpose: The Double Opposing Z- plasty ± Buccal Flap Approach (DOZP ± BFA) is a proven alternative to traditional palate repairs. A published article has demonstrated that using the DOZP ± BFA achieves excellent speech outcomes regardless of the patients cleft width or classification. However some surgeons are slow to change from traditional approaches fearing the possibility that severe complications will frequently occur when using a more anatomic reconstruction where outside tissue is added to the surgical plan. The purpose of this presentation is to review the complications seen during the first 29 years using the DOZP ± BFA.

Methods/Description: This study was a retrospective review of complications and care management for 505 patients over a 29 year time frame. Measures include but were not limited to Veau classification, postoperative trauma, syndrome status, Pierre Robin, fistula type, and additional surgeries required.

Results: Of the 505 patient cohort, there were 32 complications and 30 fistulas. For nonsyndromic, non-Pierre Robin patients (N = 333) there were 16 fistulas. The overall fistula rate was 4.8%, however fistulas > 2 mm comprised only 1.8%. There was 1.2% postoperative trauma rate. The number of fistulas requiring more than one surgery was zero. The number of fistulas requiring outside flaps was also zero. The number of nightmare complications was zero. One syndromic patient required two closures. All fistulas were closed with local tissue rearrangement. No external flaps required for repairs. Secondary speech surgeries were required more often for patients with fistulas than for patients without fistulas. However, the final speech resonance scores for patients with fistulas were similar to the scores achieved in patients without fistulas.

Conclusions: The DOZP ± BFA is a safe approach with few complications. The types of fistulas seen over 29 years, indicates that the majority of the fistulas seen are of the small nuisance variety and are easily managed with low impact on the patients. The fact that zero fistulas occurred requiring outside flaps demonstrates that DOZP ± BFA patients are at low risk of experiencing the morbidity of multiple surgeries. Additionally, all fistulas had limited effect on long-term speech outcomes.

(1) Seattle Children’s Hospital, Seattle, WA, (2) Phoenix Children’s Hospital, Phoenix, AZ

Background/Purpose: Current models of the uCLND are based upon subjective observations and study of a limited numbers of cadavers. The various descriptions are confusing, contradictory, and often lack objective data to validate them. Given that optimal treatment of the uCLND remains a challenge, a better understanding of the deformity is critical to devising ways to improve care. The purpose of this study was to develop a model of the uCLND that is based upon objective 3D data and that spans the spectrum of presentation.

Methods/Description: We assessed landmark displacements, anthropometric dimensions, and shape-base measures on 3D images infants with unrepaired unilateral cleft lip at 6 months of age (N = 100). Lateral displacement of subnasale (sn), the surface landmark for the anterior nasal spine and caudal septum, had the greatest magnitude abberation of any measure and was therefore used as the independent variable in a linear regression analysis model. In order to accommodate for age-related alterations, we corrected for the age at image capture. Significance was assessed by ANOVA with P < .05 considered significant.

Results: Significant changes were expressed for each 1 mm deviation of sn from midline (P < .05). Nose: The columella tilts 4.2°, the cleft nostril widens (1.1 mm), and, to a lesser degree, the nose broadens (0.7 mm). The noncleft alar base is driven lateral, away from midline (0.8 mm), while the cleft alar base gets left behind and migrates posteriorly (0.6 mm). The nasal dorsum follows sn deviation, swinging 1.8° from midline, toward the noncleft side. The noncleft alar base does not deviate as much as subnasale resulting in constriction of the noncleft nasal sill (−0.2 mm). Collapse of the dome occurs progressively with these changes with measureable alterations in the nose tip volume ration (+0.15) and alar-cheek definition (+2.09). Lip: The entire philtrum deviates away from midline, toward the noncleft side. The lip heights adjacent to the cleft diminish (−0.2 mm) and the lateral lip lengths shorten (−0.3 mm). Meanwhile, the non-cleft medial lip height grows (+0.2 mm). Face: Facial height and width do not change, however, inter-endocanthal distance widens (+0.4 mm).

Conclusions: Deviation of sn can be used as a measure of cleft severity to produce a progressive model of the uCLND and associated changes. Contrary to previous descriptions there is no normal side, the cleft alar base is not displaced lateral, and the nasal dorsum does not deviate toward the cleft. Rather, displacement of the noncleft alar base, deviation of the dorsum that follows sn, and retrusion of the cleft alar base are associated with progressive collapse of the nasal form. Based on this data, treatment should aim to centralize the columella and balance the alar base relationships by moving the noncleft alar base medial and the cleft alar base anterior. Correction of the “twist” in the nasal foundation may be critical to long-term stability of primary repair.

(1) Children’s Mercy Kansas City, Kansas City, MO

Background/Purpose: Due to increasing misuse of and dependence on opioids, medicine has begun to evaluate areas where exposure to prescription opioids can be decreased including in vulnerable pediatric populations. As infants emerge from anesthesia after palatoplasty, there appears to be a predictably large degree of agitation and disorientation. We hypothesized that directed treatment of postoperative agitation could improve recovery and reduce pain and opioid use in these patients. Oral clonidine was identified as a readily available nonopioid medication used in children that decreases postoperative agitation. This study aimed to evaluate the effectiveness of clonidine as an adjunct to postoperative pain management with regard to decreased opioid use and hospital length of stay after primary palatoplasty.

Methods/Description: This is a single-center IRB-approved study from a tertiary pediatric hospital reviewing the use of postoperative opioids after primary cleft palate repair. Two cohorts were compared, one before and one after the implementation of a postoperative management protocol using scheduled doses of clonidine. The study retrospectively evaluated the use of pain medication, length of stay, and pain scores for each group.

Results: The preclonidine cohort included 26 patients undergoing primary palatoplasty for 9 months prior to protocol initiation who did not receive any clonidine. The 6-month pilot phase of the clonidine protocol included 17 patients. Postoperative medications included a combination of IV fentanyl, oral acetaminophen, ibuprofen, and oxycodone ± oral clonidine. Oxycodone was dosed according to weight as needed for moderate-severe pain not responding to other oral measures. In all, 42 of 43 received postoperative oxycodone. The preprotocol cohort took on average 4.61 doses over 19.46 hours while the clonidine cohort required slightly less with an average of 3.86 doses over 22.30 hours (P = .41). The mean length of postoperative hospital stays increased from 1.24 days preprotocol to 1.38 days postprotocol (P = .49). Although bedside caregivers subjectively reported smoother emergence and immediate postrecovery phases in the clonidine group, no significant differences were seen between the groups for recorded pain scores or Pediatric Anesthesia Emergence Delirium (PAED) scales.

Conclusions: The data from this pilot study indicates clonidine use was not associated with any significant reductions in estimated pain scores or reductions in immediate postoperative opioid consumption prior to discharge. There was also no decreased length of stay documented. This suggests that while clonidine may have some clinically observable benefits for postoperative agitation, there was no clear evidence that it was helpful in measurably improving recovery experiences for patients undergoing primary cleft palate repair.

(1) Yale University School of Medicine, New Haven, CT, (2) Johns Hopkins School of Medicine, Baltimore, MD, (3) Stanford School of Medicine, Palo Alto, CA, (4) Department of Plastic and Reconstructive Surgery, Yale University School of Medicine, New Haven, CT

Background/Purpose: While late age presentation of cleft lip (CL) and cleft palate (CP) in international settings has been associated with increased surgical morbidity, little is known about the prevalence of or risks associated with these procedures in the United States. The current study aimed to assess the prevalence of and risks associated with older age CL and CP repair in comparison to repair at younger ages.

Methods/Description: Primary CL and CP repairs of patients under the age of 18 were identified in the National Surgical Quality Improvement (NSQIP) pediatric database from 2014 to 2018 using CPT codes. Total postoperative complications, rates of readmission and reoperation, OR time, and length of stay were recorded. T tests were used to compare these variables across the following age groups: 0 to 5, 6 to 10, and 11 to <18.

Results: In all, 7909 primary CP and 5649 primary CL procedures were identified. Surgery after the age of 5 was rare in both cohorts: 147 (2.6%) children receiving CL and 713 (9.1%) receiving CP repair. Among both CL and CP patients, there were no significant differences in the number of unplanned readmissions, reoperations, or complication rates following surgery (P < .05) for patients aged 6 to 10 or 11 to 18. Patients ages 6 to 10 in both surgical cohorts had decreased operating room time (P < .001), as did CP patients ages 11 to 18. Older CP patients also had decreased hospital length of stay (P < .001). Complications were rare in all subgroups, but were most commonly surgical site infections (CL = 0.49%, CP = 0.19%), dehiscence/fistula (CL = 0.36%, CP = 1.3%), and respiratory complications (ie, prolonged intubation) (CL = 0.76%, CP = −1.3%). In comparison to younger children, patients over the age of 5 were less likely to experience respiratory complications following CP (P = .03) repair.

Conclusions: A small cohort of patients in the United States may be significantly delayed when receiving cleft care, likely due to adoption/immigration or missed diagnosis submucosal palates. Our data suggest a high level of surgical safety among these patients when treated in the United States, with no increased short-term morbidity among patients treated after 5 years of age, and fewer short term respiratory risks. Further efforts should be made to ensure similar safety profiles among older age patients treated abroad, and to better understand and address implications of delayed care in relation to longitudinal speech and/or functional care.

(1) Osaka University, Graduate School of Dentistry, Osaka University Cleft Center, Suita city, Japan, (2) Osaka University Cleft Palate Team, Suita, Japan, (6) Graduate School of Dentistry, Osaka University, Suita, Japan

Background/Purpose: Velopharyngeal structure augmentation methods are used as alternatives to pharyngeal flap operations. We recently evaluated the sites and materials of velopharyngeal structure augmentation in dogs because they naturally exhibit velopharyngeal insufficiency, and we found that augmentation was most effective at the nasal mucosa of the soft palate than at the posterior pharyngeal wall or bilateral pharyngeal walls using autogenous fat tissue. However, performing injections correctly was difficult because the injection points of velopharyngeal space could not be observed directly. In this presentation, we report a novel method of injecting materials for velopharyngeal structure augmentation using an endoscopic device, while observing the injection points directly.

Methods/Description: Endoscopic soft palate augmentation was performed on 4 patients (aged 8-16 years) under general anesthesia. All clinical protocols were reviewed and approved by the Clinical Ethics Committee of Osaka University Graduate School of Dentistry (approval number H30-E30). Subcutaneous fat was collected from the left thigh and centrifuged to separate into 3 layers, and only the middle layer was used as the injection material. The autogenic fat tissue was injected into the nasal mucosa of the soft palate using an endoscopic needle-type device. We then evaluated the effect by comparing the endoscope observation and speech assessment before and after the operation.

Results: Through endoscopic injection of materials, the injection part could be directly observed, and it was also easy to adjust the amount. The materials were successfully injected, and patients could achieve velopharyngeal function immediately. They were able to maintain it for more than 6 months, and we are further continuing our follow-up.

Conclusions: Velopharyngeal insufficiency augmentation using an endoscopic device was found to be a useful new technique. Therefore, our results may aid in the treatment of patients with velopharyngeal insufficiency, although long-term follow-up is needed for further evaluation.

(1) Kanagawa Children's Medical Center, Yokohama, Japan, (2) Hirakawa Orthodontic Clinic, Yokohama, Kanagawa, (3) Fukawa Orthodontic Office, Kamakura, Kanagawa

Background/Purpose: We have been using our own treatment for bilateral cleft lip and palate (BCLP) patients. After presurgical orthopedics (PSO), the subsequent surgical procedures depend on the result of PSO. The procedures consist of PSO, gingivoperiosteoplasty (GPP), Furlow double-opposing Z-palatoplasty (FDOP), and maxillary protraction appliance (MPA). Our purpose is to evaluate the outcomes including speech, alveolar bone formation, and the maxillary growth after our treatment.

Methods/Description: Twenty-seven nonsyndromic BCLP patients without mental disorders and serious anomalies were treated by PSO, cheiloplasty, GPP, and FDOP at Kanagawa Children’s Medical Center. After the treatments, their speech outcomes, alveolar bone formations, and maxillary growths were evaluated by CT imaging at 5 years of age. MPA was used as part of the protocol for 6 months to 1 year for postoperative retardation of maxillary growth cases.

(1) University of Utah Health, Salt Lake City, UT, (2) Intermountain Healthcare, Salt Lake City, UT

Background/Purpose: Malocclusion has been widely associated with cleft palate (CP) and is defined as misalignment of individual teeth within each dental arch or of the dental arches relative to normal occlusion. Although there is a clear association between CP and severe malocclusion, there are little data in the literature on risks or protective factors associated with development of malocclusion. Thus, the aim of this study is to evaluate how single-stage versus 2-stage palate repair affects the risk of developing malocclusion among CP patients.

Methods/Description: A retrospective cohort study with follow-up of between 3 and 18 years among patients who underwent CP repair between 1999 and 2015 at an academic, tertiary children’s hospital. Interventions included CP repair, evaluating differences in the rate of malocclusion based on a single- versus 2-stage repair. We excluded patients with submucous CP and lack of complete cleft repair records within the study hospital system. Analysis included univariate, unadjusted Cox regression analyses to assess each variable’s association with time to malocclusion. Variables with significant associations were entered into multivariate Cox regression analyses in a hierarchical manner. Nonsignificant variables were removed, and the models rerun each time a new variable was added. Cox regression analyses were time-stratified at ≤5 years and >5 years to satisfy the proportional hazard assumption.

Results: Nine hundred sixty-seven patients were included; 60.1% had staged CP repair, and 39.9% had single stage. Malocclusion was diagnosed in 28.2% of patients. In the model examining all patients at ≤5 years (n = 659), non-Caucasian patients had a higher risk of malocclusion (HR 2.46, P = .004); staged repair was not protective against malocclusion (HR 0.98, P = .55). In all patients >5 years that had a staged repair (n = 411), higher Veau (P = .001) and more recent year of birth (P = .01) were significantly associated with higher hazard rates, while having public insurance approached significance (HR 1.36, P = .058). Staged repair was not protective of developing malocclusion (HR 0.9, P = .60). In the model examining patients with staged repair ≤5 years (n = 414), higher age at hard palate closure was associated with a lower risk of malocclusion (HR 0.67, P < .001) and non-Caucasian patients had a higher risk (HR 2.56, P = .01). In patients with staged repair >5 years, more recent birth year may be associated with a higher risk of malocclusion (HR 1.06, P = 0.06), while syndrome may be associated with a lower risk of malocclusion diagnosis (HR 0.46, P = .07).

Conclusions: Our data suggest that undergoing 2-stage CP repair is not protective against malocclusion.

(1) Amsterdam UMC, the Netherlands

Background/Purpose: Tongue-lip adhesion (TLA) is commonly used as a surgical treatment for upper airway obstruction (UAO) in patients with Robin sequence (RS). The effect on speech and articulation outcomes after TLA and subsequently cleft palate (CP) repair in patients with RS is insufficiently investigated. The aim of this study was to investigate long-term speech and articulation outcomes after cleft palate repair in patients with RS and the possible effect of TLA.

Methods/Description: All consecutive patients with RS (with or without TLA, 1993-2014) who underwent CP repair according the von Langenbeck technique with intravelar veloplasty at the Amsterdam UMC were retrospectively reviewed and compared to patients with isolated cleft palate (ICP). Variables analyzed were sex, CP-type, age at TLA, associated syndromes with RS, and age at CP repair. Speech and articulation errors were assessed as binary outcomes by a senior craniofacial speech pathologist of multidisciplinary cleft team and included all assessments between the age of 3 and 6 years. These outcomes included secondary speech operation, velopharyngeal insufficiency (VPI), hypernasality, and articulation errors by assessment of cleft type characteristics (CTC), including 4 categories: (1) passive, (2) nonoral, (3) anterior oral, and (4) posterior oral.

Results: Forty-one patients with RS (56% syndromic, 44% isolated RS) and 61 ICP patients, underwent CP-repair (at median ages of 9.3 and 9.4 months, P = .207, respectively) with sufficient speech follow-up. Of the patients with RS, 23 (56%) underwent a TLA at a median age of 12 days (range: 2-100 days). CP characteristics were different: soft palate only 24% RS versus 59% ICP, soft + hard palate 76% RS versus 41% ICP, P = .001. Median age at time of all speech evaluations was 4.5 years (range: 3.0-6.3) for RS versus 4.6 years (range: 3.3-6.3) for ICP, P = .581. VPI was diagnosed in 73% of the RS-group versus 62% of the ICP group, P = .253. Rates of hypernasality and secondary speech operations to resolve VPI were higher in RS compared to ICP, both P = .003. In RS, the posterior oral CTC demonstrated higher rates compared to ICP, P = .038. When comparing RS + TLA versus RS-TLA, no significant differences were observed in variables CP-type and underlying syndromes between the 2 groups. Patients with RS + TLA did not demonstrate differences in VPI, hypernasality, secondary speech operations, or any CTC’s (all P > .05) compared to patients with RS without TLA, except for the anterior oral CTC’s (74% RS + TLA vs 28% RS-TLA, P = .003).

Conclusion: Our data demonstrated that patients with RS have higher rates of hypernasality and needed more secondary speech operations to resolve VPI compared to patients with ICP. In patients with RS, our findings demonstrated that the surgical procedure TLA does not affect long-term velopharyngeal function. However, patients with RS and TLA demonstrated higher rates of anterior oral CTC/articulation errors that might be related to a different positioning of tongue after TLA.

(1) McGovern Medical School at The University of Texas Health Science Center at Houston (UTHealth), Houston, TX, (2) University of Texas Health Science Center at Houston, TX

Background/Purpose: Velopharyngeal insufficiency (VPI) following primary palatoplasty can result in hypernasality of the voice and may require surgical revision of the palate to address palatal length or function. The conversion Furlow procedure not only improves muscle positioning but also lengthens the soft palate using a double-opposing Z-plasty technique. Buccal flaps have been described to improve cleft palate repair and can provide additional well-vascularized local tissue to augment palate length. Although previous studies have shown some benefits to using the buccal flap approach with primary Furlow palatoplasty, the effectiveness of buccal flaps with conversion Furlow in secondary management of VPI is unknown.

Methods/Description: A retrospective chart review of consecutive nonrandomized patients undergoing revision palatoplasty for VPI after primary straight-line repair from 2014 to 2020 was performed. VPI was diagnosed in patients by a speech therapist and confirmed with nasoendoscopy. Two cohorts were identified: those who received the conversion Furlow palatoplasty alone (FA) versus those with conversion Furlow with buccal flap(s) (FB), which was initiated in 2018 at our institution. Speech assessments measured preoperative and at least 3-month postoperative hypernasality and total parameter scores using Universal Parameters for Reporting (UPR). Secondary outcomes, including fistula rates, need for revision surgeries for recurrent VPI, infections, and length of stay, were also examined. Univariate analyses of the data were performed.

Results: Of the 77 patients who underwent revision Furlow palatoplasty for VPI, 21% (n = 16) had a repair that incorporated at least one buccal flap. The median age at cleft palate revision surgery was 8.76 years, with patients in the FB group being younger (7.96 years vs 8.97 years, P = .337). Four (7%) patients in the FA cohort developed a postoperative fistula of the soft palate (Pittsburgh 2-3), compared to zero patients in the FB cohort. Nine (15%) patients in the FA cohort required additional revision surgery for persistent hypernasality, compared to zero patients in the FB cohort. The average time between revision surgery and most recent assessment of speech for all patients was 1.8 years (range: 3 months to 4.8 years). Both cohorts demonstrated a decrease postoperatively in hypernasality (0.82 for the FA group and 0.66 for the FB group) and total parameter scores (3.47 for the FA group and 0.25 for the FB group).

Conclusions: Our study demonstrates that while there are no significant differences in speech outcomes between these 2 reconstructive modalities, there are reduced fistula rates and revision surgery rates among patients in which buccal flaps were used. Longer term follow-up will be necessary to determine whether these speech results continue to improve with time. This information may be useful for cleft surgeons in choosing appropriate procedures to correct VPI, though a larger cohort or randomized trial would be ideal to determine any true significance.

(1) University of Illinois at Chicago, IL, (2) University Of Illinois at Chicago/Shriners Hospitals for Children-Chicago, IL

Background/Purpose: Recently, primary cleft rhinoplasty has become much more common and accepted, potentially even standard of care. However, several authors still have voiced concerns regarding scarring, nasal growth restriction, and lack of avoidance of rhinoplasty at skeletal maturity. The principal goal of this study is to determine the amount and quality of evidence for primary rhinoplasty. Secondary goals include review of evidence regarding how primary rhinoplasty effects nasal development, if it can eliminate the need for additional rhinoplasty, and compare the different surgical approaches described in the literature.

Methods/Description: This systematic review followed the PRISMA protocol guidelines of conduct for systematic review and meta-analysis. PubMed was utilized to search the US National Library of Medicine database in January 2020. Studies were screened by title and abstract. If the study satisfied criteria, the entire article was reviewed. Criteria for inclusion were (1) human subjects undergoing rhinoplasty at the time of unilateral cleft lip repair, and (2) subjective or objective evaluation of the nasal outcome. Exclusion criteria were (1) studies of syndromic patients, (2) case reports, (3) editorials, (4) letters, (5) reviews, and (6) studies not written in English. Additional studies were included through screening the citations of reviewed articles.

Results: Twenty-two articles met inclusion criteria. Results were assessed subjectively in 10 publications with generally favorable results. Fifteen publications measured results objectively by evaluating septal deviation, direct or indirect anthropometric measurements, or nasal endoscopy. Objective evaluations revealed improved symmetry when compared with children who did not undergo primary rhinoplasty. Nine studies evaluated nasal growth and development over time and only one presented evidence for growth restriction in nasal height: dorsum ratio and columellar angle. Eight studies calculated the percentage of patients in which secondary rhinoplasty was avoided, ranging from 43% to 100%. A few studies compared surgical techniques, finding evidence that open repair, overcorrection of the cleft nostril, septoplasty, and intranasal incisions can lead to superior outcomes. Many studies had short periods of follow-up and the largest studies relied mainly on subjective analysis.

Conclusions: There is mounting evidence that the primary rhinoplasty techniques do not restrict growth of the nose, can lead to acceptable aesthetic outcomes, superior functional outcomes, and can eliminate the need for a secondary rhinoplasty in some individuals. Weaknesses of the evidence for primary rhinoplasty include lack of prospective, objective, and comparative studies with follow-up to skeletal maturity.

(1) Children’s Hospital of Philadelphia, PA

Background/Purpose: Cleft lip repair requires meticulous surgical markings that take significant experience to appropriately master. Simulation of these maneuvers could supplement available case volume for residents to achieve these competencies. Moreover, achieving these fundamentals prior to hands-on experience in the operating room could compound the learning experience within actual surgical procedures. The purpose of this study is to pilot a novel iPad application for teaching cleft lip incision markings and assessing surgical trainee performance.

Methods/Description: Trainees voluntarily enrolled to beta test a novel surgical simulator application. Surgical specialty, years of residency training, number of cleft lip repair procedures performed, and previous textbook knowledge of cleft lip repair were surveyed. Participants took an interactive pretest, which consisted of marking points of prompted anatomic landmarks for cleft lip repair incisions upon a high-resolution digital image of a patient with unilateral cleft lip. Participants then viewed a tutorial on how to appropriately delineate these landmarks using the anatomic subunit approximation method, and thereafter participants took an interactive posttest of marking these incisions on a similar patient image. Accuracy of trainee surgical markings was calculated as a cumulative deviation distance from ideal incision locations established by attending surgeons. Confidence was assessed using a visual analog scale along a spectrum of no knowledge (0 units), knowledgeable but no experience (1 unit), confident with supervision (2 units), and confident without supervision (3 units).

Results: Thirteen individuals completed the study, including medical students (n = 2), junior residents (PGY 1&2, n = 6), midlevel residents (PGY 3&4, n = 2), and senior residents (PGY 5&6, n = 3). Residents included both plastic surgery residents (n = 7) and other surgical residents rotating on plastic surgery services (n = 4). Overall, the surgical marking deviation of the posttest (389 pixel deviation) was improved compared to the pretest (401 pixel deviation; P = .046.) Overall, trainee confidence also improved from prior to the training course (0.00 units) to after completion (0.50 units; P = .041). PGY 6 residents demonstrated relatively stable performance from the pretest to the posttest (PGY 6 deviation difference: +1.872 pixels), whereas junior and midlevel trainees seemed to benefit more from the tutorial (PGY ≤5 deviation difference: –19.14 pixels). Correspondingly, these younger trainees demonstrated an improvement in their confidence after training on this simulator (P = .041; confidence difference: +0.22 units).

Conclusions: Cleft lip incision marking accuracy is significantly improved with an interactive touch screen training simulator. Plastic surgery trainees in their final year of residency seem to have plateaued at appropriate competency, but junior trainees continue to show significant improvement in their accuracy and confidence utilizing this training simulator.

(1) Children’s Hospital of Philadelphia, PA, (2) University of Pittsburgh Medical Center, PA

Background/Purpose: Rhinoplasty to correct distorted nasal architecture secondary to orofacial clefting may be desirable at a younger age to alleviate resulting aesthetic and functional impairment, but early intervention must be weighed against consequent local growth disruption, leading to a variety of practice patterns. The purpose of this study is to describe the epidemiology of cleft rhinoplasty timing, technique, and concurrent cleft procedures.

Methods/Description: The ACS NSQIP-Pediatric data set was comprehensively queried for cleft rhinoplasty procedures performed among all primary procedures coded as cleft lip repair, cleft palate repair, and/or cleft rhinoplasty. Patient characteristics, operative details, and postoperative complications were compared with appropriate statistics.

Results: During the study interval, 3317 rhinoplasties were performed for nasal deformity secondary to congenital cleft lip and/or palate. Median age at cleft rhinoplasty was 3.4 years, with 44.7% (n = 1483) performed during the initial cleft lip repair, 19.9% (n = 660) performed during repeat cleft lip repair, and 35.4% (n = 1174) were performed independent of cleft lip repair. Up to 28.2% (n = 1357 of 4811) cleft lip repairs performed in the first year of life had concurrent tip rhinoplasty, and none utilized cartilage during these early procedures. Rhinoplasty for bilateral cleft lips was performed at a significantly older age than for unilateral cleft lips (P < .001; 4.8 years vs 0.8 years). Cartilage use during rhinoplasty demonstrated a bimodal distribution at 4.5 years and 17.5 years. More extensive reconstructions involving the septum and/or osteotomies (29.3%, n = 971) were delayed until a significantly older age (P < .001, 11.7 years vs 0.8 years). These more extensive rhinoplasty procedures were significantly more likely to utilize cartilage grafts (P < .001, 15.9% vs 4.9%). Interestingly, presence of unilateral versus bilateral cleft lip did not affect need for more extensive reconstruction (P = .974). Tip rhinoplasties were significantly more likely to utilize ear cartilage (P < .001, 70.5% vs 29.5%), whereas more extensive rhinoplasties involving the nasal septum were significantly more likely to utilize costal cartilage (P < .001, 67.1% vs 32.9%). Rhinoplasty with costal cartilage grafts took significantly longer to perform than those with ear cartilage grafts (P < .001, 151 minutes vs 102 minutes), but reassuringly, ear versus costal cartilage grafts did not demonstrate statistically significant difference in graft failure or any other indexed postoperative adverse event (P all ≥.307).

Conclusions: Rhinoplasty and concomitant cartilage grafts have unique utilization profiles across the spectrum of orofacial cleft phenotypes. More extensive reconstructions involving the nasal septum are delayed until an older age. Tip rhinoplasties are more likely to utilize ear cartilage, whereas more extensive rhinoplasties involving the nasal septum are more likely to utilize costal cartilage.

(1) Children’s Hospital of Philadelphia, PA, (2) Operation Smile Nicaragua, Managua, Nicaragua, (3) Operation Smile Ecuador, Quito, Ecuador

Background/Purpose: Oronasal fistulas after palatoplasty can result in significant morbidity including hypernasal speech, symptomatic nasal regurgitation, and scarring that may impede maxillary growth. Limitations of reconstructing large, anterior hard palate fistulas with tongue, or facial artery myomucosal flaps have been well recorded in the literature. The purpose of this study is to evaluate our short-term results using the buccal myomucosal flap—a robust, reliable, single-stage technique—for closure of large, recurrent, and anterior palatal fistulas.

Methods/Description: Inclusion criteria were the presence of a large, recurrent anterior palatal fistula that had failed reconstruction with local tissues and complete medical record. Patients undergoing buccal flap closure first had approximation of apposing nasal turnover flaps of vomer mucosa medially and nasal wall mucosa laterally. The oral closure is performed with a single buccal myomucosal flap. The flap is designed with its pedicle and point of rotation just posterior to the retromolar trigone. The flap is at least 1 cm wide at its base and extends just proximal to the oral commissure. An inset incision is made immediately lingual to the alveolar ridge, and the flap is inset to the lateral palate to avoid interference with occlusion.

Results: The modified buccal myomucosal flap was performed on 11 patients aged 3 to 22 years, with average anterior palatal fistula size of 2.35 cm ² (range: 0.8-3.5 cm ² ) and average follow-up of 5 months (range: 2-13 months). Ten patients completing follow-up showed decreased nasal speech and regurgitation; 8 healed expediently, 1 flap exhibited delayed tip mucosalization, and 1 flap experienced partial anterior flap dehiscence treated successfully with flap readvancement. Nine of 11 flaps were successfully performed as a single stage; in addition to the above revision, one flap required delayed flap division to achieve comfortable molar occlusion. No patients exhibited facial asymmetry in smile or repose, limitation in lip pucker, or restriction of mouth opening.

Conclusions: The modified buccal myomucosal flap shows promising short-term results as a single-stage reconstruction for large, recurrent, anterior palatal fistulas with low morbidity. Although longer term follow-up is needed, this flap’s utility and reliability has led us away from tongue and FAMM flaps for large, anterior, recurrent fistulas.

(1) Children’s Mercy Hospital, Kansas City, MO

Background/Purpose: Hypocalcemia and hypoparathyroidism are common features of 22q11.2 deletion syndrome (22q11DS) and are classically considered a transient feature in the neonatal period. Latent hypocalcemia and hypoparathyroidism have been reported, possibly triggered during times of acute biologic stress. The published guidelines for managing children with 22q11DS specifically reference the risk of developing hypocalcemia during times of biologic stress, highlighting surgery as examples of biologic stress. Despite this, little has been published on the effect of surgery on children with 22q11DS and their risk of postoperative hypocalcemia. To date, there are no known reports of postoperative hypocalcemia in noncardiac surgeries for children with 22q11DS. We present 3 cases of children with 22q11DS who developed postoperative hypocalcemia after undergoing posterior pharyngeal flap.

Methods/Description: This is a case series of 3 patients. Written consent was obtained from the patients’ families. As this is a case series of only 3 patients, IRB approval was not required. Chart review was completed for each patient including demographics, surgical data, and preoperative and postoperative details including lab values and medications.

Results: Three pediatric patients with history of 22q11DS, hypernasality, velopharyngeal insufficiency (VPI), and a neurogenic soft palate underwent posterior pharyngeal flap for treatment of VPI. All 3 patients were prepubertal. One patient had history of congenital hypoparathyroidism and transient hypocalcemia as an infant. At the time of the procedure, none of the patients were taking any calcium supplementation or calcitriol. Preoperative serum calcium was obtained on all patients and found to be in the normal range. The surgical procedures themselves were uneventful and operative time ranged from 146 to 170 minutes. All 3 patients had a significant postoperative decline in serum and/or ionized calcium. At the time of hypocalcemia, intact parathyroid hormone (iPTH) was obtained and found to be inappropriately normal in each patient. Mild neuromuscular symptoms were observed in 2 patients, and no severe signs or symptoms of hypocalcemia were encountered. Pediatric endocrinology was consulted for each patient and medical therapy was initiated in 2 of the 3 patients. All patients had normalized their calcium by postoperative day 4 and were discharged home without any complications.

Conclusions: This case series is the first to highlight the presence of postoperative hypocalcemia in patient with 22q11DS after posterior pharyngeal flap. The hypocalcemia was transient and not associated with significant signs or symptoms. Surgeons caring for children with 22q11DS should be aware of the risk for postoperative hypocalcemia and consider checking calcium levels both pre- and postoperatively. Future studies are needed to better understand the physiologic cause, risk, and the clinical significance of postoperative hypocalcemia in patients with 22q11DS.

(1) Washington University in St. Louis, MO, (2) Washington University, School of Medicine, St. Louis, MO, (3) St. Louis Children’s Hospital, MO

Background/Purpose: Achievement of velopharyngeal competence is a primary measure of success after palatoplasty. A palatal fistula, an additional adverse outcome of cleft palate repair, causes nasal air escape, regurgitation, and speech impairment. However, it is unknown if a palatal fistula will influence velopharyngeal closure. The purpose of this study is to determine the effect of a soft palate fistula on the risk of developing velopharyngeal insufficiency (VPI) after cleft palate repair.

Methods/Description: With institutional review board approval, a retrospective chart review was conducted on patients who underwent primary cleft palate repair between 2000 and 2015. Only patients with complete records at 4 years of age were included. Exclusion criteria were submucous cleft palate or a palatal repair performed elsewhere. Patients were diagnosed with VPI by video nasoendoscopy and/or speech videofluoroscopy. Fistulae involving the secondary palate following primary palatoplasty were classified as soft or hard palate. A forced-entry multivariate logistic regression model was built to detect predictors of VPI.

Results: Of 466 candidates, 117 patients were excluded due to insufficient follow up. An additional 20 patients were excluded due to missing records, submucous cleft palate, or repairs performed elsewhere. The remaining 329 records were analyzed. Repairs were performed by 8 surgeons; 245 (74%) patients were nonsyndromic and 84 (26%) were syndromic. Mean age at primary repair was 13.2 months. There was a mean interval of 8.7 years between primary repair and most recent follow-up; 89 (27%) patients developed a palatal fistula: 39 (44%) were in the hard palate and 50 (56%) in the soft palate; 29 (8.8%) patients had an independent fistula repair; 90 (27%) patients developed VPI: of which 52 patients had no palatal fistula (22% of patients without fistula had VPI), 10 patients had only a hard palate fistula (10 of 39 patients, 26%), and 28 patients had a soft palate fistula (28 of 50 patients, 56%). Soft palate fistula was associated with increased incidence of VPI (OR 3.875, CI 1.964-7.648, P < .001); hard palate fistula was not (OR 1.140, CI 0.497-2.613, P = .757). VPI rate decreased significantly over the course of the study (OR 0.880 per year, CI 0.824-0.940, P < .001). Veau class, age at primary repair, and syndromic status were not significant predictors of VPI (.128 ≤ P ≥ .975).

Conclusions: A soft palate fistula is a significant predictor for the development of VPI after primary palatoplasty. We propose that this is a result of decreased mobility of the palate caused by scarring from a soft palate fistula, which in addition, can impact the orientation of the velar muscles. Surgical intervention, at the time of fistula repair, to add vascularized tissue may be indicated to prophylactically decrease the risk of VPI.

(1) University of Michigan, Ann Arbor, MI

Background/Purpose: Although parameters of care have been developed for patients with craniosynostosis, imaging remains up to the discretion of the treating surgeon. To inform the development of best practices, data surrounding current practices must be obtained. Therefore, we sought to measure the frequency at which craniofacial surgeons order computed tomography (CT), as well as surgeons’ primary indications for CTs. We hypothesized that we would identify considerable variation in use of imaging, as well as the associated indications.

Methods/Description: After obtaining IRB exemption, we surveyed surgeons in the American Society of Maxillofacial Surgeons and the American Society of Craniofacial Surgeons to measure the frequency at which surgeons obtain pre- and postoperative head CTs, as well as indications. Initial items were piloted with 2 craniofacial surgeons and 1 neurosurgeon, utilizing interviews to elicit feedback and ensure content validity. Chi-square tests were used to measure associations between operative volume, years in practice, and imaging. Operative volume was dichotomized into high volume (>20 operations per year) and low volume (20 or fewer operations per year). Years in practice was similarly dichotomized into greater than 10 years of practice, and 10 or fewer years of practice.

Results: A total of 85 craniofacial surgeons responded (13.8% response rate), with the majority (63.5%) having performed a craniosynostosis operation in the last month. Only 9.4% of surgeons never order preoperative CTs. Of those who do, the most common indications included diagnosis confirmation (31.2%) and preoperative planning (27.3%). About a quarter (25.9%) of surgeons always obtain postoperative head CTs, the most common indication being evaluation of surgical outcomes (46.7%), with only 13.3% of respondents ordering 2 or more postoperative scans. Nearly half (47.1%) never obtain postoperative CTs. Surgeons reported the most significant influences on their imaging decisions were fellowship training (30.6%) and current colleagues (28.2%), more so than recommendations in the literature (23.5%). Higher operative volume was associated with a lower likelihood of ordering preoperative head CTs (P = .008), but years in practice was not associated with preoperative head CTs (P = .82). Neither operative volume (P = .61) nor years in practice (P = .17) were associated with postoperative head CTs.

Conclusions: The majority of surgeons obtain preoperative head CTs. Although nearly half never obtain postoperative head CTs, 25.9% always do so, usually to evaluate surgical outcomes. Given that surgical outcomes can be evaluated clinically, the use of postoperative CTs for the evaluation of outcomes is a poor use of resources and unnecessarily exposes children to radiation. Given the high variability in practice patterns, consensus guidelines are needed to create best practices and limit unnecessary studies.

(1) Johns Hopkins All Children’s Hospital, Saint Petersburg, FL, (2) University of South Florida, Tampa, FL, (3) University of South Florida Morsani College of Medicine, Tampa, FL

Background/Purpose: Surgical approaches to the craniofacial skeleton and corrective osteotomies of the facial bones are considered technically challenging and potentially morbid procedures in plastic surgery. However, they represent only a small component of training and many residents’ first exposure to these operations is in the operative suite. Surgical simulators permit trainees to learn the nuances of surgical procedures with full autonomy and increase patient safety. We aimed to create a 3-dimensional (3D)-printed craniofacial model for surgical training which could be potentially customized to represent common craniofacial pathology and/or patient-specific anatomy.

Methods/Description: Simulator models were designed utilizing Materialise Innovation Suite software consisting of Mimics 22.0, 3-Matic 14.0 (Materialise) and printed on a Stratasys J735 3D printer with SUP 706 support media, and Bone Matrix model material. Axial computer tomography data were segmented to render 3D models of relevant anatomy. Successive reviews with the craniofacial surgeon involved design of tissue zones of adhesion, assignment of Shore-A values and varying of tissue thicknesses to improve handling and authenticity. Development began with design of a simple calvarial segment with tissue planes representing skin/dura, periosteum, cranial bone, and support media filling the cranial void. Subsequent models have incorporated cranial sutures and advanced to a cranial/orbital model including the temporalis muscle, cranial sutures, supraorbital nerve/foramen, and zone of adherence at the temporal fusion line, orbital rim, and medial/lateral canthal attachments. Each model has been assessed by the craniofacial surgeon via appropriate surgical dissection for interval modification and improvement culminating in performance of a fronto-orbital on calvarial/orbital models.

Results: The current surgical simulator represents high anatomic fidelity for performance of a bicoronal incision, subgaleal or subperiosteal approach to the orbits, frontal craniotomy, and fronto-orbital osteotomies. To achieve adequate tissue flexibility to allow reflection of the bicoronal flap, simulated tissues must be thin and are prone to tearing. Additionally, the support media creates a large amount of debris that is distracting from the simulated experience. Tactile feedback from simulated burr hold placement, craniotomies, and endocranial dissection of the dura accurately represents true surgical procedures.

Conclusions: We have successfully created a 3D-printed surgical simulator using a Stratasys J735 3D printer for craniofacial dissections and osteotomies to help surgical trainees learn to perform these procedures independently. We intend to use newly available print materials, which may be printed with more variable Shore-A values to improve tissue handling and fidelity of the simulated surgical experience. A psychometric assessment of surgical trainees for educational validation of our simulator is anticipated.

(1) The Children’s Hospital of Philadelphia, PA

Background/Purpose: Depending on the technique with which posterior vault distraction osteogenesis (PVDO) is performed, some patients may develop a significant bony step-off deformity in the low occipital vault. In this study, we aim to identify risk factors for the development of an occipital step-off deformity and correlation with functional problems such as Chiari malformation.

Methods/Description: Patients who underwent PVDO for treatment of craniosynostosis between 2009 and 2019 were identified and inclusion required preoperative and postoperative imaging within 180 days of PVDO. Demographics and variations in surgical technique—height of osteotomy, performance of barrel stave osteotomies, lagging of barrel staves to transport segment—were recorded and analyzed using Materialize Mimics v21 (Materialise). Occipital step-off deformity was rated as absent, mild, moderate, or severe when viewed on 3D reconstructions in the lateral and PA planes independently by 2 craniofacial surgeons. Appropriate statistics were performed to identify factors associated with occipital step-off deformity and correlation with functional problems.

Results: One hundred ten subjects underwent PVDO at our institution during the study period and 22 met inclusion criteria. All patients were diagnosed with craniosynostosis, with multisuture synostosis being the most common phenotype (n = 17, 77%) and 6 (27%) had Chiari malformation, 1 (17%) of which was decompressed before PVDO. Average age at PVDO was 3.07 years (range: 3 months to 14 years). Sixteen (73%) patients had intraoperative barrel staves in the low occipital vault, of which 6 (38%) were lagged to the transport segment; 6 (27%) patients had osteotomies made below inion, and the remainder were above. Surgeons’ ratings were relatively consistent, and they rated 8 (36%) patients as having no step-off, 5.5 (25%) as mild, 6.5 (27%) as moderate, and 2 (9%) as severe. There was a significant negative correlation of osteotomy below inion and severity of occipital step-off rating (P = .031, r = −0.460, 95% CI: −3.64 to −0.189). Age at PVDO (P = .072, r = 0.391) and performing barrel stave osteotomies (P = .052, r = −0.420) trended toward significance. There was no significant correlation between severity of occipital step-off rating and AP distraction distance (P = .793, r = −0.059), barrel staves lagged to the transport segment (P = .183, r = −0.295), and presence of Chiari malformation at surgery (P = .824, r = 0.050). None of the patients with Chiari malformation had complete resolution following PVDO.

Conclusions: This is the first study to measure risk factors for occipital step-off deformity in patients undergoing PVDO, demonstrating an association of less occipital step-off deformity with height of the low transverse osteotomy made below the inion. It is possible that younger age and performance of barrel staves may also be protective, though further study is required.

(1) McGovern Medical School at The University of Texas Health Science Center at Houston (UTHealth), TX

Background/Purpose: Timing of repair for patients with craniosynostosis remains varied with some patients undergoing early “minimally invasive” strip craniotomy (SC), while those presenting later undergoing traditional cranial vault remolding (CVR). Recent studies have suggested improved outcomes and lower morbidity with earlier treatment. The impact that sociodemographic disparities have on time to presentation for craniosynostosis and treatment is unknown. Herein, we examined sociodemographic factors among a heterogenous patient population at a single institution that services a highly diverse population who underwent craniosynostosis repair and subsequently compared this cohort to a national database to examine differences in regional trends.

Methods/Description: A retrospective review of patients at UTHealth who underwent craniosynostosis repair from 2016 to 2020 was performed. Data were abstracted including: repair type, race, ethnicity, and age at time of surgery. The patients were divided into cohorts based on type of operation: SC or CVR. Next, the Kid Inpatient Database (KID) database was used to assess sociodemographic factors in relation to craniosynostosis. Univariate and multivariate logistic regression were used to determine predictors in differences in the age at time of craniosynostosis repair including race, ethnicity, median zip code, and income.

Results: Single institution (regional): 94 patients met inclusion criteria. Compared with non-Hispanic white (NHW) patients, Hispanic (OR 0.11, P = .005), and non-Hispanic black (NHB; OR 0.14, P < .001) had significantly lower odds of undergoing SC. Compared to those on private insurance, patients on Medicaid had significantly lower odds (OR 0.36, P = .039) of undergoing SC. Using zip code median income levels, patients with a higher median income level had significantly higher odds (P = .006) of undergoing SC compared to patients with a lower median income. KIDS (National): 2527 patients met inclusion criteria. Compared with NHW patients, NHB (OR 0.32, P < .001) and Asian patients (OR 0.47, P = .008) had significantly lower odds of undergoing repair before the age of 1. Compared to patients with private insurance, those with Medicaid (OR 0.67, P < .001) and self-pay (OR 0.58, P = .021) had significantly lower odds of undergoing repair before the age of 1. Patients in the lowest income quartile by zip code (OR 0.68, P = .002) and second lowest quartile (OR 0.71, P = .007) had significantly lower odds of undergoing repair before the age of 1 compared to the highest quartile.

Conclusions: Our findings indicate that sociodemographic factors may play a role in diagnosis of craniosynostosis and access to care. Patients of NHW race, lower income quartiles by zip code, and those who use public insurance are less likely to undergo early repair, both nationally and at our institution. Further research is needed to delineate the casualty of this disparity in presentation and timing of surgery.

(1) Division of Plastic Surgery, Emory University, Atlanta, GA, (2) Children’s Healthcare of Atlanta, GA, (3) Emory University School of Medicine, Atlanta, GA

Background/Purpose: Mandibular distraction osteogenesis is the preferred treatment at many centers for micrognathic patients with upper airway obstruction in the setting of Pierre Robin sequence. The timing of extubation following placement of mandibular distractors is variable across institutional algorithms. Postoperative maintenance of intubation allows for an improvement in both airway dimension and tongue positioning prior to extubation, theoretically decreasing the deleterious impact of acute postoperative airway edema. Prolonged intubation, however, has a well-described risk profile. We look at our institutional experience with neonatal mandibular distraction followed by immediate extubation to assess feasibility and safety profiles.

Methods/Description: A 4-year retrospective review of patients diagnosed with Pierre Robin sequence who underwent mandibular distraction within the first 3 months of life was performed. All patients were treated at a tertiary children’s hospital and had failed preoperative positioning and airway adjuncts. Patients intubated preoperatively were excluded. Analytic end points included patient demographics, comorbidities, pre- and postoperative respiratory support, rates of immediate extubation, need for reintubation, progression to tracheostomy, correlative polysomnography, direct laryngoscopic grade view, and functional nasoendoscopy. Fifty-two patients (29 males, 23 females) met inclusion criteria; 38 (73%) patients were extubated immediately in the operating room. In those who remained intubated (27%), the mean intubation interval was 7.2 days (range: 1-14 days). No significant differences between cohorts were found when comorbidities, syndromic status, cleft pathology, preoperative respiratory support, or grade of view on direct laryngoscopy were analyzed. Case duration >120 minutes and the subjective designation of a difficult airway by the anesthesiologist were associated with the decision to remain intubated (P < .05). Eight (21%) patients in the extubated group required an increase in respiratory support in the postoperative interval. Four (11%) of these patients required reintubation. Increased postoperative respiratory support was more likely in patients with other congenital anomalies, a syndromic diagnosis, cardiac anomalies, GERD, and in those who required greater than low-flow nasal cannula preoperatively (P < .05). Six patients in the cohort progressed to tracheostomy in long-term follow-up. There was one mortality which was remote and unrelated to surgical intervention. The mean follow-up interval was 18 months. Our data suggest that immediate extubation following neonatal mandibular distraction is feasible in patients who are not intubated preoperatively. Careful consideration should be given to patients who required significant respiratory support prior to distraction and in those with specific comorbidities, as they were more likely to necessitate an increase in postoperative respiratory support and reintubation.

(1) University of Pennsylvania, Philadelphia, PA, (2) Children’s Hospital of Philadelphia, PA

Background/Purpose: Linton A. Whitaker is a pioneer of craniofacial surgery. He served as chief of plastic surgery at the Children’s Hospital of Philadelphia and University of Pennsylvania and director of the craniofacial training program. Herein, the authors reflect on his legacy by studying the accomplishments of his trainees.

Methods/Description: Dr. Whitaker’s trainees were identified who completed (a) craniofacial fellowship training while he was director of the program or (b) residency training while he was chief. Curricula vitae were reviewed. Variables analyzed included geographic locations, practice types, academic leadership positions, scholarly work, and bibliometric data.

Results: Forty-five surgeons were included from 1980 to 2011: 34 completed craniofacial fellowship training under Dr. Whitaker, while 11 completed residency training under him as chief of plastic surgery at the University of Pennsylvania followed by cleft/craniofacial fellowship training. Mean time since completion of training was 22 ± 9 years. Overall, their mean number of publications was 76 ± 81 (range: 2-339), citations 1565 ± 1636 (3-6590), book chapters 23 ± 29 (0-135), and H-index 18 ± 12 (1-45). Eighteen individuals had grant data available, averaging 13 ± 16 career total grants (0-66) with mean total funding of $8 628 811 (0-$69 111 489). Of those who completed craniofacial fellowship training under Dr. Whitaker, 80% had academic positions following training, 77.4% had active craniofacial surgery practices, and 51.6% performed aesthetic surgery as well as reconstructive procedures. With regard to academic titles, 32.4% were residency program directors, 41.2% were chief/chair, and 55.9% served as directors of craniofacial programs. Overall, mean number of publications for this group was 69 ± 72 (range: 2-276), citations 1585 ± 1705 (36-6590), book chapters 25 ± 33 (0-135), books 2 ± 3 (0-7), H-index 18 ± 11 (2-45), patents 1 ± 1 (0-4), and grants 11 ± 12 (0-47) with mean total funding of $10 204 825 ($0-$69 111 489). On subgroup analysis of the trainees who pursued academia, the majority practiced cleft/craniofacial surgery (83.9%), while 41.9% also incorporated aesthetic surgery into their practice. More than half (53.1%) were promoted to full professor, 46.9% had program director roles, 75.0% directed craniofacial programs, 53.1% achieved the rank of chief/chair, 71.0% served on journal editorial boards, 54.8% had leadership roles in surgical societies, and 19.4% had NIH funding. Overall mean number of publications for this group was 94 ± 85 (range: 9-339), citations 1839 ± 1702 (67-6590), book chapters 24 ± 30 (0-135), books 2 ± 2 (0-7), H-index 20 ± 11 (3-45), patents 1 ± 1 (0-4), and grants 15 ± 16 (0-66) with mean total funding of $9 531 735 ($0-$69 111 489).

Conclusions: Equally important to Dr. Whitaker’s clinical contributions in plastic and craniofacial surgery is the development and success of his trainees who will undoubtedly continue the legacy of training the next generation of craniofacial surgeon leaders.

(1) The Children’s Hospital of Philadelphia, PA, (2) The University of Pennsylvania, Philadelphia, PA

Background/Purpose: Mandibular distraction osteogenesis (MDO) is a commonly performed treatment for Robin sequence (RS) and syndromes involving micro/retrognathia and upper airway obstruction. There is a paucity of midterm data regarding the postoperative outcomes and complications of infants treated with MDO. We analyzed comprehensive dental, neurologic, and facial functional outcomes of previously distracted patients during the period of mixed dentition.

Methods/Description: Patients who underwent MDO between 2008 and 2019 were prospectively evaluated with minimum 4-year follow-up. Perioperative characteristics were retrospectively abstracted. Abstracted data included demographics, respiratory status, dental outcomes, nerve function (motor and sensory), postsurgical scarring, and TMJ function as measured by maximal incisal opening (MIO).

Results: In total, 110 patients met inclusion criteria. We present preliminary results of 15 patients: Twelve (80%) were syndromic; 5 Treacher Collins (41.7%), 2 Goldenhar (16.7%), 4 undescribed (33.3%), and 1 Cat eye syndrome (8.3%). Average patient age at distractor placement was 30.6 months (range: 0.3-79.2) for syndromic and 1.1 months (0.4-1.7, P ≤ .009) for nonsyndromic. The average age at follow-up visit for syndromic patients was 10.5 years (6.0-16.6) and 7.3 years (6.5-8.6, P ≤ .026) for nonsyndromic. Syndromic patients on average had 2 (0-7) number of absent teeth, defined as agenesis or extraction of deciduous and permanent dentition, compared to 1 (0-2, P ≤ .292) for nonsyndromic. Four (33.3%) patients, all syndromic, underwent MDO more than once. Subgroup analysis showed higher likelihood of dental absence for those that had multiple MDO procedure (mean 3.75) compared to MDO once (1.18; P ≤ .074). Maximum incisal opening did not differ between syndromic status nor for the number of times distracted, P > .5. The average MIO for those with permanent first or second molar injury was on average decreased (28, 25 mm) compared to those without injury (36 mm; P ≤ .027). Marginal mandibular nerve impairment was significantly associated with permanent first or second molar injury compared to those without injury, P ≤ .033. Inferior alveolar nerve impairment was associated with permanent first molar injury compared to those without injury, P ≤ .066. Marginal mandibular nerve function and inferior alveolar nerve function did not differ between syndromic status nor for the number of times distracted, P > .5. Preliminary results are limited by contrasting deciduous dentition among nonsyndromic patients and permanent dentition among syndromic patients.

Conclusions: In the period of mixed dentition, patients exhibit common findings of first or second dental molar absence. Among nonsyndromic patients, this finding applies to deciduous teeth, and among syndromic patients who have been followed to an older age, it applies to permanent teeth, and appears associated with repeat MDO procedures. We plan to present a significantly expanded cohort analysis if selected.

(1) Division of Plastic Surgery, Children’s Hospital of Philadelphia, PA, (2) Division of Plastic Surgery, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, (3) University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, (4) Department of Radiology, Children’s Hospital of Philadelphia, PA

Background/Purpose: Patients presenting with fusion of cranial-base sutures present a clinical conundrum, because it is often unclear when these sutures “normally” close. This study comprehensively investigates the physiologic timeline of cranial-base suture fusion.

Methods/Description: We retrospectively analyzed head computed tomography (CT) scans of subjects aged 0 to 18 years from an institutional database of normocephalic patients. Subjects were stratified into 23 age intervals with 10 subjects per interval. Eight cranial-base sutures (frontoethmoidal [FrEt], frontosphenoidal [FrSp], sphenoparietal [SpPa], sphenosquamosal [SpSq], sphenopetrosal [SpPe], parietosquamosal [PaSq], and parietomastoid [PaMa], and occipitomastoid [OcMa]) and 5 synchondroses (sphenoethmoidal [SpEt], spheno-occipital [SpOc], petro-occipital [PeOc], anterior intraoccipital [AIO], and posterior intraoccipital [PIO]) were assessed for patency bilaterally in each scan. The “midpoint” of suture fusion was achieved when incomplete suture closure was detected in ≥50% of subjects but complete bilateral closure did not exceed 50% of subjects. Exploratory factor analysis was performed to identify the latent structure underlying the fusion timelines of the 13 sutures and synchondroses.

Results: A total of 230 subjects were analyzed. Order of fusion of the 8 cranial-base sutures progressed in the anterior-to-posterior direction. The fusion midpoint and completion occurred at 0 to 2 months and 4 years for FrEt, 6 to 8 months and 12 years for FrSp, and 6 to 8 months and 4 years for SpPa, respectively. SpSq, SpPe, PaSq, and PaMa reached their respective fusion midpoints at 6 to 8 months, 8 years, 9 to 11 months, and 12 years; these sutures remained patent in ≥50% of subjects at 18 years. OcMa was partially fused in up to 30% of subjects by 18 years and completely fused in no subject. In contrast, the latency of closure of the 5 synchondroses was not associated with location along the anteroposterior axis. The fusion midpoint and completion age were 3 to 5 months and 5 years for SpEt, 9 years and 17 years for SpOc, 4 years and 10 years for AIO, and 18 to 23 months and 4 years for PIO, respectively. PeOc reached the fusion midpoint at 11 years; however, bilateral complete fusion never exceeded 50% of subjects. Exploratory factor analysis suggested the following 3 fusion patterns, which explained 60% of the variance in the fusion timelines: (1) FrEt, FrSp, SpPa, SpSq, SpEt, AIO, and PIO (R ² = 0.96); (2) SpPe, PaMa, OcMa, PeOc, and SpOc (R ² = 0.85); and (3) PaSq (R ² = 0.86).

Conclusions: Cranial-base sutures and synchondroses showed characteristic timelines of fusion during childhood. These findings may help surgeons and radiologists interpret CT data of patients with head shape abnormalities. More posteriorly located cranial-base sutures tended to close later; however, synchondroses did not follow this trend. Future work should further elucidate the mechanisms and clinical sequelae of cranial-base suture fusion that deviates from the “normal” timelines.

(1) University of Alabama at Birmingham School of Medicine, AL, (2) New York University, New York City, NY, (3) University of Alabama at Birmingham, AL

Background/Purpose: A variety of studies have independently investigated primary calvarial reconstruction, but few have examined high-risk complex cranial defects. Up to 33% of patients who undergo reconstruction have hostile preexisting conditions with coexisting soft tissue and osseous defects due to history of radiation, prior failed cranioplasty, or concurrent infections. Although studies have evaluated autologous reconstruction for hostile craniums, there is a paucity of studies comparing alloplastic and autologous reconstruction for hostile defects. Our objective was to retrospectively evaluate the use of alloplastic reconstruction alongside autologous reconstruction for hostile cranial defects.

Methods/Description: An IRB-approved retrospective review of patients who underwent cranioplasty of a hostile site at a southeastern tertiary care center between January 2008 and December 2018 was performed. The patients were stratified into 3 groups based on the type of implant used: autogenous (bone), alloplastic (PEEK, Titanium, PMMA), or mixed (combination of both types of graft). The primary outcome metric was a complication in the year following cranioplasty, identified by flap or bone graft failure, necrosis, or infection. Statistical analysis included t tests and χ ² tests where appropriate using SPSS.

Results: There were 43 total cases in this time period; 15 autogenous, 23 alloplastic, and 5 mixed. The purely autogenous group had the highest complication rate (85%) and the alloplastic group had the lowest complication rate (38%). When stratified by specific material used for reconstruction (15 bone, 14 PEEK, 10 titanium, and 5 PMMA), overall complication rate was statistically significant (P = .009; χ ² test) with PEEK implants having the lowest complication rate (21%). The analysis documented an overall complication rate that was statistically different between the 3 groups (P = .012).

Conclusions: This analysis interestingly found that in the setting of hostile cranial defects, cranioplasties would benefit from the use of prosthetic implants instead of autologous bone grafts, not only for avoidance of donor site morbidity but also for decrease in overall complications.

(1) Craig R Dufresne, MD, PC, Fairfax, VA

Background/Purpose: Freeman-Burian syndrome (FBS), formerly Freeman-Sheldon syndrome, is a rare congenital complex myopathic craniofacial syndrome that frequently involves extremity joint deformities, abnormal spinal curvatures, and chest wall mechanical problems that, together with spinal deformities, impair pulmonary function. Although potentially disabling, proper treatment can considerably improve patient outcomes, but clinical guidance does not exist, accurate up-to-date information limited, and clinical understanding of the syndrome at a professional and lay level is guided mostly by outdated literature. The lack of available clinical guidance is known clinically to impair care and outcomes. To improve care and patient outcomes, we developed a clinical practice guideline.

Methods/Description: From 2015 to 2018, we conducted 2 systematic reviews that included a meta-analysis of individual patient data. From 2006 to 2018, we also convened discussions to arrive at expert consensus among 10 experienced multidisciplinary participants, which included family and patient representatives. No formal deliberative procedure was used.

Results: Unsurprisingly, there was a great paucity of literature on FBS, and much of what was existent was of poor quality or not current. The resulting recommendations are heavily based on the clinical consensus of the participants, informed by appropriate literature. directed toward health status, and quality of life improvement for affected individuals. Primary goals in evaluation and management of FBS must be the (1) betterment of quality of life by mitigating deformities’ burden and (2) reducing respiratory-related morbidity and mortality. Goals are best achieved with a combination of limited surgical procedures and an emphasis on therapeutic and maintenance physiotherapy, guided by a craniofacial team.

Conclusions: In FBS, craniofacial muscles may be preferentially impacted by fibrous tissue replacement. The lack of available objective data should not reduce clinical vigilance to the possibility that fibrous tissue replacement may influence almost any aspect of the patient’s presentation, thus necessitating nonstandard treatment deviations. Based on our decades of experience with this challenging patient population, we feel much can be done to afford FBS patients a good and productive quality of life through exquisite medical surveillance, rapid intervention in acute upper respiratory disturbances, conservative operative intervention, and longitudinal lifestyle structuring by the patients.

(1) Children’s Hospital of Philadelphia, PA, (2) Children’s Hospital of Philadelphia, University of Pennsylvania, PA

Background/Purpose: Due to advances in 3D and 4D ultrasonography, it is possible to detect CF anomalies at 10 weeks of gestation as the facial bones begin to ossify. Rates of prenatally diagnosed craniofacial anomalies differ by region and country partially due to screening policies and level of technician expertise. Isolated craniosynostosis is a particular diagnostic challenge. The purpose of this study was to identify the diagnostic accuracy of ultrasound and MRI for various craniofacial anomalies at our tertiary care center associated with a high volume fetal diagnostic unit.

Methods/Description: Our institutional programmatic fetal imaging database was queried to identify patients with suspected craniofacial conditions from January of 2002 through August of 2019. Parental and demographic data, prenatal imaging, postnatal examination findings, and outcomes (delivery, termination, fetal demise, infant demise) were obtained. Sensitivity, specificity, positive predictive value, and negative predictive value of prenatal diagnoses were calculated using postnatal clinical examination as the gold standard.

Results: Of the 73 parent/fetus dyads identified, 43 fetuses met all inclusion criteria. The mean maternal age at consultation was 32.8 ± 5 years (range: 22.4-41.3), mean gestational age 26w ± 4w6d (range: 19w-36w5d). 70% (30) of patients were prenatally suspected to have craniosynostosis, 14% (6) micrognathia, 7.0% (3) Binder syndrome, and 9.3% (4) a variety of other conditions; 69.7% (30) of patients received fetal ultrasound and fetal MRI, 23.3% (10) received fetal United States only, and 7.0% of patients (3) received fetal MRI alone. Seven fetuses were terminated (craniosynostosis n = 6, microophthalmos n = 1) and 4 infants with multiple congenital anomalies passed away in infancy. For the diagnosis of any craniofacial anomaly, ultrasound: sensitivity 90%, specificity 43%, PPV 82%, NPV 60%; MRI: sensitivity 86%, specificity 50%, PPV 86%, NPV 50%. For craniosynostosis specifically, ultrasound: sensitivity 100%, specificity 43%, PPV 71%, NPV 100%; MRI: sensitivity 100%; specificity 50%; PPV 82%, NPV 100%. Ultrasound had a sensitivity and PPV of 100% for both micrognathia and Binder syndrome. There were 4 false-positive diagnoses of isolated craniosynostosis on prenatal ultrasound that were found to be overriding sutures without synostosis or normal head shape variants on postnatal examination. In the setting of syndromic craniosynostosis, careful attention was paid to associated anomalies (ie, hands and feet in Apert syndrome) to support the diagnosis.

Conclusions: Although CF anomalies can be detected as early as 10 weeks gestation, most anomalies are diagnosed in the second trimester after the fetal anatomy scan. Finding ways to maximize diagnostic accuracy is paramount given the profound consequences of parental decision-making subsequent to diagnosis. Additionally, it is essential to communicate the degree of doubt associated with each prenatal diagnosis, especially in the setting of isolated anomalies.

(1) Children’s Hospital of Pittsburgh, PA, (2) University of Pittsburgh, School of Medicine, PA, (3) Department of Plastic Surgery, University of Pittsburgh Medical Center, PA

Background/Purpose: Dynamics of structural fat graft (SFG) growth during adolescence is unknown. Adolescent growth is characterized by rapid changes in weight and body habitus. Many challenges and confounders exist inherent to studying SFG outcomes in children, including need for long-term objective follow-up, grafting technique, various donor sites, unique recipient sites, disease severity/progression, absence of adequate experimental controls, and normal facial growth. To circumvent these challenges, we designed and implemented a longitudinal, split-face analysis using serial 3-dimensional photogrammetric images to quantitatively assess facial growth after hemifacial fat transfer.

Methods/Description: Using serial 3-dimensional photogrammetric images, we performed a split-face analysis of 8 patients with linear scleroderma after hemifacial fat grafting. At least 2 photogrammetric scans separated by at least 12 months with the first scan captured no earlier than 4 months following surgery. Anthropometric measurements, recorded at the time of each image acquisition, were used to determine growth over discrete time intervals. We performed repeated quantitative volumetric measurements of the grafted hemiface (treatment group) and the contralateral hemiface (control group). Volumetric changes were measured relative to weight change over each interval.

Results: Patients were followed longitudinally after hemifacial SFG on average for 41.0 months. Twenty-3 discrete postoperative time intervals were analyzed (2.9 intervals per patient; mean interval time = 14.6 months). Control and grafted facial soft tissues changed as a function of weight, weight velocity, and triponderal mass index (TMI, an index to estimate body fat levels in adolescents). TMI strongly correlated with volumetric changes of grafted hemifaces and control hemifaces (P < .0001 and .0004, respectively). Compared to the control side, grafted hemifaces were more sensitive to interval TMI changes (P < .0001). As TMI increased over an interval, facial growth was significantly greater on the grafted side. Conversely, as TMI decreased over an interval, the decrease in facial volume was significantly greater for the grafted side compared to the control side. There was a significant interaction between fat graft volume and TMI on grafted tissue response in a dose-dependent manner (P = .002).

Conclusions: Using a longitudinal, quantitative, split-face-controlled analysis with high-resolution surface imaging, we found that structural fat grafts are dynamic tissues that are hyperresponsive to changes in body fat levels. Compared to the control side, grafted tissues were not only more sensitive to increasing body fat levels but facial volume also decreased more substantially with loss of body fat. There is a predictable relationship between adiposity changes and graft response, which is augmented by fat graft volume in a dose-dependent manner.

(1) Université Gustave Eiffel, Marseille, France, (2) Lapeyronie Hospital, Montpellier, France, (3) YooMed, Montpellier, France, (4) APHM, Marseille, France

Background/Purpose: Mandibular hypoplasia is an anatomic abnormality of the facial complex that could affect approximately 1/5600 to 1/8000 births per year. Because of anatomical connections between the mandible, the tongue and the superior airways, this pathology may be associated to feeding and/or respiratory troubles. Thus, the early management of the pathology is crucial to relieve this airway obstruction. These therapeutic strategies vary from functional therapies as prone positioning, nasopharyngeal tube or continuous positive airway pressure to surgical options as glossopexy (or tongue-lip adhesion), tracheotomy, or mandibular osteodistraction. However, the success rates of these therapeutic strategies are variable and some of them may imply some potential risks. The aim of this study was to improve our understanding about how the hypoplasic and healthy mandibles are different so the pathology could be, eventually, identified and managed the earliest and the most efficiently as possible.

Methods/Description: A morphometric method was applied on 3D mandibular models of children aged from 39 gestational weeks to 7 years old. The study sample was composed of 31 hypoplasic children and as many sex- and age-matched healthy children.

Results: Morphological distinctions between pathological and healthy mandibles were highlighted only from the first year of life. The same morphological evolution was observed for the hypoplasic and healthy mandibles, correlated with the tooth development stages. Bilateral and unilateral mandibular hypoplasia were also distinguished. Supervised classification trees were built to identify the pathology and discriminate unilateral from bilateral mandibular hypoplasia (prediction rates = 81% and 84%, respectively).

Conclusions: Our results suggest that the pathology significantly impact the mandibular morphology only when the first deciduous teeth begin to appear, without affecting its global growth pattern. Thus, the relevance of surgical intervention to correct the mandibular morphology from the very first months of life may be questioned.

(1) University of Pittsburgh, PA, (2) University of Pittsburgh School of Medicine, PA, (3) Children’s Hospital of Pittsburgh, PA

Background/Purpose: Optimizing operating room (OR) utilization is a critical component of health care system efficiency. OR workflow performance may be evaluated by metrics such as turnover time, start time tardiness, utilization of operative staff/supplies, block time allotment, and rate of case cancellation. A few international institutions have sought to analyze OR cancellation in places that cater to the adult patient. However, there is a paucity of literature on cancellation among the pediatric plastic surgery population. Therefore, the purpose of our study was to analyze the extent of OR cancellation over a span of one year and its effect on raw utilization of OR block time allotted to surgeons in the pediatric plastic surgery department of a children’s hospital.

Methods/Description: We retrospectively reviewed the cases of 4 plastic surgeons at a tertiary pediatric hospital between 2018 and 2019. Data collected included patient demographics, type of surgery, time of cancellation, reason for cancellation (preoperative clearance fail, “no-show,” illness, physician reason, and other), length of surgery, and minutes of block time allotted to each surgeon per year. Percent of cases cancelled, scheduled times lost, and block times lost in one year were calculated.

Results: Surgeons A, B, C, and D scheduled 170, 416, 305, and 474 cases, respectively. Among all 4 surgeons, 28.2% of cases were cancelled, 37.2% of scheduled time was lost, and a total of 21.3% of block time was lost due to cancellation over the span of one year. Patients of surgeon A and D were more likely to cancel due to reasons classified as “other,” including causes such as failure of NPO or a family’s last-minute decision to forego elective surgery (33.3% and 37.2%, respectively), whereas patients of surgeon B and C were more likely to cancel due to illness (67.9% and 36.4%, respectively). Surgeons A, B, and D experienced significant day-of-surgery cancellation (33%, 29%, 28%, respectively). Notably, surgeon D had the highest rate of case cancellation overall (9.1%) and was the most likely of the 4 surgeons to perform procedures under local sedation without requiring general anesthesia.

Conclusions: Operating room utilization and cancellation is largely understudied, especially among pediatric cohorts. There have been no studies conducted on the effectiveness of OR utilization and cancellation specifically within the scope of pediatric plastic surgery. We ultimately believe that most of our patients’ reasons for cancellation, even those classified as “illness,” are to some extent avoidable. Implementation of patient education, close follow-up, and the inclusion of the patient in discussions regarding logistics of surgery have the power to drastically decrease OR cancellation rates in the pediatric plastic surgery population.

(1) The Children’s Hospital of Philadelphia, PA

Background/Purpose: Posterior vault distraction osteogenesis (PVDO) has gained acceptance as a part of the armament for treatment of craniosynostosis, but the role of PVDO in nonsynostotic (non-CS) diagnoses has not been published. The purpose of this study was to compare perioperative safety and efficacy of PVDO in patients with nonsynostotic cephalocranial disproportion, namely slit ventricle syndrome and idiopathic intracranial hypertension (IIH) (also known as pseudotumor cerebrii), to a “control” cohort of patients with craniosynostosis.

Methods/Description: A retrospective case-controlled study of patients undergoing PVDO from 2009 to 2019 at our institution was performed, and controls were matched by sex and age at PVDO. Operative details, perioperative outcomes, and distraction patterns were analyzed with appropriate statistics.

Results: One hundred nineteen subjects underwent PVDO during the study interval, of which 9 patients met inclusion criteria for the non-CS cohort. In the non-CS cohort, 5 (56%) patients were male, 6 (67%) were Caucasian, and 4 (45%) carried genetic diagnoses. Six (67%) patients underwent PVDO for slit ventricle and the remaining 3 (33%) patients underwent PVDO for IIH. Five (56%) patients were found to have Chiari malformation and 7 (78%) patients had VP shunts. The majority of controls were syndromic (n = 6, 67%) and had multisuture synostosis (n = 7, 78%). The non-CS cohort underwent PVDO at a median 56.1 months old (Q1 41.1, Q3 86.6) versus the control cohort at 55.7 months (Q1 39.6, Q3 76.0; P = .931). The non-CS cohort had a median operative time of 168 minutes (Q1 113, Q3 216) versus the control cohort at 152 minutes (Q1 132, Q3 245; P = .387). The non-CS cohort had median percent total blood volume loss at 17.4% (Q1 10.5, Q3 34.8) versus the control cohort at 25.5% (Q1 12.8, Q3 35.3; P = .340). All 9 subjects in the non-CS cohort were distracted in a vector along Frankfurt horizontal (100%) for a median distance 27 mm (Q1 25, Q3 31) versus the control cohort at 31 mm (Q1 26, Q3 35; P = .258). The non-CS cohort spent a median of 2 days (Q1 1, Q3 5) versus the control cohort at 1 day (Q1 1 Q3 2; P = .174) in the ICU. Total length of stay was significantly longer in the non-CS cohort (median days 5 [Q1 4, Q3 5] vs median days 3 [Q1 3, Q3 4], P = .031). Two non-CS patient required shunt externalization within 30 days of surgery. One of 6 slit ventricle patients required shunt manipulations and a repeat PVDO within 1 years of PVDO, while the others did not require manipulation 2.5 years post-PVDO for intracranial pressure concerns. Two of 3 IIH patients are symptom-free while 1 patient is treated medically for occasional headaches.

Conclusions: In this small, case-controlled pilot study of the utility of PVDO in treating slit ventricle syndrome and IIH, safety appears similar to PVDO in the synostotic setting. The dataset lacks adequate follow-up to assess efficacy, although short-term follow-up demonstrates promising results with regard to need for shunt revision and symptomatic headache relief.

(1) Instituto Sírio-Libanês de Ensino e Pesquisa, Instituto de Responsabilidade Social Sírio-Libanês, São Paulo, Brazil, (2) Hospital Sírio-Libanês, São Paulo, Brazil

Background/Purpose: Although first reports of the clinical use of adipose-derived stromal cells (ADSC) suggest that this approach may be feasible and effective for soft tissue augmentation, there is a lack of randomized, controlled clinical trials in the literature. Hence, this study aimed to investigate whether a faster protocol for isolation of ADSC and their use in combination with fat tissue improve the long-term retention of the grafts in pediatric patients with craniofacial microsomia.

Methods/Description: Patients with craniofacial microsomia (n = 30) were grafted with either supplementation of ADSC (experimental group) or without supplementation of ADSC (control group). Number of viable cells isolated before and after the supplementation of the grafts was calculated, and these cells were examined for mesenchymal cell surface markers using flow cytometry. 3D photogrammetry was performed to assess both hemifaces preoperatively and at 1 week, 2 weeks, 1 month, 3 months, 6 months, and 12 months postoperatively. Cost-effectiveness analysis was performed and the Youth Quality of Life—Facial Differences (YQOL-FD) was applied at both preoperative and 12 months postoperative period.

Results: Average number of viable cells isolated before and after the supplementation of the grafts was 4.8 × 106 and 8.1 × 106 cells/mL of fat tissue (P = .015). Flow cytometry analysis revealed that the ADSC were positive for mesenchymal cell markers (>95% for CD 73 and CD 105). For experimental group, surviving fat volume at 6 months was significant higher than for control group (P = .003).

Conclusions: These results suggest that this strategy for isolation and supplementation of ADSC is effective, safe and superior to conventional lipoinjection for facial recontouring in patients with craniofacial microsomia. Clinical question/Level of evidence: Therapeutic, II

(1) Yale University School of Medicine, New haven, CT, (2) Mayo Clinic Florida, Jacksonville, FL, (3) Department of Plastic and Reconstructive Surgery, Yale University School of Medicine, New Haven, CT, (4) Department of Craniofacial Surgery, Hospital for Rehabilitation of Craniofacial Anomalies, University, Bauru, São Paulo, Brazil (5) Department of Plastic Surgery, University of São Paulo, Brazil

Background/Purpose: Visual impairment secondary to orbital and periorbital dysmorphology is frequent in Pfeiffer syndrome patients. The etiopathogenesis of this aberrancy, however, remains unclear.

Methods/Description: Untreated Pfeiffer syndrome patients (n = 31) and normal controls (n = 43) were compared. Craniometric and volumetric analyses related to the orbit and periorbital anatomy were performed using Materialise software.

Results: Overall, orbital cavity volume of Pfeiffer patients is reduced by 28% (P < .001), compared to normal, starting before 3 months of age (P = .004). Globe volume diminished by 10% (P = .011) before 3 months of age, yet caught up to normal thereafter. However, the retrobulbar soft tissue volume remained smaller after 1 year of age (17%, P = .002). Globe volume projection outside of the orbit increased in all observed ages (82%, P < .001). The volume of sphenoid bone, maxilla and mandible proportionately were restricted by 24-25% (P = .005-.036) before 3 months of age. The volume of maxilla and mandible gradually approximated normal; however, the sphenoid bone volume in Pfeiffer patients remained less than normal (P < .001) into childhood. The anteroposterior length of both the zygoma and maxilla was reduced by 14% (P < .001). The anterior positioning of the zygoma is shorter by 23% (P < .001) in Pfeiffer patients overall, while the anterior positioning of maxilla is reduced by 23% (P < .001).

Conclusions: Pfeiffer syndrome patients develop decreased retrobulbar soft tissue and globe volume, along with a restricted orbital cavity volume in infancy. Significant hypoplasia of the sphenoid bone results in a more severe central facial retrusion (maxilla), compared to lateral facial structures (zygoma).

(1) 3D Craniofacial Image Research Laboratory, University of Copenhagen, Denmark, (2) Department of Oral and Maxillofacial Surgery, 3D Craniofacial Image Research Laboratory, RH, Copenhagen, Denmark, (3) Department of Pediatrics, Center for Rare Diseases, Copenhagen University Hospital, RH, Copenhagen, Denmark, (4) Department of Plastic Surgery, Cleft Palate and Craniofacial Center, Helsinki University Hospital, Helsinki, Finland, (5) Department of Clinical Genetics, Center for Rare Diseases, Copenhagen University Hospital, RH, Copenhagen, Denmark, (6) 3D Laboratory and Department of Pediatric Dentistry and Clinical Genetics, University of Copenhagen, Denmark

Background/Purpose: The purpose of this study was to quantify and compare differences in facial asymmetry (FA) in children with nonsyndromic unicoronal synostosis (NS-UCS) and Muenke syndrome associated unicoronal synostosis (MS-UCS). It was hypothesized that children with MS-UCS present with significantly more severe FA compared to children with NS-UCS.

Methods/Description: The sample consisted of 20 children with UCS (7 MS-UCS and 13 NS-UCS) acquired from the primary care centers in Copenhagen and Aarhus, Denmark (N = 14) and Helsinki, Finland (N = 6). Data consisted of CT scans taken preoperatively (mean age 0.6 years; range: 0.1-1.4 years) from which soft tissue surfaces were constructed. Twenty-two soft tissue landmarks were placed manually by 2 independent examiners who were both blinded to the results of each patient’s genetic testing. Guided by the 22 landmarks, a symmetric facial template was deformed to match each patient’s surface, obtaining spatially detailed left-right point correspondence in all patient surfaces. FA was defined as a vector A between a point on one side of the midsagittal plane (MSP) and the corresponding point on the other side after mirroring across the MSP. The length of A provided the total amount of asymmetry (in mm) while it’s Cartesian components provided asymmetry in the transverse, vertical, and sagittal directions, respectively. Mean FA values were calculated for the full-face region and for 6 subregions: forehead, eye, nose, cheek, mouth, and chin. Comparisons of means were carried out using Mann-Whitney U test (level of significance: 5%).

Results: The nature of the facial asymmetry was similar in the 2 groups, but the magnitude of asymmetry was significantly greater in the MS-UCS group than in the NS-UCS group for all regions studied. For the full-face region, a significant difference between means of 2.3 mm was calculated between the 2 groups and the largest difference was documented in the transverse direction (1.7 mm). Region-wise, the largest significant difference between means was seen in the forehead (3.2 mm) region, followed by the chin (2.9 mm), eye (2.2 mm), cheek (2.0 mm), nose (1.7 mm), and mouth (1.3 mm) regions. The transverse direction presented with the largest significant difference between means in the following regions: forehead (2.3 mm), chin (3.9 mm), nose (1.8 mm), and mouth (1.3 mm). In the eye and cheek regions, the largest significant difference was found in the sagittal direction (1.4 mm and 1.8 mm, respectively).

Conclusions: The hypothesis, that children with MS-UCS presented with significantly larger FA in all studied regions compared to children with NS-UCS, could not be refuted. The largest significant difference between means was found in the forehead and chin regions, where the transverse direction presented with the largest significant difference.

(1) Spectrum Health/Michigan State University, Grand Rapids, MI, (2) Helen DeVos Children’s Hospital, Grand Rapids, MI

Background/Purpose: Von Willebrand disease (VWD) is one of the most common inherited bleeding disorders. The disorder is autosomal dominant and occurs due to an absent or defective von Willebrand factor (VWF), an adhesive glycoprotein. VWF binds to platelets and sites of vascular injury to aid in platelet adhesion and also assists in fibrin formation through the reduction of factor VIII inactivation. VWD often presents as excessive bleeding after trauma or surgery. In order to minimize bleeding and reduce transfusion requirements during highly invasive surgical procedures, such as craniosynostosis repair, preoperative identification of these patients can be helpful.

Methods/Description: Prior to undergoing operative repair of craniosynostosis, patients were screened for von Willebrand disease as a part of the pediatric blood management protocol. The laboratory screening tests used were von Willebrand factor antigen, ristocetin cofactor, and von Willebrand factor multimers. Those who tested positive for von Willebrand disease were evaluated by a hematologist and the pediatric blood management team. These patients received Humate-p immediately preoperatively and tranexamic acid intraoperatively. Postoperatively, Amicar was given and ristocetin cofactor levels were checked. Humate-p was given for low ristocetic cofactor levels.

Results: Of the 100 patients screened from 2014 to the present, 14 patients tested positive for von Willebrand disease. At 14%, this is significantly higher than the up to 1% prevalence in the general population. All of these patients were found to be type 1, the mildest and most common form of the disorder.

Conclusions: Identification of patients with von Willebrand disease prior to cranial vault surgery allows for appropriate preoperative, intraoperative, and postoperative measures to be taken to minimize blood loss. By following our team protocol, these patients did not require an increased transfusion rate. Given the high prevalence of VWD found in this group of patients with craniosynostosis, further studies are needed to investigate a potential genetic link between von Willebrand disease and craniosynostosis.

(1) Washington University, Saint Louis, MO, (2) Washington University in St. Louis School of Medicine, MO, (3) St. Louis Children’s Hospital/Washington University in St. Louis, MO

Background/Purpose: The reconstructive goals in sagittal craniosynostosis are to relieve growth restriction and ameliorate biparietal narrowing and frontooccipital bossing. Repair is more challenging in older patients due to thicker bone and decreased growth potential requiring more extensive remodeling. The aim of this study is to assess the safety and efficacy of different open repair techniques in patients older than 1 year.

Methods/Description: The multi-institution Synostosis Research Group database (SynRG) was queried for open repairs of nonsyndromic sagittal craniosynostosis from 2017 to 2019 and supplemented with retrospective chart review at a single participating institution between 2006 and 2016 (total N = 42). Inclusion criteria required primary calvarial vault remodeling (CVR) performed after 12 months of age. Length of stay (LOS), estimated blood loss (EBL), transfusion rates, operating room (OR) times, cephalic index (CI), and complications were reviewed.

Results: Patients’ mean age (±SD) at surgery was 28.7 ± 16.7 months. Fourteen were treated by barrel stave osteotomy, 14 by barrel staves extending to the posterior vault, 7 by clamshell osteotomy, and 7 by parietal craniotomy. As a cohort, mean OR time was 249 ± 66 minutes; EBL was 326 ± 191 mL; and LOS was 3.3 ± 0.9 days. Thirty-five (83%) patients required intraoperative transfusions. There were no readmissions, complications, additional surgery, or mortality within the first 30 postoperative days. At an average follow-up time of 387 days, cranial index was improved by 7.3, from 67.0 ± 4.3 to 74.4 ± 4.4. Postoperative CI for the majority of patients (57%) remained below normal range (75-80).

Conclusions: Calvarial vault reconstruction is a safe method to treat sagittal craniosynostosis in older children, but later surgical intervention after the age of one often results in suboptimal correction of CI. Although EBL and OR time appear to be greater than values reported for earlier repair, there was no increase in readmissions, complications, or need for secondary repair in this multi-institutional cohort with repairs after 1 year of age. Additional investigation is needed to characterize effective strategies in treating older patients.

(1) University of Texas Southwestern Medical Center, Dallas, TX, (2) University of Texas Southwestern, Dallas, TX

Background/Purpose: Alloplastic implants such as porous polyethylene (MEDPOR) for external ear reconstruction are an alternative to traditional autologous techniques. The benefits of porous polyethylene include technical ease of construct formation, high tensile strength, lack of donor site, rapid tissue ingrowth, and lack of resorption and degeneration. In this study, we present a comprehensive review of patient outcomes and revisions to evaluate the safety and efficacy of the use of porous polyethylene for external ear reconstruction.

Methods/Description: We performed a retrospective chart review of all patients undergoing external ear reconstruction with porous polyethylene (MEDPOR) implants at Children’s Health Medical Center between 2012 and 2019. Patients were identified using an electronic medical record search. Chart review was performed to extract biographical information, operative surgical information, and postoperative revisions and complications.

Results: We identified 52 patients who underwent ear reconstruction with porous polyethylene (MEDPOR) implant by the senior author (CAD). Twenty-eight patients had isolated microtia, 18 had craniofacial microsomia, and 3 were due to trauma/revisions. Thirty were males and 20 were females, with 22 left-sided and 28 right-sided ears. Thirty-eight were Hispanic, 9 Caucasian, 2 African American, and 1 Asian. Marx classification was 1 class I, 15 class II, 26 class III, and 3 class IV. The average age at surgery was 5.82 years. Average operative time was 1.59 hours, which improved with surgeon experience. Average length of stay was one postoperative day. The first follow-up was an average of 5.61 days. Thirty-two patients (61.54%) underwent one or more revision following reconstruction. Twenty-two (42.31%) patients for complications, 10 (19.23%) patients for aesthetics, and 9 (17.31%) patients for elevation. Isolated microtia had the highest rates of revisions for aesthetics (21.43%) and for repositioning (21.43%), while craniofacial microsomia had the highest rate of revision for therapeutics (45.00%).

Conclusions: Alloplastic implants have emerged as a novel solution in overcoming the aesthetic, functional, and psychological challenges associated with microtia. Porous polyethylene implants are a reliable means for achieving successful external ear reconstruction with an acceptable complication profile. Although there are multiple advantages over autologous procedures, further study is needed to mitigate complications as the majority of patients required a secondary revision procedure.

(1) Children’s Hospital of Philadelphia, PA, (2) University of Pittsburgh Medical Center, PA

Background/Purpose: As surgical techniques improve, surgical complexity and outcomes continue to evolve, thus relative procedure compensations need to be periodically revisited. The purpose of this study is to define the relationship between craniofacial surgical procedure relative value units (RVUs) and metrics of surgical complexity and physician workload.

Methods/Description: The ACS NSQIP-Pediatric dataset was queried for the most common craniofacial procedures. Perioperative variables were analyzed with a multivariate regression model to assign corresponding correlation coefficients, which were then used to redistribute RVUs for each procedure code based on surgeon time and assumed risk. RVUs are reported as total work RVUs incorporating all additional secondary procedures by the primary team, which is necessary given that all procedure variables such as operative time encompass the entire procedure. Concurrent procedures by additional surgical teams, or cases where the additional procedures by the primary team had greater RVU than the primary procedure were excluded from analyses.

Results: During the study interval, 19 445 pediatric patients underwent craniofacial procedures meeting inclusion criteria. Multivariate regression yielded the following equation to predict RVUs for a given CPT code: work RVUs = 0.025*(operation time in minutes) + 0.035*(blood transfusion required in mL) + 0.084*(postoperative days until discharge) + 0.058*(serious adverse events) + 0.641*(related readmission) + 0.027*(related reoperation) + 13.898 constant. The P values of these parameters are as follows: P < .001 for operation time, P < .001 for transfusion, P < .001 for length of stay, P = .955 for serious adverse events, P = .641 for related readmission, and P = .027 for related reoperation. CPT 21159 “Le Fort III…” continues to remain the craniofacial procedure with the highest RVUs using our regression at 33.93 units, given lengthy procedure duration (357 minutes), greatest requirement for blood transfusion (283 mL), greatest length of stay (10 days), highest rate of serious adverse events (1 in 4 patients), and highest rate of related reoperation within 30 days (1 in 4 patients). The most underestimated procedure is CPT 42235 “Repair of anterior palate, including vomer flap” which has a current average total RVU of 8.84, but adjusted RVU using our regression is 17.11 units, representing a suggested change of +8.27 units.

Conclusions: Increasingly complex midface procedures remain among the highest RVUs given physician workload, surgical complexity, and risk for serious adverse events. Increasingly complex palatal reconstructions are the most underestimated in compensation from current reimbursement standards. Despite limitations of any surrogate for surgeon time, procedure risk, and hospital cost, these variables represent reasonable nationwide estimates to help guide reconsideration of appropriate insurance compensation.

(1) Children’s Hospital of Philadelphia, PA

Background/Purpose: Mandibular distraction osteogenesis (MDO) and continuous positive airway pressure (CPAP) may effectively treat more and less severe forms of tongue-based airway obstruction (TBAO) in Pierre Robin sequence (PRS), respectively. This study aims to describe longitudinal outcomes for these 2 approaches in selected patient populations.

Methods/Description: Patients with syndromic and nonsyndromic PRS treated with MDO or CPAP during 2009 to 2019 were reviewed. CPAP was typically employed in patients with more mild dyspnea or feeding difficulty, contraindications to surgery, or based on family preference. Subjects were included if they received baseline and at least one follow-up polysomnogram (PSG) examinations. Only patients initiating treatment within the first year of life were included. Appropriate tests of comparison were performed.

Results: A total of 134 patients were treated during this study period; 55 who underwent MDO and 9 who received CPAP only treatment met inclusion. Mean age at first PSG was 41 days (range: 2-269) and 24 days (2-63) for MDO and CPAP only groups (P = .288). Mean baseline OAHI was greater in the MDO group (x¯     = 40.95[34.8-47.1]) compared to (x¯     = 25.17[14.3-36.1], P = .033) in the CPAP group. Baseline maximum ETCO2 was significantly higher in MDO (x¯     = 55.05 [95% CI: 52.6-57.5]) compared to the CPAP only group (x¯     = 48.33[43.9-52.8], P = .024). Central apnea hypopnea index (CAHI) and Spo ₂ nadir did not significantly differ between the 2 groups (P > .05). Syndromic status, presence of cleft palate, and presence of any airway anomalies were not statistically different between the groups (P > .09). More patients treated with MDO required nasogastric tube feeding at time of discharge (40, 80%) compare to CPAP (4, 44.4%, P = .036); however, at 6 months follow-up, there was no difference in feeding outcome (P = .376). 26 patients in the MDO group (47.3%) utilized CPAP at home with 7 (26.9%) weaned off of it by the time of their last PSG compared to the 8 (88.9%) in the CPAP only. Mean duration of CPAP usage was 552 days (range: 109-842) and 428 days (335-522, P = .553) for MDO and CPAP cohorts, respectively. There was a higher likelihood of those who received CPAP at home after having MDO to remain on CPAP at the time of their last PSG (P = .002) compared to those who were treated with CPAP only. Final PSG variable means did not differ between the 2 groups, P > .05. MDO significantly reduced OAHI, Spo ₂ nadir, and max ETCO2, P < .001. CPAP significantly reduced OAHI and Spo ₂, P < .001; however, there was no statistically significant decrease in max ETCO2, P = .183.

Conclusions: CPAP appears to be similarly effective at treating TBAO in infants with less severe PRS with outcomes similar to those patients who underwent MDO. Linear mixed modeling is being applied to this data and would also be presented.

(1) Baylor College of Medicine and Texas Children’s Hospital, Houston, TX

Background/Purpose: Surgical options in patients with craniosynostosis can be time dependent due to the rapid brain growth that drives cranial growth during the first year of life. Minimally invasive techniques require that early excision of the synostosed suture be performed such that this rapid growth phase, with the aid of helmeting, can correct the aberrant head shape that has resulted. Interventions prior to 6 months age have shown good results with these minimally invasive techniques. Older patients require larger cranial vault remodeling surgeries to achieve satisfactory outcomes. The purpose of this study is to identify factors that result in a delayed presentation to a craniofacial clinic that would obviate certain surgical options for patients.

Methods/Description: Retrospective chart review was conducted from November 2011 to September 2018 to identify patients with a documented diagnosis of craniosynostosis who presented for initial evaluation by the craniofacial team at our institution. We excluded patients with a syndromic diagnosis or a history of correctional surgery. Patients who presented at 6 months of age or older were considered to have presented in a delayed fashion. We analyzed 14 potential variables that could be associated with delayed presentation.

Results: A total of 208 patients were identified for chart review with 142 (68.3%) meeting our inclusion criteria. Of the 142 patients, 70 (49.3%) presented prior to 6 months of age, while 72 (50.7%) presented in a delayed fashion. Nonwhite patients were more likely to present in a delayed fashion compared to white patients (61.0% vs 36.1% P = .004). Patients who had Medicaid insurance were more likely to present in a delayed fashion compared to those who had other types of insurance (60.7% vs 43.8% P = .047). Compared to patients who presented before 6 months of age, patients who presented in a delayed fashion were also more likely to have other documented comorbidities (48.6% vs 30%, P = .023), developmental abnormalities (23.6% vs 5.7%, P = .003), and were also more likely to have symptoms associated with increased intracranial pressure (11.3% vs 1.4%, P = .017). Sex (P = .501), ability to speak English (P = .251), income (P = .565), distance from our hospital (P = .729), family makeup (P = .791), being the first born (P = .951), type of referring provider (P = .192), and the presence of preappointment imaging (P = .070) were not significant.

Conclusions: Delayed presentations of patients with nonsyndromic craniosynostosis occur frequently. This is disproportionately true with minority patients and patients with Medicaid insurance. These populations are at increased risk for requiring larger cranial vault remodeling procedures which are associated with increased perioperative morbidity. Interventions to improve timely presentations to craniofacial centers should be explored with particular focus on minority populations and patients with Medicare insurance.

(1) Center for Regenerative Medicine, Shriners Hospital for Children/Massachusetts General Hospital, Boston, MA, (2) Massachusetts General Hospital, Boston, MA

Background/Purpose: Genome-wide association studies have greatly contributed to the understanding of the correlation of certain genetic mutations with cleft lip and palate formation. Unfortunately, they do not explain the development of such anomalies at the cellular level. Due to the pertinence of such comprehension to preventative and therapeutic intervention, we attempted to model a complex syndromic orofacial cleft in a dish. Deriving embryonic-like pluripotent stem cells from a patient born with a malformation allows us to retrace the arc of development and grants us the opportunity to dissect the regulatory networks involved in the formation of the cleft.

Methods/Description: Patient and parent blood samples were used to generate induced pluripotent stem cells (iPSCs). We infected peripheral blood mononuclear cells derived from a whole blood draw of the patient and her father with Sendai Viruses leading to an overexpression of 4 factors highly expressed in early embryonic development: OCT-4, KLF-4, c-MYC, and SOX2. Following a 21-day period of cellular reprogramming, 78 colonies were chosen for the patient and 51 were chosen for the father. Following 5 passages, the 3 superior looking colonies from patient and father were expanded. At passage 10, we confirmed the genetic stability, cellular pluripotency and genomic purity of our cells. We supplemented our downstream experimental design by performing all inquiries in a sex- and ethnicity-matched batch of fully characterized iPSCs as a control.

Results: We derived induced pluripotent stem cells from a single child born of unrelated parents in Honduras with an oblique facial cleft with anophthalmia and a coloboma, developmental delays, and leucomalacia, as well as the child’s father. All iPSCs displayed properties typical of embryonic stem cells, such as persistent self-renewal and the expression of markers of pluripotency. Furthermore, all iPSCs proved capable of 3 germ layer differentiation and differentiation into neural crest cells. Whole-genome sequencing of the patient and her parents identified a variant in TLE2, a gene with roles in epithelial differentiation and neurogenesis. The iPSCs of the patient and her father proved equally capable to differentiate into chondrocytes, adipocytes, Schwann cells, and osteoblasts.

Conclusions: We could demonstrate that the gene regulatory networks involved in brain and face development appear to be dispensable for iPSC reprogramming, as well as the formation of embryoid bodies. Utilizing CRISPR technology will allow us to further dissect the networks involved in the interwoven processes of brain and face development. The identification of the regulatory networks involved will help us to better understand the developmental biology of syndromic cleft lip and palate and lay the ground work for future innovation.

(1) College of Medicine, University of Lagos, Nigeria, (2) University of Iowa/Postdoctoral Research Fellow, IA, (3) University of Iowa, Iowa City, IA, (4) Department of Orthodontics University of Dundee, Scotland, UK (5) University of Lagos, Nigeria, (6) KwaZulu-Natal University, Durban, South Africa, (7) University of Iowa, IA, (8) University of PortHarcourt, Rivers, Nigeria, (9) University of Pittsburgh, PA, (10) Utah State University, Logan, UT, (11) NIH, Bethesda, DC, (12) Department of Oral Pathology, Radiology and Medicine, College of Dentistry, University of Iowa, IA

Background/Purpose: Based on epidemiological and experimental data, OFC etiology is considered to be multifactorial and determined by interaction between many loci genes and environment. Fetal environment is the mother, thus etiology of cleft can be affected by the genes, lifestyle, environmental exposure, and gene–environment interaction (GEI) of the mother. Several studies have reported associations between obesity and diabetes mellitus with risk of CL/P. Congenital malformations are reported to occur approximately 3 times more often in newborns born to diabetic mothers than to nondiabetic patients. Although Africa is critical for understanding modern human origins and genetic risk factors for disease, it has been underrepresented in human genetic studies. This study is the first to answer the research question: Is there a role of genome-wide significant polymorphisms for type 2 diabetes mellitus and obesity in OFC in the African population?

Methods/Description: This is a case–control triads study where cases are subjects with OFC and controls are subjects without OFC. The study was conducted using saliva samples from 3 African countries—Nigeria, Ghana, and Ethiopia. The approval for the African sample collection was gotten from the IRB of the 3 Hospitals involved. DNA extraction and analysis were done at the Butali lab and 48 SNPs selected from GWAS database for diabetes mellitus and BMI (proxy for obesity). Statistical analyses were done using FBAT and Plink statistical software and P > .05 was considered nominal significance and Bonferroni corrected P value was .001 The genotypes at different loci were called and a test of association was done. Case–control analysis and transmission disequilibrium test (TDT) using was done using PLINK (http://pngu.mgh.harvard.edu/∼purcell/plink/). We also did the TDT analyses using the family-based association test (FBAT) software (www). For this test, we set α at .05 to denote statistical significance and 1.04 E-03 as Bonferroni corrected P value.

Results: There were 1000 cases and 2000 controls analyzed with 48 SNPs associated with type 2 diabetes mellitus and BMI genotyped. The case–control analysis of BMI SNPs showed that rs7903146 on TCF7L2 (OR = 4.5; CI: 0.97-20.8, P = .03), rs3810291 near gene ZC3H4 (OR = 2.5; CI: 0.97-6.6, P = .04), and rs2287019 near gene QPCTL (OR = 4.3; CI: 1.6-11.8, P = .001) were strongly associated with cleft palate. Only TCF7L2 was significantly associated when family-based associated test was done. On analysis for diabetes mellitus, SNPs showed rs7756992 near gene CDKAL1 was associated with all cleft cases in both case–control and family-based association analysis.

Conclusions: Our significant findings suggest possible association between metabolic syndromes and risks for OFC. Further prospective studies need to be conducted to confirm these relationships where the information on the status of maternal metabolic syndrome is considered in the analyses.

(1) Children’s National Hospital, Washington, DC

Background/Purpose: Sagittal craniosynostosis (SC) is usually diagnosed during early childhood due to the presence of scaphocephaly. Nevertheless, latent forms of premature sagittal fusion with mild or even absent phenotypic presentation have been reported. A recently presented study from our group demonstrated a prevalence of incidental sagittal fusion of over 3% in otherwise phenotypically normal children from 1 to 5 years of age. The purpose of this article is to validate this preliminary data with a larger cohort and to analyze factors that may be associated with incidental finding of early suture fusion, at our institution.

Methods/Description: We evaluated computerized tomography (CT) scans of patients from 0 to 71 months of age who presented to the emergency department (ED) between 2008 and 2020. Patients who were found to have syndromes related to craniosynostosis, phenotypes consistent with craniosynostosis, VP shunt placement, brain or cranial abnormalities, were excluded. A panel of 2 craniofacial surgeons reviewed the CT scans for presence of CS. Demographic information, gestational age, past medical and family history, medications, chief complaint, and fusion type were recorded as covariates. Patients were classified into 2 groups based on the presence or absence of any craniosynostosis (group A vs group B, respectively) and Unpaired t test and χ ² /Fisher exact test were carried out to identify differences between groups. Firth logistic regression was performed to determine the association between the independent covariates and the presence of CS in 2 subcohorts; patients ≤24 months of age and patients older than 24 months of age.

Results: We identified 870 patients with a mean age of 30.1 SD ± 21.1 months. Overall, 44 (5.06%) patients were found to have bony fusion along the sagittal suture; besides the normal closure of the metopic suture, no other sutures showed any fusion. Twenty-six of the fusions were complete and 18 were incomplete, with partial fusions ranging from 10% to 95% of total suture length. All patients had a normal cranial index. The prevalence increased from 0 to 24 months, then was constant from 25 to 71 months. Affected patients were more likely to have a family history of neurodevelopmental disease, and those who received neurotropic medications had 33.28 (95% CI [3.37, 328.64], P = .003) and 25.78 (95% CI [1.80, 369.88], P = .017) times higher odds of developing SC, respectively. Furthermore, subcohort encompassing patients above 24 months of age showed premature subjects had 2.21 (aOR: 2.21, 95% CI [1.02, 4.77], P = .043) times higher odds of developing CS than the full-term babies.

Conclusions: Normocephalic SC in young children is common. Although the impact of this condition is unknown, the correlation with the use of neurotropic medication use and family history of neurodevelopmental disease is concerning.

(1) University of Iowa/Postdoctoral Research Fellow, IA, (2) Division of Biostatistics and Computational Biology, College of Dentistry, University of Iowa, IA, (3) Department of Biostatistics, University of Iowa College of Public Health, IA, (4) University of Iowa, IA, (5) Department of Orthodontics University of Dundee, Scotland, (6) Kwame Nkurumah University of Science and Technology, Kumasi, Ghana, (7) Addis Ababa University, Ethiopia, (8) University of Lagos, Nigeria, (9) KwaZulu-Natal University, Durban, South Africa, (10) Iowa Institute of Oral Health Research, Iowa City, IA, (11) University of Ferrara, Italy, (12) University of PortHarcourt, Rivers, Nigeria, (13) Knust-Kath, Kumasi, Ghana, (14) University of Pittsburgh, PA, (15) NIH, Bethesda, DC, (16) Department of Oral Pathology, Radiology and Medicine, College of Dentistry, University of Iowa, IA

Background/Purpose: Nonsyndromic cleft lip with or without cleft palate (NSCL±P) is the most common craniofacial defect and occurs secondary to multiple mechanisms. As a complex trait, many genetic studies have been done to identify the genetic variants that contribute to the risk of the birth defect. However, only about 25% heritability have been explained thus far.

Methods/Description: In this study, we used whole genome sequencing (WGS) analysis to identify de novo variants that may explain the missing heritability of NSCL±P. A total of 150 African trios were involved in this study. After QC, 130 trios were analyzed for high impact de novo variants: We filtered for novel/rare (minor allele frequency <0.01) coding variants that are deleterious to the protein function (loss of function and missense variants). We then prioritize the variants that play roles in craniofacial morphogenesis that may explain the birth defect.

Results: Our de novo analysis identified novel loss of function (LOF) variants in TTN and MINK1 genes, and missense variants in DHRS3 and TULP4. These genes are involved in the development of the face and some have been shown to play roles in the syndromic CL±P. A LOF variant was found in ARHGAP10 a gene encoding one of the members of the Rho GTPase-activating protein family. Although no craniofacial phenotype is available in animal models investigated, this gene interacts with CTNNB1: a key component of the canonical Wnt signaling pathway. Additionally, we found novel variants in TP63 and SHH which are well reported CL±P candidate genes. We also identified a novel de novo mutation in TCTN1 whose variants have been reported to cause Joubert syndrome: a disorder due to primary ciliary defect. This syndrome include brain stem malformation and cerebellar hypoplasia features with some cases reporting CL±P phenotypes.

Conclusions: WGS analysis of de novo variants in NSCL/P African families identified novel genes and variants in candidate genes that may explain the development of this birth defect. Our study provides additional evidence for the role of de novo variants in the etiology of NSCL/P. Findings from this study also show the power of WGS analysis of trios in genetic variants discovery.

(1) University of Pittsburgh, PA, (2) University of Iowa, College of Dentistry, IA, (3) Department of Surgery, Plastic and Reconstructive Surgery, University of Colorado School of Medicine, Denver, CO, (4) Department of Pediatrics, College of Medicine, University of the Philippines Manila, Manila, Philippines, (5) ECLAMC (Estudio Collaborativo Latino Americano Malformaciones Congenitas), Patagonia, Patagonia, (6) Department of Genetics, Institute of Biology, Federal University of Rio de Janeiro, Brazil, (7) Dental and Craniofacial Genomics Core, School of Dental Medicine, University of Puerto Rico, San Juan, (8) Department of Pediatrics, McGovern Medical SchooUniversity of Texas Health Science Center at Houston, TX, (9) Department of Health Management and Policy, College of Public Health, University of Iowa, IA, (10) Lancaster Cleft Palate Clinic, PA, (11) Department of Oral Biology, School of Dental Medicine, University of Pittsburgh, PA, (12) University of Iowa, IA, (14) Department of Orthodontics, College of Dentistry, University of Iowa, IA

Background/Purpose: Dental caries is a common oral disease, which is a global public health problem that can lead to serious complications. The risk for developing dental caries is hypothesized to increase in children born with orofacial clefts (OFCs) due to abnormalities that affect tooth structure, shape, and numbers. Previous studies investigating dental caries incidence in people with OFCs compared to the general population have reported mixed results, with some, but not all, studies showing increased risk of caries in individuals with OFCs. The aim of this study is to evaluate the relationship between dental caries and OFCs and investigate possible shared genetic risk by means of a genome-wide association study (GWAS) of caries in a multiethnic cohort from the Pittsburgh Orofacial Cleft study (POFC).

Methods/Description: The sample of OFC cases and their family members (N = 3686) included families recruited in Argentina, the Philippines, Guatemala, Hungary, and the United States. Participant ages ranged from 2 to 12 years for analysis of the primary dentition, and 18 to 60 years for analysis of the permanent dentition. Dental caries was assessed by counts of decayed and filled teeth (dft/DFT) and was analyzed as a quantitative trait. Age, gender, recruitment sites, and orofacial cleft status were treated as covariates. Genetic variants (single nucleotide polymorphisms, SNPs) were genotyped or imputed across the entire genome. Mixed-models implemented in the EMMAX software were used to test genetic association with dental caries in the OFC families while simultaneously accounting for covariates, relatedness, and population structure.

Results: Mean caries scores for both permanent (DFT) and primary (dft) dentitions were analyzed. Binary OFC status (affected vs unaffected) was associated with dental caries in both the primary (β = 0.80861, SE = 0.20120, P = 6.24 × 10E-5) and the permanent dentition (β = 1.4312329, SE = 0.3496957, P = 4.39 × 10E-5). In each case, the presence of an OFC increased the caries mean score. Further, preliminary GWAS results of DFT and dft in the OFC families identified several loci (P ≤ 10E-7) within or near genes with plausible biological roles in craniofacial morphogenesis, tooth development, and dental caries, which support our hypothesis of some shared genetic underpinnings for these conditions.

Conclusions: This study identified risk genetic loci associated with dental caries susceptibility in a large multiethnic population of OFC families. Our results will lead to better understanding of cariogenesis among people with nonsyndromic OFCs. In addition, this study will help in improving the clinician predictions, prevention, and/or treatment measures of dental caries for people affected with OFCs.

(1) University of Missouri-Kansas City, School of Medicine, MO, (2) Children’s Mercy Kansas City, MO

Background/Purpose: Nutrition and growth assessments are important factors in the early care of children with congenital orofacial clefting diagnoses. It has long been believed that children with congenital clefting conditions do not grow as well as children without clefts for reasons related to both nutrition and potential intrinsic growth issues. More recent investigations of growth and nutrition in these patients would suggest however that in reality, these children should compare favorably with their noncleft peers given the right interventions and support. Long-term population growth studies are needed however to demonstrate the expected longitudinal growth over time for these children.

Methods/Description: This study is a retrospective review of a cohort of patients undergoing cleft palate repair to assess longitudinal growth parameters over the first 2 years of life. This pilot study worked to identify growth patterns by dividing the cohort into cleft palate only (CPO) and combined cleft lip and palate (CLP) groups to compare outcomes for these different diagnoses. Weights, including percentiles and z-scores, were reviewed at approximate time points of birth, 1st team visit at 1 to 2 weeks, and then at 2, 4, 6, 9, 12, 15, 18, 21, and 24 months. These data were then visualized individually and as averaged data to compare with World Health Organization (WHO) published growth charts for healthy children aged 0 to 2 years.

Results: The initial cohort of patients examined included 28 patients, 12 with CPO and 16 with CLP. Average birth weights for CPO was 3.41 kg (SD 0.71) and for CLP was 3.31 (SD 0.57). Each group demonstrated typical growth curves patterns and maintained relative cohesiveness among their diagnosis cohort. When compared with the WHO growth curves, the average data of the CLP group fell between the 3rd and 50th percentile curve for the first 6 to 8 months of age and then matched or surpassed the 50th percentile for 0 to 2 years. Average data for CPO fell more directly along the lower third percentile of the WHO curve until 10 to 12 months of age and then more closely approximated the 50th percentile curve. Overall the data demonstrated approximation of normal growth by 12 months of age for both groups, even before most patients had undergone formal cleft palate repair (mean repair = 13.7 months).

Conclusions: This initial growth curve data support the idea that these infants are not growth delayed or impaired. In general, these patients should be expected to have normal growth potential over time. With maximal nutritional care and intervention as needed these patients should be expected to meet or exceed noncleft standards for weight gain in the first 2 years of life.

(1) Erasmus MC, Rotterdam, the Netherlands, (2) Boston Children’s Hospital, MA, (3) Erasmus University Medical Center, Rotterdam, MA, (4) Duke University, Durham, NC, (5) Karolinska University Hospital, Stockholm, Sweden, (6) Erasmus University Medical Center, Rotterdam, the Netherlands, (7) International Consortium for Health Outcomes Measurement, Boston, MA

Background/Purpose: The ICHOM Cleft Lip and Palate Standard Set (Set) is available free of charge and designed to be implemented across cultures. Over the past 4 years, a few cleft centers in North America and Europe have implemented this set of outcomes in their daily clinical practice. The outcome data collected will be used to facilitate quality improvement efforts and learning initiatives through intercenter comparisons. However, before meaningful comparisons can be generated, widespread adoption and implementation of the Set in multiple cleft centers is needed. Many cleft institutions have faced challenges implementing the Set. Known factors hampering implementation efforts include lacking a defined implementation strategy or a clear understanding of the overarching conditions that promote or hinder implementation in routine clinical care. Therefore, the aim of this study is to describe and identify facilitators and barriers for the implementation of the ICHOM Standard Set for cleft care in routine clinical practice, based on the implementation experiences of 4 pilot centers.

Methods/Description: A qualitative study including an exploratory survey and in-depth interviews among clinicians, nurses, Health Information Technology professionals, project coordinators, and leads employed by cleft teams of Boston Children’s Hospital (USA), Duke University Hospital (USA), Erasmus University Medical Center (NL), and Karolinska University Hospital (SE). Thematic content analysis was performed, and themes were organized according to the 5 dimensions of the RE-AIM evaluation framework for interventions and health care programs: reach, effectiveness, adoption, implementation, and maintenance.

Results: In total, 13 participants (n = 20) completed the exploratory survey and 5 interviewees were selected. Content analysis revealed that teams reach patients either via email or during the clinic visit to capture patient-reported outcomes. The adoption of the Standard Set is enhanced by creating importance and aligning motivation and priorities at the organizational and cleft team level. Aligning workflows and developing an efficient HIT platform for the collection of outcomes is necessary early on, followed by pilot testing or stepwise implementation. Regular meetings and financial resources are crucial for implementation as well as sustainability together with analyzing the collected data and providing feedback to health care professionals and patients. Stimulating patient-centered care was articulated as a positive outcome, whereas time was a challenging factor for all RE-AIM dimensions.

Conclusions: The identified themes can inform ongoing and starting implementation efforts. Intentionally investing time to lay a sound foundation early on will benefit every phase of the implementation process and help overcome barriers such as lack of support or motivation. The RE-AIM framework can be used by teams to scrutinize and improve their implementation process of an outcomes set in a structured fashion.

(1) Global Smile Foundation, Norwood, MA, (2) Amsterdam UMC, the Netherlands, (3) Sri Ramachandra Institute for Higher Education and Research, Chennai, India, (4) Southern Highlands Speech Pathology, Sydney, Australia, (5) Hansjörg Wyss Department of Plastic Surgery, NYU Langone Health, New York, NY, (6) Orthodontics Department, Akdeniz University Faculty of Dentistry, Antalya, Turkey, (7) Medical University of South Carolina, Charleston, SC, (8) New York University Langone Medical Center, NY, (9) Duke University, Durham, NC, (10) Children’s Hospital of Pittsburgh, PA, (11) Department of Plastic and Reconstructive Surgery, Yale University School of Medicine, New Haven, CT, (12) Cincinnati Chidren’s Hospital Medical Center, OH, (13) NYU Langone Health, New York, NY, (14) E. Rebagliati Hospital Essalud Lima Peru, Peru, (15) Bauru Dental School, University of São Paulo, Bauru, Brazil, (16) University of São Paulo, CT, (17) Spectrum Health Medical Group, Grand Rapids, MI, (18) Hospital Gea Gonzalez, Mexico City, Mexico, (19) Alemana Clinic, Santiago, Chile, (20) San Jose University Children’s Hospital, Bogota, Colombia, (21) Boston Children’s Hospital, MA, (22) Brazilian Craniofacial Society, Brazil, (23) Great Ormond Street Hospital for Children, London, England, (24) Seattle Children’s Hospital, WA, (25) Hospital de Clinicas, School of Medicine, National University of Buenos Aires, Argentina, (26) Rio Grande do Sul Federal University, Porto Alegre, Brazil

Background/Purpose: Newborns with cleft lip and/or palate (CLP) exceed 100 000 per year in low- and middle-income countries (LMICs). Patients, if left untreated, are at high risk of morbidity, due to functional deficits, malnutrition, aspiration, and infections. Limited resources in LMICs create barriers for establishing “Interdisciplinary Cleft Care” programs. Surgical missions driven by nonprofit organizations have been able to partially address this need, but their ability to promote long-term sustainable cleft care has come to a question. Simulation-based training has emerged as an essential tool for enhancing medical education and training. Global Smile Foundation, a nonprofit organization, is a leader in the establishment of Interdisciplinary Cleft Care programs, with its volunteers being involved in cleft care for over 3 decades. We were able to demonstrate the efficacy of our first Simulation-Based Comprehensive Cleft Care Workshop (SBCCW), in the Middle East-North Africa (MENA) region, and its wide acceptance by our recipients. In the current study, we want to prove the effectiveness and successfulness of our second SBCCW, in Latin America.

Methods/Description: Our second SBCCW took place in Lima, Peru, in October 2019. Hands-on simulations of CLP repair using high-fidelity CLP simulators were also provided to our participants. Participants were asked to complete a satisfaction survey at the end. Attendees were also asked about the obstacles facing cleft care in their countries and the possible interventions to overcome these obstacles. Short-term (at the end of the SBCCW) and medium-term (6 months later) follow ups were conducted by our team collecting data about improvements in the participants’ competence, performance, outcomes, clinical care, and whether the SBCCW has changed their practice. Procedural confidence for pre- and postsimulation was evaluated using the psychometrically validated tool for measuring self-confidence during surgical learning. Descriptive statistics were used for the collected data. Data analyses were performed using the Statistical Package for the Social Sciences.

Results: Ninety-eight of the 198 participants from 29 different countries filled the satisfaction survey at the end of the workshop. The 2 most common barriers to cleft care in LMICs identified by our participants are the absence of financial support and the absence of multidisciplinary teams. Respondents claimed an improvement in their procedural confidence after the simulation sessions. Respondents had consistent short-term and medium-term impressions about the SBCCW positively impacting their competence, performance, outcomes, clinical care, and even changing their practice.

Conclusions: This study provides evidence that implementation of a SBCCW leads to a significantly improved procedural confidence, as well as a sustained positive impact on the clinical practice of the participants, reinforcing its role as a cleft care capacity-building tool.

(1) University of Bristol, United Kingdom

Background/Purpose: Our understanding of why children are born with clefts and how well they do in life is limited but we know there is a strong genetic component for some and that environmental factors, including variations in treatment received, are also involved. Investigations of risk factors to date have tended to be small observational studies which cannot be used to assign causality and do not provide strong evidence for any modifiable factors. Large datasets are needed to address inconsistencies in findings and to identify genetic and environmental risk factors as well as to investigate outcomes for varying patterns of treatment and the impact of cleft on affected individuals and their families over time. The Cleft Collective Cohort Study started in response to a request from those involved in cleft care in the United Kingdom to collect data that could be used to address clinically meaningful research questions for this population, including: What causes clefts? Which treatments are best? How are children impacted by cleft?

Methods/Description: The cleft cohort study comprises 2 cohorts of children born with cleft lip and/or palate: a birth cohort (recruited either in the prenatal period or postnatally); and a 5-year cohort recruited when children attend for a routine clinical audit appointment. Children born with all cleft types, including those with and without identified syndromes and comorbidities, are recruited together with their parents and siblings from all 16 cleft operating sites in the United Kingdom. Participants provide biological samples which are genotyped. Additional data include parent questionnaires and clinical records which provide information on surgery, early development, speech, dental health, psychology, and well-being of parents and children. Further data are available from linkage with health and education records.

Results: Recruitment started in 2013 and is strong with biological samples collected from 1825 trios (affected child, mother, father) to date. In total, there are currently 9032 participants in the study, comprising: 3209 children born with a cleft; 220 siblings; 5603 parents (3173 biological mothers, 2413 biological fathers, 17 mother’s partners). The distribution of broad cleft subtypes in the sample reflects UK prevalence rates (cleft lip only 24.8%, cleft palate only 38.8%, unilateral cleft lip and palate 25.6%, and bilateral cleft lip and palate 10.8%). The data we collect allow us to classify clefts with greater detail using the LAHSHAL coding which specifies which tissues are affected. Current figures suggest 13% of cases are syndromic. Our sample represents a uniform distribution across all of the Index of Multiple Deprivation (IMD) deciles. Data on ethnicity are currently held on 1341 cases, of these, 91% are classed as white, 6% as Asian, and 3% as “other.”

Conclusions: The Cleft Collective is a unique resource, providing an unprecedented collection of biological, environmental, demographic, and clinical data to the research community to transform our understanding of cleft.

(1) Phoenix Children’s Hospital, AZ

Background/Purpose: Numerous surgical procedures have been described for the treatment of velopharyngeal insufficiency (VPI). Although pharyngeal flap and sphincter pharyngoplasty are the most established procedures, palatoplasty (including straight-line rerepair and Furlow techniques) and palatal lengthening are more recently proposed alternatives. We sought to characterize the current utilization of each VPI procedure in the United States.

Methods/Description: A retrospective cohort study was performed using the MarketScan Commercial database for 2015 to 2017, which contain a nationally representative sample of health care delivery in the United States. Patients undergoing VPI surgery were included if they had a diagnosis of hypernasality or VPI and were 3 to 24 years old. Procedure type was determined by physician procedure billing.

Results: Information was available on 573 VPI surgeries performed between 2015 and 2017, representing approximately 10% of all VPI surgeries in the United States during that period. Pharyngeal flap was the most frequently performed procedure, accounting for 40.3% of all VPI surgeries. Sphincter pharyngoplasty was performed in 25.0% of surgeries. Palatal procedures were less frequent with palatoplasty performed in 14.5% of surgeries and palatal lengthening in 5.4%. Combinations of these procedures were performed in 14.8% of surgeries. The average age at surgery was 8.8 years (SD 4.5), but there were significant differences in age between the procedure types (P < .0001): patients undergoing palatoplasty were younger (7.7 years [SD 4.3]) while those undergoing sphincter pharyngoplasty were older (9.6 years [SD 4.9]). Procedure type did not vary significantly by year of surgery (P = .14), gender (P = .96), or for patients with a diagnosis of 22q11 deletion syndrome (P = .28). Among surgeons performing more than one VPI surgery during the observation period, 85% performed more than one type of procedure and 65% performed palatoplasty and/or palatal lengthening in at least some patients.

Conclusions: Pharyngeal flap and sphincter pharyngoplasty are the most frequently used procedures for treating VPI in the United States. Although palatoplasty has been broadly adopted in the United Kingdom, and multiple surgeons have promoted use of palatoplasty for treatment of VPI in patients with small velopharyngeal closure patterns or sagittal levator muscle orientation, utilization of palatoplasty is much lower than pharyngeal flap and sphincter pharyngoplasty. This may be due, in part, to an absence of studies directly comparing the effectiveness of these VPI surgical procedures.

(1) Global Smile Foundation, Norwood, MA, (2) University of California, Irvine, Orange, CA, (3) Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, (4) The University of Maryland Medical Center/Shock Trauma Center, Baltimore, MD

Background/Purpose: Clefts of the lip and palate are leading congenital anomalies, affecting 1 in 700 births. Access to basic medical care and psychological support is poor in low- and middle-income countries (LMICs), which adds to the economic and social strains created by the disease. Even though nongovernmental organizations (NGOs) address the need for cleft care in LMICs, significant challenges facing international cleft care persist.

Methods/Description: A review of the literature was performed using PubMed, identifying the various building blocks in achieving a sustainable cleft care model. English language articles were included, and texts and references were reviewed.

Results: Surgical outreach programs are encouraged to develop and/or implement protocols to maximize patient safety such as the GSF emergency response protocols and quality assurance guidelines. The WCC is a collaboration of organizations that aims to develop an approved set of standards and guidelines to deliver standardized, sustainable, comprehensive cleft care. Since implementation of the modified World Health Organization surgical safety checklist in 2010, GSF has operated on more than 1800 patients with no mortalities reported. The Diagonal Care Delivery model provides the temporary workforce and resources needed, empowers local teams, and invests in infrastructure. Yearly interdisciplinary Comprehensive Cleft Care Workshops (CCCW) empower practitioners through educational initiatives including simulation-based hands-on sessions and was shown to be well received among participants. Fellowships and training programs such as the GSF-Cleft Surgery Training Program and the NasoAlveolar (NAM) training program promote education and mentoring by experts in the field. Continuous research and quality assessment are pillars of sustainable outreach cleft care. Emerging technologies such as Augmented Reality can be used to demonstrate procedures and supervise and empower surgeons.

Conclusions: International interdisciplinary/comprehensive cleft care requires continuous concerted endeavor between local and visiting teams, as well as national and global organizations. The standardization and regulation of current practices, in addition to the promotion of capacity building initiatives, is essential to achieve sustainable cleft care.

(1) Global Smile Foundation, Norwood, MA, (2) Oregon Health & Science University, Portland, OR, (3) Medical University of South Carolina, Charleston, SC, (4) Ochsner Hospital for Children, New Orleans, CA, (5) Yale New Haven Hospital, CT, (6) The University of Maryland Medical Center/Shock Trauma Center, Baltimore, MD

Background/Purpose: Clefts of the lip and palate are among the most common congenital birth defects with varying incidence in different geographical regions and populations. Surgical outreach programs temporarily provide the essential resources and expertise to address health care needs in underserved communities. However, such initiatives have traditionally been criticized for a lack of guidelines and appropriate regulations, raising ethical concerns around standards of care. In this study, we propose updated quality assurance guidelines to complement existing guidelines for mission-based cleft lip and/or palate care.

Methods/Description: A review of the literature was performed using PubMed, identifying published protocols and guidelines for cleft care delivery in underserved settings. Articles in the English language were included, and texts and references were reviewed.

Results: Based on American Cleft Palate-Craniofacial Association (ACPA) quality standards, published protocols by Global Smile Foundation (GSF), and the senior author’s 3-decade experience, updated guidelines for mission-based care were synthesized. We integrated 10 axes for safe, effective, and sustainable cleft care delivery in underserved settings: (1) site assessment; (2) team composition and credentialing; (3) establishment of community partnerships; (4) team training and mission preparation; (5) implementation of quality assurance guidelines, operative safety checklists, and emergency response protocols; (6) immediate and long-term postoperative care; (7) medical record keeping; (8) outcomes evaluation; (9) education; and (10) capacity building and sustainability. We further analyzed these axes to describe experience-derived and evidence-based recommendations.

Conclusions: The implementation and continuous improvement of quality assurance guidelines would ensure reproducible processes and outcomes. We hope that these guidelines will promote further discussion toward delivery of safe and effective interdisciplinary cleft care.

(1) Wake Forest School of Medicine, Winston-Salem, NC, (2) Medical University of South Carolina, Charleston, SC

Background/Purpose: Guidelines recommend that children with a cleft lip or cleft palate undergo primary repair prior to 12 months and 18 months of age, respectively. Patient race and ethnicity have been shown to affect timing and outcomes of cleft surgery to varying degrees. The aim of this study was to characterize racial differences in primary cleft lip and cleft palate repair utilizing a national pediatric surgical database.

Methods/Description: Patients younger than 2 years who underwent primary cleft lip or cleft palate repair from 2013 to 2018 were identified in the National Surgical Quality Improvement Program Pediatric (NSQIP-P) dataset. Descriptive statistics described patient preoperative risks, characteristics, and short-term outcomes. Multivariate logistic regression models were performed to identify predictors for readmission, and Cox hazard regression models were used to identify factors predictive of age at surgical repair.

Results: In total, 6021 primary cleft lip and 6938 primary cleft palate repairs were identified. Black (odds ratio [OR] 1.81 (95% CI 1.16-2.83) and Hispanic (OR 1.82, 95% CI 1.27-2.61) race were independent predictors of readmission following primary cleft lip repair. Multivariate Cox hazard regression analysis of time to surgical repair demonstrated that Hispanic (adjusted hazard ratio [aHR] 0.90, 95% CI 0.84-0.96) and Asian (aHR 0.62, 95% CI 0.55-0.70) patients received primary cleft lip repair later than white patients, whereas black (aHR 0.87, 95% CI 0.79-0.95), Hispanic (aHR 0.83, 95% CI 0.77-0.89), and Asian (aHR 0.47, 95% CI 0.43-0.53) patients received cleft palate repair later than white patients. Asian children experienced the greatest delays to surgery.

Conclusions: Racial disparities were identified in timeliness to surgery for both primary cleft lip and cleft palate repair. Black race and Hispanic ethnicity were associated with readmission following cleft lip repair. Further research is warranted to elucidate causes of these difference to ensure equitable cleft care for all children.

(1) The University of Bristol, United Kingdom, (2) North Bristol NHS Trust, United Kingdom

Background/Purpose: Covid-19 has impacted on the delivery of health care and education to children. Those with underlying medical conditions, including children born with clefts, are particularly vulnerable. During the lockdown period in the United Kingdom, some interventions were delivered using remote methods and other innovative ways. Nevertheless, delays in surgery and other treatments may have increased anxiety in parents and children and adversely influenced outcomes. Previous reports show that children born with clefts typically perform worse on measures of educational attainment relative to their peers. During the pandemic, “home schooling” replaced conventional teaching and it is not yet known what impact that has had specifically for children born with clefts. The primary aim of this study was to determine patients’ and families’ views of cleft care and educational support received over lockdown. The secondary aim was to identify how the well-being of children with cleft was affected during this period.

Methods/Description: The cleft collective study is a large prospective longitudinal UK cohort study of children born with cleft lip and/or palate available as a resource to researchers. To date, the study has recruited over 9000 participants from over 3000 families. Children within the study are now aged between 0 and 15. The Cleft Collective recently invited parent participants to complete a questionnaire about the impact of Covid-19. A mixed methods approach was used to address the study aims. Free-text data from the questionnaire was used to undertake a thematic analysis on responses to questions about interventions received, delayed treatment, educational support, and more general questions about Covid-19 and lockdown. Data on the child’s emotions and worries were collected retrospectively for the first (complete) and second (partial) phases of lockdown. The emotions and worries data were analyzed using a repeated measures technique for the 2 different time periods. Data were also compared as independent groups, to identify which characteristics helped children cope better during lockdown.

Results: Over 1100 families responded and preliminary analysis has identified several themes from the data so far. These include both negative and positive experiences of participants during lockdown. The data also highlight the concerns of parents, especially with regard to delays in receiving face-to-face speech and language therapy. Analysis is ongoing and will be reported in full at the time of the presentation.

Conclusions: In conclusion, this study provides real-time data to cleft clinicians and education providers highlighting parental concerns and interventions that have gone well. The results provide important information for cleft care in the event that further lockdowns are imposed. The study findings also identify which groups may need additional support with regard to a child’s well-being and anxieties. Future analyses will determine the impact of the pandemic and associated lockdown on longer term outcomes.

(1) Global Smile Foundation, Norwood, MA, (2) Joan C. Edwards School of Medicine, Huntington, WV, (3) Tufts University School of Medicine, Boston, MA, (4) The University of Maryland Medical Center/Shock Trauma Center, Baltimore, MD, (5) University of Arkansas for Medical Sciences, Little Rock, AR

Background/Purpose: Health care systems worldwide have suffered tremendous consequences due to the COVID-19 pandemic. Low- and middle-income countries (LMICs), that already had unmet surgical needs prior to the pandemic, have been especially affected. Although surgical outreach programs provide temporary increases in workforce and resources in LMICs, these programs were severely impacted by the pandemic due to precipitous travel bans, flight changes, and cancellations of unprecedented magnitude. Global Smile Foundation (GSF) volunteers have provided interdisciplinary cleft care through surgical outreach programs for over 3 decades, with the success of these programs heavily relying on volunteers and their continued willingness to provide global care. GSF has had to promptly acclimate to surging challenges. Moreover, little has been discussed regarding the impact of the pandemic on LMICs. This study is the first to assess how the pandemic might affect surgical outreach programs providing care in LMICs.

Methods/Description: ERB approval was obtained to collect data from study subjects. Inclusion criteria comprised of individuals who have participated in at least one global surgical outreach program with GSF and had a current email address in the organization’s database. The total number of study subjects was 403. There were no exclusion criteria. The data were collected in the form of a 6-question survey that gathered responses in the form of multiple-choice, checkbox, and free-text answers.

Results: The survey received 223 responses, corresponding to a 55% response rate. Although most volunteers (64%) plan on joining future GSF outreach programs, 31% remain unsure of their future commitment. Volunteers’ main concerns to join future outreach programs were worries of COVID-19 infection (85%) and mandatory quarantine in their home-country post-trip (69%) and in the host country (60%). A lot of uncertainty remains regarding safety to resume outreach programs. Many volunteers would not consider traveling until a vaccine is available (30%). Additionally, 70% of respondents report current travel restrictions implemented by their institution, with 78% of these respondents disclosing that no time frame has yet been determined to resume traveling.

Conclusions: This study shows that, while the COVID-19 pandemic does not completely dissuade GSF volunteers from their commitment to providing cleft care in LMICs, a lot of uncertainty remains regarding upcoming surgical outreach programs. One would therefore worry that the existing burden of disease highlighted by the 2015 Lancet Commission on Global Surgery will only increase.

(1) Children’s Hospital of Wisconsin, Milwaukee, WI

Background/Purpose: This presentation will focus on an effective and quick procedure to determine family identified priorities for multidisciplinary cleft care as well as data regarding specific family identified priorities gathered through a quality improvement initiative at a Midwestern multidisciplinary cleft lip and palate team. This team utilizes half-day multidisciplinary visits during which patients see any combination of the following providers: plastic surgery, ENT provider, social work speech language therapy, audiology, genetic counseling, pediatric psychology, orthodontics, prosthodontics, dentistry, nursing, and pulmonology. The specific providers who will see the patients for their team visit are identified ahead of time through chart review and standard practice; however, scheduling is flexible and team members may be added or removed throughout the day based on patient/family preferences, scheduling, and team member recommendations. This model of care is in accordance with ACPA parameters of care calling for an interdisciplinary team of specialists to provide management of patients with craniofacial differences; however, it does not take into account family identified priorities in any standardized manner. In an attempt to ensure incorporation of family identified priorities into the multidisciplinary visit, this team began asking families “What matters most today?” at the start of their team visit. The goal of this presentation is to review the procedure this team implemented as well as to present data regarding family identified priority on the day of their child’s multidisciplinary team visit gathered through asking this question.

Methods/Description: All patients and families presenting for a multidisciplinary cleft lip and palate team appointment were asked “What matters most today?” at the start of their team visit with the intent of identifying their main concern(s) for that visit. The current sample includes responses from 344 families, with data collection ongoing. Families were asked this question by the provider who saw them first on the multidisciplinary clinic day. Attempts were then made to address these identified concerns as soon in the course of the visit as possible, which at times included adding an additional team provider to their clinic visit. For example, a family that identified a behavioral concern would be offered the opportunity to see Pediatric Psychology during their team visit even if they had not previously been identified as needing to see Pediatric Psychology that day. At the end of each visit, the last team provider seeing the patient confirmed that the family’s main concern for the day had been addressed and/or the family was aware of a plan in place to address this concern in the future. This presentation will review the above-described procedure, rational for this procedure and data regarding family identified priorities gathered through asking this question.

(1) Children’s Hospital Los Angeles, CA

Background/Purpose: Although most children with craniofacial diagnoses do not experience significant psychological symptoms, they are at risk for social issues, self-esteem, and subclinical anxiety and depression. Social support groups for children with craniofacial diagnoses and their families provide an opportunity for peer normalization and learning social and coping skills. The group modality is well suited to address the mutual social support and skills practice in a shared therapeutic setting; however, given COVID-19, groups have been cancelled or held via telehealth. This presentation aims to outline the process used at a large urban children’s hospital for holding patient (ages 6-17) and caregiver (English and Spanish) groups by telehealth and offer suggestions to address common difficulties.

Methods/Description: Some of the advantages of adapting long-standing craniofacial support groups to telehealth included ease of access for families by removing several barriers, such as distance, transportation, travel time, childcare, and financial costs. Multiple logistical and clinical challenges were also encountered in the use of telehealth. The logistical issues included inconsistent internet connectivity, varying family computer fluency, technical issues on telehealth platforms, recruitment in context of screen fatigue, scheduling, and obtaining consent. Clinical challenges included setting group rules, establishing group rapport, maintaining engagement, approximating naturalistic group member interactions, allowing for bilingualism, managing simultaneous conversations, and following up on ethical concerns, such as suspected child abuse. The process of adapting groups started with acknowledging the limitations of telehealth for group modalities and establishing realistic expectations. Logistical suggestions include allowing for multiple telephone calls prior to starting the group to address technical questions, mailing bilingual step-by-step instructions for use of telehealth platform along with group rules, scheduling separate times for caregivers and patients, splitting caregiver group by language and patient group by size or age, shortening the number of sessions and meeting time, setting platform functionalities to minimize disruption, documenting verbal consents, and planning for individual follow-up phone calls. Some clinical recommendations are setting member guidelines, using a high degree of structure and turn taking, using minimal screen sharing of written materials, incorporating engaging age appropriate activities, facilitating interactions between group members, including simple yoga exercises for movement, and providing participation rewards. Family and patient feedback about the telehealth groups was generally positive, with frequent appreciation expressed for continuing the service during COVID-19. Despite drawbacks inherent to the telehealth format, there are benefits from offering social support groups for craniofacial populations with adaptations.

(1) Nationwide Children’s Hospital, Columbus, OH

Background/Purpose: Infants with cleft lip and/or palate often have challenges with feeding. Although guidance is provided on the feeding strategies required for liquids, little information is available regarding the initiation of solids. The American Academy of Pediatrics recommends the introduction of solid foods at 6 months of age (AAP, 2012). This generally begins with purees, followed by meltable and soft (melt/soft) solids at 7 to 9 months, and easy table foods at 9 to 12 months (Miller et al., 2020). These time frames frequently coincide with cleft-related surgical repairs and restrictions, which may delay food introduction and advancement, resulting in long-lasting feeding difficulties (Northstone et al., 2001; Coulthard et al., 2009). Our goal was to determine the timing and parent-reported feelings and challenges related to solid food provision for infants with clefts.

Methods/Description: Mothers of infants 8 to 14 months of age with cleft lip and/or palate were recruited to complete an electronic REDCap survey on feeding practices across the United States. Demographics, infant health history, and information regarding timeline, maternal comfort, and observed challenges of solid food introduction were collected. Descriptive statistics were used to describe the sample and information collected about solid food introduction. ANCOVA was performed to determine whether the initiation of solids differed between cleft types, controlling for infant age at survey completion.

Results: Mothers (n = 137) from 37 states completed the survey. The sample included infants with cleft lip (n = 18, 13%), cleft palate (n = 41, 30%), and cleft lip and palate (n = 78, 57%). Average age at solid food initiation was 5.64 ± 1.59 months for purees, 7.82 ± 1.72 months for melt/soft solids, and 9.10 ± 2.09 months for table foods. These ages did not differ based on cleft type when controlling for infant age. Ten infants had not yet had purees (10 ± 2.45 months), 28 had not begun melt/soft solids (9.25 ± 2.01 months), and 46 had not started table foods (9.35 ± 1.91 months) at survey completion. Although excitement was reported (36% purees, 33% melt/soft solids, and 42% table foods), there was also a great level of worry (54% purees, 39% melt/soft solids, and 39% table foods). Primary difficulties observed included nasal regurgitation with purees (64%) and gagging with melt/soft solids (41%) and table foods (31%). Feeding services were received by 47% of infants and were primarily provided by feeding therapists on the cleft team.

Conclusions: Infant age at solid food introduction was not found to vary based on cleft type and fell close to or within the general timelines for solid food advancement for those who had started them. Although families were excited to begin solids, worry was the prevailing feeling reported by mothers, particularly when introducing purees. Feeding providers on cleft teams should ensure education and support is provided throughout the latter half of infancy to support texture progression and decrease the risk of later feeding problems.

(1) University of Michigan, Ann Arbor, MI

Background/Purpose: Nasoalveolar molding (NAM) can be a challenge for both patient and families as well as the practitioner providing NAM. Utilizing nurse practitioners is a great way to implement NAM at any institution. Our nurse practitioner lead NAM with the assistance of a dental assistant has made NAM an option for our patient population and assists our surgeons with cleft lip and palate repairs. Implementing new treatment options always has a learning curve for the providers. Several lessons were learned during the implementation of NAM. The lessons learned include setting up a NAM program and working with families to make NAM successful.

Methods/Description: This presentation will review lessons learned over the past 5 years as our institution implemented Nurse Practitioner lead NAM through past cases and experiences. Discussion topics include an overview of implementing nurse practitioner lead NAM team model, reviewing guidelines set up to make NAM successful and challenges along the way.

(1) Children’s Hospital Los Angeles, CA

Background/Purpose: There is ample evidence-based information regarding bottle-feeding an infant with an orofacial cleft, but comparatively little information is available for introduction of solids in the unrepaired cleft population. Therefore, families are left without standard guidelines regarding introduction of solids as well as cup drinking, leading some families to delay trials of a variety of tastes and textures until after the palate is repaired. These reduced exposures of solids during appropriate developmental stages may result in oral aversions and possible delays in oral motor skills necessary for eating and drinking. Practitioners often have similar questions about the progression of feeding and would benefit from some uniform guidelines for feeding older infants and toddlers with unrepaired clefts so that they can better educate families.

Methods/Description: A review of literature was completed to compile information for introduction of solids in the typical population for the age range in question. These results were then incorporated with evidenced-based knowledge of the cleft population to create recommendations. A family-friendly handout was developed to share this information with patients in a consistent manner. Main Objective of Presentation: The purpose of this presentation is to establish guidelines for feeding of solids and open cup drinking for infants with an unrepaired orofacial cleft. Strategies to promote a positive feeding environment and a suggested list of solids (created in collaboration with a registered dietician), will be provided. Information will be included to identify patients who are exhibiting signs of feeding concerns.

(1) Phoenix Children’s Hospital, AZ, (2) Cincinnati Children’s Hospital Medical Center, OH

Background/Purpose: Standardized outcome measurement is an essential component of evaluating and improving health care services. Although cleft centers in the United Kingdom have implemented standardized evaluations of aesthetic, speech, and dental outcomes for children with cleft lip and palate; to date, there has not been widespread adoption of standardized outcome measurements at cleft centers in the United States. To overcome this gap, we sought to identify facilitators and barriers to adoption of standardized outcome measurements in cleft care.

Methods/Description: Individual semistructured qualitative interviews were conducted with 24 providers and staff representing all treatment disciplines at a single US-based cleft center and with 3 key stakeholders from the United Kingdom. Interviews explored the barriers and facilitators to implementing standardized outcome measurements, focusing on collection of standardized facial photographs, dental models, and speech evaluations at age 5. Interviews were audio recorded, transcribed, and analyzed for content using the Consolidated Framework for Implementation Research (CFIR).

(1) Oregon Health and Science University, Portland, OR

Background/Purpose: The craniofacial disorders (CFD) clinic at Oregon Health & Science Universities’ Child Development and Rehabilitation Center (CDRC) has been seeing children with deformational plagiocephaly and congenital muscular torticollis for the past 25 years by our nurse practitioner team. Deformational plagiocephaly (DP), which is the most common type of plagiocephaly, refers to asymmetric flattening of an infant’s skull due to external forces. With the successful implementation of the “Back to Sleep” campaign the prevalence of DP has increased from 5% in the 1990s to 20% to 30% presently. Clinically, DP is mostly seen as a cosmetic condition; however, there has been research that shows that these children may be at risk for developmental delay and require further screening by clinicians. Congenital muscular torticollis (CMT) is the most common type of torticollis and it can be present at birth or in the first few months of infancy. The incidence of CMT ranges from 0.3% to 2% of newborns but has been reported as high as 16%. Over the past 6 years, a physical therapist was added to the team due the high incidence of CMT and DP occurring together. During this session, we plan to discuss our interdisciplinary clinic model, specific evaluation components, and explain the observed benefits of the interdisciplinary team approach such as decreasing health care costs, reducing needing intervention, and providing a more comprehensive evaluation. Per the research, if intervention for CMT begins before 1 month of age, 98% achieve near normal range within 1.5 months, but waiting until after 1 month of age prolongs intervention to about 6 months, and waiting until after 6 months can require 9 to 10 months of intervention, with progressively fewer infants achieving near normal range. Early referral to physical therapy can lead to prevention of secondary sequelae, reduces treatment duration and avoids more invasive interventions, which in turn proves to be most cost effective. During this presentation, we plan to outline the steps we are taking to implement a prevention plan to help educate families early about the prevalence of DP and CMT. Over the past year, our clinic began to collect data by a creation of repository and utilizing our electronic medical record system. The goal of the repository is to help guide our clinic team by analyzing outcomes and trends, improve quality of care, show overall impact of our clinic program, and better match children with plagiocephaly and/or torticollis to appropriate treatment protocols. Further we hope this interdisciplinary clinic model and demonstration of a clinical repository will in turn improve continuity of care, documentation of children with torticollis, and/or plagiocephaly within our practice setting at OHSU and can be carried forward to multiple settings.

Methods/Description: The objectives are to describe the interdisciplinary clinical model and summarize clinic data collection and research agenda.

(1) University of Washington, Seattle Children’s Hospital, WA, (2) Seattle Children’s Research Institute, WA, (3) Seattle Children’s Hospital, WA, (4) University of Washington, Seattle, WA

Background/Purpose: Breastfeeding has numerous far-reaching benefits in early life. Children with oral clefts are often unable to feed exclusively at the breast, yet little is known about the mother’s early experiences with feeding-related stress and breast milk feeding that does not occur at the breast. We set out to learn about the extent, motivation, and stress related to breastfeeding and breast milk feeding of mothers of infants with a cleft lip only (CLO) or with a cleft palate only or cleft lip with palate (CP/CLP).

Methods/Description: We conducted a case series study among mothers of infants with CLO or CP/CLP. We administered a web-based survey on feeding experiences in the infant’s first 4 months of life and abstracted the chart for clinical details. Mothers of infants with CLO or CP/CLP up to 36 months of age were eligible to participate in the study. We generated descriptive statistics and compared experiences between infants with CLO and CP/CLP using t tests, Fisher exact, and χ ² tests. We stratified findings based on CLO and CP/CLP because we anticipated cleft palate involvement would have a substantial effect on feeding. Breast milk feeding was defined as feeding a baby milk from the mother’s breast using a method other than at-breast.

Results: Our sample included 75 mothers and had a response rate of 30%. Over 40% of mothers were >35 years old, had a 4-year degree or greater, and no prior live births. Among infants, 61% were male, 81% were white, 41% had a CLO, and 59% had a CP/CLP. Most mothers (84%) intended to exclusively breastfeed prior to learning of their child’s diagnosis and 77% were disappointed when they learned they may not be able to do so. Based on a 0 to 100 stress scale, feeding-related stress was higher among mothers of an infant with CP/CLP than CLO at week 1 (78.8 vs 41.5, P < .001) and at week 4 (41.0 vs 32.4, P < .001) after the infant’s birth. When the infant was 1 week old, mothers of infants with a CLO versus CP/CLP had higher prevalence of any breastfeeding (71% vs 12%) and exclusive breastfeeding (52% vs 2%). At 16 weeks, mothers of infants with CLO versus CP/CLP also had higher prevalence of any breastfeeding (61% vs 7%) and exclusive breastfeeding (35% vs 2%). The number of mothers predominantly breast milk feeding their infant decreased between 1 and 16 weeks for infants with CLO (42%-30%) and infants with CP/CLP (77%-64%). Fewer mothers of infants with CLO versus CP/CLP ever used a breast pump (74% vs 96%, P = .01). More mothers with CP/CLP versus CLO used a hospital grade pump (50% vs 26%, P = .06).

Conclusions: There are opportunities to improve access to mother’s own breast milk for infants with CLO and CP/CLP. Mothers of infants with CLO may benefit from strategies to improve duration and exclusivity of breastfeeding. Mothers of infants with CP/CLP may benefit from strategies that increase their ability to provide breast milk, including obtaining a hospital grade pump, and providing additional support throughout the first months after birth.

(1) Kaiser Permanente, Oakland, CA

Background/Purpose: The purpose of this presentation is to share how we modified our Infant Cleft Palate Clinic (ICP) to a virtual service delivery model during the COVID-19 global pandemic so that other Craniofacial Centers may also be able to use a similar format for their patients. Goals are to briefly describe the purpose of ICP Clinic, the Craniofacial specialists involved, and the preventative approach we use.

Methods/Description: The Infant Cleft Palate Clinic is held in a group/class format and includes presentations by our craniofacial clinic social worker, registered dietitian, nurse, and speech pathologist. Goals of ICP are to provide a parent support group, monitor the infant’s feeding, weight gain and development, prepare parents for cleft palate surgery, and provide strategies to facilitate speech development while increasing parents’ awareness of compensatory speech errors and how to prevent early maladaptive speech development in babbling and first words. Will discuss the virtual platform we used and how we plan to continue using this virtual platform for those families who may not be able to attend ICP in person in the future.

(1) All India Institute of Medical Sciences, Rishikesh, India

Background/Purpose: The global COVID-19 pandemic has severely affected the management of patients with cleft lip/palate. Our center is providing comprehensive cleft care to the remote hilly region of Uttarakhand, India, for the past 3 years. Before the pandemic, about 15 to 20 patients of cleft lip+/palate were operated per month. At the wake of the pandemic, there was uncertainty about how to alleviate anxiety and provide follow up care to these patients. Telemedicine has been effectively used for providing global cleft care. However, in the absence of dedicated telemedicine hubs in the remote localities we adopted a cost-effective, mobile device-based teleconsultation service to provide timely and regular expert advice to the caregivers of these patients. The present study describes our experience.

Methods/Description: We dedicated a single mobile device with 3G connection for the teleconsultation services. Initially, a whatsapp group was created which included our cleft care team members, postoperative patients, and Community Health Workers (ASHA workers) from different villages who provided information directly about any newborn or new patient with cleft deformity. Then their phone numbers were included in the group. Patients were divided into 2 groups: group 1: Newborn/New patient; group 2: Postoperative patients which included patients operated for lip only, patients where lip and palate surgery has been done and follow up of other surgeries. Algorithms were developed for each group. In group 1, feeding advice was given, taping was shown on a model and weight gain was monitored. In group 2, speech evaluation was done. Speech therapy sessions were conducted through video calls. Feedback was obtained in the common phone number.

Results: Over a period of 5 and a half months, we were able to provide cleft care support to 78 patients. Twenty newborns and 7 new patients were registered. Among the 51 patients who has already been operated at our center 9 were of cleft lip waiting for palate surgery. We found an average weight gain of 2.2 kg in boys and 1.56 kg in girls over a period of 3 months. Of 36 patients who had both lip and palate operated 21 were less than 5 years of age and 15 were more than 5 years. These 15 children had one online speech training session every month and at the end of 3 sessions were asked to send an audio clip of sentences provided by our speech therapist. We found the cost advantage of teleconsultation to be Rs 2658 by comparing the mobile data plans needed to the cost of public transport to travel from the patients residence pin code to our institute.

Conclusions: Teleconsultation is an effective stop gap method in bringing patients under the ambit of comprehensive cleft care during COVID-19 pandemic. This low-cost mobile-based telemedicine service and algorithms developed for diagnosis and follow-up can be used effectively during normal times to reduce the financial burden to the caregivers.

(1) Children’s Hospital Colorado, Aurora, CO

Background/Purpose: The presenter will discuss creating a 3-week virtual summer camp experience in replacement of the traditional summer camp program held at a camp facility. The virtual camp allows children to participate who otherwise cannot attend camp in-person due to travel distance.

Methods/Description: This urban pediatric hospital has offered for the past 20 years a summer and winter camp experience for children affected with a cleft anomaly. The COVID pandemic in 2020 led our state to ban in early spring any overnight camp programs. Our team had to reimagine what a camp experience could be virtually and decided to offer a 3-week virtual camp experience via a secured Zoom site. An email letter was sent to the camp mailing list, introducing the opportunity of Virtual Cleft Camp and inviting children to participate. Parents had to RSVP about their child’s interest to attend our virtual camp. Once parents confirmed participation, they were sent the dates and times of the planned activities. The team’s outreach coordinator partnered with the past summer/winter camp facility staff to plan the activities. Eighteen children participated in at least one of the camp activities; most participated in almost all activities. Each camper received a mailed package that contained a welcome letter, bingo cards, a box of Skittle candies labeled as bingo markers, package of word search activities, and other items. Some of the activities done over the 3 weeks included an arts and crafts project where they made a life map, Skittle Bingo, yoga, Happy Hour Word Search Races, and a scavenger hunt. Despite having a virtual camp, the traditional bonding Sharing Circle, was more poignant, as campers shared their struggles and joys. We concluded with a Cleft Camp favorite, costume dance party, done virtually with 2 teams. Each team created a dance to a song of their choice and performed for everyone. After virtual camp, each camper and parent received evaluation forms to complete. Feedback was positive from both parents and campers; they commented on meaningful connections created, especially during these challenging times. Campers want to continue to have monthly virtual gatherings. This virtual program model can be applied to other programs such as parent support meetings or social family gatherings.

(1) University of Illinois Hospital and Health Sciences System, Chicago, IL, (2) Wheaton College, Chicago, IL

Background/Purpose: Our craniofacial center, just as many health care systems, adjusted its delivery of care in response to the current pandemic in order to reduce the risk of transmitting SARS-CoV-2, the virus the causes COVID to providers and patients. Considering the craniofacial (CFC) population, patient’s treatment plan often requires at least annual follow-up visits with multiple interdisciplinary providers. Many of our patients undergo long commutes; moreover, 70% of the patients are from the underserved and diverse population and receive state-funded medical insurance. Thus, the maintenance of continuity of care is important for the CFC population as appointments are used to prepare and schedule upcoming treatments and surgeries. In response, our center has offered telehealth appointments for all our patients and families. The purpose of this study is to evaluate patient and caregiver experience of using telehealth services to determine its benefits and barriers as the center considers providing virtual care during and beyond the COVID-19 pandemic.

Methods/Description: A bilingual, English and Spanish, survey was created using Qualtrics and emailed to the caregivers of patients who recently attended a virtual appointment with any of our providers from our craniofacial team. Families without emails were interviewed via phone call. A total of 60 families were invited to complete the survey. The survey assessed the following: The feasibility of attending telehealth services, benefits and barriers to attending the appointment, patient and caregiver satisfaction with each provider, preference of in-person versus telehealth meeting, and preference of future telehealth appointments (ie, seeing all providers in one day vs separate days).

Results: Preliminary data suggest that caregivers are overall satisfied with telehealth appointments. Many participants reported liking the convenience of telehealth visits, specifically the flexibility, the shorter wait time and duration, and not needing to travel. They were overall satisfied with the time spent with the providers and indicated that they received the necessary information during the appointment. In addition, many caregivers indicated that they would rather attend virtual provider appointments on different days instead of the traditional full day of appointments at our center. No barriers or negative experiences were reported.

Conclusions: The current findings indicate that with telehealth, caregivers are experiencing a reduction of burden of care without compromising the quality of care. This is extremely valuable considering the diverse and underserved patients our center aims to serve. Although preliminary, the data suggest that telehealth visits could be beneficial even beyond the pandemic as means to increase treatment engagement and decrease barriers to appointments.

(1) Department of Orthodontics, Faculty of Dentistry, Cairo University, Egypt

Background/Purpose: Recently, the era of “digital orthodontics” is invading the profession and it seems that the nasoalveolar molding (NAM) was not missed in this new paradigm. Digital activation of the NAM appliances was introduced previously under the name of “computer aided design/nasoalveolar molding” (CAD/NAM). In this technique, a software was used to produce a series of 3D-printed models encompassing the sequence of the activation steps. On these physical models, a set of appliances were manually constructed for each patient. Virtual construction and 3-D printing of the appliance was not doable at this time. The aim of current presentation is to introduce a new 3D-printed nasoalveolar molding (D-NAM) appliance, in which both the activation steps and appliance construction were done virtually followed by 3D-printing of the appliance itself and not the models. The effectiveness of the new D-NAM appliance in improving the maxillary arch dimensions (MADs) in infants with unilateral complete cleft lip and palate (UCLP) before surgical lip repair was assessed using digital models.

Methods/Description: The new D-NAM was delivered to 14 nonsyndromic infants with UCLP. In each infant, the maxillary models were 3D-scanned into virtual models, on which segmentation and alveolar segments approximation were digitally performed using 3shape software. Approximation movements were divided into 3 models representing 3 activation steps. On each of these models, virtual appliance construction was performed followed by 3D-printing of the appliance. Nasal stent was added to the appliances of the second and third steps. Horizontal tapes were applied to approximate the 2 labial segments. All the MADs measurements were done virtually on digital models collected at the beginning (T1) and after (T2) treatment.

Results: Clinically and/or statistically significant improvements in all the measured MADs were recorded at T2 before surgical lip repair.

Conclusions: The introduced D-NAM/3D-printed appliance is a simple and efficient technique to improve the MADs in infants with UCLP before surgical lip repair.

(1) University of Rochester Medical Center, Eastman Institute for Oral Health, NY, (2) Dentistry Faculty, Feira de Santana State University, Bahia, Brazil, (3) Seattle Children’s Research Institute, WA, (4) Department of Biostatistics, University of Iowa College of Public Health, IA, (5) University of Iowa College of Dentistry, IA, (6) University of Pittsburgh, PA, (7) University of Rochester Medical Center, NY

Background/Purpose: Conduct a systematic review and meta-analysis to assess whether individuals with nonsyndromic orofacial clefts (OCs) display a higher frequency of dental anomalies (DA) compared to individuals without OCs.

Methods/Description: A literature search of indexed databases (PubMed, Cochrane, Web of Science, Embase, Scopus, and LILACS) was conducted without language restriction up to and including February 1, 2020. Cross-referencing was used to further identify articles. Several cleft teams across the United States and Europe were contacted to obtain unpublished data. The eligibility criteria were observational studies with original data that statistically compared individuals with OC without syndromes to those without OC on any type of DA in primary and/or permanent dentition. Random effects meta-analysis through the Mantel-Haenszel estimator was used to evaluate the association between OC and DA using the odds ratio (OR) with 95% CIs. The methodological quality of the studies was assessed according to the modified Newcastle-Ottawa Scale.

Results: The literature search generated 933 records, and 75 full-text articles were reviewed. Twenty-six studies encompassing 15 213 individuals, with ages between 1 and 74 years, from all the world regions fulfilled our selection criteria and were included in the meta-analysis. Previously unpublished original data from the United States, Guatemala, Hungary, Nigeria, Argentina, and the Philippines were included through collaboration with several cleft teams. In summary, 1, 19, and 6 studies had high, moderate, and low-quality assessment scores, respectively. The meta-analysis revealed statistically significant associations between OC and agenesis (OR, 14.2; 95% CI, 9.4-21.3), supernumerary teeth (OR, 5.7; 95% CI, 3.3-9.7), developmental enamel defects (OR, 5.6; 95% CI, 3.5-9.0), microdontia (OR, 14.8; 95% CI, 4.0-54.6), peg-shaped anterior teeth (OR, 12.2; 95% CI, 3.6-41.2), taurodontism (OR, 1.7; 95% CI, 1.0-2.7), tooth malposition and/or transposition (OR, 5.6; 95% CI, 2.8-11.5), tooth rotation (OR, 3.2; 95% CI, 1.3-8.2), and tooth impaction (OR, 3.6; 95% CI, 1.1-12.2). No association was observed between OC and dens invaginatus, root dilaceration, and fusion and/or germination. In general, the OR estimates of the reviewed studies exhibited significant heterogeneity (P < .0001) with the I ² statistic ranging from 0% to 95%. The funnel plot suggested evidence of publication bias.

Conclusions: Within the limitations of this study, the available evidence suggests that individuals with OC are more likely to present with a range of DA than their unaffected peers.

(1) Instituto Sírio-Libanês de Ensino e Pesquisa, Instituto de Responsabilidade Social Sírio-Libanês, São Paulo, Brazil, (2) Hospital Sírio-Libanês, São Paulo, Brazil, (3) Instituto de Responsabilidade Social Sírio-Libanês, São Paulo, Brazil

Background/Purpose: The goal of presurgical nasoalveolar molding is to align and approximate the alveolar cleft segments while at the same time achieving correction of the nasal cartilage and soft tissue deformity. These corrections are achieved by adding a nasal stent to the labial vestibular flange of a conventional intraoral molding plate as described by Grayson. However, because of small number of orthodontists trained in the procedure and the costs, its use in developing countries is quite difficult. Looking for alternative treatment protocols, in 2014 we began to use a simple nasal elevator made with a paper clip lined with plastic, and an elastic band fixed to the forehead, combined with paper tape to approximate the cleft edges, as proposed by Monasterio, and promising results have been obtained. From 2014 to 2019, a total of 115 patients with complete unilateral cleft lip and palate (CLP) or complete cleft lip and alveolus (CLA), and 46 patients with complete bilateral CLP or CLA, have been successfully treated by this protocol.

Methods/Description: Newborn patients with complete CLA and CLP presurgically treated and then submitted to cleft lip repair by one single surgeon from 2014 to 2019 were analyzed. Using Image J software, columella deviation angle and nostril width ratio were the primary outcomes of interest to evaluate patients with unilateral CLP/CLA before and after the presurgical treatment. For bilateral deformities, it was considered the nostril width and height ratio, and the nose width and height ratio before and after treatment. Secondary outcomes of interest were the number of total appointments, complications, drop off index, and for bilateral clefts it was also considered if surgical repair could be performed in 1 or 2 stages. After cleft lip repair, symmetry of the nares and the lip were evaluated for both patients who received and did not receive presurgical treatment. Cost-effectiveness analysis was performed and its long-term effect to the maxillary growth is being evaluated through the Atack index.

Results: Approximately 160 patients with unilateral or bilateral complete CPA and CLP have been treated between 2014 and 2019. Severe deformities became mild ones, and more than 90% of patients presented good/excellent results. Mean number of appointments were 5 (once a month). Comparing patients who received or did not receive presurgical treatment it was observed that 18% in the experimental group but 68% of the control group end up with some sort of asymmetry. For bilateral patients, 36% needed a 2-stage surgical repair. Facial growth analysis (N = 78) showed 68% of Atack 1+2, 15.5% of Atack 3 and 15.5% of Atack 4+5 for unilateral deformities.

Conclusions: Nasal elevator plus lip taping is an effective, reliable and simple alternative of providing presurgical molding in developing countries.

(1) University of Rochester Medical Center, Eastman Institute for Oral Health, NY

Background/Purpose: Conduct a systematic review and meta-analysis to assess the periodontal health profile of children, adolescents, and adults with cleft lip and/or palate (CL/P).

Methods/Description: A literature search of indexed databases (PubMed, Cochrane, Web of Science, Embase, Scopus, and LILACS) was conducted without language restriction up to and including August 1, 2020. Cross-referencing was used to further identify articles. The eligibility criteria were original observational studies that statistically compared the periodontal profile of individuals with CL/P to those without CL/P. The periodontal health assessment included plaque index (PI), gingival index (GI), bleeding index (BI), periodontal pocket depth (PPD), and clinical attachment loss (CAL). Random effects models with inverse variance weighting were applied to obtain an overall estimate of the effect size. Heterogeneity across studies was investigated using the χ ² test and I ² statistic. Quality assessment was performed based on Joanna Briggs criteria.

Results: The literature search generated 851 records, and 43 full-text articles were reviewed. Nineteen studies comprising 2397 individuals, with age ranging between 2 and 28 years, fulfilled our selection criteria. The meta-analysis revealed significant difference in mean PI scores (mean difference [MD] = 0.34, 95% CI = 0.23-0.45) and GI scores (MD = 0.50, 95% CI = 0.24-0.77) between individuals with and without CL/P. Significant heterogeneity (P < .0001) was observed among the reviewed studies. More bleeding on probing was detected among individuals with CL/P compared to control as well as a slight increase in PPD and CAL; however, such an increase may have little clinical significance. Limited available data from long-term follow-up studies, up to 25 years, suggest that periodontal parameters can worsen over time in the absence of specialized supportive periodontal therapy. Optimal oral hygiene may be hindered by the anatomical defect of the cleft, collapse of the maxillary segments, long-term surgical, orthodontic and prosthodontic treatment, residual scar tissue, misaligned teeth, and fear of brushing the cleft area. Caregivers are usually preoccupied with satisfying the medical, surgical, nutritional, and speech development demands of the cleft population; consequently, preventive dental care may be given a low priority.

Conclusions: Individuals with CL/P are more likely to present with more plaque accumulation and gingival inflammation, and a slight increase in PPD and CAL compared to unaffected peers. Limited available data from long-term follow-up studies suggest that periodontal parameters can worsen over time in the absence of specialized supportive periodontal therapy. As age is positively related to periodontal disease progression and periodontal destruction can intensify in this population subset, clinicians should consider targeting preventive dental care from an early age and reinforcing daily oral hygiene practices.

(1) Boston Children’s Hospital, MA, (2) Duke University, Durham, NC, (3) MedStar Georgetown University Hospital, Washington, DC

Background/Purpose: Value-based health care reform requires assessment of outcomes and costs of medical interventions. In cleft care, presurgical infant orthopedics (PSIO) is still being evaluated for clinical benefits and risks; however, cost of these procedures has been largely ignored. This study employs robust accounting methods to quantify the cost of providing 2 types of PSIO: Latham and nasoalveolar molding (NAM).

Methods/Description: This is a prospective study of patients with nonsyndromic cleft lip and/or palate (CL/P) who underwent PSIO from 2017 to 2019 at 2 academic centers. Costs were measured using time-driven activity-based costing (TDABC). Personnel costs, facility costs (operating room, clinic, and inpatient ward), and equipment costs were included. Travel expenses were incorporated as an estimate of direct costs borne by the family, but indirect costs (eg, time off from work) were not considered.

Results: Twenty-three patients were treated with Latham and 14 with NAM. For Latham, average total cost was $7553/patient ($1041 for personnel, $637 for equipment, $4871 for facility, and $1004 for travel over 6.5 visits). Unilateral and bilateral costs were $6891 and $8860, respectively. For NAM, average cost totaled $2541 ($364 for personnel, $151 for equipment, $300 for facility, and $1726 for travel over 13 visits); $2120 for unilateral and $3048 for bilateral treatment.

Conclusions: The major difference in cost is attributable to operative placement of the Latham device. Travel cost for NAM is often higher due to frequent clinical encounters required. Future investigation should focus on whether outcomes achieved by PSIO justify the $2100 to $8900 expenditure for these adjunctive procedures.

(1) Tokyo Medical and Dental University, Japan

Background/Purpose: Digital impression confers several advantages since it is widely used in dentistry. The conventional impression method has the risk of aspiration of the impression materials, impaction of the material to the complicated structure of the defect, or damaging the frail tissue of the defected site. Powder-less intraoral scanning is one of the ideas that can be used to overcome these problems. For patients with cleft lip and palate who have tissue deficiency, it would be an optimized choice. However, the possibility of actual use of this approach is still unknown. The purpose of this study was to show the possibility of the data acquisition using powder-less intraoral scanner on patients with cleft lip and palate.

Methods/Description: Fifteen patients with cleft lip and palate who had tissue deficiency were participated in this study (7 females, 8 males; mean age 53.2 years). Study procedures and objectives were explained to the patients, and all provided written informed consents were obtained. Digital impressions of maxillae for all 15 patients were acquired using powder-less intraoral scanner (TRIOS 3, 3shape). The maxillary scanning process initiated occlusally on one side from the posterior area toward the anterior teeth and then moving to the other posterior side area on a zigzag manner. Once all the remained teeth were scanned, the probe was moved to the mucosal and palatal area including the defected area with a circular motion. The 3-dimensional data were observed using related software, and colored digital images were also saved.

Results: All the scans were completed without errors. Remained teeth and part of palate were well scanned in all 15 patients. It was possible to scan the defect part in maxillae of most patients although the scanning process was sometimes slow when the probe was near to the defect part. For some patients, some defect parts especially deeper parts were not acquired.

Conclusions: The results of missing data of maxillae were likely due to the restricted size and angle of the scanner probe. The vestibule was shallower near the cleft side teeth and the residual scarring might also be influenced the data acquisition. In conclusion, digital impressions were acquired using a powder-less intraoral scanner on the shallow defect of the maxilla. In the viewpoint of prosthesis fabrication, it is still necessary to combine the digital technique with the conventional steps. Improved intraoral scanning technology will increase the possibility of its use for patients with cleft lip and palate in the future.

(1) Tokyo Medical and Dental University, Japan

Background/Purpose: As patients with cleft have unique morphology on their maxillofacial region, prosthodontist must pay attention to the function, esthetics and sustainability, when the treatment is planned. Telescopic denture has been introduced and widely used since 1930s. Because of distinguished advantages of esthetics and hygiene, it has been used to habilitate patients born with cleft lip and palate (CLP). However, fabricating telescopic denture demands high dental technique skills owing to complex laboratory procedures, and to refabricate the telescopic denture is even more difficult. This report describes a patient using telescopic denture for 30 years. The patient’s intraoral condition became worse and molars were lost resulted in poor masticatory function. An overdenture was selected as recovery treatment on existing telescopic crowns. Here, we describe techniques of fabricating overdenture under the collaboration between prosthodontists and dental technicians on existing telescopic crowns in a female patient with bilateral CLP.

Methods/Description: In 2014, a 55-year-old patient with bilateral cleft palate presented at clinic for Maxillofacial Prosthetic at Tokyo Medical and Dental University (TMDU) dental hospital with a chief complaint of poor mastication. The history revealed that patient has been treated with multidisciplinary team and was referred to maxillofacial prosthetic clinic in 1983. Telescopic denture was delivered. As 30 years progressed, the oral condition changed due to dental caries and periodontitis. Teeth 15 and 16 were extracted and current telescopic denture was ill fitted. Overdenture on the existing telescopic crowns was selected as a treatment option. Fabrication of the denture (1) Discuss the denture design with a dental technician at chairside. (2) Make impression with irreversible hydrocolloid impression material and fabricate a working cast. (3) Fabricate a metal framework using metal housing with round mesh on the telescopic crown and clasps on the posterior teeth and try with artificial teeth. (4) Process the denture with acrylic and deliver.

Results: Prosthodontist, as a member of multidisciplinary team, must follow the long-term observation. In this case, after the use of telescopic denture for over 30 years, the intraoral condition was changed. Impairment of masticatory function was caused and to fabricate new prosthesis was an option. With the cooperation of highly skilled dental technician, we could deliver an overdenture with good retention, stability, and esthetic, which allow patient to switch to use new overdenture from telescopic denture without any trouble and intermission.

Conclusions: The overdenture fabrication under the collaboration between prosthodontist and dental technician on existing telescopic crown allowed the patient with cleft to improve mastication without any trouble and intermission used.

(1) Institute of Dental Studies and Technologies, Modinagar, Uttar Pradesh, India

Background/Purpose: Constricted maxilla, with a clinical presentation of anterior and posterior crossbite is one of the classical symptoms seen in patients with unilateral cleft lip and palate (UCLP) (Jain et al., 2015). Expansion can be achieved by either using rapid maxillary expansion (RME) or slow maxillary expansion (SME). SME promotes more physiological adjustment to sutural separation lowering the risk of relapse and producing greater stability. The Quad Helix and NiTi Palatal expander are 2 commonly used appliances in this regard; however, to date a comparative evaluation has not been done.

Methods/Description: This prospective study aimed to evaluate and compare the effects of quad helix and NiTi palatal expander (Npe2) appliances in all 3 planes of space using data from cone beam tomography in patients with UCLP presenting in the mixed dentition stage. The study was conducted on 44 patients with complete UCLP, between the age groups of 8 to 12 years They were equally divided into 2 groups: quad helix (22 patients) and NPE 2 (22 patients). The study design was approved by the ethical committee of the institute (IDST/IERBC/2017-20/02), and informed consents were signed by the legally responsible guardians before enrolling the participants. The sample size was calculated to a power of 80% with an α error of .05 and Cohen effect size of 0.6. Calculations suggested a sample size of 25. Patient recruitment started on December 2017 and ended on December 2019. Preoperative and postoperative CBCT scans were obtained using NewTom GiANO CBCT machine delivering high-resolution images with a large field of view (FOV) of 17 × 13.5 cm. Patient position was standardized using light markers, with transverse plane parallel to Frankfort horizontal line of the machine. (Tube voltage: 60-90 Kv, Tube current: 2-15 Ma and frequency: 140 kHz, voxel size of 0.30 mm). The CBCT scans were evaluated by importing the DICOM images in NNT software (version 1.010).

Results: Paired t and independent t tests were used to detect differences (intra and intergroup, respectively). Significant changes in the intermolar and intercanine width postexpansion were seen (4.2 mm and 5.5 mm mean change for NPE2 and QH group, respectively; the difference was statistically significant (P < .05); however, the upper molar tipped more (5.13°) in the QH group which was statistically significant (P < .05). There was a significant (P < .05) bone level change at the buccal and palatal region 6 mm below the CEJ (0.6 mm decrease and 0.8 mm increase, respectively) in the QH group with no other detrimental effects noticed, nor any other significant intergroup or gender differences.

Conclusions: Both appliances seem to be effective in expanding the constricted UCLP arches; however, more expansion is achieved using Quad Helix at the expense of tipping the anchor tooth potentially affecting the bone levels of the same.

(1) Institute of Dental Studies and Technologies, Modinagar, Uttar Pradesh, India

Background/Purpose: To assess the differences between 2 methods of presurgical infant orthopedics, that is, the modified Grayson technique and a new technique using clear aligners in complete unilateral cleft lip and palate patients. CLP management begins early on in the infancy stage (as early as 6 weeks old), in order to allow for the manipulation and molding of the malleable dentofacial tissue due to the high levels of maternal estrogen still persisting in the infant. The technique has evolved over the years and is now termed as presurgical infant orthopedics (PSIO) with the literature iterating several process and techniques that use this physiological facet to initiate the process of reconstruction, with the modified Grayson approach supposedly gaining worldwide acceptance and practice over the course of time (Cuzalina A & Jung C., 2016; Grayson BH et al., 1993). The authors had documented and submitted a case series that documents one of the world’s first PSIO cases to be treated by CAT (Batra et al, 2019).

Methods/Description: This prospective comparative cohort study which was done on 30 patients which were divided into 2 groups, that is, group A (modified Grayson technique) and group B (clear aligner trays—Compass 3D; Belo Horizonte) consisting of 15 patients each. Scanned cast measurements were taken before and after treatment and assessed using a laser scanning machine and 3-dimensional software (C500 SOLUTIONIX and GOM INSPECT, respectively). This was supplemented with standardized anterior posterior, worm’s-eye view, and profile photographs. Furthermore, the number of visits for each patient group were also tabulated and assessed. The differences were compared using independent “t” test and Mann-Whitney U test for the respective parameters. The study sample was calculated using the software Medcalc 19.1 (MedCalc Software Ltd) software to a power of 80% and α of .05 with a 0.6 effect size with the reduction in the soft tissue cleft gap to be the primary outcome, and a 1-mm mean difference was considered significant which gave the minimum sample number per group to be 12; however, to further increase the power, 15 patients were taken in each group; these data were derived from studies previously undertaken in the department along with previously published literature (Monasterio et al., 2013; Chang et al., 2014).

Results: Both group A and group B showed similar posttreatment changes, with no overt significant differences. However, there was a statistically significant difference in the number of visits (P < .05) with group B having much lower visits to the center as compared to group A. The posttreatment values matched previously established growth reference values. The analysis of photographic measurements showed improved nasal asymmetry in both groups without any significant difference.

Conclusions: Since there was no difference in the treatment results between the 2 methods along with a reduction in the number of required patient visits, PSIO via clear aligners.

(1) Hospital for Rehabilitation of Craniofacial Anomalies, USP, Sao Paulo, Brazil, (2) Department of Orthodontics, Hospital for Rehabilitation of Craniofacial Anomalies, USP, Sao Paulo, Brazil, (3) Hospital for Rehabilitation of Craniofacial Anomalies, Sao Paulo, Brazil, (4) Department of Speech, Language and Hearing Disorders, Bauru School of Dentistry, University of Sao Paulo, Brazil, (5) Bauru Dental School, University of Sao Paulo, Brazil

Background/Purpose: To longitudinally evaluate, by cephalometric analysis, the craniofacial growth in 349 individuals with CUCLP, and the influence of techniques for cheiloplasty (Spina (S) vs Millard (M)) and 1-stage palatoplasty (Furlow (F) vs von Langenbeck (VL)), and age at accomplishment of palatoplasty (9-12 months vs 15-18 months). The study also analyzed the influence of the 4 operating surgeons on craniofacial growth, and the percentage of cases with orthodontic-surgical prognosis in the total sample.

Methods/Description: The study analyzed 698 lateral cephalograms on the software Dolphin Imaging 11.8, with angular/sagittal measurements of 349 individuals with CUCLP registered at the Hospital for Rehabilitation of Craniofacial Anomalies, at phases T1 (mixed dentition before orthodontic intervention, between 6 and 10 years old) and T2 (permanent dentition over 14 years old). The results were analyzed by descriptive statistics and multivariate analysis. The percentage of cases with orthodontic-surgical prognosis was calculated by association with cephalometric measurements using the χ ² test.

Results: In T1, the number of cases with ANB<0 was higher than in T2. After orthodontic–orthopedic management, in T2, the ANB>0 increased due to more anterior maxillary positioning and clockwise mandibular rotation. In T1, there was predominance of SNA80° increased, while SNB>80° was reduced, with tendency to clockwise mandibular rotation with downward and backward positioning. In T2, 9.73% of individuals presented probable surgical-orthodontic prognosis. The cheiloplasty technique did not influence the SNA, SNB, or ANB in T1 and T2. The palatoplasty technique did not influence the SNA and SNB in T1 and T2. However, the VL technique had more negative influence on ANB than the F technique in T1, while in T2 the ANB was similar for both. The SNA and SNB did not present statistical difference between subgroups operated by S-F, S-VL, M-F, and VL techniques, both in T1 and T2. Only the ANB in T1 presented statistical difference between techniques, with less favorable results for M-VL (−2.58) compared to M-F (−0.58). In T2, after orthodontic/orthopedic treatment, all subgroups had the sagittal relationship corrected and presented mean angles ANB>+3° (similar to individuals without CLP) without statistical difference. Palate repair by the single-stage technique, both early (9-12 m) and late (15-18 m), did not show statistical difference in SNA and SNB operated by different techniques, in T1 and T2. Only in T1, the ANB presented more negative discrepancy in the group operated earlier (ANB = −2.2) than the group operated later (ANB = −1.3, P = .03), which was corrected in T2 after orthodontic and orthopedic management. The mean of both groups changed from negative ANB to ANB>3.5. There was no statistical difference in SNA between the 4 surgeons in T1 and T2.

Conclusions: From the total sample, in T2, 9.73% had ANB from −7° to −1° with probable need for orthodontic-surgical treatment, and 70% had ANB>2°.

(1) Hospital for Rehabilitation of Craniofacial Anomalies, Sao Paulo, Brazil, (2) Bauru Dental School, University of São Paulo, Brazil, (3) Department of Orthodontics, Hospital for Rehabilitation of Craniofacial Anomalies, USP, Sao Paulo, Brazil, (4) Hospital de Reabilitação de Anomalias Craniofaciais, Universidade de São Paulo, Brazil, (8) USP, Sao Paulo, Brazil

Background/Purpose: Primary surgeries interfere, to a variable extent, on the maxillary growth and development in patients with cleft lip and palate (CLP). The aim of the present study was to evaluate the influence of 2-stage palatoplasty—“2Stg” (first stage: lip repair + nasal ala + hard palate with vomer flap at 3-6 m; second stage: soft palate at 12-18 m) on the maxilla development of children with unilateral cleft lip and palate (UCLP), without previous orthodontic intervention. The 2Stg group was compared to a control group of children submitted to 1-stage palatoplasty (1Stg), with simultaneous repair of hard and soft palate (12-18 m). Both protocols refer to the current (2Stg) and prior (1Stg) protocol of the Hospital for Rehabilitation of Craniofacial Anomalies (HRAC/–USP). The influence of the technique, expertise of the plastic surgeon, and timing of lip, hard palate, and soft palate closure was verified and compared.

Methods/Description: A total of 349 plaster models of patients in complete deciduous or mixed dentition phase (2Stg: 169; 1Stg: 180) were categorized by 3 orthodontists as to severity of maxillomandibular discrepancy using the 5-year-old occlusal index.

Results: The intraexaminer reliability was very good (0.81-0.98) and interexaminer reliability varied from satisfactory to very good (0.56-0.83). The mean occlusal index of 2Stg and 1Stg groups was 2.77 and 3.03, respectively. The variability of the mean index between surgeons varied from 2;38 to 3.2 (2Stg) and 2.91 to 3.2 (1Stg). There was significance in the distribution of G5 index (P = .002) in which the study group (2Stg) presented more cases of index 1 (10.65%) and fewer cases of index 5 (1.18%), while the control group (1Stg) presented 11.11% of index 5. The grouped index showed a higher percentage of good to excellent results (indexes 1 + 2 = 36.68%) and lower percentage of poor index with probable need for orthognathic surgery (indexes 4 + 5 = 25.44%) compared to the 1Stg group (1 + 2 = 31.67%; 4 + 5 = 30.56%), though not statistically different.

Conclusions: Individuals who underwent 2Stg palatoplasty had lower percentage of severe maxillomandibular discrepancy (index 5) compared to 1Stg palatoplasty; however, it is not possible to state that the 2Stg protocol is better than the 1Stg protocol, considering that there was no statistical difference between the mean occlusal indexes. The percentage distribution of occlusal indexes showed considerable variation between surgeons. Surgeons operating in both groups (1 Stg and 2 Stg) had similar mean occlusal indexes in both groups. Future studies with longer follow-up should be carried out to monitor the growth of children in the present sample.

(1) Hansjörg Wyss Department of Plastic Surgery, NYU Langone Health, New York, NY, (2) New York University Langone Health, NY, (3) Oregon Health and Science University, Portland, OR, (4) New York University Langone Medical Center, NY

Background/Purpose: Nasoalveolar molding (NAM) was developed to decrease the severity of cleft lip/nasal deformity and improve the alignment of alveolar segments in order to ease surgical repair. Numerous studies have shown that NAM can benefit patients during their cleft care; however, it requires caregivers to actively participate in patient care in order to successfully complete treatment. The purpose of this study is to determine the burden of care of NAM for patients and caregivers seeking treatment at our institution.

Methods/Description: This is a retrospective cohort study of patients with unilateral or bilateral cleft lip and/or palate (CLP) treated with NAM at our institution, a tertiary referral center specializing in cleft and craniofacial care, between 2012 and 2019. Burden of care variables were assessed for patients and caregivers participating in NAM treatment. All patients with complete medical records were included in the study. Assessment of physical burden of care included duration of NAM treatment, distance traveled, and evaluation of orthodontic visits, such as number of scheduled, unscheduled, cancelled, and unattended visits.

Results: A total of 184 patients (63% male, 72% unilateral cleft lip, 82% with cleft palate, 95% nonsyndromic) met inclusion criteria. The mean duration of NAM was 14 ± 4 weeks. Patients and caregivers had a mean of 16 ± 5 scheduled orthodontic visits and 1 ± 1 unscheduled visits made urgently to assess any caregiver concerns. Caregivers were found to cancel 1 ± 2 visits and rarely did not show up for appointments (0.3 ± 0.9). In total, a mean of 16 ± 6 visits were completed (scheduled and unscheduled), and 1 ± 2 were missed (cancelled and unattended). Patients and caregivers had a mean travel distance from their home to treatment center of 23 ± 29 miles, resulting in a mean travel time of 39 ± 31 minutes. When stratifying by cleft lip type, patients with bilateral cleft lip had significantly longer duration of NAM treatment (18 ± 5 vs 13 ± 3 weeks; P < .001) and greater number of scheduled visits (19 ± 6 vs 14 ± 4; P < .001), but had similar need for unscheduled visits due to caregiver concerns (0.76 ± 1.3 vs 0.74 ± 1.4; P = .930).

Conclusions: NAM is a finite orthodontic intervention that requires caregivers to actively participate in patient care for approximately 3 months of their early life. The decision to pursue NAM as an intervention to improve surgical outcomes should be considered alongside the burden of care on caregivers to complete treatment. The results of this study will help our institution appropriately counsel families and inform decision-making. Future studies should explore barriers to completing NAM treatment and implement strategies to improve access to care.

(1) University of Oxford, United Kingdom, (2) MD Anderson, Houston, TX, (3) McMaster University, Hamilton, Ontario, Canada, (4) Hospital for Sick Children, University of Toronto, Ontario, Canada, (5) The Spires Cleft Centre, Oxford, United Kingdom

Background/Purpose: Computerized adaptive testing (CAT) delivers short, accurate, and personalized versions of the CLEFT-Q, a patient-reported outcome measure (PROM) for people with cleft lip and/or palate. Regression trees (RTs, machine learning algorithms) may be able to make these assessments even shorter and/or more accurate, by harnessing clinician-reported data in addition to PROM responses. This is an innovative approach. We compared the accuracy of CAT and RT techniques, hypothesizing that RTs would achieve better performance by including additional non-PROM data.

Methods/Description: We used clinician-reported data (eg, cleft type, planned future treatments, etc), and patient-reported item responses from the CLEFT-Q field test (2434 participants) to train RTs that comprised binary decision nodes. We compared their prediction accuracy to CATs of similar length. Accuracy was assessed through correlation between shortened CAT or RT estimates and full-length scores, root mean squared error, and 2-tailed Wilcoxon signed-rank test comparing mean absolute error.

Results: RTs generally used PROM item responses to create decision nodes, rather than clinician-reported variables, contrary to what was hypothesized. Most trees demonstrated poorer accuracy than CAT comparators (P < .001). Examining the model structures demonstrated the greater resolution of CATs that used all 3 or 4 possible item responses, rather than the binary decisions made by the RTs.

Conclusions: When predicting CLEFT-Q scores using RTs, PROM item responses were more informative than clinician-reported data. Conventional RTs comprising binary nodes risk underfitting and had poorer performance than CATs here. Future work may investigate RTs better able to harness polytomous PROM responses.

(1) University of Oxford, United Kingdom, (2) MD Anderson, Houston, TX, (3) McMaster University, Hamilton, Ontario, Canada, (4) Hospital for Sick Children, University of Toronto, Ontario, Canada, (5) The Spires Cleft Centre, Oxford, United Kingdom

Background/Purpose: The CLEFT-Q is a patient-reported outcome measure (PROM) with 7 subscales that assess facial appearance in cleft lip and/or palate. We propose a novel model to describe conceptual relationships between these subscales. In the model, the subscales for appearance of the nose, nostrils, teeth, jaw, lips, and cleft lip scar all reflect a second order construct: overall appearance of the face, which is independently reflected by the face subscale items directly. We test our hypothesis using structural equation modeling (SEM).

Methods/Description: We used the lavaan package in R to build clinical concept-driven SEM models, to investigate relationships between questions in the CLEFT-Q appearance scales. We used modification indices to judiciously refine our model alongside clinical logic. We included 640 participants from the international CLEFT-Q field test and tested the reproducibility of our results by repeating the analysis with a further 452 participants.

Results: Our model demonstrated excellent fit to the data (comparative fit index 0.999, Tucker-Lewis index 0.999, root mean square error of approximation 0.036, and standardized root mean square residual 0.036). The repeat analysis confirmed excellent fit of the hypothesized model.

Conclusions: Our structural model, which was based on a clinical understanding of appearance in orofacial clefting, is supported by data from the CLEFT-Q field test. This is evidence that the CLEFT-Q measures facial appearance in a way that is consistent with our clinical understanding. In future, multidimensional computerized adaptive tests could build on this model to accurately predict CLEFT-Q scores from fewer, more focused questions.

(1) The University of Manchester, England

Background/Purpose: This study aimed to assess the effect of dental restorative treatments on the oral health-related quality of life (OHRQOL) of adults with cleft lip and/or palate (CLP) and compared to adults with no orofacial cleft.

Methods/Description: The study was composed of a sample of 70 adult participants who received and completed dental treatments at the University Dental Hospital of Manchester. Thirty-five participants with CLP (CLP group) and 35 participants with no orofacial cleft (control group) agreed to participate. Each participant completed the Oral Health Impact Profile (OHIP-49) with no missing data. The OHIP-49 data were analyzed using the Mann-Whitney U test, and a P value <.05 was considered as statistically significant.

Results: Adults with CLP (23 females and 12 males) had reported higher scores than adults with no orofacial cleft (21 females and 14 males) in all the 7 subscales of the OHIP-49. The highest mean scores in the CLP group were in the psychological discomfort subscale (mean scores of 2.28), followed by psychological disability (mean scores of 1.66); however, these results were not statistically significant (P > .05) except in physical disability (mean scores of 1.22, P = .017) and social disability (mean scores of 0.93, P = .046).

Conclusions: Adults with CLP reported poorer OHRQOL in all of the 7 subscales of OHIP-49 compared to the control group; however, these were only statistically significant in the physical and social disability subscales. Further studies with a larger population are needed to confirm the result of this study.

(1) Children’s Hospital Los Angeles, CA, (2) Seattle Children’s Hospital, WA, (3) Shriners Hospitals for Children, University of Illinois, Chicago, IL, (4) Children’s Hospital of Philadelphia, PA, (5) University of North Carolina, School of Medicine, Chapel Hill, NC, (6) University of Washington, Seattle, WA

Background/Purpose: Craniofacial microsomia (CFM) is characterized by underdevelopment of the ear(s) and mandible. School-aged children with CFM can be at risk for learning difficulties compared to peers; however, less is known about early neurodevelopment and behavior. This study examines development in preschoolers with and without CFM as part of the multisite CFM: Longitudinal Outcomes in Children Pre-Kindergarten (CLOCK) study.

Methods/Description: Preschoolers with CFM (n = 92) and without CFM (n = 76) were evaluated at an average age of 38 months (SD = 1.9). Assessments were completed in English (73%), Spanish (8%), or both languages (19%) using the Bayley Scales of Infant and Toddler Development—Third Edition (Bayley-III) and the Clinical Evaluation of Language Fundamentals Preschool—Second Edition (CELF P-2). Parents completed the Child Behavior Checklist (CBCL). Medical information was gathered from caregivers and chart review. Scores were converted to z-scores based on the controls’ score distribution. Group differences were estimated using linear regressions adjusted for age, sex, socioeconomic status (SES), study site, race, and test language. In categorical analyses, a delay was defined as ≥1 SD below the mean.

Results: Most participants were male (55%), white (68%), and Latino (46%), and a range of family SES was represented. The phenotype of cases was categorized using OMENS as well as microtia with mandibular hypoplasia (51%), microtia only (29%), and other CFM-features (20%). Nearly 70% of cases received intervention services and 81% had hearing loss. After adjustment for covariates, scores were similar for children with and without CFM on the Bayley-III subtests and on the CELF P-2 receptive language subtests of Sentence Structure and Basic Concepts. Children with CFM scored significantly lower than controls on the CELF P-2 expressive language subtest of Recalling Sentences (β = −.40, P = .04) and receptive language subtest of Concepts and Following Directions subtest (β = −.57, P = .01). Among cases, those with microtia only and with other CFM features had lower language scores. An area of delay was present in 37% of children with CFM compared to 15% of controls. There were no group differences on the CBCL composite scales; however, there were more parental concerns reported for male cases.

Conclusions: On average, development in preschoolers with CFM was comparable to controls, with the exception of selected language measures. In categorical analyses, children with CFM were more likely than controls to have an area of delay. Relative to previous cohorts, the CLOCK sample had fewer participants with more severe CFM presentations and a high rate of early intervention, which may attenuate differences. In addition, learning/developmental differences may become more pronounced over time. Providers should monitor for language, learning, and behavioral concerns as children with CFM enter school and assist in advocating for appropriate school and community services.

(1) University of Iowa Children’s Hospital, IA, (2) Shriners Hospitals for Children Chicago, IL, (3) Nationwide Children’s Hospital, Columbus, OH, (4) Shriners Hospitals for Children, University of Illinois at Chicago, IL, (5) Children’s Medical Center, Dallas, TX, (6) The Hospital for Sick Children, Toronto, Ontario, Canada, (7) Lancaster Cleft Palate Clinic, PA

Background/Purpose: Research on iCL/P has demonstrated a higher risk of learning concerns. Extensive work has evaluated biological factors (eg, sex and cleft type) and neuropsychological patterns (eg, phonological awareness, auditory working memory). However, very little work has addressed the potential relationship to school environment, psychosocial functioning, or school services.

Methods/Description: A total of 391 children, ages 8 to 10 years old (192 male; 59 iCLO, 72 iCPO, and 260 iCLP) and their guardians were recruited during a regular clinic visit to complete questionnaires designed to evaluate psychological functioning. Participants were recruited from 6 centers across North America from August 2016 through September 2019. Measures included Academic Functioning (ABAS-III and CBCL subscores), General School Functioning (Cleft-Q and PedsQL subscores), Global Psychosocial Functioning (PedsQL and CBCL total scores), and guardian report/chart review of diagnoses and services received.

Results: Academic Functioning was predominantly in the average to high average range, although boys had significantly lower functioning than girls (F _1,368 = 5.355, P = .021). This difference was driven by boys with iCPO (38% were at or below the 16th percentile). Of the total sample, 33% fell within the at risk range for academics. Academic Functioning was significantly correlated with ratings of General School Functioning on the PedsQL (r range of 0.144-0.326). Academic Functioning was also correlated with ratings of Global Psychosocial Functioning on the PedsQL and CBCL (r range of 0.127-0.272). Thirty-four (9%) participants had a formal diagnosis of a learning or language disability; 41% of these children had co-occurring autism (3%), mood (9%), behavior (12%), or ADHD (38%) diagnoses. Seventy-one percent of those with a formal diagnosis were identified as at risk by parent ratings. However, only 19% of those rated at risk by parents had a formal diagnosis. Finally, 67% of all participants had a history of receiving either or academic or language services; the most common was speech therapy (51%). Three quarters of these services had been referred by the cleft team. Among those rated as at risk, 74% had a formal IEP; goals included speech therapy (66%), reading (29%), math (21%), and writing (8%).

Conclusions: Parent ratings of academic functioning for the majority of children with iCL/P are within the average range or higher. Results support previous research that boys, particularly those with iCPO, are at a higher risk for poorer performance. Academic functioning was correlated with psychosocial functioning. Of those at risk, few have a formal language or learning disorder diagnoses. Despite lower diagnosis rates, the majority of those rated at risk were receiving services through the school system. Screening of academic performance should continue and guardians should be guided in risk factors and ways to advocate for their children within the school system.

(1) Children’s Mercy Hospital, Kansas City, MO

Background/Purpose: In 2010, suicide became the second leading cause of death among teenagers. However, it’s been shown that many people who die by suicide have seen a health care provider in the months prior to their death. The Joint Commission issued National Patient Safety Goal #15, which calls all hospitals to identify safety risks inherent in its patient population by identifying patients at risk for suicide. To date, most hospitals have not adopted methods for suicide screening (Inman et al., 2019). Furthermore, patients diagnosed with a clefting condition have been found to have a significantly higher risk of suicide. This study describes the preliminary data for the implementation of a suicide screening program within a Cleft Clinic.

Methods/Description: An IRB approved, retrospective study of the implementation of suicide screening in children with cleft lip and/or palate at a tertiary pediatric institution.

Results: In January 2019, universal suicide screening was implemented for all patients aged 12 years or older presenting to the Cleft Clinic for tertiary care at a pediatric hospital. The Ask Suicide-Screening Questionnaire (ASQ) is used to identify patients who are currently at risk of suicide. The ASQ is a validated tool developed for use in emergency departments, inpatient and outpatient settings within pediatric institutions. Two hundred and sixty-two patients were screened for suicide risk. Of these patients, 14 (5.3%) have been identified as at risk for suicide. No patients have been identified as acutely positive. All positive screens were identified by nursing staff and addressed by a social worker through mental health assessment and utilization of the C-SSRS. As with all new processes, incorporation into standard clinic flow can take some time. Over time, we have been able to screen more eligible patients, as we incorporated standard work.

Conclusions: In response to the increasing incidence of suicide among teenagers, the implementation of suicide screening at outpatient visits can be critical in identifying at risk patients. This is further supported by the identification of 14 patients at a Cleft Clinic visit within 12 months of the implementation of the screening process.

(1) McMaster University, Hamilton, Ontario, Canada, (2) Hospital for Sick Children, University of Toronto, Ontario, Canada, (3) Great Ormond Street Hospital For Children NHS Trust, London, United Kingdom, (4) Sobrapar Hospital, Sao Paulo, Brazil, (5) University of Pittsburgh School of Medicine, PA, (6) University Medical Center Utrecht, the Netherlands, (7) Children’s Health Ireland at Temple Street, Dublin, Ireland, (8) University of British Colombia, Vancouver, (9) Great Ormond Street Hospital, London, England, (10) Western University, London, Ontario, Canada, (11) CHEO, Ottawa, Ontario, Canada, (12) Boston Children’s Hospital, MA, (13) Queensland Children’s Hospital, Brisbane, Australia, (14) Oxford University Hospitals NHS Foundation Trust, United Kingdom, (15) The Spires Cleft Centre, Oxford, United Kingdom, (16) Alder Hey Children’s Hospital, Liverpool, England, (17) Oxford University Hospitals, United Kingdom, (18) Memorial Sloan-Kettering Cancer Center, New York, NY, (19) Modus Outcomes, Letchworth Garden City, United Kingdom

Background/Purpose: Patient-reported outcome measures (PROMs) are important for capturing the patient perspective. These tools can aid in treatment evaluation and clinical decision-making. The CLEFT-Q is a PROM that includes 12 independently functioning scales that measure appearance (face, nose, nostrils, teeth, lips, jaws), health-related quality of life (psychological, social, school, speech distress) and speech function, and an eating/drinking checklist. This PROM was originally develop for patients with cleft lip and palate, however, in a previous study, the content of the CLEFT-Q scales was found to be valid for patients with other craniofacial conditions. The current aim is to examine the psychometric properties of the CLEFT-Q scales internationally in patients 8 to 29 years of age with a broad range of facial conditions.

Methods/Description: Patients aged 8 to 29 years with a facial condition completed relevant CLEFT-Q scales between October 2016 and December 2019. Rasch measurement theory (RMT) analysis was used to examine differential item function (DIF) between the original CLEFT-Q sample and new craniofacial sample (n = 2036). Properties of the scales were also examined in a combined cleft and craniofacial sample (n = 4743) including test of item fit, targeting, DIF, and reliability.

Results: When the cleft and noncleft samples were compared, DIF was observed for 23 CLEFT-Q items. However, when items with DIF were split by sample, correlations between the original and split person locations DIF had negligible impact on scale scoring (correlations >0.995). In the combined sample, RMT analysis led to retention of original content for 10 CLEFT-Q scales, modification of the Teeth scale, and the addition of an Eating/Drinking scale. Data fit the Rasch model for 11 of the 12 CLEFT-Q scales (exception School, P = .04). Person separation index, and Cronbach α values met criteria.

Conclusions: The scales described by this study can be used to collect and compare patient-reported outcomes from children and young adults with cleft and noncleft craniofacial conditions.

(1) Children’s Medical Center, Dallas, TX, (2) Nationwide Children’s Hospital, Columbus, OH, (3) Shriners Hospitals for Children, University of Illinois at Chicago, IL, (4) The Hospital for Sick Children, Toronto, Ontario, Canada, 5) Lancaster Cleft Palate Clinic, PA, (6) Shriners Hospitals for Children Chicago, IL, (7) University of Iowa Children’s Hospital, IA

Background/Purpose: Interrater agreement for health-related quality of life (HRQoL) has been found to be variable, particularly for child and parent-proxy reports. This construct has been explored in pediatric populations with various health conditions (eg, cancer, postoperative pain), but examination of interrater variance has been limited for youth with cleft lip and/or palate (CL/P).

Methods/Description: Participants (363 children, ages 8-10, and their parents) from 6 centers, recruited between August 2016 and May 2019, completed a measure of HRQoL, the Pediatric Quality of Life Inventory Generic Core Scales (PedsQL) as part of a larger study. Analyses included 315 children (147 male; 46 iCL, 55 iCP, and 214 iCLP), along with parent-proxy reported scores from 258 female caregivers and 83 male caregivers (parent-proxy report scores were collected from 2 caregivers for 30 patients). Separate analyses were conducted for male and female parent-proxy report, such that each child report score was used only once in each set of analyses.

Results: Two-way mixed effect intraclass correlations (ICC) revealed poor to fair agreement (r < .04) between child and parent-proxy raters for most PedsQL subscales and composite scores. Of note, moderate agreement was detected for child and female caregiver ratings of school functioning (r = 0.43) and for both male and female caregiver proxy scores for the overall psychosocial composite (r = 0.42 and 0.41, respectively). Overall means were also compared for child report and parent-proxy report by male and female caregivers; there was no difference between groups across all PedsQL subscales and composite scores. Child and parent-proxy reported scores were also compared to published normative data for the PedsQL Generic Core Scales for both chronically ill and healthy samples. Child report and parent-proxy report scores were significantly higher than scores from the chronically ill standardization sample across all domains (P < .01 for all); however, when compared with healthy controls, child and parent-proxy scores were significantly lower (P < .01), with the exception of the Physical Functioning scale, which was not significantly different from healthy controls.

Conclusions: Although parent-proxy report may provide valuable information about children’s observed functioning, the lack of agreement between parents and children with CL/P about the child’s own level of functioning supports the need for both parent-proxy and child self-reported outcomes to be included when evaluating HRQoL for youth with CL/P. In addition to providing important information about children’s own perceived HRQoL, differences between self-report and parent-proxy report may be clinically meaningful. Children with CL/P and their parents may endorse better HRQoL when compared with youth with other conditions defined as chronic illness; however, results also suggest that psychosocial functioning is negatively impacted for this population when compared with healthy youth.

(1) McMaster University, Hamilton, Ontario, Canada, (2) McMaster University, Hamilton, Ontario, Canada (3) Hospital for Sick Children, University of Toronto, Ontario, Canada, (4) Sobrapar Hospital, Campinas, Sao Paulo, Brazil, (5) Children’s Health Ireland at Temple Street, Dublin, Ireland, (6) University of British Colombia, Vancouver, Canada, (7) Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, (8) Great Ormond Street Hospital, London, England, (9) University of Pittsburgh School of Medicine, PA, (10) Western University, London, Ontario, Canada, (11) University Medical Center Utrecht, the Netherlands, (12) Oxford University Hospitals NHS Foundation Trust, England, (13) Boston Children’s Hospital, MA, (14) University of Gothenburg, Sweden, (15) Clinica Las Condes, Santiago, Chile, (16) Alder Hey Children’s Hospital, Liverpool, England, (17) CHEO, Ottawa, Ontario, Canada, (18) University of Colorado School of Medicine, Aurora, CO, (19) Memorial Sloan-Kettering Cancer Center, New York, NY, (20) Modus Outcomes, Letchworth Garden City, United Kingdom

Background/Purpose: The FACE-Q Craniofacial Module represents a comprehensive set of scales designed to measure outcomes including appearance, function, and health-related quality of life for patients aged 8 to 29 years with facial conditions. Psychometric findings for the original CLEFT-Q scales that are included in this module were tested in patients with cleft and noncleft facial conditions, and results are described elsewhere. The aim of this study is to examine psychometric performance of the newly developed scales of the FACE-Q Craniofacial Module.

Methods/Description: Patients aged 8 to 29 years with conditions associated with a visible or functional facial difference completed relevant FACE-Q Craniofacial module scales between December 2016 and December 2019. Rasch measurement theory (RMT) analysis was used to assess psychometric properties of each scale including: item fit, targeting, differential item functioning, and reliability. Scores were transformed from 0 (worst) to 100 (best) for tests of construct validity that included looking at differences by severity, and correlations between scales measuring related constructs.

Results: A total of 1495 participants were recruited with a broad range of conditions (eg, birthmarks, facial paralysis, craniosynostosis, etc). RMT analysis resulted in the refinement of 7 appearance scales (birthmark, cheeks, chin, eyes, forehead, head shape, smile), 2 function scales (breathing, facial), and an appearance distress scale. Three checklists were also formed (eye function, and eye and face adverse effects). For 9 scales, significantly lower scores were reported by participants whose appearance or functional difference was rated as a major compared to those with minor or no difference. There was a significant correlation between higher appearance distress scores and lower appearance scale scores.

Conclusions: The FACE-Q Craniofacial Module scales can be used to obtain evidence-based outcomes data in conditions associated with a facial difference.

(1) Global Smile Foundation, Norwood, MA, (2) University of Arkansas for Medical Sciences, Little Rock, AR, (3) Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, (4) Global Smile Foundation, Guayaquil, Guayas, Ecuador, (5) The University of Maryland Medical Center/Shock Trauma Center, Baltimore, MD

Background/Purpose: One in 500 to 700 newborns per year worldwide, and 250 000 newborns per year in developing countries, had congenital cleft lip and palate (CLP). These congenital anomalies have detrimental psychosocial, economical, and aesthetical manifestations on the patients’ lives. Global Smile Foundation (GSF) is a nonprofit organization committed to provide adequate comprehensive cleft care for those in need, with its volunteers being involved in cleft care for more than 3 decades. GSF provides both education and clinical care, through surgical outreach programs to low- to middle-income countries (LMICs), trying to establish interdisciplinary programs for cleft care in local centers. In this report, we provide an assessment on the clinical and economic outcomes of GSF’s outreach programs worldwide.

Methods/Description: We reviewed all the primary cleft lip and cleft palate repairs performed in the last decade during our surgical missions in Ivory-Coast, Guatemala, Ecuador, Lebanon, Mali, Burkina-Faso, El Salvador, Senegal, Brazil, and Peru. A total of 1509 patients were reviewed, 951 with primary cleft lip repair and 558 with primary cleft palate repair. The mean of age of the people included in this study was 3.3 ± 4.3 years for all, 3.0 ± 5.3 years for those who underwent primary cleft lip repair and 3.7 ± 2.5 years for those who underwent primary cleft palate repair. Averted disability-adjusted life years (DALYs) were calculated. The disability weight for patients with primary cleft lip before treatment was measured at 0.098 with a residual disability weight of 0.016 following treatment. The disability weight for patients with primary cleft palate before treatment was measured at 0.231 with a residual disability weight of 0.015 after treatment. Life expectancy was derived from the World Health Organization life tables. The economic value of the averted DALYs was then calculated by multiplying the number of averted DALYs by gross national income per capita.

Results: Global Smile Foundation has prevented 12 922 years lived with disability (DALYs) for patients who underwent primary cleft lip or primary cleft palate repairs over the last decade. The approximate overall financial gain from these interventions is between US$64 651 261 and US$124 001 435, with an average financial gain per patient estimated to be between US$42 844 and US$82 175.

Conclusions: These figures show the significant impact reflected by Global Smile Foundation, through our surgical missions, on our patients’ lives in low- and middle-income countries. We hope that future efforts will provide us with more resources to help us get access to a larger number of patients.

(1) Children’s Hospital of Philadelphia, PA, (2) Seattle Children’s Hospital, WA, (3) University of North Carolina, School of Medicine, Chapel Hill, NC, (4) Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru, (5) Pontificia Universidad Javeriana, Cali, Colombia, (6) Universidad Icesi, Cali, Colombia, (7) Clinica Comfamiliar Risaralda, Pereira, Colombia, (8) Pontificia Universidad Javeriana, Bogota, Colombia, (9) Children’s Hospital Los Angeles, CA

Background/Purpose: Craniofacial microsomia (CFM) is associated with wide clinical variability and often includes underdevelopment of the ear(s) and mandible. Approximately 1:1100 US newborns with CFM are born annually. In addition to complex health care needs, children with CFM can be at risk for social and educational difficulties, which may be related to speech and hearing status. This presentation describes caregiver reports of social experiences and educational placement, services, and academic performance of children ages 3 and older with CFM.

Methods/Description: As part of the NIH-funded prospective CAUSE study at 4 sites in the United States and 5 sites in South America, caregivers of children with CFM were interviewed about their child’s demographics, health history, their child’s awareness of their diagnosis, experiences of teasing, educational placement and services, and overall school performance. Clinical phenotype was determined by the integration of data from medical chart review, caregiver report of medical history, and standardized photos. Participants ages 3 to 18 were included in this analysis.

Results: Children with CFM (N = 143) had a mean age of 9.9 years (SD = 4.5), 63% were male, and most were born in the United States (41%) or Colombia (36%). CFM features and experiences were generally similar for United States and South American participants. Unilateral microtia (76%) was the most common CFM feature and 90% had hearing loss, with 55% using hearing aids. Caregivers reported children’s mean age of diagnosis awareness was 4.4 years, and 14% felt that their child was too young to notice at the time of study participation. Teasing was reported for 42%, which started at an average age of 6.1 years and took place at school/day care (85%), by classmates (85%) and community peers (30%). Teasing was most often described as taking place some of the time (49%). Of those enrolled in school, 81% in the United States and 95% in South America were in general education. Parents described their children’s grades as well above average (40%), above average (29%), or average (19%). Individualized Education Programs (IEP) were in place for 54% in the United States compared to 5% in South America, most often for speech, language, and/or hearing needs. Preliminary analyses did not identify relationships between teasing and health or school status.

Conclusions: Findings from this multinational study are consistent with smaller US-based CFM studies of psychosocial and educational outcomes. As caregivers reported children’s diagnosis awareness at age 4 and teasing starting around age 6 for about 40% of children, team members are encouraged to integrate coping strategies for families into team visits starting in early childhood. The majority of children were reported to be average or above academically in general education classes and half of US children had an IEP. However, given high rates of hearing loss and speech/language needs, it may be that additional support services are indicated and team members should assist in facilitating appropriate services.

(1) DePaul University, Chicago, IL, (2) University of Kentucky, Lexington, KY, (3) University of Illinois Hospital and Health Sciences System, Chicago, IL

Background/Purpose: Children with craniofacial conditions are at risk for experiencing negative impacts on psychological adjustment and functioning. Psychosocial risks include emotional, behavioral, and social challenges; learning; speech; language and hearing difficulties; and stressors related to medical treatment. Caregivers play an important part in supporting their children’s coping efforts. Families from diverse backgrounds would benefit from a tool that both increases children’s coping skills and parents’ confidence in managing and supporting their child’s coping. No such tool currently exists for the pediatric craniofacial community, thus, the current project aims to develop a low-cost, accessible, evidence-based intervention in English and Spanish that can be individually tailored to build coping skills and promote resilience in families of children with craniofacial conditions from diverse backgrounds. The Cellie Coping Kit (CCK) intervention was originally developed for children with cancer and has been successfully adapted for use with multiple specialized pediatric populations (eg, Sickle cell disease, eosinophilic esophagitis). Through this presentation, we will discuss how we will adapt the CCK for the pediatric craniofacial population.

Methods/Description: Drawing from existing research on the CCK, a multidisciplinary treatment team with expertise in working with predominately Latinx families and other underserved communities, will follow the kit specific evidence-based adaptation process to create a CCK for children with craniofacial conditions. This presentation will describe the kit, lay out the adaptation process, and discuss future project goals. Kit components include a stuffed toy named Cellie, coping cards that provide cognitive-behavioral coping techniques, and a caregiver book that parallels coping card topics to support caregivers’ efforts to assist their child’s coping and address their own challenges. First, a literature review of effective coping strategies for children with craniofacial conditions will be conducted to identify appropriate coping techniques to incorporate into the kit. Then craniofacial experts (ie, medical and mental health providers) will review materials for thoroughness and developmental appropriateness. We will then seek out feedback from clinic families in a focus group to finalize kit materials. Afterward, we will translate materials into Spanish. Future directions for the project include kit production and evaluation of CCK acceptability, feasibility, and effectiveness in improving coping and psychosocial functioning among families with children with craniofacial conditions from diverse backgrounds.

(1) The Children’s Hospital at Westmead, Sydney, Australia, (2) Pacific University, Forest Grove, OR

Background/Purpose: In formal clinical research, audit, benchmarking, and outcome studies, the perceptual rating of performance for specific speech constructs including hypernasality, nasal emission, and nasal turbulence, as well as the phonetic transcription of speech disorder is undoubtedly the role of a highly trained, experienced speech-language pathologist (SLP). Although SLPs can also make judgments about global measures of speech performance for constructs such as speech understandability and speech acceptability, it is argued that lay or naive listeners represent a more ecologically valid group of raters for judging these concepts. A comparison of lay listener and SLP ratings of global speech measures from the same speech samples could also potentially determine how valid SLPs are in making global judgments about speech performance. The aim of this study was to investigate the interrater and intrarater reliability of untrained lay listener judgments of global measures of speech performance from spontaneous (connected) speech samples from children with differing degrees of velopharyngeal function and to compare the reliability of lay listener ratings to a group of SLPs’ ratings of the same speech samples.

Methods/Description: Audio recordings of spontaneous speech stimuli were elicited from 40 children aged between 4 and 14 during routine attendance at a Cleft Palate Clinic. The speech samples were selected to reflect the typical clinical presentation of velopharyngeal function (and velopharyngeal insufficiency). Ordinal and visual analogue scale (VAS) ratings of both speech understandability and speech acceptability using scales from the Rhinocleft Speech Assessment were made by a group of 4 SLPs and 12 adult lay listeners. Reliability of ordinal scale measures were estimated using κ and Fleiss’ κ statistics, and VAS measures were estimated using the intraclass correlation coefficient (ICC). Spearman ρ was used to estimate the strength of correlation between lay listener and SLP mean VAS ratings.

Results: Intrarater reliability was fair to good for ordinal scale ratings (κ: 0.25-0.72), and good to excellent for VAS ratings (ICC: 0.69-0.95). Interrater reliability was fair to moderate for ordinal scale ratings (Fleiss’ κ: 0.32-0.51) and excellent (ICC: 0.97-0.99) for VAS rating ratings. There was a high to very high positive correlation (Spearman ρ: 0.89-0.93) between the mean VAS ratings for the lay listener and SLP ratings.

Conclusions: This study has established that untrained, lay listeners demonstrated good to excellent degrees of intrarater reliability and excellent interrater reliability for VAS ratings of global measures of speech performance which were superior to the results achieved on ordinal scale measures. Furthermore, these reliability estimates were consistent with the estimates derived from the experienced SLPs’ ratings of the same speech samples using the same rating scales.

(1) Hadassah Academic College, Jerusalem, Israel

Background/Purpose: To describe the occurrence of phoneme-specific nasal emission (PSNE) and phoneme-specific hypernasality (PSH) in patients with and without structural disorders of the velopharyngeal mechanism. To examine the relationship of this speech behavior to coexisting phonological processes and the differences in its patterns of occurrence between patients with and without structural disorders of the velopharyngeal mechanism.

Methods/Description: In this retrospective case analysis, 1252 patients were grouped into 3 groups: group A—patients with overt palatal anomalies (n = 350) having cleft lip and palate (n = 126) or cleft palate only(n = 126), group B—patients with occult anomalies of velopharyngeal mechanism (n = 736) having occult submucous cleft palate (n = 345), submucous cleft palatal ((n = 167), and patients with velopharyngeal insufficiency but no sign of palatal cleft anomaly (n = 226), and group C—patients with PSNE and PSH in the absence of structural anomaly of the velopharyngeal sphincter (n = 166). Perceptual VPD tests including Hebrew articulation and phonological assessment following endoscopy were conducted. Differences were analyzed by χ ² test.

Results: In all, 36% of the studied patients had phonological disorders; 35% of patients in groups A and B had compensatory articulation disorders. Patients in group A with compensatory disorders had significantly (P < .006) lower prevalence of phonological errors (49%) in comparison to the prevalence of phonological errors (63%) in patients with compensatory articulation disorders in group B. In group A, 6% had PSNE of which 53% had phonological errors; 2% had PSH of which 25% had phonological errors. In group B, 4% had PSNE of which 25% had phonological errors; and 3% had PSH of which 64% had phonological errors. In group C, 56% had PSNE of which 42% had phonological errors and 44% had PSH of which 42% had phonological errors. Patients in group C had significantly (P < .000) higher prevalence of atypical backing phonological error (22%) in comparison to 18% in group B, and no backing error in group A.

Conclusions: The current study provides additional data about the etiology and the use of phoneme-specific nasal resonance and/or nasal air emission. Our results about relationship between phonology errors and compensation disorders in relation to PSNE and PSH in patients with and without structural disorders of the velopharyngeal mechanism can help clarify the etiology of this specific speech disorder as an articulatory structural constraint, or an underlying phonological/organizational disorder. Understanding the etiology of PSNE and PSH can affect treatment options for patients.

(1) St. Louis Children’s Hospital, MO, (2) Washington University School of Medicine, St. Louis, MO

Background/Purpose: Research in velopharyngeal (VP) function dating from 1986 has shown that compensatory articulations (CAs) including glottal stops, pharyngeal fricatives, and nasal fricatives are produced with incomplete VP closure, even if the velopharynx is capable of complete closure with appropriate articulation. Further studies showed that patent oronasal fistulas can influence VP movement and closure. Therefore, valid assessment of VP function, perceptual or instrumental, should control for both fistulas and CAs. Failure to do so could cause invalid assessment leading to unnecessary surgery or inadequate speech therapy. Studies that do not stipulate exclusion of compensatory articulations or fistulas may be of questionable validity. At ACPA 2018, we reported the result of a literature review of 157 published articles from 42 journals to evaluate how well literature on VP assessment or management outcome stated control for fistulas and CAs. Only 6% of the articles stated control for both fistulas and CAs; 12% controlled for one or the other, but not both; 37% described speech sample validity by describing the speech pathologist (eg, “experienced, licensed”) 24% made no statement concerning the speech sample used. Would articles peer reviewed by speech-language pathologists (SLPs), be more likely to report speech sample validity? The purpose of this study was to determine how many journals from the prior study used SLP peer reviewers.

Methods/Description: The editorial staffs of the 42 identified journals were contacted with a standard email message to inquire whether a submitted manuscript concerning cleft palate speech or surgical speech outcome would be peer reviewed by a SLP. Responses were recorded in both Word logs and Excel database. Nonresponding journals were sent follow-up queries, with 3 standard queries sent over 5 months. Spearman ρ tested for association between SLP reviewers and reported speech sample validity.

Results: Sixteen (38%) journals did not respond, or declined to give information, including 5 that have either been discontinued or merged with other publications. Nine (21%) journals confirmed SLP reviewers; 9 (21%) stated possible use of SLPs. Four (9.5%) journals reported unlikely use of SLP reviewers, and another 4 (9.5%) reported that they do not use SLPs. The articles that described full speech sample validity came from just 4 journals: 2 which confirmed SLP peer reviewers, 1 stated possible use of SLPs, and 1 nonresponsive journal. There was a small but significant association between inclusion of SLPs as reviewers and VP validity (Spearman ρ = 0.179, P = .034, n = 105) when validity was categorized as none, partial, or complete.

Conclusions: Analysis suggests journals using SLP peer reviewers may be more likely to report speech sample validity, but many journals do not use SLPs as peer reviewers. Discernment and caution may be valuable tools for the reader of the evidence base concerning speech and surgical outcomes in children with cleft palate and VP dysfunction.

(1) Oregon Health and Science University, Portland, OR, (2) University of Utah, Salt Lake City, UT, (3) Kaiser Permanente, Portland, OR

Background/Purpose: Every year up to 250 000 new babies with cleft lip and/or palate are born in the world, many of whom are born in areas where resources are limited, scarce, or nonexistent. A variety of international cleft care programs have been created in an effort to provide care for individuals who may not otherwise receive surgical intervention. Best practice guidelines assert that interdisciplinary team approaches should be utilized in providing care in international settings, and speech-language pathologists play a crucial role in supporting long-term outcomes for individuals experiencing craniofacial differences. Because of the underdeveloped nature of the medical infrastructure in many low- and middle-income countries (LMIC), rehabilitation services including speech-language pathology services may be scarce or unavailable. The inclusion of a speech-language pathologist on an international team allows for speech assessment of previously treated patients, improved triage of surgical candidates, and instruction of therapy techniques for patients of the program. Involvement of speech pathology is essential to differential diagnose of velopharyngeal dysfunction. The speech-language therapist aids in differentiating the need for speech therapy to remediate articulation errors, or surgery to correct hypernasal resonance, or potentially, the combination of speech therapy and surgery.

Methods/Description: This presentation will discuss crucial elements of speech-language pathology programming abroad and the role of the speech pathologist on international cleft care trips, including screening surgical patients, evaluating nasal resonance balance and velopharyngeal function to inform surgical decision-making, training families and caregivers in language and articulation, and providing services for children with atypical speech patterns. Case study examples from mission experiences in Peru will be utilized to exemplify these elements and the importance of collaboration between the surgical team and the speech pathologist in identifying appropriate surgical candidates. The American Cleft Palate-Craniofacial Association also created guidelines to inform clinicians working abroad, noting standards for training requirements and an emphasis on sustainability with the goal of creating viable and ethical programs. Discussion will also include efforts for program sustainability, to include training family members and other local personnel in delivering speech-language pathology treatment programs. Specifically, SLPs will discuss how they have delivered capacity building trainings with local medical providers. Unique ethical and training considerations when practicing internationally will also be discussed.

(1) Eastern Washington University, Spokane, WA

Background/Purpose: Speech of individuals with velopharyngeal insufficiency (VPI) is characterized by nasalized vowels. It is well known that velopharyngeal coupling significantly alters the acoustic spectrum of vowels. Centralized F1 and lowered F2 due to effects of nasalization could change the dimensions of the quadrilateral space. Such variations might cause the vowel space area to be compressed which might contribute to reduced speech intelligibility. This study was conducted to address the relation between speech intelligibility and spectral characteristics vowels in nasal speech.

Methods/Description: Twenty children aged 4 to 10 years (10 with hypernasality and 10 typically developing peers) repeated the vowels /a/,/i/,/u/,/ae/ within a /hVd/ context. F1 and F2 midpoint frequencies were measured from the spectrogram. Vowel space area (VSA) was computed using the formula for the area of an irregular quadrilateral (Vorperian & Kent, 2007). In order to determine intelligibility, 50 naive listeners were asked to orthographically transcribe the sentences produced by the participants. Correlational analysis was used to determine the relationship between the VSA and intelligibility deficits. An independent t test was also used to determine the difference between VSA in children with VPI and typically developing peers.

Results: There was a significant positive correlation between intelligibility scores and VSA (r = 0.67, P = .004). VSA was significantly different between the 2 groups (t(18) = −4.02, P = .001). Children with VPI had significantly smaller VSA than their age-/gender-matched peers.

Conclusions: This is the first study to report data on acoustic vowel space area for children with VPI between the ages 4 to 10 years. Vowel space area was smaller in VPI speech; therefore, assessment on the contribution of the different vowels on VPI speech is warranted as the results could have treatment implications.

(1) Great Ormond Street Hospital NHS Trust, London, United Kingdom, (2) Speech at Home, Dublin, Ireland

Background/Purpose: Speech@Home (S@H), an evidenced-based, integrated online intervention package designed specifically for children with cleft palate type speech disorders, provides the speech and language therapist (SLT) with a therapy program builder and linked resources which can be used to deliver therapy at home via telehealth, ideally following parent training. COVID-19 has driven the need for SLTs to move rapidly from a face-to-face delivery model to telehealth service delivery. The aim of this presentation is to describe how S@H addressed this problem, evaluated using a quality improvement (QI) methodology framework of plan, do, study, act cycle.

Methods/Description: Six speech pathologists from 4 cleft centers in the United Kingdom/Ireland volunteered to participate in the project and completed a S@H training webinar. Following assessment and consent to participate, 16 naive parents in the S@H approach and their child received 10 teletherapy sessions. Each session was followed up with an emailed link to the parent, with that week’s program, and all the resources required to complete the activities. Data collection of therapists’ and parents’ views of the website’s usability/functionality, content (therapy activities and resources), together with their emotional responses using the virtual platform, were collected at weeks 1, 5, and 10, on a 1 to 5 point Likert scale. The nature of the children’s speech disorders and speech changes was documented. The S@H team provided feedback to the SLTs at week 5 to inform the second cycle of intervention.

Results: In cycle 1, therapists and parents rated usability, activities, and resources with almost all high positive scores of 4 or 5 (mean 4.4, mode, 4, median 4). Minor functionality errors identified by the therapists were corrected, including the parent/therapist ongoing notation system. The speech sound videos and cleft speech–specific resources were especially highly praised. The need for downloading resources on an iPhone and iPad was identified, and this functionality added. Therapists also suggested improvements to the Program Builder, such as reducing the need to enter the same consonants across activities and the imbalance in activities at different levels.

Conclusions: Following one training webinar, Speech@Home was found to be very suitable for use in teletherapy, with therapists and parents reporting positive experiences, especially encouraging given these were “untrained” parents. Furthermore, the QI methodology has provided excellent insights into the S@H platform by 6 novel therapists, building the evidence for its effectiveness. This project has already improved the virtual platform but also informed the S@H team as to other areas for improvement, suggestions for ways to address some of these, and identified further training items for therapists.

(1) Great Ormand Street Hospital, London, United Kingdom, (2) Speech at Home, Dublin, Ireland

Background/Purpose: The Parent Led Articulation Therapy study has shown that parent-led intervention for cleft palate articulation therapy could be an alternative model of service delivery. An integral aspect of this intervention was parent training and the development of speech therapy programs with their resources, all labor-intensive activities for busy therapists. Based on this, Speech@Home has been developed an online integrated, innovative speech intervention package for children with cleft palate and related conditions. This approach shifts the onus of speech therapy delivery to parents at home under the supervision of a speech and language therapist (SLT) using telehealth. Parents are an untapped resource where services are very stretched. With the Covid crisis, teletherapy has become a safe way to deliver speech and language therapy services. The aim of this presentation is to provide an overview of the Speech@Home approach.

Methods/Description: Speech@Home provides (1) in depth parent training courses and (2) a novel online Therapy Programme Builder and linked resources which can be used in conjunction with therapist led teletherapy. Parent training is available in 2 formats: (1) A 2-day course for parents undertaking regular therapy activities, working in partnership with a therapist. Everything an SLT requires to run a training course for a group of parents is available including videoed lectures, lecture handouts, sound videos, parent quizzes and answers, and course evaluation forms, (2) A stand-alone 1-day course to support parents who are able to access therapy, whose child is on the waiting list or is too young to receive direct therapy but therapy is highly likely to be required. The Speech@Home Therapy Programme Builder enables the SLT to produce a child-specific program in a short space of time. It provides the SLT with a drop-down menu software package containing over 150 therapy activities each linked to the appropriate picture and video resources. The parent is emailed the link to their child’s therapy program with all the picture resources required for each activity. This presentation will provide an overview, with illustrations, of the parent training courses, a demonstration of the Therapy Programme Builder with resources and the professionally presented parent programme.

(1) Children’s Hospital of Pittsburgh of UPMC, PA, (2) University of Pittsburgh, PA, (3) Case Western Reserve University School of Medicine, Cleveland, OH, (4) University of Pittsburgh School of Medicine, PA

Background/Purpose: This investigation evaluates velopharyngeal function and articulation differences in patients with clefts who have undergone Lefort 1 advancement. Patients with cleft lip and palate often have midface hypoplasia with malocclusion which may result in obligatory articulation changes. Surgical maxillary advancement may worsen velopharyngeal function, with previous reports citing rates of VPI of 0% to 84%. Obligatory articulation changes have been observed to resolve/improve after correction of malocclusion. The data presented will aid in informed consent discussions surrounding orthognathic surgery and speech changes.

Methods/Description: This retrospective review evaluates speech parameters in patients with a history of complete cleft lip and palate or palatal cleft who had conventional Lefort 1 maxillary advancement or maxillary distraction osteogenic advancement. Pre- and postoperative speech measures include the Pittsburgh Weighted Speech Score (PWSS) to identify velopharyngeal incompetence (VPI) and formalized speech evaluation with clinical notation of articulation patterns. Articulation categorized as (1) normal, (2) obligatory distortions secondary to malocclusion, or (3) retained speech distortions/misarticulation following orthognathic surgery. Cephalometric parameters will be evaluated to assess for significant trends. Fifty-six patients were included in the study group (23 female, 33 male). Cleft categories were 20 BLCLP, 9 right ULCLP, 25 left ULCLP, and 2 complete palatal cleft. Average age at time of surgery was 16.95 with postoperative speech assessment occurring an average of 6.8 months after intervention.

Results: PWSS averaged 2.68 before surgery and 4.41 after surgery (P < .001). Two patients had improved PWSS; 13 (23%) patients had worsening of PWSS of clinical significance. Of 10 patients with VP competence (PWSS 0): 2 developed VPI (PWSS >7), 5 developed borderline VP incompetence (PWSS 3-5). 2 patients changed from borderline competence to borderline incompetence. Four patients changed from borderline VP function to overt VPI. Of this, 49 (87.5%) of 56 patients had presurgical obligatory speech distortions attributed to malocclusion; 30 (61%) of 49 had normal articulation postoperatively, 15 (30.6%) of 49 had retained distortions, correctable with speech therapy.

Conclusions: Of this, 87.5% of patients requiring Lefort 1 osteotomy had articulation differences attributed to malocclusion; most resolved with correction of malocclusion or could resolve through speech therapy for retained distortions. On average, symptoms of velopharyngeal dysfunction increased in patients undergoing Lefort 1 advancement (23% of patients experienced clinically significant change).

(1) University of Toronto, Ontario, Canada, (2) The Hospital for Sick Children, Toronto, Ontario, Canada

Background/Purpose: Nasalance scores are an important tool in the assessment of nasality disorders. They are calculated from dual-microphone recordings of the oral and nasal speech signals. This requires carefully balanced microphones and dedicated signal processing, so the equipment is not available to all clinicians. If nasalance could be calculated from separate single-microphone recordings, the approach would become feasible for more clinicians. The goal of the present study was to evaluate how well nasalance scores could be calculated from temporally asynchronous single-channel recordings of the oral and nasal speech signals.

Methods/Description: Twenty-one participants (9 female) with unilateral cleft lip and palate, aged 7 to 11 years (mean 9.13 years), were recorded repeatedly with a nasometer headset. This resulted in a total of 488 recordings, or 122 repeated recordings each of the nasal Jam sentence and the non-nasal Zoo sentence. A macro was written in Praat that approximated the filter characteristics of the nasometer software and provided very similar nasalance scores, which formed the ground truth for the study. The average intensity levels of the oral and nasal channels from matching recordings were used to calculate time-free nasalance scores. Finally, the average intensity levels of the first oral and the second nasal signal as well as the second oral and the first nasal signal from the repeated recordings from the same session were used to calculate asynchronous nasalance scores. Results were analyzed in terms of the mean differences from the ground truth nasalance scores.

Results: For the ground truth nasalance scores, the mean differences were 4.24 (SD 3.62) between Jam recordings and 2.81 (SD 2.68) between Zoo recordings from the same session, and 80% of the data were within 5 nasalance points. The time-free nasalance scores differed on average 4.1 (SD 3.0) for the Jam and 1.65 (SD 1.53) for the Zoo sentence, and 83% of the data were within 5 nasalance points. Finally, the asynchronous nasalance scores differed on average 4.4 (SD 3.3) for the Jam and 2.09 (SD 2.21) for the Zoo sentence, and 65% of the data were within 5 nasalance points.

Conclusions: The ground truth nasalance scores showed a level of variability between repeated nasalance recordings that could be expected for a clinical data set. The time-free and the asynchronous nasalance scores approximated the ground truth scores reasonably well. More research is needed to develop a robust methodology for the assessment of nasalance based on single-microphone recordings.

(1) University of North Carolina at Chapel Hill, NC, (2) Nationwide Children’s Hospital, Columbus, OH

Background/Purpose: Young children with cleft palate often produce apical-alveolar sounds such as /t/ and /s/ by elevating the midportion of the tongue to the hard palate rather than the tip of the tongue to the alveolar ridge. Palatalization (backing) of alveolar sounds may be due to (a) anterior anomalies including oronasal fistulae and maxillary arch collapse, or (b) developmental articulatory imprecision. The purpose of this preliminary study was to determine articulation and spectral characteristics of children with cleft lip and palate (CLP), children with cleft palate (CP) only, and typically developing (TD) children without clefts. It was hypothesized that if maxillary anomalies contribute to backing of alveolar targets, then children with CLP would show the most backing errors and reduced spectral distinctions. Conversely, if backing is a developmental phenomenon reflecting general articulatory imprecision, then backing errors and spectral distinctions would be similar across the 3 groups of children.

Methods/Description: Participants consisted of 27 children, 34 to 38 months of age, in 3 cohorts: (a) 10 with repaired unilateral CLP (5 RCLP, 5 LCLP; 9 males), (b) 9 with repaired CP (7 soft only, 2 soft and hard; 2 males), and (c) 8 TD children without clefts (4 males). The average age of palate repair was 10.0 months (range: 7.2-13.1) and included both 2-flap palatoplasty and double-opposing z-plasty. None of the children had oronasal fistulae, known sensorineural hearing loss, known syndromes, or Pierre Robin sequence. Children were audio recorded during administration of the Goldman-Fristoe Test of Articulation-3 (GFTA3); the recordings were scored by an experienced speech-language pathologist (SLP) following guidelines recommended by the Americleft group. Scoring agreement with another experienced SLP was 94% or better. TF32 software was used to obtain the first spectral moment (mean frequency) for /t/-/k/ and /s/-/ʃ/ targets from 14 words produced by each child during the GFTA3.

Results: There were significant differences in GFTA3 standard scores between TD children (mean = 101, sd = 9) and children with CLP (mean = 83, sd = 8; P = .011) and between children with CP (mean = 97, sd = 16) and children with CLP (P = .044). Only one child with CLP (10%) backed 2 or more productions of /t/; none of the TD children or children with CP backed /t/. None of the TD children backed /s/, 1 child with CP (11%) backed /s/, and 6 children with CLP (60%) backed /s/. There was a significant /t/-/k/ spectral difference between TD children (mean = 2.3 kHz, sd = 0.9) and children with CLP (mean = 1.1 kHz, sd = 1.0) (P = .011); there was a significant /s/-/ʃ/ spectral difference between children with CP (mean = 0.7 kHz, sd = 0.8) and children with CLP (mean = −0.2 kHz, sd = 0.6; P = .006).

Conclusions: Three-year-old children with repaired CLP are more susceptible to backing /s/ and exhibit reduced spectral distinction of /t/-/k/ and /s/-/ʃ/ compared to children with CP only and TD children. Velopharyngeal and hearing status are discussed.

(1) East Carolina University, Greenville, NC

Background/Purpose: The levator muscle is the primary muscle for velar elevation. Understanding the typical morphology of this muscle is critical for improving knowledge about atypical velopharyngeal (VP) mechanisms. Variation in the levator muscle length, thickness, or VP portal depth has been reported to contribute to VP dysfunction. Studies have reported the levator muscle dimensions and found minimal asymmetry between the length and thickness of the levator muscle bundles in typical anatomy and in cleft anatomy. However, these studies represent relatively small sample sizes (less than 10 participants per group for in vivo studies). In addition, it is not clear what degree of asymmetry of the right and left levator muscle length and thickness is considered “typical” in typical population. Furthermore, it is not known whether asymmetric features of the levator muscle bundles result in an observable impact on VP function among individuals with normal speech. The goal of the current study is to determine the typical range of asymmetry between the length and thickness of the levator veli palatini muscle bundles and to explore the impact of the observed asymmetry on VPl closure. Another aim was to report normative length and thickness of the levator veli palatini muscle among adults with typical VP anatomy.

Methods/Description: Magnetic resonance imaging data and Amira 5.5 Visualization Modeling software were used to evaluate the levator veli palatini muscle bundles among 89 participants with typical VP anatomy. Flexible nasopharyngoscopy was used to determine the function of VP closure among 39 of the 89 participants with typical VP anatomy to examine the functional impact of observed asymmetry.

Results: Matched paired t tests demonstrated a nonsignificant difference between the length and thickness of the right and left levator muscle bundles. The mean difference between the right and left length of the levator muscle bundles was 2.28 mm, but ranged from 0.09 to 10.37 mm. In all cases where individuals displayed asymmetry in the levator muscle through MRI, there was no observed impact on the symmetry of VP closure.

Conclusions: Results of this study suggest that differences in the right and left levator veli palatini muscle are not significant among individuals without cleft palate. However, among individual cases where asymmetry was sizeable, there was no direct impact on the closure pattern. This may suggest there are multiple factors that contribute to asymmetrical VP closure that are beyond the level of the levator veli palatini muscle.

(1) Ian Jackson Craniofacial Clinic, Beaumont Royal Oak, MI, (2) Hospital Gea Gonzalez, Mexico City, Mexico

Background/Purpose: Moebius syndrome (MS) is characterized by congenital bilateral paralysis of the facial and abducens nerves. Clinical features include feeding problems, dysarthria, dysphagia, sialorrhea, strabismus, and lack of facial expression. Patients with MS frequently present with dysphagia during infancy. Further on, during childhood, a severe speech disorder is a common feature. However, articulation deficits in patients with MS are scarcely reported in the related scientific literature.

Objective: The aim of this study is to describe speech deviations, intelligibility and sialorrhea in patients with MS.

Methods/Description: Eighty-seven patients with MS were prospectively studied. Age ranged from 4 to 18 years. A complete speech and language pathology (SLP) evaluation was performed in all cases. The evaluation focused on articulation placement, sialorrhea, and intelligibility of speech.

Results: Sialorrhea was detected in 23% of the patients. Abnormal articulation placement of bilabial phonemes was observed in 68% of the patients. Another 50% of the patients presented with articulation placement errors in other phonemes. Intelligibility was classified as adequate in 18% of the cases. Mildly affected intelligibility was found in 51% of the patients. Speech was considered moderately unintelligible in 20% of the cases. Unintelligible speech was found in 11% of the patients.

Conclusions: From the results of this prospective study, it can be concluded that a high percentage of patients with MS are at high risk of presenting with moderate to severe speech disorders. Thus, an early SLP intervention should be provided for this population in order to enhance speech development and reducing the risk of severe oral communication impairments.

(1) Arizona State University, Tempe, AZ, (2) Vanderbilt University, Nashville, TN, (3) The George Washington University, DC

Background/Purpose: Young children with repaired cleft palate are a diverse clinical population, who vary in their acquisition of speech and vocabulary skills during early development following palatoplasty. The purpose of this study was to examine an early intervention database for participant characteristics associated with changes in speech and vocabulary growth of children with repaired cleft palate who received an Enhanced Milieu Teaching Plus Phonological Emphasis (EMT+PE: Scherer, Kaiser & Frey, 2020) intervention compared to children in a “business-as- usual” (BAU) control condition who were receiving routine interventions and follow up in the community.

Methods/Description: Participants 29 children with repaired cleft palate were randomly assigned (stratified by gender and age) at initial screening to a business-as-usual (BAU) condition or to the EMT + PE intervention condition. Participants were between the ages of 15 and 36 months, received their primary palatoplasty prior to 12 months of age, and had cognitive scale composite scores of 80 or greater on the Bayley Scales of Infant and Toddler Development-III. Measures and Assessments: The language and speech skills of the children were evaluated at 3 time points: (a) preintervention, (b) immediately postintervention, and (c) follow-up 3 months after postintervention. Assessments included a history, single-word articulation test, language sample, parent questionnaire of vocabulary production, and standardized language test. Intervention Procedure: In the Enhanced Milieu Teaching (EMT; Kaiser et al., 1998) plus Phonological Emphasis (EMT + PE) condition, a trained SLP used naturalistic intervention strategies to facilitate vocabulary and speech development (Scherer & Kaiser, 2010). Participants in the EMT + PE group received individualized therapy during 30-minute clinic sessions twice weekly for 6 months.

Results: Overall, children in the EMT + PE group had better outcomes for language and speech. Speaking rate (words per minute), vocabulary size, and NDW from the language sample showed moderate to large effect sizes as predictors of consonant inventory and speech accuracy. Children in the EMT + PE group who had higher speaking rates showed greater change in speech production from pre to postintervention than children who were low rate talkers. Given the importance of rate of word use as demonstrated by WPM’s high correlation with intervention outcome, we examined the descriptive statistics for WPM in the whole sample (M = 6.81, SD = 5.94, range = [0-17.10], Median = 6.10).

Conclusions: This study provided evidence for an adapted EMT + PE intervention that would focus on early vocabulary growth for children who are just beginning to use words (10-50 words) and have a talking rate of <10 words per minute. For children with skills above that benchmark, interventions targeting speech outcomes appear to be most effective for children who are using around 50 words and have a talking rate of >10 words per minute.

(1) Nationwide Children’s Hospital, Columbus, OH, (2) Aichi Gakuin University, Nagoya, Japan, (3) Department of Otolaryngology, Head and Neck Surgery, The Ohio State University Wexner Medical Center, Columbus, OH

Background/Purpose: Although communication disorders are a hallmark feature of 22q11.2 deletion syndrome (22qDS), few have studied speech-language development of 22qDS in the first few years of life (Scherer et al., 1999; Scherer et al., 2001). Children with 22qDS may display limited vocabulary, restricted sound repertoire, and general language delays; yet little is known about their early vocal characteristics, social interactions, and parent vocal input. The purpose of this study is to examine these early communicative characteristics in young children with 22qDS.

Methods/Description: Thirty children with 22qDS (17 males) and 21 typically developing peers (TD group, 8 males), ages 10 to 35 months (mean 22 months), were enrolled in this prospective study. All participants completed a daylong audiorecording (mean 13 hours) in their home using a Language Environment Analysis (LENA) digital language processor. LENA software algorithms and statistical modeling were used to analyze conversations and estimate AVA scores (an index of speech output complexity that reflects the degree of organization of a child’s vocalizations as speech-like sounds), and quantify child vocalizations, conversational turns, and caregiver vocal input. Data regarding the child’s home environment, vocabulary size, therapies, and hearing history, were also collected. Mann-Whitney U tests were used to examine group differences.

Results: Children with 22qDS produced significantly less frequent vocalizations (115/hour vs 160/hour, P < .022) and conversational exchanges as compared to TD peers (35/hour vs 52/hour, P < .007). Age was not predictive of quantity of child vocal output (P = .25). AVA speech complexity scores were significantly lower in children with 22qDS (83.7 vs 100.6, P < .001). In the 22qDS group, there was a significant negative correlation between age and AVA scores (r = −0.453, P < .05). Quantity of child vocal output was not associated with AVA scores (r = 0.092). Children with 22qDS were exposed to less caregiver input than TD peers, but this did not reach significance (1087/hour vs 1356/hour, P = .063). Although maternal education level was similar across groups (P = .269), higher maternal education level was correlated with higher caregiver vocal input, conversational interactions, and child vocal output (r = 0.36, 0.43, 0.32, all P < .01).

Conclusions: Young children with 22qDS are significantly less vocal and interactive than TD peers starting at very early ages. Speech output complexity is also significantly reduced in young children with 22qDS. An unexpected finding of this study was the negative relationship between age and AVA scores in children with 22qDS, suggesting that older children in this study had less complex speech output compared to the younger children. The AVA score may serve as a very early index of emerging speech disorders in infants and toddlers in 22qDS. Implications for early diagnosis and intervention for speech-language disorders in 22qDS will be discussed.

(1) University of Bristol, United Kingdom, (2) North Bristol NHS Trust, United Kingdom, (4) Charles Sturt University, Bathurst, New South Wales, Australia

Background/Purpose: Speech intelligibility is a core outcome of treatment for children born with cleft palate and is included in the International Consortium for Health Outcomes Measurement Standard Set of outcome measures for cleft lip and/or palate (CL/P). The specific measure used is the Intelligibility in Context Scale (ICS), a free parent-rated measure of intelligibility with 7 different communication partners on a 5-point scale (http://www.csu.edu.au/research/multilingual-speech/ics). The ICS has been validated as a screening tool with children born without clefts who speak a range of languages; however, no comparator data are available for children born with CL/P to enable its use in outcome measurement. The aim of this study was to investigate performance on the ICS for children with CL/P using data from the Cleft Collective, a large-scale longitudinal clinical cohort study, and to provide comparison data for a sample of 3-year-old English-speaking children born with any cleft type.

Methods/Description: The study used cross-sectional data from parent questionnaires administered in the Cleft Collective Birth Cohort Study. Scores for the ICS range from 1 (never understood) to 5 (always understood). Data were available for 412 children aged 3 years born with any type of cleft from questionnaires completed by mothers. Descriptive and inferential statistics were carried out to determine difference according to children’s cleft type and syndromic status.

Results: The average ICS score for the total sample was 3.75 (sometimes-usually intelligible; SD = 0.76, 95% CIs: 3.68-3.83) of a possible score of 5 (always intelligible). Children’s speech was reported to be most intelligible to their mothers (M = 4.33, SD = 0.61, 95% CIs: 4.27-4.39) and least intelligible to strangers (M = 3.36, SD = 1.00, 95% CIs: 3.26-3.45). There was strong evidence (P < .001) for a difference in intelligibility between children with cleft lip only (n = 104, M = 4.13, SD = 0.62, 95% CIs: 4.01-4.25) and children with any form of cleft palate (n = 308, M = 3.63, SD = 0.76, 95% CIs: 3.52-3.71). Children born with cleft palate with or without cleft lip and an identified syndrome were rated as less intelligible (n = 63, M = 3.28, SD = 0.85, 95% CIs: 3.06-3.49) compared to children who did not have a syndrome (n = 245, M = 3.72, SD = 0.71, 95% CIs: 3.63-3.81).

Conclusions: These results provide preliminary data to support use of the ICS in outcome measurement in 3-year-old children born with CL/P using the outcome measures recommended by the International Consortium for Health Outcomes Measurement. Future analyses will adjust for syndromic status and will be repeated with a larger sample size and with older children (age 5), as children in the Cleft Collective mature.

(1) Speech at Home, Dublin, Ireland, (2) Great Ormand Street Hospital, London, United Kingdom

Background/Purpose: Fifty percent of children with cleft palate have persistent speech problems at 5 years of age requiring speech and language therapy. However, there is a lack of access to regular therapy in many countries. Although parent training/involvement has positive outcomes in early communication skills in cleft palate and noncleft speech disorders, little is known about parents undertaking intervention for cleft speech disorders. The aim of the study was to evaluate an alternative method of facilitating improvements in speech by engaging parents.

Methods/Description: Forty-six children, aged 2.9 to 7.5 years, were included in this 2-phase, 2-center randomized controlled trial (RCT). In the parent-led arm, parents attended a 2-day training course, were given a speech program and conducted home-based therapy for 12 weeks. Parents were supported by a cleft speech and language therapist (CSLT) using telehealth (FaceTime) and a one-to-one face-to-face session. In the control arm, the group received 6 therapy sessions with a research speech and language therapist comparable to that provided by local services. Speech recordings were undertaken pre- and postintervention. Percent consonant correct (PCC) for words and sentences was the primary outcome measure. Activity and participation were analyzed using the Intelligibility in Context Scale (ICS) and the Focus on Outcomes for Children Under Six (FOCUS). Postintervention, parent experience was evaluated. Nineteen (83%) parents participated in telephone interviews or focus groups conducted by an independent psychologist/speech and language therapist.

Results: Both groups showed a statistically significant improvement in PCC for words (P < .001; 95% CI: 7.51-14.24; d = 0.62), PCC for sentences (P < .001; 95% CI: 11.70-21.26; d = 0.3), activity (P < .001; 95% CI: 1.45-3.15; d = 0.46), and participation (P < 01; 95% CI: 7.63-25.03; d = 0.36). There was no statistically significant difference between the groups. Parents praised the content, flexibility, and pace of the intervention program. They commended FaceTime, particularly when activities were not working. Challenges included how they felt “somewhat daunted when taking on the course” but “were very happy to have participated.” Some parents reported being overwhelmed by the materials and concern at being viewed by their child as a teacher. Parents provided excellent critical feedback on how to improve training and fine-tune the therapy programs.

Conclusions: Parent-led articulation therapy, when supported by a CSLT using telehealth, achieves comparable outcomes to standard speech therapy and is acceptable to parents. This study has informed the development of an innovative intervention package called Speech@Home.

Reference

Sweeney T, Hegarty F, Powell K, Deasy L, O’Regan M, Sell D. Randomised controlled trial comparing parent led therapist supervised articulation therapy (PLAT) with routine intervention for children with speech disorders associated with cleft palate. Int J Lang Commun Disord. 2020. Available online. doi:10.1111/1460-6984.12542

(1) University of Bristol and Cambridge University Hospitals NHS Foundation Trust, United Kingdom, (2) University of Bristol, United Kingdom

Background/Purpose: Children born with cleft palate with or without cleft lip (CP+/-L) are risk of persisting speech output problems at age 5 years. There is also increasing evidence that children born with clefts have poorer education outcomes than their peers. Previous research has linked speech input processing skill to speech output and literacy development in children without clefts. To date, the speech output of children born with CP+/-L has been examined and reported extensively. There has been much less attention on speech input processing skills with limited evidence available to inform our understanding of these skills in this population. This study aimed to examine the profile of speech input processing skills in children born with CP+/-L.

Methods/Description: Speech input processing skills were assessed in 61 children with CP+/-L (mean age 69 months). Participants were recruited from 5 regional cleft center sites in England. Speech input processing skills assessed were mispronunciation detection of cleft speech characteristics (CSCs), auditory lexical discrimination, auditory and silent alliteration awareness, and auditory discrimination of nonwords (with and without CSCs). Additional variables were collected via direct assessment (receptive vocabulary and working memory), parent questionnaires, and clinical records. Performance on speech input processing assessments was categorized as “low” or “high”’ and χ ² and t tests used to examine associations between (1) factors associated with speech development in all children, (2) factors associated with speech outcome in children born with CP+/-L and speech input processing. Pearson correlation explored relationships between speech input processing tasks.

Results: Lower scores on all speech input processing tasks were associated with at least one variable representing aspects of language, communication, or learning. There were also associations between some speech input processing skills and other variables including working memory and hearing. Associations varied across speech input processing skills. Correlations between speech input processing tasks were weak to moderate in most cases suggesting they generally tested different skills but with some overlap. Correlations were stronger between the auditory discrimination tasks and between the alliteration awareness tasks. There was little evidence of associations between speech input processing and factors associated with speech outcomes in children born with CP+/-L.

Conclusions: The results provide preliminary evidence to suggest speech input processing skills should be considered in children born with CP+/-L alongside other speech and language skills. Associations between speech input processing and factors relating to aspects of learning and education would benefit from further exploration.

(1) Birmingham Children’s Hospital, Coventry University, United Kingdom, (2) The University of Nottingham, United Kingdom, (3) Great Ormond Street Hospital NHS Trust, London, United Kingdom, (4) Coventry University, United Kingdom, (5) Oxford Brookes University, United Kingdom

Background/Purpose: Over the last decade, there has been an international focus on the reporting of speech outcomes at age 5 years in the cleft population. However, to identify those children most at risk of a poor speech outcome at this age, early assessment and intervention must precede this. At present, there are no published, valid, and reliable assessment protocols for the assessment of speech at age 3 years +/- cleft lip (CP±L) in English. The aim of this study was to investigate the impact of different speech samples, rating methods, and scales on listener reliability when assessing speech outcomes in 3-year-old children with CP±L.

Methods/Description: Twenty-five participants were recruited from a single cleft center; 20 with CP±L and 5 typically developing children without a cleft. In the CP±L group, 50% had cleft palate (n = 10), 35 had UCLP (n = 7), and 15% had BCLP (n = 3). The age range for the group with CP±L was 10 months; for the noncleft group this was 5 months. One speech and language therapist (SLT) carried out all the assessments which were video recorded. Two speech samples were used (sample A: spontaneous speech and picture naming; sample B: short phrase repetition), both designed specifically for the study. The order in which the participants completed the speech samples was randomized. Seven specialist SLTs from 5 different UK cleft units analyzed the speech samples. Cleft speech characteristics, phonology, resonance, nasal airflow errors, and overall ratings of velopharyngeal function were determined. Intrarater reliability was calculated on 23% of the recordings. Following the study, the SLTs completed a questionnaire on the usability and acceptability of the speech samples and rating scales.

Results: **This study is currently in the data analysis stage, and the findings will be updated by December 15 2020**. The results will include comparisons of listener reliability between the 2 speech samples; comparisons of listener reliability of an overall perceptual measure of velopharyngeal function compared to separate measures of resonance and nasal airflow errors; comparisons of listener reliability between ordinal and visual analogue scales; and feedback from the SLT questionnaire.

Conclusions: This study will determine which speech sample and rating methods and scales are most reliable in the assessment of 3 year-olds with CP±L and the acceptability of these to SLTs.

(1) Kaiser Permanente Northern California, Oakland, CA, (2) Kaiser Permanente Regional Craniofacial Multispecialty Program, Northern California, Orinda, CA, (3) Kaiser Permanente Regional Craniofacial Program, Northern California, Oakland, CA

Background/Purpose: As our world faced a global infectious disease pandemic that included social distancing, craniofacial clinics had to reassess how to continue to deliver safe quality care for our clients. This presentation will outline how our clinic was able to transition our physical appointments to virtual team clinics through telemedicine and teamwork. Our team was able to reopen our clinics virtually 2 months after we were required to shelter in place. The goal of this presentation is to show what our team did during this unprecedented time and give other ACPA members working ideas if this situation occurs again.

Methods/Description: The presentation will include background information about the clinics we offered prior to the COVID-19 pandemic and then a description of how our work transitioned into creating a virtual clinic. There will be data presented in terms of number of patients seen in comparison to our in-person clinics prior to the pandemic. There will be a focus on the team members, their roles, and how each person’s contributions added to the overall execution of a new method of care delivery. The format will be power point slides.

(1) Texas Tech University, Lubbock, TX, (2) Department of Otolaryngology, Head and Neck Surgery, Texas Tech University Health Sciences Center, Lubbock, TX

Background/Purpose: To our knowledge, there remain limited studies closely examining supply and distribution of OFC care in the United States. This study sought to investigate ACPA member input on barriers to Cleft Team care in the United States. Survey was distributed to ACPA members involved in team cleft care. One hundred thirty-two respondents with responses capturing subjective barriers to Cleft Care as well as opinions on Geographic areas with underserved populations in the United States.

Methods/Description: Survey was created with help of Clinic Research Institute using Qualitrix software. Questions began with demographic data on respondents including gender and level of training. Questions included “Do you perform OFC surgery in US,” “Do you do cleft surgery on international mission trips,” “What type of Fellowship training,” “Do you work with ACPA accredited team,” “Where is your surgical practice located,” “Top barrier to cleft care access in US,” and “Demographic area in US think is most underserved.” Survey was administered to ACPA members. Total of 132 respondents to survey.

(1) Nationwide Children’s Hospital, Columbus, OH, (2) University of Utah, Salt Lake City, UT

Background/Purpose: Management of children with cleft lip and/or palate is best provided by an interdisciplinary team (ACPA Parameters of Care, 2018). Although the team care needs of children with cleft palate, with or without cleft lip (CP/L), are well known, there remains ambiguity whether children with cleft lip/alveolus without cleft palate (CL/A) require the same care pathway. The aims of this study are to (1) describe current cleft team care practices for children with CL/A, and (2) assess team coordinator perception of the care needs of children with CL/A.

Methods/Description: A REDCap-based survey was distributed by email to all ACPA-approved team coordinators in the United States. Survey questions focused on whether children with CL/A routinely received team visits, which team members routinely saw children with CL/A, differences in team care delivery for children with CL/A versus CP/L, type and frequency of assessments offered to children with CL/A, etc. Coordinators were asked about their perception of the risk of speech, hearing, dental, and appearance concerns in children with CL/A. Responses were collected anonymously. Data were exported into SPSS for analysis.

Results: Fifty-seven surveys were completed (35% response rate), with 71% of surveyed coordinators indicating that they were a nurse; 19.6% of team coordinators reported they were not familiar with the ACPA parameters of care; 78.9% indicated they routinely see children with CL/A for team visits, with 40% seeing them annually. Children with CL/A were most likely to see the plastic surgeon (94%), feeding specialist (90%), and orthodontist (78%). Half of teams employ a “different” approach to team care for children with CL/A compared to children with CP/L; 50% routinely referred children with CL/A for hearing testing and 35% reported that they receive a speech evaluation every 1 to 2 years. 47% of teams only evaluated speech “as needed,” yet 48% of coordinators thought children with CL/A had a higher risk for speech disorders than the general population. The majority felt that children with CL/A had a high risk of appearance- and dental-related concerns. Qualitative analysis of open-ended responses revealed that most coordinators felt children with CL/A should receive help with psychosocial needs (28%), dental/jaw needs (59%), and speech-language or hearing (38%). Overall, 70% of respondents felt that all children with CL/A should receive routine team visits, regardless of need.

Conclusions: There is considerable variability in current team care practices for children with cleft lip/alveolus, without cleft palate, among ACPA Approved teams. Teams may benefit from further education regarding care needs for children with CL/A. Screening tools that can assist with needs assessment may also be helpful to guide team care decision-making.

(1) Nationwide Children’s Hospital, Columbus, OH, (2) Nemours Children’s Hospital, Orlando, FL, (3) Unaffiliated, Columbus, OH

Background/Purpose: 22q11.2 deletion syndrome (22q11DS), a condition associated with multiple health and neurodevelopmental sequelae, is best managed though comprehensive centers that offer coordinated interdisciplinary team care. Potential benefits of team care include ensuring completion of evaluations and screenings recommended by the International 22q Care Guidelines (Bassett et al., 2011). A quality improvement (QI) project was initiated at our institution to increase 22q guideline adherence among patients with 22q11DS. Using electronic medical record (EMR) and clinic-based interventions, we aimed to increase the proportion of patients with 22q11DS who were adherent to key 22q guidelines.

Methods/Description: Patients with confirmed 22q11DS seen by the 22q team between October 2017 and January 2018 were included in the project. The QI team assessed adherence to 8 22q care guidelines including those satisfied by one-time assessments and those requiring repeat surveillance (eg, labwork). Adherence was measured as a “bundle” (adherence to all 8 guidelines). A cause–effect analysis was completed and key drivers identified. The project aimed to increase the proportion of patients with 22q11DS with adherence to 8 key care guidelines from 71% (baseline) to 95% (goal). Several educational and EMR-based interventions were implemented. These included (1) an expanded after-visit summary explaining the value of 22q surveillance testing to families; and (2) EPIC-based Best Practice Advisory (BPA) alerts for providers. These included a Health Maintenance BPA reminding providers to order surveillance testing when indicated, as well as a Referral BPA to encourage referrals to the 22q Center. Monthly BPA reports were generated to track utilization of EMR tools as a process measure. Pre-post assessments of one-time and repeat surveillance assessments were performed.

Results: The proportion of 22q11.2DS patients adherent to all 8 guidelines increased from 71% to 84% at 10 months postintervention. Stratification based on the frequency of assessments showed adherence for repeat surveillance measures (eg, labwork) increased from 76% at baseline to 89% postintervention. In just 5 months, the Referral BPA fired 301 times, leading to 68 referrals to the 22q Team. The Health Maintenance BPA was acted upon 74 times (11.7% provider interaction).

Conclusions: Educational and EMR-based interventions for both patients and providers have improved 22q care guideline adherence in a short period of time at our 22q Center. Variations in guideline adherence for one-time screenings versus repeat labwork will be discussed, as well as new interventions which are currently underway to improve adherence to specific care recommendations. EMR-based interventions offer 22q centers new options to employ automated technology to facilitate increased referrals to team care and improve provider adherence to 22q surveillance testing at appropriate intervals.

(1) The University of Glasgow, Scotland

Background/Purpose: Although the well-being of people affected by cleft lip and/or palate (CLP) is a well-researched area, there is a paucity of research concerning the medical experiences of patients during cleft treatment. The current study aims to address this gap. In the United Kingdom, multidisciplinary cleft teams treat patients in structured care pathways. Surgical interventions aim to improve functional and aesthetic outcomes. However, with appointments and surgeries spanning the timeline of a child’s youth, one can see that cleft care can place a heavy burden on an affected child. Although comprehensive medical care is essential for positive long-term outcomes, important domains of well-being such as education and leisure time may be affected by lengthy and frequent hospital stays. This study therefore aims to explore the potential impact that treatment for CLP may have on the well-being of affected children.

Methods/Description: A qualitative approach was adopted for this study, allowing for a deep exploration of the research topic. Carers of children with CLP were deemed appropriate participants given the early age at which cleft care commences. Following advertisement by Cleft Lip and Palate Association, 12 self-selected participants were recruited. Semistructured telephone interviews were conducted, loosely following an interview guide which covered relevant domains. The interviews were transcribed and analyzed using Braun and Clarke’s 6-phase approach to thematic analysis (2006). The study was approved by the University of Glasgow, School of Psychology Research Ethics Committee.

Results: Five master themes were generated following data analysis. Participants felt that living with CLP disrupted many domains of a child’s life, including sleep, education, feeding, and one’s sense of normality. A lack of awareness of CLP was also experienced both in school settings and in health care workers. Participants also discussed the importance of child-centered care, discussing the advantages of staff continuity, and the benefits of child friendly environments. Effective communication was also deemed important by participants, both with health care workers, with other families affected by CLP, and with their own children to prepare them for the future. Finally, the influence of age on living with a cleft was discussed, with changes in coping and difficulties associated with evolving autonomy becoming important issues as their children grew older.

Conclusions: Findings of the current study suggest that the medical treatment of CL/P may cause disruptions to a child’s life and raise age-specific challenges. Negative outcomes of cleft care may be minimized by effective communication, child-centered health care, and raising awareness of CL/P. A finding that was not included in the literature that informed the study is also presented, in that the medical treatment of CL/P may emphasize difference in young people with craniofacial anomalies.

(1) Massachusetts General Hospital, Boston, MA, (2) Shriners Hospitals for Children, Boston, MA

Background/Purpose: Multidisciplinary and longitudinal care are key attributes of cleft teams. During COVID-19, all cleft centers had to adapt to deliver care while observing new regulations on physical distancing. We used Zoom as a telehealth platform and replicated our native in-person multidisciplinary team workflow to a virtual format. We preserved key aspects of our in-person clinic and this virtual workflow using Zoom was so successful, that we continue to utilize the telehealth option for several key advantages it provides.

Methods/Description: We used a telehealth Zoom platform with patient-specific secure password access. The Cleft team coordinator served as the Zoom “host” who preassigned all specialists into individual “breakout rooms” on the Zoom application. After the patient joins the main session, the Cleft Team Coordinator can then navigate the patient through the different breakout rooms with various specialists via the “Breakout Rooms” tab that is seen in the bottom right of the call.

Results: Our Cleft Clinic carried out 33 in-person patient consultations before the COVID-19 shutdown in Boston (January 2020-February 2020). As Covid-19 regulations mandated closure of all nonessential clinical activity, we operationalized the telehealth clinic. During this time, we saw 10 patients via the telehealth platform (March 2020-May 2020). As conditions evolved, we incorporated telehealth in a hybrid model, where we saw a total of 43 patients (June 2020-August 2020), of which 19 (44%) were in person and 24 (56%) were via the telehealth platform.

Conclusions: Implementation of a virtual cleft and craniofacial clinic has proven to be an effective substitute for the native in-person patient experience. Ongoing work will include patient-reported survey of these clinic experiences. The Zoom telehealth system negated the distance between provider and patient and allowed our cleft team to broaden our reach to international patients. Furthermore, young patients with cleft malformations can by accompanied by family members in the same Zoom session even though the family members may not be physically together. This style of clinic also allowed greater access to specialists who are still asked by the hospital to work remotely. We found the ability to set individual “Break out rooms” for each specialist to be a key feature available on Zoom but not in other telehealth applications. We describe this Zoom telehealth workflow as a framework to integrate efficient multidisciplinary and longitudinal care in the treatment of patients with cleft abnormalities during COVID-19 but also as a paradigm shift in cleft care delivery.

(1) Washington University School of Medicine in St. Louis, MO

Background/Purpose: Correction of a cleft or craniofacial anomaly can be complex, and treatment often spans several years. Team care, including multiple specialists, is standard practice. Teams include plastic surgeons, speech pathologists, audiologists, dentists, psychologists, orthodontists, otolaryngologists, and others. However, multispecialty visits can be costly to families. In turn, high costs lead to elevated attrition rates for children with cleft, potentially compromising patient outcomes. We aim to examine the direct financial burden of a cleft-craniofacial team visit on the patient’s family.

Methods/Description: Retrospective analysis of billing records was conducted on a curated group of patients (n = 21) who received care from an ACPA-approved cleft-craniofacial team at an academic children’s hospital during the first half of 2019. Out-of-pocket costs resulting from facility fees and some departmental charges were included. Patients were selected to represent a variety of commercial insurance plans, and encounters included both “mini-team” visits (n = 10), where patients saw providers from 4 or fewer disciplines, as well as “full-team” visits (n = 11), where patients saw providers from 5 or more disciplines. The Mann-Whitney U test was used to evaluate cost differences between different groups. A cost and satisfaction survey was distributed to the families of a second cohort of patients (n = 6) who received cleft-craniofacial team care between January and June 2020. The survey collected self-reported out-of-pocket costs and responses regarding perceived value of team care.

Results: Out-of-pocket costs for a single team care visit averaged $350 ± 410, mean ± SD. Costs ranged from $0 to approximately $1230 and had a median (IQR) of $120 (0, 590). Notably, out-of-pocket costs for full-team visits ($120 [0, 780]) were not significantly greater than those for mini-team visits ($130 [10, 500], P = .457). Family-reported satisfaction was high, with all survey respondents reporting excellent quality of care. The self-reported out-of-pocket costs ranged from $0 to $1000. All survey respondents agreed or strongly agreed with the following statement: “Cost affects how likely we are to return for all recommended team care visits.”

Conclusions: Out-of-pocket costs to patients and their families vary widely, from patients on Medicaid with no out-of-pocket costs, to patients with high deductible health plans that are responsible for almost the entire balance. In the words of one survey respondent, “the costs that are required for treatment do become a bit of a financial burden, and insurance does not always cover as much as we would like.” Collaboration with multiple stakeholders including providers, insurers, and patient guardians, as well as hospital administrators, is critical to increase patient retention and improve final clinical outcomes.

(1) Children’s Hospital Los Angeles, CA

Background/Purpose: Health care providers have always experienced high levels of burnout and compassion fatigue, but during this incredibly unique time (COVID-19), burn out, stress, and feelings of hopelessness in the health care setting are even more prevalent. Additionally, working in a health care setting, providers are 4 times more likely to be victims of workplace violence than those working in private industry. Providers may also find themselves in situations that are ethically questionable or morally distressing. These experiences result in increased levels of stress. Craniofacial teams also have their own unique stressors, with providers caring for complex patients with multiple diagnoses who may be difficult to treat, possibly in the setting of social or cultural barriers to care. Families who seek out information on the internet or through social media may have unrealistic expectations or demands, leading to families feeling disappointed in outcomes and/or frustrations. The purpose of this presentation is to acknowledge the challenges of providing care to patients with Craniofacial differences in the 21st century and to identify the ways in which providers can protect against stress and burnout.

Methods/Description: A review of current data regarding stress among health care providers was conducted. Resources to support health care providers in various types of adversity, both locally and nationally, were reviewed and compiled. Main Objective of the Presentation: The main objective of the presentation is to reach providers who are experiencing professional burnout and help them identify their need for self-care, provide suggestions for supporting their Team members and to be an advocate for institution wide “caring for the caregiver” programs.

(1) University of Missouri-Kansas City School of Medicine, MO, (2) Children’s Mercy Kansas City, MO

Background/Purpose: Management of children with diagnoses of cleft lip and/or palate requires long-term commitment and follow-through on the part of family and medical caregivers. Patients can have medical challenges starting in infancy and continuing as they develop over time. Coordinated cleft team care involves the recurrent assessment of these children to ensure best outcomes. This often occurs on a yearly basis, or more frequently depending on age and diagnosis. Nonetheless, for a variety of reasons many children experience early attrition and become lost to follow-up (LTFU). In this case, the Cleft Team is unable to ensure complete and adequate management of their cleft issues.

Methods/Description: This single-center retrospective review evaluated all patients undergoing primary palatoplasty at a tertiary pediatric hospital for a diagnosis of cleft palate only (CPO) or cleft lip/palate (CLP) between January 2015 and May 2018. Records were reviewed for cleft diagnosis, type of surgery, and timing of post-palatoplasty visits. All patients with unplanned absence from their scheduled Cleft Team evaluations for 2 years or more were considered LTFU. Clinic notes were examined for any documentation to explain the separation.

Results: During the study period, 126 children underwent primary cleft palate repair (male = 55, 43.6%; female = 81, 64.3%). Diagnoses included 33 (26.2%) unilateral CLP, 31 (24.6%) bilateral CLP, 50 (39.7%) CPO, and 12 (9.5%) submucous cleft palate (SMCP). From this group, 26 (20.6%) of 126 patients requiring long-term cleft-related management have been absent from clinic for at least 2 years. Records identified 8 documented reasons including death of patient (n = 1), family relocation (n = 4), loss of insurance (n = 1), and family refusal of care (n = 1). In the other 18 cases, patients were recommended for routine follow-up or even additional surgery but have not returned to clinic for unknown reasons. Many records indicate multiple unsuccessful attempts to reconnect with these families. Characteristics of the LTFU group include 8 (30.7%) males and 18 (69.2%) females. Patients with CPO are relatively overrepresented among the LTFU group (14/26; 53.9%) compared to those with CLP (bilateral = 4/26; 15.4% and unilateral = 5/26; 19.2%) or SMCP (3/26; 11.5%). No gender or age-related differences were found. The LTFU group traveled further than the remainder (mean: 112.9 vs 85.6 miles), but this difference is not statistically significant (P = .24).

Conclusions: This study identifies a rate of 20.5% of patients who have undergone cleft palate repair who do not continue with planned follow-up through the multidisciplinary cleft team. For 69.2% of these patients, the reasons for attrition from clinic are not understood. It appears that patients with CPO may be more likely than those with CLP or SMCP to prematurely lose connection with the team. Other specifically significant risk factors for attrition were not readily identified from the records and can only be surmised without further investigation.

(1) Spectrum Health Medical Group, Grand Rapids, MI, (2) Spectrum Health, Michigan State University, Grand Rapids, MI

Background/Purpose: The reconstructive surgeon’s challenge is to achieve a complete restoration for all anatomical variations of the cleft palate. Traditional beliefs hold that there is sufficient tissue within the cleft to accomplish this in all cases. Presently, the vast majority of surgeons use single pattern repairs which are highly inflexible and may only work well on certain anatomic presentations. Many surgeons revert to older, more growth restricting techniques on complete or wide clefts. The Anatomic Cleft Restoration Philosophy (ACRP) believes that as tissue deficiency is identified in the cleft defect it must be replaced to achieve a complete palate restoration. The Double Opposing Z-pasty +/- Buccal Flap Approach (DOZP+/-BFA) fits within the ACRP and has demonstrated excellent speech results regardless of cleft width or classification. In addition to excellent speech results, excellent growth results have also been presented. Nonanatomic secondary VPD surgeries such as pharyngeal flaps and sphincteroplasties are fraught with problems. Whereas, the ACRP inspired buccal flap palate lengthen procedure has proven successful, leaving patients with more normal anatomy. The purpose of this workshop is to teach the Anatomic Cleft Restoration Philosophy. This workshop will also demonstrate how to use the buccal flap and other useful flaps to achieve a complete restoration of the cleft palate, thus improving outcomes in both primary and secondary palate repair.

Methods/Description: The buccal myomucosal flap has proven its great applicability in cleft palate repair. The use of this flap and others will be taught via video, slides, didactic presentation, and audience interaction. Participants may bring cases for discussion. Specific surgical details will be taught to maximize successful outcomes. The senior presenter will relate his experiences with over 1500 buccal flaps surgeries used in various cleft palate applications. The Anatomic Cleft Restoration Philosophy (ACRP) will be described. Using the ACRP has led to the development of multiple reconstructive procedures that leave the patients with more normal anatomy.

(1) Children’s Mercy Hospital, Kansas City, MO

Background/Purpose: Most literature and educational content on alveolar bone grafting in the patient with cleft lip and palate is focused on the unilateral situation. The bilateral case, while occasionally straightforward, is unarguably more complicated from a technical perspective, and results are typically less favorable than in the unilateral case. Bilateral cases are often complicated by a malpositioned, often diminutive, premaxilla, large anterior palatal fistulae, or multiple previous failed attempts at grafting. Surgical access for proper soft tissue closure and maintenance of blood supply to the premaxilla can be a challenge. The purpose of this presentation is to discuss the management of alveolar clefts in patients with bilateral cleft lip and palate, starting with the conventional and moving to the more complex.

Methods/Description: The basic technique of bilateral alveolar cleft grafting will be described. Splint fabrication will be discussed. More complex cases will be highlighted, including advanced methods for closure, such as premaxillary osteotomy, segmental osteotomies, distraction osteogenesis, and soft tissue flaps. The presentation will be case based with photographic and video descriptions, with audience participation welcomed.

(1) Helen DeVos Children’s Hospital, Grand Rapids, MI, (2) Cincinnati Children’s Hospital Medical Center, OH, (3) University of Michigan Oral and Maxillofacial Surgery, Ann Arbor, MI, (4) Beaumont Hospital, Detroit, MI

Background/Purpose: The most challenging reconstructions in craniomaxillofacial surgery are hypoplastic asymmetries. Hemifacial microsomia (HFM) is the most common. HFM is a variable and heterogeneous malformation involving the lower two-thirds of the face and ear. This represents embryologic hypoplasia of the structures arising from the first and second brachial arches. The challenges of reconstructing severe grades of HFM are well recognized. Numerous protocols for autologous reconstruction have been advanced. Unfortunately, these have not been substantiated in patient series with consistently acceptable results. Coordinated care often proceeds through puberty and can involve orthodontics, soft tissue restoration, skeletal orthognathic surgery, TMJ reconstruction, and auricular correction or prosthetics. Purpose: To improve the outcomes for children with HFM and discuss the multiple stages of surgical/orthodontic interventions. New customizable techniques will also be presented.

Methods/Description: This upper-level course will start with a brief discussion of HFM and its etiology. We will then review our extensive experience with distraction osteogenisis. Emphasis will be placed on long-term follow-up and the significant relapse that is experienced with these unique patients. For patients with class IIB and III HFM, we will present our techniques for customized TMJ reconstruction. We will also present customized TMJ options for other conditions such as arthritis. The role of orthodontic coordination is emphasized. The soft tissue envelope will be addressed next. Comparisons between microsurgical interventions and fat grafting will be presented. Finally, our preferred method for autologous ear reconstruction and the unique technical challenges of the patient with HFM are reviewed.

(1) Children’s Hospital of Philadelphia, PA, (2) Nationwide Children’s Hospital, Columbus, OH, (3) Children’s Hospital, Clinical Genetics Center, Philadelphia, PA, (4) Institute of Neuroscience and Physiology, Speech and Language Pathology Unit, Gothenburg, Sweden, (5) Great Ormond Street Hospital NHS Trust, London, United Kingdom, (6) NHS, Essex, London, United Kingdom

Background/Purpose: Speech and language disorders are hallmark features of 22q11.2 deletion syndrome (22qDS). Learning disabilities, cognitive deficits, palate abnormalities, velopharyngeal dysfunction, behavioral differences, and various medical and psychiatric conditions are also major features of this syndrome. The complexity and variability in the phenotypic presentation of 22qDS poses significant management challenges to craniofacial teams and speech-language pathologists (SLPs). Although general medical care guidelines for management of 22qDS have been available for several years (Bassett et al., 2011), specific suggestions for speech-language pathologists, and others engaged in the management of velopharyngeal dysfunction and communication disorders in 22qDS, were still needed. The purpose of this session is to summarize the state of the art of current clinical and scientific knowledge regarding speech-language disorders in 22qDS and provide recommendations for best practices for clinical management.

Methods/Description: An expert international group of SLPs and health care providers, with extensive clinical and research experience in 22qDS, was formed, and over a period of several years developed a consensus set of principles and practical suggestions for management of communication disorders in 22qDS (Solot et al., 2019). In this session, a general overview of the phenotype and care needs of individuals with 22qDS will be presented. 22qDS best practices for diagnosis and management of speech-language disorders in 22qDS will be discussed. In this session, members of the expert consensus group will present and discuss the best practices, including diagnostic procedures, treatment protocols, and associated management recommendations for SLPs, surgeons, and other practitioners caring for this population across the life span. Approaches to implementing 22qDS best practices will be discussed, as well as strategies to optimize outcomes and minimize long-term communication impairments.

(1) Seattle Children’s Hospital, WA, (2) UCLA, Los Angeles, CA

Background/Purpose: Surgery is often the first-line approach in the management of velopharyngeal dysfunction (VPD) for individuals with cleft and craniofacial conditions. When surgical options have been exhausted or in cases where surgery is contraindicated, prosthetic management can be an effective alternative. With advancements in surgical techniques, the use of prosthetics for the management of VPD has been eliminated from many centers; however, not all patients with VPD are optimal surgical candidates. Contraindications for surgical VPD management include obstructive sleep apnea, inadequate tissue due to previous surgeries, or serious medical conditions that contraindicate anesthesia or surgery (ie, certain cardiac disorders). Overall, the decision to pursue surgical versus prosthetic management of VPD should be determined by the multidisciplinary team. At our craniofacial center, the multidisciplinary team including otolaryngologists, plastic surgeons, speech-language pathologists (SLP), pediatricians, and orthodontists determine the patient’s surgical versus prosthetic candidacy. Once an individual is referred for prosthetic management, the otolaryngologist performs ongoing instrumental evaluation in order to guide management. The orthodontist fabricates and modifies the speech appliances as needed. The SLP works in conjunction with the orthodontist to adjust the appliance to meet the specific VPD needs of the patient. The goals of this foundational course are to (1) discuss rationale for use of speech prosthesis in lieu of surgical management, (2) facilitate multidisciplinary discussion about the decision-making process, and (3) provide an overview of the process involved in prosthetic management of VPD, from the initial evaluation to the final stages of prosthetic modification.

Methods/Description: This foundational course will include perspectives from a pediatric dentist/orthodontist, speech-language pathologist, and otolaryngologist with experience treating children with complex craniofacial conditions. Patient populations will be described, processes will be defined, and protocols will be discussed. Cases will be used to highlight key points. The presentation will provide an overview of the working relationship between the team members that are integral to the success of the speech prosthesis as well as specific information about holistic patient assessment, appliance selection and fabrication, and medical coding. The information presented in this session is meant to be of interest to SLPs, surgeons, pediatric dentists, orthodontists, prosthodontists, and any others involved in treating VPD in children with complex medical and/or craniofacial conditions.

(1) Children’s Mercy Kansas City, MO, (2) Children’s Mercy Kansas City, KS

Background/Purpose: It is well understood that orofacial clefting and craniofacial disorders can be syndromic or isolated, resulting from either a single genetic variation or a combination of multiple genetic and environmental factors, respectively. We commonly treat both isolated and syndromic anomalies within a multidisciplinary team setting. A genetic specialist may not always be readily available at the time of initial presentation. Therefore, it is imperative that the evaluating clinicians be able to recognize certain constellations of features associated with known cleft and craniofacial syndromes. Having a basic understanding of the etiology will allow for appropriate navigation of the numerous genetic tests and interpretation of the results. This can allow for a more streamlined process and utilization of resources. Ultimately, understanding the genetic contribution to the clinical presentation will help clinicians provide better medical management for each individual patient.

Methods/Description: This lecture-based foundational course is designed to help explain the basics of genetic and environmental causation of common cleft and craniofacial conditions. This will serve as a primer on topics such as inheritance patterns, genetic family trees, basic genetic testing strategies, and interpretation of results. We will discuss the role of a genetic counselor versus a clinical geneticist and what a formal genetic consult entails. Attention will be given to different specific syndromic and nonsyndromic diagnoses and describe how a clinician might work to recognize a child at risk for a formal genetic diagnosis. As a part of this education, we will explain how different genetic tests may or may not help identify a suspected problem, when and how certain tests should be ordered, and how those results are interpreted. Lastly, we will touch on how genetics researchers are working to identify new candidate genes which may have a role in cleft and craniofacial conditions.

(1) Children’s Mercy Kansas City, MO, (2) Children’s Mercy Kansas City, KS

Background/Purpose: Children born with oral clefting face a variety of challenges related to feeding and weight gain in the first year of life. These children are at increased risk for growth disturbance, malnutrition, and even delays in surgical care or complications as a result of slow growth or poor weight maintenance. In the neonatal period families often struggle with learning or applying appropriate feeding techniques to maximize their infant’s nutritional intake. Neonatal feeding challenges are compounded by eventual transition to solids, cup training, and perioperative dietary restrictions that can unnecessarily increase family stress during the first year. Nutritionally, there is also concern for children losing weight after surgical interventions as a result of pain, dietary restrictions, and increased nutritional requirements. Multidisciplinary management of feeding and growth challenges throughout childhood is crucial to support best outcomes for these children and their families. In our experience, comprehensive team-centered nutritional care should include the cooperation of a committed group of feeding specialists that evaluate and manage patients in both inpatient and ambulatory settings with an organized and multifaceted approach as the child ages. Our primary aims are to avoid malnutrition and support healthy growth and development while easing the stresses associated with feeding challenges in this special population.

Methods/Description: This multidisciplinary presentation will identify the role of each feeding-related specialty involved in our cleft nutritional management approach and discuss nutritional needs, pearls, and pitfalls at each stage of infant and child development. The importance of early, frequent, and supportive interactions with families will be stressed and we will detail the appropriate assessments and interventions necessary to help these children and their families meet age-appropriate nutrition and lactation goals throughout the first year of life. We will highlight ways in which different team compositions can maximize their newborn feeding support, teach food, and cup transitions and provide comprehensive growth and feeding recommendations. We will also discuss our strategies for perioperative nutritional interventions in the inpatient and ambulatory settings to support families, respect required dietary restrictions, and identify appropriate high calorie nutrition sources to help prevent significant postoperative weight loss and help ensure the children will recover and heal appropriately after lip and palate surgery. The target audience for this presentation will be anyone involved with or interested in learning about nutrition, feeding, or lactation education and assessment/support of patients with a cleft or craniofacial condition.

(1) Nationwide Children’s Hospital, Columbus, OH, (2) Children’s Medical Center, Dallas, TX, (3) University of Michigan Medical School, Ann Arbor, MI

Background/Purpose: Families of children with craniofacial conditions face numerous treatment-related decisions. Over time, youth themselves become increasingly involved in decision-making about their care, particularly for procedures with primarily aesthetic goals. Tension between (and among) caregivers, youth, and provider team members can emerge around treatment decisions. There can also be uncertainty about treatment outcomes which can impede patients’ and/or families’ abilities to make informed decisions. Further, there can be challenges related to patients’ abilities to assent and participate in decision-making because of developmental and other factors. Shared decision-making (SDM) is a central feature of patient/family-centered care and is associated with patient satisfaction with treatment outcomes and reduced likelihood of decisional regret. SDM is an evidenced-based approach grounded in ethical principles that can be used to help providers, patients, and families make craniofacial care-related decisions consistent with family values and preferences. It is often assumed that providers practice SDM, yet research suggests that the key elements of SDM are often absent from treatment-related discussions with patients and/or caregivers. For this reason, use of decision aids, or tools that enable patients and/or caregivers to become active participants in the decision-making process, is recommended. The purpose of this course is to provide an overview of the principles of SDM and how it can be operationalized and implemented in the context of craniofacial care through the use of decision aids.

Methods/Description: This course will (1) provide an overview of SDM and evidence for its effectiveness, (2) review use of patient decision aids as facilitators of SDM and benefits and barriers to their use, (3) apply SDM to case examples across various ages, (4) discuss strategies for integrating SDM into routine clinical practice.

(1) Universidad Autonoma de Manizales, Pereira, Risaralda, Colombia

Background/Purpose: The Problem: Despite continuous improvement in human, technical, and financial resources, why is it that in certain specialties, we continue to struggle to deliver the ideal clinical results in large-scale management of patients with cleft-related sequelae? In my opinion, the problem is 2-fold: The first reason is that many of the contemporary treatments available to cleft patients necessarily demand recurrent monthly appointments at the treatment center. A good example is Mixed Dentition Corrective Orthodontics, in which a highly specialized professional must see the patient once a month, for as long as 2 years, meticulously performing at each visit, minute adjustments in orthodontic appliances to obtain the desired clinical results. The nature of current treatments is highly dependent on the manual dexterity and clinical expertise of a small number of professionals. The second reason, which only exacerbates the first, is the practical dilemma posed by the distance patients must travel in order to receive treatment, in most cases with very limited transportation infrastructure, as described in many research articles. The Solution: As was the problem, the solution is also 2-fold: First of all, to correct the position of our patients’ teeth and bone structure, without mandatory visits to treatment centers, we propose the development of protocols for digitally manufactured sequential aligners that modify oral structures, according to a computer-generated treatment plan. The digitally designed tooth movements will be accomplished by a series of plastic thermoformed aligners, worn 24 hours a day for 2 weeks per stage. The second strategy, to assure that our treatments are progressing without complication, or to recognize when the patient must come to the treatment center for an occasional intervention by the specialist, we propose the development of a mobile phone–based remote monitoring application. With a simple kit and a few minutes of training, we can empower our patients and their caretakers to become our clinical eyes from afar. Presentation Goals: After attending the session, attendees will have a clear understanding of the rationale behind digital technology–based treatment of cleft patients in mixed dentition and the enormous possibilities of remote treatment monitoring. After this experience, it is expected that many cleft treatment providers will be inticed to continue the learning process that will permit the implementation of these novel applications in their own practices.

Methods/Description: Presentation Format: Virtually delivered Keynote presentation. Presentation Content: (1) Current dilemmas in delivery of orthodontic treatment to cleft patients. (2) Innovative approach to orthodontic treatment for cleft patients, (3) Fundamental concepts of CAD-CAM technology in orthodontic care. (4) Remote monitoring of orthodontic treatment. (5) Presentation of clinical cases. (6) Future perspectives for digitally based cleft treatment.

(1) Riley Hospital for Children at IU Health, Indianapolis, IN, (2) West Virginia University, Morgantown, WV

Background/Purpose: The use of oral motor exercises (OME) has a long history in speech-language pathology that continues, despite the fact that our knowledge base has greatly expanded. Our treatment literature contains a number of controlled studies, which support the use of different speech-based treatments in modifying the speech sound disorders of children both with and without velopharyngeal dysfunction (VPD). This proposal will discuss issues that contraindicate the use of OME in the treatment of children with VPD. It will examine arguments that proponents of OMEs have used and contrast these arguments with current literature. Practical therapy examples will be discussed as well as ways for the Team SLP to engage caregivers and community SLPs into the discussion. The topics that will be covered include the following: (1) What are OMEs? (2) Are OMEs relevant to the speech treatment process? (3) Are their theoretical issues underlying the employ of OME? (4) Is there a body of research that identifies differences in the development and use of speech and oral motor tasks? (5) Is strengthening muscles a legitimate goal for children with VPD? (6) Can OMEs aid in the development of oral awareness? (7) Why does OME remain a treatment used by a number of SLPs? (8) Is irrefutable evidence the only metric in evidence-based decision making? (9) So what do I do now?

Methods/Description: This submission will begin with presentations that discuss first, the background and development of oral motor exercises; next, a brief description of cleft palate speech errors; and finally, the theoretical framework for cleft palate–related speech therapy. It will then transition to more of a scripted question and answer format representing questions/points from both sides of the oral motor exercise argument. The goal is to leave the attendee with the theoretical framework for critical thinking in the SLP clinical practice, but also practical suggestions for engaging in conversations with community SLPs and caregivers that may be proponents for oral motor exercises.

(1) The Children’s Hospital at Westmead, Sydney, Australia, (2) Pacific University, Forest Grove, OR, (3) University of Iowa, IA, (4) St. Louis Children’s Hospital, MO

Background/Purpose: In 2014, a draft set of professional competencies for the perceptual evaluation of cleft palate speech was published from the Speech Assessment Task Force of the 2013 International Cleft Palate and Craniofacial Congress. Additionally, the task force generated a series of general and competency-specific questions that could guide subsequent appraisal and study of these competencies. The publication included a recommendation for further review and revision of these competencies, with scope for expansion of this document. The aim of this focus group will be to seek input and discussion from members of the ACPA on required refinements and amendments to these draft competencies.

Methods/Description: A focus group will be facilitated by a group of experienced Speech-Language Pathologists with knowledge of the original development of the draft competencies. The presentation will include a review of the scope of the perceptual assessment competencies; a review of the existing competencies; and a facilitated discussion around the requirements of each. The application of these competencies to crosslinguistic speech assessment will also be considered. Consensus agreement will be recorded and items requiring further discussion or review will be identified for broader evaluation. Participants will be supplied with a copy of the publication from the Speech Assessment Task Force for review. A new professional competencies document will be generated from the focus group based upon consensus agreement of the participants. In addition to this, a process for the subsequent dissemination of this document for review by the wider ACPA membership will also be established.

(1) Seattle Children’s Hospital, WA, (2) University of California Los Angeles, CA, (3) Children’s Hospital Los Angeles, CA, (4) University of California San Francisco, CA

Background/Purpose: The majority of patients cared for by multidisciplinary craniofacial teams have conditions with orofacial clefting, craniosynostosis, or branchial arch anomalies. Team providers must determine whether the patient’s findings represent an isolated condition or possible underlying genetic syndrome. For each patient, the provider must make recommendations for additional assessments ranging from laboratory tests, imaging, and subspecialty consultation to assure that the child is safe and ready for surgical procedures and to guide medical management. This includes need for and timing of genetics consultation and testing. The goals of this workshop are to (1) provide a framework for providers regarding indications for genetic assessment of children with orofacial clefting, craniosynostosis, and branchial arch anomalies and impact of the genetic diagnoses on medical and surgical management, and (2) determine utility of proposed genetic testing algorithms for children with orofacial clefting, craniosynostosis, and branchial arch anomalies.

Methods/Description: A panel of experts in Craniofacial medicine (pediatricians and geneticists) will discuss their approach to the genetic assessment of patients with orofacial clefting, craniosynostosis, and branchial arch anomalies. Presenters will provide workshop participants with (1) a summary of frequency of underlying genetic causes, (2) potential impact of genetic testing on patient care, and (3) an algorithm for genetic assessment and testing for each major category. Workshop participants, assisted by experts will have the opportunity to apply the algorithms in the assessment of provided case scenarios.

(1) East Tennessee State University, Johnson City, TN, (2) Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE

Background/Purpose: A recent development in Cleft Palate Team Care is the transition of care for young adults with cleft lip and palate (CLP) and their families moving from child to adult-centered care. CLP Teams are increasingly becoming aware of the needs of these young adults with CLP as they are discharged from the pediatric team. To date, few teams in the United States have acted on this need and provide a transition of care plan to their patients aging out of their pediatric team care (Bisceglia et al., 2017). Although the ACPA Parameters of Care (ACPA, 2018) allude to services for adults with CLP, transition of care has not yet been included in this document. In opening this door, Vallino and Louw (2017) proposed an evidence-based conceptualized model for the transition of care for young adults with CLP. Yet, there is more to do. In order for CLP team care to advance to meet the needs of patients across the age spectrum, there is an urgent need for future professionals to be trained in transition of care for young adults with CLP. Interprofessional Education (IPE) is the ideal approach to follow in jointly preparing future professionals in transition of care since it is a team endeavor. According to the WHO (2010), IPE ensures that the future health workforce is collaborative-practice ready It is timely for teams to embrace the concept of transition of care and to train future professionals to provide services that recognize the importance of personal and environmental factors in facilitating holistic transition planning and service delivery and are developmentally appropriate to young adults with CLP (Farre & McDonagh, 2017). Training future professionals can be approached through problem-based learning (PBL). Anchored to PBL is experiential learning (EL). Central to both is to encourage critical and independent thinking in the student. Teams who provide transition of a care to their patients and commit to training future professionals will contribute to sharing the national health burden of these young adults, strengthen the skills of future professionals, and contribute to an optimal health system with improved health outcomes. The aim of this presentation is to describe a model for preparing future professionals in the transition of team care for young adults with cleft palate within an ICF framework following student-centered approach.

Methods/Description: An overview of adolescents/young adults with CLP and a service delivery model for their transition of care will be described (Vallino & Louw, 2017). The key components of the proposed IPE training model of transition of care to be discussed are (1) The concept of IPE; (2) The International Classification of Functioning, Disability and Health; (3) Strategies for PBL and EL; (4) Suggestions to guide future professionals through the 4 integrated processes of EL. Practical strategies for teams to involve future professionals in developing and providing transition of care to young adults with CLP will be presented.

(1) NYU Langone Health, Mount Kisco, NY, (2) Wyss Department of Plastic Surgery, NYU Langone Health, New York, NY, (3) NYU Langone Health, New York, NY, (4) myFace, New York, NY

Background/Purpose: It is well known that pediatric cleft and craniofacial patients encounter multiple challenges in their lives. Our goal is to present to mental health professionals and craniofacial center providers a Panel Workshop for helping parents/caregivers learn how to best advocate for their school age children with craniofacial conditions who experience academic and social challenges. Information about various types of schools, choosing a school, enrollment and school placement, in-school special services, home tutoring following surgery, parent–teacher conferences, how to transfer to another school, and related topics will be discussed. In addition, we will address how to decrease student stress, develop realistic schedules, bullying interventions, technology, and social media guidelines and provide families with relevant resources.

Methods/Description: The panelists focused on topics including academic advocacy, academic testing, neuropsychological and behavioral assessments, IEP’s (Individual Educational Plans), optimal classroom placement, academic modifications and accommodations, and in-school special services. Social advocacy focused on issues such as starting and changing schools, transition to new grades, returning to school after surgery, and best ways to help their child cope with social issues and bullying. Panelists included our craniofacial team’s psychologist, senior social worker, nurse practitioner, and a nonprofit funding organization’s director of family programs. In addition, the parent of a school age child, an adult patient, a school administrator, and an educational/advocacy representative from a community organization participated.

(1) Augusta University, GA

Background/Purpose: Conventional interdisciplinary care involving orthognathic surgery and extensive orthodontic treatment, occasionally fails to achieve optimal results in occlusion, and dental and facial esthetics, in patients with cleft lip and palate and other craniofacial deformities. Restorative treatment and prosthodontic rehabilitation are often required to address hard and soft tissue deficiencies presented in this type of cases. These patients can present significant maxilla-mandibular discrepancies, severe anterior bony defects, and multiple missing or malformed teeth. The macro and micro esthetics of the smile can be severely affected. The purpose of this presentation is to review the challenges involved in the interdisciplinary treatment of craniofacial patients and present treatment alternatives related to the management of such complex cases.

Methods/Description: In this clinical session, we will discuss the diagnosis, treatment planning, and ideal treatment sequence in adolescents and adults with cleft lip and palate and other craniofacial deformities. We will also demonstrate the integration and challenges of the different medical and dental specialties to obtain satisfactory results and will present a variety of cases treated by the interdisciplinary team.

(1) University of Nevada, Reno School of Medicine, NV, (2) St. Louis Children’s Hospital, MO

Background/Purpose: ASHA Special Interest Group 5, Craniofacial and Velopharyngeal Disorders, offers this session that includes case presentations/research. In 1988, an international, multidisciplinary working group of craniofacial professionals convened to devise standard methods for reporting findings from velopharyngeal imaging studies. The intent was to provide seamless communication from one center to another, and to facilitate consistency in reporting research and treatment outcomes. The recommendations were published in 1990 with a call for validation studies. As time has passed, newer professionals have entered the field without awareness of the standards, and validation studies are scarce, causing concern for our evidence base. The intent of this session is to review these standards, demonstrate their use, and discuss recent validity research in detail.

Methods/Description: The rationale for developing a standardized reporting procedure for VP imaging will be reviewed, and the basic method will be presented. Video examples of nasopharyngoscopy and multiview videofluoroscopy will illustrate the methods of reporting, as well as the advantages and limitations of using the standardized approach. The results of a multicenter study that investigated intrarater and interrater reliability in the evaluation of video clips of VP endoscopy for speech will be reviewed. In that study, 5 raters at 3 different centers rated video clips using metrics recommended by the International Working Group: the size of VP opening, location of opening in the VP port, and the pattern of VP closure. There were a total of 37 speakers in the study: 21 were normal speakers and 16 were patients being evaluated for VPD. Intrarater reliability, measured by percentages of exact agreement, was high for each rater and for all metrics. However, interrater reliability varied from slight-to-moderate depending on the metric. Clinical application of findings will be discussed. Directions for future research will be proposed.

(1) Children’s Mercy Hospital, Kansas City, MO, (2) Children’s Hospital of Philadelphia, PA

Background/Purpose: Velopharyngeal dysfunction (VPD) is one of the most common clinical features of 22q11.2 deletion syndrome (22q) and often requires surgical intervention. Treating VPD in these patients is complex and prior studies have demonstrated poorer speech outcomes postoperatively in children with 22q compared to those without. Part of the complexity in managing these patients derives from the variety of anatomic and functional abnormalities that contribute to their VPD including cleft palate, palatal hypotonia, adenoid hypoplasia, platysbasia, and differences of the cranial base and upper cervical spine. Due to the multifactorial nature of VPD, these patients present unique challenges for both the treating surgeon and the speech-language pathologist. Patients with 22q also have specific considerations both pre- and postoperatively that are crucial to the safety and success of speech surgery. It is imperative that the surgeon and speech pathologist work together to address the nuances specific to patients with 22q and VPD. The purpose for this study session is to provide strategies and approaches in the evaluation and workup, surgical management, and postoperative care for these patients. The ultimate goal is to help providers caring for children with 22q optimize their VPD outcomes.

Methods/Description: Case-based discussions, video demonstration, and audience participation will be the foundations of this study session. This multidisciplinary presentation will be led by 2 surgeons and 2 speech pathologists, each who treat a large number of patients with 22q and VPD. The speech pathologists will highlight approaches to the timing of VPD surgery in light of speech development, age, developmental status, and prior therapy. They will also discuss strategies for therapy both pre- and postoperatively as well as overall expectations. The surgeons will discuss important steps in the preoperative evaluation including the assessment for medialized carotid arteries, when/if to obtain a sleep study, and the potential need for staged preoperative adenotonsillectomy. Surgical management of VPD in patients with 22q will be examined through video demonstration and case-based scenarios. The surgeons will review factors to consider when selecting the type of surgery to perform. Specifically, the pharyngeal flap procedure and technique will be highlighted, as it is the current workhorse for management of VPD for these patients. Postoperatively, inpatient care will be discussed as well as the importance of monitoring for postoperative hypocalcemia and sleep apnea. Lastly, complex cases will be presented detailing some of the presenters’ most difficult patients; these will demonstrate the teamwork and collaboration required from both the surgeon and speech pathologist to optimize outcomes. Throughout the presentation, audience participation will be encouraged to generate a productive multidisciplinary discussion.

(1) University of Manchester, United Kingdom, (2) Department of Orthodontics, University of Dundee, Scotland, Scotland

Background/Purpose: Cleft palate is among the most common birth abnormalities. The success of primary surgery in the early months of life is crucial for successful feeding, speech, hearing, dental development, and facial growth. Over recent decades, age at palatal surgery in infancy has reduced. This has led to palatal closure in one-stage procedures being carried out around the age of 12 months, but in some cases as early as 6 months. The primary objective of the Timing of Primary Surgery for Cleft Palate (TOPS) trial is to determine whether surgery for cleft palate performed at 6 or 12 months of age is most beneficial for speech outcomes. Aim: To investigate the effect of the timing of palatal surgery for cleft palate children on dentofacial development by assessing facial soft tissue profile development and maxillary arch constriction at 5 years of age. Main Outcomes: Compare facial development between the 2 study groups at 5 years age using the soft tissue ANB angle; Compare maxillary arch constriction between the 2 study groups at 5 years age using the modified Huddart and Bodenham (MHB) automated scoring system. Secondary Outcomes: Identify factors that influence facial development (soft tissue ANB) using the full study sample, for example, gender, ethnicity, site, surgeon; identify factors that influence maxillary constriction (MHB scoring) using the full study sample, for example, gender, ethnicity, site, surgeon.

Methods/Description: This article is part of the TOPS trial. This publication will report on the influence of early versus late palatal surgery on the dentofacial development as a secondary outcome. About 520 eligible participants diagnosed with cleft palate who met the inclusion criteria were randomized and allocated to either early palatal surgery at 6 months or late surgery at 12 months. At the age of 5 years, colored profile photographs and impressions for study models were taken for all study participants. Outcome measure include (1) Facial development using soft tissue ANB angle and (2) Maxillary constriction using MHB automated scoring.

(1) Sydney Children’s Hospital Network, Westmead, Australia, (2) Pacific University, Forest Grove, OR, (3) University of Iowa, IA

Background/Purpose: Infants with cleft palate and/or related craniofacial anomalies often require supplementary supports to achieve positive feeding outcomes. Additionally, children with a syndromic diagnosis associated with their cleft or craniofacial condition may present with feeding or swallowing difficulties related to the anatomical and physiological characteristics of that specific condition. Despite the high prevalence of orofacial clefting worldwide, limited evidence exists to support best practices in feeding for these at-risk populations. Although there is evidence guiding the use of specific feeding bottles in children with cleft palate (Bessell et al., 2011; Reid, 2004), there remains a dearth of evidence to support other feeding interventions. This lack of evidence is often reported as a reason why speech-language pathologists (SLPs) feel uncomfortable or less confident in managing feeding and swallowing disorders in these infants. The aim of this presentation is to provide a practical overview of the similarities and differences between the feeding disorders commonly seen in infants presenting with cleft and craniofacial anomalies and the feeding patterns seen in typically developing infants. By drawing on the attending SLPs’ knowledge of normal anatomy, and normal feeding patterns, in conjunction with case-based examples of the common feeding difficulties seen in the clinic setting, it is envisaged that attendees will better understand the origin and nature of these feeding disorders and be better placed to provide preliminary support for these infants and their families.

Methods/Description: This presentation will provide a review of the normal anatomy and physiology of infant feeding patterns, together with an overview of the specific aspects of feeding that can be adversely affected by the presence of a cleft or craniofacial condition. A series of video recordings demonstrating both normal and disordered feeding patterns in infants with cleft and craniofacial conditions will be used to highlight the difficulties seen in these clinical populations, with videos selected to reflect various age-related stages of feeding development. Case examples will include infants with Pierre Robin sequence (PRS) and the 22q11.2 deletion syndrome. Practical strategies to support feeding in relation to each case will be explained, coupled with a discussion of when to refer for specialist intervention or videofluoroscopy swallow study (VFSS).

(1) California State University San Marcos, CA

Background/Purpose: Literature has revealed that children with cleft lip and/or palate (CLP) may be at risk for psychosocial challenges associated with their diagnosis. It has been reported that children with CLP experience psychosocial challenges such as teasing, bullying, low self-esteem, social rejection, and unwanted attention (Alansari, Bedos, & Allison, 2014). It has also been reported that typically developing peers make negative judgments toward children with CLP (Lee, Gibbon, & Spivey, 2017). There is a need for a school-based program for children with CLP to encourage positive attitudes and reduce barriers that children with unintelligible speech face in their daily functioning (Lee et al., 2017). Schools play an essential role in developing children’s awareness of communication disorders to reduce stigmatization and encourage children to understand and relate to their peers with CLP (Lee et al., 2017). It is crucial to develop prevention and intervention approaches that promote realistic self-awareness and facilitate continuous open communication between parents and children with CLP (Richman, McCoy, Conrad, & Nopoulos, 2012). The purpose of the FACE program is to increase positive and meaningful interactions between children with CLP and their peers and teachers, as well as improving their own self-perception and self-esteem. This program will promote awareness and acceptance of children with craniofacial disorders and encourage children and adults to refrain from making assumptions about this population. There is a lack of educational psychosocial programs for children with CLP, and most education surrounding the characteristics of craniofacial disorders (eg, speech) arises from speech-language pathologists or the children with CLP themselves. FACE consists of multiple parts that are designed for children with CLP and their parents, teachers, and peers. Each person that the child with CLP interacts with on a daily basis plays an important role regarding their psychosocial functioning. It is crucial to foster positive peer relationships, as peer attitudes pose strong implications for the social–emotional wellness of children with CLP. Furthermore, it is essential to educate teachers on the speech, language, and psychological factors surrounding CLP, since literature has shown that teachers may have inaccurate perceptions surrounding children with communication disorders.

Methods/Description: The FACE program contains a letter to teachers/staff, a child-centered presentation for students between first and fifth grade, an interactive coloring activity focused on the anatomy of the speech mechanism, an evidence-based in-service for first- through fifth-grade teachers, quality of life questionnaires, and a take-home program that includes 2 activities that emphasize parent/caregiver involvement to facilitate open discussions regarding a child’s feelings and attitudes toward CLP.

Conclusions: The FACE program has been piloted within 2 elementary schools and to graduate students at CSUSM.

(1) The Children’s Hospital at Westmead, Sydney, Australia, (2) Middlemore Hospital, Auckland, New Zealand, (3) John Hunter Hospital, Newcastle, NSW, Australia, (4) Pacific University, Forest Grove, OR

Background/Purpose: Speech-language pathologists (SLP) new to the perceptual evaluation of cleft palate speech require a coordinated approach to their education, training, clinical supervision and ultimately, the development and establishment of their clinical competence. The monitoring of these competencies also requires a mechanism where both the SLP and their managers can “check-off” a core competencies document and track required competency-based training. There is a need for a computerized solution to this monitoring which can be easily tailored to the individual SLP’s needs; shared with the SLP; and also allow managers to evaluate the ever-changing progress of potentially more than one SLP’s skills.

Methods/Description: A Microsoft Excel (Microsoft Corp. 2010) workbook was constructed to provide a computerized solution to the tracking and monitoring of SLPs’ clinical competencies. The workbook was based on, and populated with, a draft set of clinical competencies for the perceptual assessment of cleft palate speech (Fitzsimons, 2014). Each SLP’s worksheet within the workbook includes provision to record the SLP’s progress against both standardized and customizable competencies. Information able to be added for each competency includes the specific criteria for each competency, the target and completion dates for achieving that competency, the staff member(s) responsible for facilitating the necessary training and education of that competency, and the status of each competency. The worksheet includes a summary section where the SLP and the manager(s) can see an “at-a-glance” overview of the completed, pending and overdue competencies which forms the basis of a development plan for each SLP.

Results: This computerized workbook represents a standardized approach to the evaluation and monitoring of professional competencies for SLPs. The document utilizes a widely available computerized format (Microsoft Excel) and as such, the constituent data can be exported and shared in any digital form to facilitate cross-center benchmarking projects. The workbook can be tailored to the needs of both an individual SLP and/or a specific center/hospital and provides a framework to support a new SLP’s skill development and professional competence.

Conclusions: A computerized solution to the tracking and monitoring of speech assessment competencies for speech-language pathologists new to the evaluation of cleft palate speech has been developed. The document will be available to ACPA members and feedback on the scope and inclusions of the document will be sought.

(1) Harvard School of Dental Medicine, Boston, MA, (2) Boston Children’s Hospital, MA

Background/Purpose: Infants with Robin sequence (RS) commonly have obstructive apnea (OA) and impaired oral feeding. Mandibular distraction osteogenesis (MDO) is efficacious in resolving OA, but its effects on feeding remain unknown. The objectives of this study were to (1) evaluate weight gain in patients with RS before and after MDO and (2) identify factors that impact feeding and weight gain in this population.

Methods/Description: This is a retrospective cohort study of infants with RS treated by MDO at Boston Children’s Hospital from 1995 to 2019. To be included, growth data had to be available in patient health records. Subjects were excluded if they had a tracheostomy. Predictor variables included demographic characteristics, presence of cleft palate (CP), genetic syndrome, gastroesophageal reflux disease (GERD), aspiration, feeding tube use, and caloric supplementation. The primary outcome variable was weight gain (g/d). Growth data were collected for every available time point between birth and 12 months after distraction device removal and grouped as follows: birth weight (T0), birth to MDO (T1), MDO to device removal (T2), device removal to 6 months post-removal (T3), and 6 months post-removal to 12 months post-removal (T4). All data points were graphed and the slope of a best-fit line was used to represent the data point for each time period. Published age-appropriate growth curves were used for comparison. Wilcox signed-rank tests and Mann-Whitney U tests were performed, and P < .05 was considered statistically significant.

Results: Twenty-two subjects were included: 9 (41%) female; 14 (64%) Caucasian; 14 (64%) with a CP; and 11 (50%) syndromic. Mean age at MDO was 6.6 ± 9.1 months, with distraction devices removed 9.9 ± 3.2 weeks post-placement. Mean distraction length was 18.0 ± 2.9 mm. Mean apnea–hypopnea indices (AHI) before and immediately after MDO were 14.8 ± 6.3 and 1.8 ± 1.3, respectively (P < .05). From T0-T1, mean weight gain was 18.9 ± 9.3 g/d (9.47 ± 12.61 g/d or 33.3% less than norms, P < .05). From T1-T2, weight gain was 22.2 ± 10.8 g/d (3.48 ± 6.17 g/d or 18.6% greater than norms, P < .05). From T2-T3, weight gain was 12.3 ± 5.5 g/d (2.19 ± 4.47 g/d or 21.7% greater than norms, P < .05). From T3-T4, weight gain was 10.3 ± 4.5 g/d (1.83 ± 3.25 g/d or 21.8% greater than norms, P < .05). Subjects using feeding tubes at T1 had a higher growth percentile compared to those exclusively fed orally (P < .05). Conversely, continued use of a feeding tube by T4 was associated with a lower growth percentile (P < .05). Cleft palate and syndromic status did not significantly affect growth.

Conclusions: Infants with RS have poor weight gain prior to correction of OA but experience greater weight gain than their peers after successful MDO and for at least the following 12 months after device removal. Although use of a feeding tube for caloric supplementation was associated with increased weight gain prior to MDO, patients with continued feeding tube use after MDO had a lower growth percentile than those who were exclusively fed orally.

(1) Rush University Medical College, Chicago, IL, (2) Rush University Medical Center, Chicago, IL, (3) Rush Craniofacial Center, Rush University Medical Center, Chicago, IL

Background/Purpose: Velopharyngeal insufficiency (VPI) occurs in 5% to 36% of patients following primary cleft palate repair and is defined as the inability to fully seal the velopharyngeal sphincter. Since most sounds in the English language require complete closure of the velopharyngeal sphincter, surgical treatment is often required for full rehabilitation. Stress velopharyngeal insufficiency (SVPI) occurs when high intraoral pressure prevents the soft palate from sealing and is reported in 7% to 34% of collegiate brass and woodwind musicians. Although normal speech seldom causes intraoral pressures to exceed 5 to 6 mm Hg, playing a woodwind or brass instrument can cause pressures to surpass 130 mm Hg. This study investigated the impact of woodwind and brass instrument use on VPI in patients with a history of cleft palate.

Methods/Description: Patients aged 8 to 24 years with a history of a cleft palate reconstruction and woodwind or brass instrument use were recruited from a craniofacial center. Participating patients and families were administered a questionnaire pertaining to their musical instrument usage and speech competence. A subsequent retrospective chart review utilizing the electronic medical records system was then performed.

Results: Participants ranged in age from 10 to 17 years with an average age of approximately 14 years. On average, the patients had played for 2 years and practiced less than 2 h/d and 5 h/wk. Approximately, 75% of participants had a history of speech pathologies requiring speech therapy with the average time spent in therapy being slightly less than 4 years. None of the participants noticed voice changes such as hypernasality or nasal air escape after playing their instrument.

Conclusions: Low time commitment (<2 h/d) woodwind or brass instrument playing does not appear to put patients with a history of a repaired cleft palate at greater risk of developing VPI. Therefore, school-aged participation in these instruments should not be restricted unless the patient becomes symptomatic.

(1) Fordham University, New York, NY, (2) NYU Langone Health, Mount Kisco, NY, (3) myFace, New York, NY

Background/Purpose: Studies exploring well-being among adolescents with craniofacial conditions (CFCs) have yielded equivocal results, with levels of psychological functioning and stress varying. For example, some studies suggest those with CFCs may experience declines in their quality of life, resiliency, and self-esteem, while others have found that adolescents with CFCs do not differ in psychological adjustment or stress compared to their peers. Given these contradictory findings and the complexity of psychological functioning, more research is needed to untangle these conflicting results. Studies have proposed resiliency and social skills training for young adults with CFCs as potential interventions to improve psychological well-being and coping, but the avenues to best address these concerns remain understudied. The current study sought to describe the psychosocial needs of adolescents diagnosed with a CFC for intervention development through the use of focus groups.

Methods/Description: The current mixed-methods study combined qualitative focus groups with quantitative measures. Fourteen adolescents, aged 14 to 18, with a confirmed diagnosis of a CFC joined one of 2 semistructured focus groups in an outpatient clinic at a major medical center in New York City. Focus groups consisted of topics relevant to growing up with a facial difference and incorporated various activities to facilitate conversation and to trial potential interventions (ie, icebreakers, role-playing, mindfulness meditations). Participants were also asked to complete measures assessing a range of psychological constructs. Average scores were compared to normative data for adolescents. The 2 focus groups were coded using an inductive approach to assess pertinent themes. Additionally, the acceptability and feasibility of a proposed intervention was measured.

Results: When compared with a normative sample, adolescents with CFCs displayed lower quality of life, self-esteem, mindfulness, perceived stress, and coping, but higher resiliency, perceived social support, body image quality of life, and PTSD symptoms. Based on qualitative analyses, 6 themes emerged: stress, bullying, coping, resiliency, mindfulness, and social support. Both qualitative and quantitative analyses revealed that most participants were highly supportive of a future intervention for this population.

Conclusions: The present study identified several factors associated with psychological well-being of adolescents with facial differences and demonstrates the importance of creating interventions to target specific psychosocial needs. These findings offer guidance in developing and refining a useful program for this population and provide vital information to help those with CFCs who are experiencing psychosocial challenges.

(1) The University of Alabama at Birmingham, AL

Background/Purpose: Previous studies suggested obstructive sleep apnea (OSA) may be more prevalent in children with isolated cleft lip and/or palate (CLP), and children with a concomitant syndrome, craniofacial malformation, or genetic mutation. This study aims to describe the prevalence of OSA in symptomatic children with a CLP and to determine whether associations exist between OSA and the presence of said comorbidities.

Methods/Description: We identified 31 of 645 children with CLP referred for diagnostic polysomnogram (PSG) because of snoring or witnessed apneic event between 1997 and 2011. Cutoff values of AHI ≥1 and ≥5 were applied to establish the diagnosis of OSA. Patients were considered to have a syndrome if diagnosed with Treacher Collins, Pierre Robin, DiGeorge, Alport, Kabuki, or any chromosomal abnormality. We used χ ² or Fisher exact test when any expected cell size was <5 to find associations between OSA and syndrome status and controlled for race and gender.

Results: Of the 31 children, 15 (48.4%) were male; 16 (51.6%) were Caucasian, 11 (35.5%) were African American, and 4 (12.9%) were Hispanic or Asian. Thirteen (41.9%) had a sequence, malformation, syndrome, or chromosomal abnormality and 18 (58.1%) did not. Four patients had Pierre Robin sequence, 3 had DiGeorge, 1 had Treacher Collins, 1 had Alport, 1 had Kabuki, 1 had Stickler, and 2 had a known chromosomal abnormality. Twenty-six patients had “Two flap palatoplasty,” 3 had Furlow Z-palatoplasty, and 2 had some other or unknown procedure. Eight patients had a documented secondary repair prior to their PSG. Twenty (64.5%) patients had an AHI ≥ 1, and 6 (19.4%) patients had an AHI ≥ 5. There was no significant difference in the number of patients with an AHI ≥ 1 or AHI ≥ 5 in patients with or without a syndrome (P = .276 and P = .676, respectively). There was no significant difference in the mean AHI when in patients with or without a syndrome (P = .388). All analyses were repeated while controlling for gender and ethnicity, separately and simultaneously. All results were far from statistical significance, in fact the P > .15 for nearly every variable in the model.

Conclusions: The 19.4% prevalence of OSA in this population exceeds the estimated prevalence of up to 5.7% in the general population. Patients with CLP post repair should undergo screening for OSA regardless of syndrome, gender, and ethnicity.

(1) University of Southern California, Keck School of Medicine, Los Angeles, CA, (2) Division of General Surgery, University of Southern California, Los Angeles, CA, (3) Division of Plastic and Maxillofacial Surgery, Children’s Hospital Los Angeles, CA, (4) Division of Plastic and Reconstructive Surgery, University of Southern California, Los Angeles, CA

Background/Purpose: A total of 20% to 50% of patients with history of cleft lip/palate (CL/P) require Le Fort 1 osteotomy (LF1) for correction of midface hypoplasia (MH). The primary goal of this study was to ascertain the rate of clinically significant MH necessitating LF1 correction in patients with CL/P. Secondary outcomes evaluated the impact of cleft phenotype and number of prior CL/P-related surgeries on the eventual need for LF1 osteotomy.

Methods/Description: A retrospective cohort study was conducted of patients with CL/P born between 1975 and 2008 presenting to our institution. Operative reports, craniofacial team notes, jaw team documentation, and dental/orthodontic records of 1022 patients were screened, and patients with CL/P who received the majority of their cleft care at our institution, had adequate documentation reflecting cleft care, and who were ≥18 years old at the time of last craniofacial/dentistry follow-up were included. Patients with no presence of operative reports confirming CL/P or LF1 surgery, nonparamedian clefts, or clefts that were a consequence of a larger craniofacial syndrome were excluded. Demographic data, cleft phenotype, total number of CL/P-related surgeries, and need for LF1 osteotomy were collected and analyzed.

Results: One hundred seventy-seven patients with CL/P met the inclusion criteria; 158 had cleft lip and palate (CLP), 7 had cleft palate only (CP), 9 had cleft lip only (CL), 2 had submucous cleft palate only (SMCP), and 1 had CL with SMCP (SMCP+L). Overall, 90 (50.9%) of 177 patients received LF1 for maxillary advancement; 86 (54.4%) of 158 of patients with CLP underwent LF1, compared to 4 (57.14%) of 7 patients with CP (χ ² = 0.02, P = .888). No patients with CL, SMCP, or SMCP+L received LF1. Patients with clefts involving the secondary palate versus those with no secondary palate involvement had a significantly higher rate of LF1 (90/165, 54.5% vs 0/12, 0%, χ ² = 13.32, P < .001). Comparatively, patients with BCLP versus UCLP also had a significantly higher rate of LF1 (22/31, 71.0% vs 64/127, 50.4%, χ ² = 4.25, P = .039). Total number of all labial, palatal, or alveolar surgeries previously received did not correlate with eventual LF1 need (t = 0.46, P = .648). Additionally, surgical repair for VPI did not correlate with eventual need for LF1 (χ ² = 0.62, P = .430). However, among patients with VPI who underwent surgical repair, those who had pharyngeal flap had a significantly higher rate of LF1 (5/5, 100%) compared to those who had received sphincter pharyngoplasty (8/18, 44.4%; χ ² = 4.92, P = .027).

Conclusions: Our institutional LF1 incidence in patients with CL/P was 50.9%. When patients with CL/P are followed by a comprehensive cleft craniofacial team with orthognathic surgery readily available, more patients may be recommended for LF1 correction of MH. Additionally, patients with clefts involving the secondary palate and patients with BLCP should be counseled about the increased likelihood of requiring LF1 compared to patients without secondary palate involvement and patients with ULCP, respectively.

(1) Augusta University, GA

Background/Purpose: Patients with cleft palate with or without lip involvement may develop a fistula between the oral and nasal cavities after the palatoplasty. The fistula requires surgical repair to minimize the adverse impact on speech and feeding. An alternative treatment to surgery is the use of an obturator prosthesis to close the opening. In order to fabricate the obturator/speech-aid appliances for children, the clinician must make a maxillary impression. The most common technique is the analog method, using either irreversible hydrocolloid (alginate) or polyvinyl siloxane impression material delivered with a tray, inserted into the patient’s mouth. Using these conventional impression techniques, there are associated risks such as pushing the impression material into the nasal cavity, or its fragmentation during withdrawal of the impression. In addition, the contact of the impression material or the tray with the posterior palatal area can be uncomfortable to the patient and cause a negative experience during the procedure, especially in young children. An alternative to the conventional impression technique would be the digital intraoral scan. Intraoral scan is considered more comfortable and preferred for the patient compared to the conventional impression technique. Also, the accuracy of both methods was similar. However, there are no previous reports in the literature about the fabrication of obturators or speech aid devices using digital technology in children with cleft lip and palate. The purpose of this case report is to show the clinical effectiveness of intraoral digital scan in the fabrication of obturator/speech-aid appliances in children with cleft lip and palate.

Methods/Description: Two cases that involved the treatment of young children (4 and 5 years old) are presented. In both cases, the children required the fabrication of a prosthetic device: an obturator/speech-aid prosthesis and an obturator appliance. Maxillary impressions were made using an intraoral scanner. The casts were printed and both appliances were delivered a week later. Minimal adjustments were needed. Patients did not have any behavioral problems during the procedure, which took approximately 2 to 3 minutes. Parents and caregivers responded positively as well. The insertion of the appliances demonstrated good stability and retention in both cases.

(1) Boston Children’s Hospital, MA

Background/Purpose: Children with craniosynostosis are considered at risk for adverse cognitive, psychosocial, or educational outcomes, all of which may be associated with lower health-related quality of life. Understanding the long-term morbidity associated with this population will allow for specified treatment plans and more comprehensive care for children and their families. This pilot study sought to examine these outcomes in school-aged children with a history of syndromic and nonsyndromic craniosynostosis.

Methods/Description: The current study included 52 children diagnosed with craniosynostosis who underwent surgical repair (mean age = 8.8 years; standard deviation [SD] = 3.3 years; range: 7-17 years; 48% male, 77% syndromic). They are followed regularly in the multidisciplinary craniofacial clinical care setting. Child self-report and/or parent-proxy measures of cognitive functioning, depressive symptoms, anxiety symptoms, and peer relationships were collected using the NIH PROMIS pediatric and parent-proxy measures. These measures were completed in-person during their follow-up visit. Educational outcomes were abstracted from the medical record.

Results: Self-report and parent-proxy ratings of cognitive functioning were in the normal range (self-report cognitive functioning mean = 52.4; SD = 9.6; parent-proxy cognitive functioning mean = 48.8; SD = 8.5). Children and parents did not report significant psychological distress on the self-report or parent-proxy ratings of depression (self-report mean = 44.4; SD = 8.1; parent-proxy mean = 46.3; SD = 9.2), anxiety (self-report mean = 42.2; SD = 8.2; parent-proxy mean = 46.3; SD = 8.6), and peer relationships (self-report mean = 49.4; SD = 11.3; parent-proxy mean = 49.9; SD = 7.7). Furthermore, no significant differences were found in child self-reported or parent-proxy outcomes when comparing syndromic status, surgery type, and age at time of assessment. Regarding educational outcomes, 32% of children with syndromic craniosynostosis received educational support compared to 10% of children with nonsyndromic craniosynostosis, although the difference is not statistically significant (P = .238).

Conclusions: In general, children and parents reported intact cognitive functioning, adaptive psychological well-being, and healthy peer relationship quality, regardless of syndromic status and were comparable to unaffected peers. Although self- and parent-proxy PROMIS measures were comparable to normative data, a greater percentage of children with a history of craniosynostosis necessitated specialized instruction via a formal education plan (eg, IEP) compared to the general population. It will be important to monitor and assess subtle cognitive and academic differences, especially among children with syndromic craniosynostosis.

(1) Children’s Hospital of Pittsburgh, PA, (2) University of Pittsburgh, School of Medicine, PA

Background/Purpose: The first year of life for children with cleft lip/palate requires careful adherence to institutional protocols to ensure the best surgical and speech outcomes. The COVID-19 pandemic during the Spring of 2020 had multiple effects on the provision of health care around the globe, including the suspension of elective and nonessential surgeries at high-volume cleft centers. The objective of this study was to determine the early effect of the COVID-19 pandemic on the surgical care of cleft lip and/or palate at a high-volume cleft center.

Methods/Description: A retrospective comparative cohort study of patients with cleft lip and/or palate undergoing lip adhesion, cleft lip and nose repair, and palatoplasty before and during the pandemic was performed. Prepandemic cohort: January 2019 to December 2019; pandemic cohort: January 2020 to July 2020. Patient information was compared between the 2 groups. Age at repair and surgical delay were calculated. Mean ± standard deviation was reported. Two-tailed Student t test was performed and a P ≤ .05 was considered statistically significant.

Results: One hundred eight patients were included in the study (50 female, 58 male). There were 49 in the prepandemic cohort and 59 in the pandemic cohort. Mean age at lip adhesion was 3.1 ± 1.2 months prepandemic (n = 8) and 3.1 ± 1.2 months in the pandemic cohort (n = 10; P = .95), including one lip adhesion delayed by 52 days. Mean age at cleft lip and nose repair was 6.7 ± 2.1 months prepandemic (n = 19) and 8.8 ± 3.2 months in the pandemic cohort (n = 30; P = .02). Six pandemic cleft lip and nose repairs were delayed; the mean delay was 73 ± 43.1 days. The mean age at palatoplasty was 14.5 ± 3.4 months prepandemic (n = 31) and 15.4 ± 4.8 months in the pandemic cohort (n = 31; P = .38). Eight pandemic palatoplasties were delayed; the mean delay was 80.4 ± 47.5 days.

Conclusions: The COVID-19 pandemic caused delays at each stage of repair for cleft lip and/or palate-related procedures; however, only primary cleft lip and nose repair was significantly affected. Although the impact of isolated delays in care may be negligible, the downstream effect of such delays on cleft outcomes remains uncertain. As the pandemic continues, further delays may be expected while providers adapt to this rapidly evolving situation, especially in those practicing in resource-constrained health systems. This study emphasizes the importance of remaining vigilant regarding the care of this vulnerable population during this challenging time.

(1) Chang Gung Memorial Hospital, Taoyuan, (2) Analytical Imaging and Modeling Center, Children’s Health, Dallas, TX, (3) Sobrapar Hospital, Campinas, Sao Paulo, Brazil, (4) UT Southwestern School of Medicine, Dallas, TX

Background/Purpose: Defining 3-dimensional (3D) normal craniofacial morphology in healthy children could provide craniofacial surgeons a reference point to assess disease, plan surgical reconstruction, and evaluate treatment outcome. The purposes of this study were to report normal craniofacial form and quantify craniofacial asymmetry of healthy children in Taiwan by implementing the 3D stereophotogrammetry technique.

Methods/Description: Healthy Taiwanese elementary school children (n = 652) aged 6 to 12 years with no known craniofacial anomaly were recruited. After the 3dMD scanning procedure, 32 landmarks were manually placed on the 3D cranial images. Thin-plate spline algorithm based on landmarks and closest point matching was applied to deform a symmetric 3D template into the scale of each scanned images. Skull asymmetry and facial asymmetry were calculated using 3dMD vultus and MATLAB. Average head shape models were also presented.

Results: Overall, the mean head transverse width, height, anteroposterior length, and circumferences were 163.02, 220.79, 179.07, and 526.55 mm, respectively. On average, the skull asymmetry and facial asymmetry were 2.47 ± 1.26 mm and 0.96 ± 0.53 mm, respectively, with no significant (all P > .05) differences found when comparing males and females. In the average head shape model, certain craniofacial areas on the right side were found to be more protruded than those on the left side.

Conclusions: This study shows that the baseline craniofacial form of the Taiwanese elementary school children is asymmetric with a tendency of more protrusion of the head on the right side.

(1) University of Kansas School of Medicine, Kansas City, KS, (2) The University of Kansas Medical Center, Kansas City, KS

Background/Purpose: Although pathology in the maxillary and mandibular bones is rare in young patients, the differential diagnosis is broad. A craniofacial surgeon must be prepared when these lesions are encountered. The World Health Organization (WHO) updated its classification of maxillofacial bone pathology in 2017. Using these updated guidelines, a systematic review of common maxillofacial bone lesions in the pediatric population was performed.

Methods/Description: A PubMed search was conducted capturing English language articles from inception to July 2020. Thirty-one articles were identified that described the frequency of maxillofacial bone pathology. Data were extracted and organized using the WHO 2017 classification of odontogenic and maxillofacial bone tumors. Prevalence data were analyzed among diagnostic categories and geographical regions. SAS version 9.4 was used to complete statistical analyses.

Results: The articles included 5709 maxillofacial bone tumors for patients ranging in age from birth to 19 years. Pooled analysis revealed the most common lesions to be cysts (57.7%), followed by benign odontogenic tumors (21.7%), benign nonodontogenic tumors (19.9%), and malignant odontogenic or bone tumors (0.7%). The most common odontogenic cysts included radicular cyst (42.7%) and dentigerous cyst (39.0%) followed by odontogenic keratocyst (15.0%). Among odontogenic bone tumors, odontoma (49.3%) was most common followed by ameloblastoma (29.1%). The most common nonodontogenic bone tumor was fibrous dysplasia (42.4%), and the most common malignant bone tumor was osteosarcoma (75.0%). Significant variations were found by geographic region, with dentigerous cyst more common than radicular cyst, and ameloblastoma more common than odontoma in African and Asian countries (P < .0001).

Conclusions: This systematic review uses the WHO 2017 guidelines to classify common odontogenic and nonodontogenic maxillofacial bone lesions reported by institutions around the world. Pathogenesis, presentation, and available treatment options for the most common odontogenic and nonodontogenic maxillofacial bone lesions are reviewed.

(1) Augusta University, GA, (2) Augusta University, Section of Plastic Surgery, GA

Background/Purpose: Restoring the smile in patients with maxillary hypoplasia, multiple missing teeth, and abnormal soft tissue morphology is a significant challenge for the clinician and the patient. Interdisciplinary care is critical in these cases. The treatment is even more complex, when maxillary hypoplasia also involves a missing premaxilla. The aim of this presentation is to illustrate an innovative fixed prosthodontic approach to restore the esthetic zone, after orthodontic treatment and orthognathic surgery, in a patient with complete bilateral cleft lip and palate (CBCLP), missing premaxilla and severe midline bone defect.

Methods/Description: An 11-year-old male patient with CBCLP and missing premaxilla of uncertain etiology, using a poorly adapted interim maxillary removable partial denture presented for treatment. The patient presented a skeletal class III with increased vertical pattern and severe maxillary constriction. Other findings involved a dental class III with multiple missing teeth in the anterior region, transposition of the upper left canine erupting on the midline and a peg lateral incisor on the left side erupting on the canine area. Retroclined mandibular incisors, anatomical open bite, and short upper lip were also present. Patient was classified as Goslon Yardstick #5. The orthodontic treatment consisted in maxillary expansion and extraction of the transposed maxillary peg lateral incisor, to distalize the upper left maxillary canine and consolidate the left posterior segment. Fixed appliance therapy in combination with an interim maxillary partial denture for esthetics and psychosocial reasons was used during the entire treatment. Orthodontic treatment allowed preparation of the patient for an asymmetric 2-piece maxillary advancement, and expansion of the posterior segments as well as, preparation of the lower arch for the replacement of a central incisor. After the orthodontic debonding, patient underwent crown-lengthening surgery to provide adequate connector height for a zirconia fixed dental prosthesis with layered porcelain, which included right and left first premolars and canines as the abutment teeth, and lateral and central incisors as pontics. Pink porcelain was used to compensate for the severe bony defect and restore the facial and dental esthetics. Maxillary second premolars received lithium disilicate crowns to allow a more harmonious smile line, and adequate occlusion. The last step was the insertion of a lithium disilicate resin-bonded fixed partial denture that restored a missing mandibular central incisor.

(1) Keck School of Medcine of USC, Los Angeles, CA, (2) Children’s Hospital Los Angeles, CA, (3) Children’s Hospital Los Angeles, Keck School of Medicine of USC, CA

Background/Purpose: Patients with 22q11.2 deletion syndrome (DS) are at risk for the development of hypocalcemia during times of stress due to hypoparathyroidism from aplasia or hypoplasia of the parathyroid gland. Several studies have shown that patients with 22q11.2 DS experience higher rates of postoperative hypocalcemia and complications following cardiac surgery compared to patients who have no deletion. However, postoperative hypocalcemia and calcium-related complications after primary or secondary palatal repair in children with 22q11.2 DS has not been well described. In 2011, Practical Guidelines for Managing Patients with 22q11.2 Deletion Syndrome recommended the assessment of ionized calcium pre and postoperatively as well periodically throughout childhood in patients with 22q11.2 DS. This study investigates if the stressful event of cleft palate surgery in patients with 22q11.2 DS is associated with hypocalcemia and/or complications related to hypocalcemia compared to age-matched controls.

Methods/Description: A retrospective chart review was performed on patients with 22q11.2 DS and age-matched controls with cleft lip and palate and cleft palate only receiving a repair of their secondary palate September 2011 to present. ICD-10 and surgical CPT codes were used to determine subjects. The following data from 26 patients with 22q11.2 DS and 191 age-matched controls was obtained: age at time of surgery, assigned gender at birth, type of cleft diagnosis, pre-, peri-, and postoperative serum calcium levels, length of surgery, length of hospital stay, ICU admission rates, postoperative complications, additional diagnoses, mortality, and rates of ED visits or rehospitalizations within 30 days of surgery. Data were analyzed using SAS Version 9.4.

Results: Preliminary analysis of the data shows that the majority of patients with and without 22q11.2 DS undergoing secondary palate repair did not have pre-, peri-, or postoperative serum calcium levels checked, 85% and 94%, respectively. When calcium levels were checked, the rate of postoperative hypocalcemia was not higher in the group with 22q11.Ds versus controls, 67% and 50%, respectively. Five patients with 22q11.2 DS experienced postoperative complications versus 6 patients in the control group. The most common postoperative complication was poor oral intake. The types of complications found in both groups did not seem to be caused by hypocalcemia.

Conclusions: This study demonstrated that 2011 recommendations to check pre-, peri-, or postoperative serum calcium were rarely completed. Despite this, it appears that patients with 22q11.2 DS undergoing repair of the secondary palate did not experience higher rates of hypocalcemia or postoperative complications compared to age-matched controls. Checking of serum calcium in patients with 22q11.2 DS may not be routinely necessary for repair of the secondary palate.

(1) University of Pittsburgh, PA, (2) University of the Philippines Manila, Philippines, (3) Department of Oral Pathology, Radiology and Medicine, College of Dentistry, University of Iowa, IA, (4) University of Lagos, Nigeria, (5) Fundación Clínica Noel, Medellin, Colombia, (6) Noel Clinic, Medellin, Colombia, (7) Department of Orthodontics, College of Dentistry, University of Iowa, IA, (8) Dental and Craniofacial Genomics Core, School of Dental Medicine, University of Puerto Rico, San Juan, Puerto Rico, (9) Lancaster Cleft Palate Clinic, PA

Background/Purpose: The unaffected relatives of individuals with nonsyndromic orofacial clefts have been shown to exhibit subtle craniofacial differences compared with the general population. In the current study, we investigate the differences in palate shape between biological parents of individuals affected with orofacial clefting in comparison to unrelated normal controls and explore whether sex and ancestry have an effect on the observed differences.

Methods/Description: We conducted a landmark-based geometric morphometric analysis to compare palate shape in the clinically unaffected parents of children with nonsyndromic clefting of the lip with or without cleft palate (NSCL/P; 49 fathers; 92 mothers) and demographically matched adult controls (356 males; 438 females). Three ancestral groups were represented, namely Asian, European, and African. We conducted pairwise group comparison using canonical variates analysis, and then confirmed and characterized findings of shape differences using Euclidean distance matrix analysis.

Results: In terms of overall size (ie, centroid size), fathers had smaller palates than male controls. In contrast, mothers had larger palates than female controls. However, the effect of allometry (defined as size-related differences shape) was minimal, contributing between 2% and 3% of the variation in shape. Significant differences in palate shape were detected in unaffected mothers (but not fathers) compared to demographically matched controls. The differences in shape were ancestry specific; mothers of Asian and African ancestry showed wider and shorter palates with higher posterior palatal vaults, while mothers of European ancestry showed narrower palates with higher anterior palatal vaults were detected.

Conclusions: Our findings suggest that altered palate shape is a subclinical phenotypic feature, which may be indicative of elevated orofacial cleft risk. The risk phenotype varied by sex and ancestry, suggesting possible etiologic heterogeneity among demographic groups. Understanding the genetic basis of these informative palate shape traits may reveal new genes and pathways relevant to nonsyndromic orofacial clefting.

(1) Department of Biomedical and Specialty Surgical Sciences, University of Ferrara, Italy, (2) School of Epidemiology and Public Health, University of Ottawa, Ontario, Canada, (3) Department of Orthodontics, University of Dundee, Scotland, United Kingdom, (4) Department of Oral Pathology, Radiology and Medicine, College of Dentistry, University of Iowa, IA, (5) Department of Obstetrics and Gynaecology, Erasmus University Medical Center, Rotterdam, the Netherlands, (6) Department of Cranio-Maxillo-Facial Surgery, Smile House Milan, University of Milan, Italy

Background/Purpose: Orofacial clefts result from early life events when the embryo is extremely susceptible to in-utero maternal environmental exposures. Among such exposures, tobacco smoke is reported to be the most consistent environmental risk factor for orofacial clefts. Tobacco smoke can affect developmental programming in the embryo. Sustained changes may be detectable in upper lip tissues in nsCL±P cases. Methylenetetrahydrofolate reductase (MTHFR) is an enzyme in the one-carbon cycle that can provide methyl groups for DNA methylation and appears to be involved in craniofacial development. The effect of MTHFR on the occurrence of nsCL±P so far has involved investigation of MTHFR variant genotypes. MTHFR promoter methylation (mMTHFR) has similar effects on MTHFR activity to those conferred by MTHFR variants, but has not been reported in relation to nsCL±P. Moreover, mMTHFR is reported to regulate methylation repair activity, and hence might contribute to individual variability in response to environmental perturbations such as smoking. Therefore, we assessed the association between level of mMTHFR and level of LINE-1 methylation in the lip tissues of cases with nsCL±P, its relationship with MTHFR c677C>T variant, and whether mMTHFR could mitigate the effect of smoking exposure.

Methods/Description: MTHFR methylation was assessed in DNA extracted from medial and lateral tissues taken from 26 infants with nsCL±P. The DNA extracted was bisulfite converted and mMTHFR measured on a pyrosequencer. LINE-1 methylation and MTHFR c.677C>T genotype data were assessed by established methods. We performed Student t test for within and between pair comparisons, and applied Pearson correlation to determine the relationship between levels of mMTHFR and LINE-1. On calculating statistical power, we observed a 1-β-error of nearly 0.60 on considering the total of 26 cases included in this study.

Results: We found a nonsignificant difference in mMTHFR (P = .733) and LINE-1 (P = .148) between medial and lateral tissues. mMTHFR was not influenced by MTHFR c.677C>T genotype, but there was weak evidence that the difference was larger among infants exposed to maternal smoking compared to nonexposed infants (P = .554). LINE-1 methylation differences were significant (P = .025) in infants born to nonsmoking mothers, but this was not apparent (P = .872) in infants born to mothers who smoked. Our Pearson correlation analysis showed a small strength of association between mMTHFR and LINE-1 methylation (r = −0.381; P = .319).

Conclusions: Our observation of differences in patterns of mMTHFR levels in lip tissue, although statistically nonsignificant, suggests the interplay of gene and environment in establishment of methylation in tissues at both sides of cleft lip. Further investigation in a larger cohort, integrated with metabolic assessment is warranted.

(1) University of Southern California, Los Angeles, CA, (2) Children’s Hospital Los Angeles, CA, (3) University of Southern California, Keck School of Medicine, Los Angeles, CA

Background/Purpose: Craniosynostosis (CS) is the premature fusion of one or more cranial sutures and occurs in 1 of every 2100 to 2500 births. Early diagnosis and surgical intervention for CS is indicated partially due to the risk of optic neuropathy secondary to elevated intracranial pressure (EICP). Papilledema (PE), optic nerve pallor (ONP), and optic nerve atrophy (ONA) are concerning for EICP. Although the syndromic CS population has been well described, less is known about the vision needs of patients with nonsyndromic CS. The purpose of this study is to track preoperative and postoperative ophthalmic EICP markers and describe ophthalmic findings across CS patients.

Methods/Description: As part of a larger IRB-approved study, chart review from 2009 to 2020 identified findings from the preoperative and first 2 postoperative ophthalmology visits (OV). Case histories for patients with significant findings were described.

Results: Patients (N = 332) were mostly male (64%), Latino (52%) or European American (22%), and had public insurance (58%). Confirmed syndromes were present in 5% of patients and the nonsyndromic patients were born with sagittal (41%), unicoronal (23%), metopic (20%), bicoronal (8%), 13 other multiple suture combinations (6%), or lambdoidal (2%) CS. Surgery was at an average age of 7.9 ± 4.1 months (range: 2-42). Preoperative OV took place for 36% of patients at a mean age of 6.8 months. Nonsyndromic patients generally had normal preoperative examinations (64%), followed most often by hyperopia (18%), exotropia (6%), and anisometropia (7%). Postoperative OV1 were at an average age of 19.8 months for 45% of the sample. The nonsyndromic patients had normal examinations (63%), hyperopia (16%), exotropia (8%), anisometropia (5%), or other findings. A patient with nonsyndromic sagittal CS had persistent ONA identified at OV1. A third of patients had a postoperative OV2 at age 26.7 months. Nonsyndromic CS findings at OV2 included normal examinations (45%), hyperopia (22%), exotropia (15%), and anisometropia (6%). Among patients with syndromic CS, a patient with sagittal and metopic CS in the setting of tetrasomy 15q had persistent PE. A patient with Apert syndrome had preoperative ONP that resolved by postoperative OV1. A patient with Saethre-Chotzen syndrome had PE postoperatively that resolved by OV2. A patient with craniofrontonasal dysplasia presented at OV1 with ONA. ONA was also identified for a patient with sagittal and bicoronal synostosis at OV2.

Conclusions: Consistent with the literature, ophthalmic markers of EICP tended to present for syndromic CS. Ongoing ophthalmology care is an important aspect of CS management not only for detection of EICP but also for identification of and intervention for multiple vision issues found in patients with isolated CS. Providers should counsel parents about the need for close follow-up for children’s visual health and ensure access to ophthalmology as part of their CS treatment plan, as recommended by international CS work groups.

(1) Smile Train, London, United Kingdom, (2) Smile Train, New York, NY, (3) London School of Hygiene and Tropical Medicine, United Kingdom

Background/Purpose: Babies born with a cleft lip and/or cleft palate need immediate attention and care, especially because they need to learn how to feed successfully in order to grow and thrive. In resource-poor countries, numerous challenges including poverty, malnutrition, high disease burden, low education, social neglect, lack of specialized breastfeeding support to mothers, and limited access to specialist medical services, place children at high risk of malnutrition, and death. To date, there is no estimate of the burden of malnutrition in children with unrepaired orofacial clefts in low- and middle-income countries (LMICs). The purpose of this study was to gain a global insight of the scale of malnutrition in children with cleft in LMICs to inform current and future nutrition and cleft care programs in these settings.

Methods/Description: Through partnerships with hospitals across >85 LMICs, Smile Train has been supporting free cleft surgery and comprehensive care provision as well as health professional training for over 20 years. We analyzed 638 988 anonymized records from Smile Train’s global clinical database. The records correspond to primary surgeries of children ≤5 years operated between 2008 and 2018 in partner hospitals. Underweight prevalence was derived from the generation of weight-for-age z-scores using WHO Stata macro. Prevalence data at country level were compared to the most recent national data on under-5 underweight prevalence extracted from the online Demographic Health Survey (DHS) program.

Results: We included 602 568 primary surgeries in our analysis. The overall prevalence of underweight at the time of surgery was 28.6%—a figure well above the global underweight estimate of 13.5% in children under-5 without cleft. The prevalence of underweight at surgery varied with the nature of cleft—cleft lip only, 25.5%; cleft palate only, 18.4%; and cleft lip and palate, 32.8%. Other determinants of underweight prevalence included age at surgery, gender, ethnicity, and geography. The prevalence of underweight in children with any cleft type was either high (20%-29%) or very high (≥30%) in two-thirds of all countries (N = 60) with at least 200 treated cases reported by partners in Smile Train’s database. In addition, for all (N = 42) but 4 countries with available DHS data, the prevalence of underweight was higher in children with cleft than in their peers without cleft (P < .05). The prevalence of underweight in children with cleft was found positively correlated with DHS country estimates for the prevalence of underweight among under-5 with no cleft (rs = 0.63, P < .0001).

Conclusions: Our findings shed light on the burden of malnutrition in children with cleft operated in Smile Train partner hospitals across the developing world. To prevent or manage malnutrition and promote timely and safe surgery, Smile Train is working toward the provision of feeding counseling and nutrition support to all children with cleft in the care of its partners.

(1) Department of Speech, Language and Hearing Disorders, Bauru School of Dentistry, University of Sao Paulo, Bauru, Sao Paulo, Brazil, (2) Hospital for Rehabilitation of Craniofacial Anomalies at University of Sao Paulo, Sao Paulo, Brazil, (3) University of Sao Paulo, Bauru, Sao Paulo, Brazil

Background/Purpose: To investigate the relationship between maxillary arch dimensions preprimary palatal repair and speech outcome in children born with unilateral cleft lip and palate.

Methods/Description: A retrospective review of patients who underwent palatoplasty was performed. Primary palatoplasty was performed by 4 surgeons in a single center, using Furlow or von Langenbeck procedures. The sample consisted of 139 patients (both genders) with isolated unilateral cleft lip and palate (both genders) who fulfilled the following selection criteria: no palatal fistula, have speech recordings between 3 and 6 years of age, and 3D-digitalized gypsum casts models performed pre-primary palatoplasty. Three experienced speech-language pathologists rated the occurrence of hypernasality (presence or absence) of the edited recorded speech samples. The data were analyzed considering the surgical variables (surgeon, surgical technique, surgical time, and gender) of each patient. Linear measurements of the maxillary arch (intercanine distance—DIC, intertuberosity distance—DIT, anteroposterior distance of the hard palate—DAP, and posterior width of the cleft—LPF) and area measurements (from cleft—AF and blades greater—ALMa and minor—ALMe) were performed on the 3D-digitalized casts, using software 3 Shape Appliance Design 2013-1 and Mimics Research 17.0. The χ ² test was used to calculate the relationship between the results of the occurrence of hypernasality and the surgical variables. The t test was used for the relationship between the occurrence of hypernasality and the maxillary measurements.

Results: With regard to the hypernasality evaluation of the recorded samples, 86 (62%) patients presented normal resonance and 53 (38%) presented hypernasality. The means of the maxillary measurements were 27.22 mm (SD = 2.72 mm) for DIC, 35.67 mm (SD = 2.65 mm) for DIT, 28.35 mm (SD = 2.7 mm) for DAP, 10.16 mm (SD = 2.76 mm) for LPF, 427.59 mm ² (SD = 203.4 mm ² ) for AF, 1335 mm ² (SD = 154.34 mm ² ) for ALMa, and 979.28 mm ² (SD = 147.56 mm ² ) for ALMe. Only the relation between the occurrence of hypernasality and the measurement of the AF was statistically significant (mean of 461.30 mm ² for patients presenting with hypernasality and 372.88 mm ² for patients without hypernasality, P = .01).

Conclusions: The area of the cleft palate (AF) was the only measure that could predict speech outcome in children born with unilateral cleft lip and palate.

(1) Boston Children’s Hospital, Boston, MA, (2) Brigham and Women’s Hospital, Boston, MA

Background/Purpose: Hemifacial Atrophy (HFA), also known as Parry-Romberg Syndrome (PRS), involves progressive hemifacial atrophy, often beginning in childhood that may include subcutaneous tissues such as skin, muscle, cartilage, and in severe cases may involve bone and brain. The cause of this syndrome has yet to be identified. Clinical follow-up of children with HFA is challenging, and most centers currently use clinical photography for this purpose. Three-dimensional photogrammetry is potentially an ideal method to monitor discrete small changes between time points, but has not been studied to date to our knowledge. Comparing 2 time points can be challenging, however, given interval facial growth. The purpose of this pilot study is to use 3D imaging to track the progression of HFA during growth and development.

Methods/Description: This pilot study reports the preliminary findings of a prospective study on pediatric patients with HFA. Subjects in all phases of active and stable disease were included. Three-dimensional photographs (VECTRA 3D, Canfield Scientific, Parsippany NJ) were taken at 2- to 3-month intervals during routine clinical care. For each separate time point, the images were analyzed for asymmetry by mirror imaging the halves of the face and creating an overlay heat map. Serial images between time points were analyzed by measuring the difference in volume and again using heat maps to illustrate areas of progressive disease.

Results: A female and a male patient were identified who had 1 year of follow-up in the study. In 1 patient, 3D photogrammetry suggested possible subtle signs of progressive brow atrophy, although the disease appeared clinically stable. Photogrammetric analysis of the other patient correlated with clinical improvement documented during the course of the study.

Conclusions: 3D photogrammetry may be a helpful adjunct to the clinical exam in HFA in determining whether there has been subtle disease progression over time. Using these 3D photogrammetry techniques, this pilot study reports early findings of HFA facial volumetric analysis in actively growing patients across a 1-year follow-up period. A similar methodology could potentially aid in diagnosis and treatment planning for other causes of acquired or congenital facial asymmetry.

(1) Children’s National Hospital, George Washington University School of Medicine, Washington, DC, (2) Children’s National Health System, Washington, DC, (3) Children’s National Medical Center, Washington, DC, (4) The Georgetown University School of Medicine, Washington, DC

Background/Purpose: Upper airway obstruction in association with micrognathia and glossoptosis are the hallmark signs of Robin sequence (RS). Mandibular distraction osteogenesis (MDO) has been increasingly used to address airway obstruction in infants with RS by enabling gradual mandibular lengthening and soft tissue stretching. While many complications associated with this procedure have been well documented, there is a paucity of literature describing the timing of facial nerve dysfunction (FND) associated with MDO.

Methods/Description: A 16 question survey was designed through REDCap and distributed via email to members of the American Cleft Palate-Craniofacial Association and International Society of Craniofacial Surgery. Questions were related to surgeon experience in treating RS, perioperative protocols, and experience with FND as a complication of MDO. Responses were collected for 280 days.

Results: A total of 84 responses were collected. Most respondents were surgeons practicing for greater than 15 years (57.1%, n = 48) in a university hospital setting (72.6%, n = 61) in North America (66.3, n = 55), and had completed a craniofacial fellowship (77.4%, n = 65). Of the 83 participants responding to questions on frequency and protocols, the majority performed between 1 and 5 neonatal MDO procedures a year (62.7%, n = 52), and most used internal, buried devices (67.9%, n = 57). Perioperative protocols varied, with participants most commonly noting a latency phase of 1 to 3 days (48.2%, n = 40), with an average total distraction length of 1.0 mm per day (45.8% n = 38), distracting twice daily (67.5%, n = 56). For most participants, end point for distraction was a Class III occlusion (56.0%, n = 47). A majority of respondents reported FND as a complication of MDO in patients with RS (65.5%, n = 55); 60.7% (n = 51) experienced transient palsy and 21.4%, (n = 18) had permanent palsy. Of the 55 respondents whose patients experienced FND following MDO, 27.4% (n = 23) noted this complication immediately following initial device placement/osteotomies, 32.1% (n = 27) during distraction, 11.9% (n = 10) during consolidation, and 28.6% (n = 24) experienced FND following removal of distraction devices. Among the 59 respondents reporting on transient palsy resolution, time to return of normal function was typically 1 to 3 months (54.2%, n = 32).

Conclusions: FND following MDO in patients with RS is a complication experienced by a majority of craniofacial surgeons who responded to our survey, but remains to be studied in detail. While most respondents noted temporary FND, over 20% reported permanent dysfunction. Further research should aim to establish risk factors associated with the development of FND, as well as identify surgical and perioperative strategies for avoiding this complication.

(1) Boston Children’s Hospital, Boston, MA, (2) Stanford University School of Medicine, Stanford, CA

Background/Purpose: Pain management strategies following palatoplasty vary substantially. Despite efforts to reduce narcotic utilization, specific analgesic regimens are typically guided by surgeon preference. The aim of this study was to define analgesic variables that affect postoperative narcotic use and time to resumption of oral intake.

Methods/Description: Retrospective review of nonsyndromic patients undergoing primary palate repair. Analgesic variables included local anesthetic, pterygopalatine ganglion nerve block, palatal pack, and postoperative use of ketorolac, dexamethasone, and nursing controlled analgesia (NCA) opioid dosing. Proxy measures for pain included time to resumption of oral intake and morphine equivalence (mg/kg/h) administered. Analgesic variables were analyzed with the Mann-Whitney U test and the impact of age and weight were assessed with regression analysis.

Results: Veau phenotypes for the 111 patients included were I (28%), II (19%), III (33%), IV (16%), and submucous (4%). Age, weight, local anesthetic, and postoperative use of ketorolac, dexamethasone, and palatal pack had no effect on either proxy measure (P > .05, all). Postoperative narcotic usage was significantly lower in patients who had an intraoperative suprazygomatic peripheral nerve block and significantly higher when NCA was utilized P < .05, both). Neither variable had a significant impact on time to resumption of oral intake (P > .05, both).

Conclusions: Several perioperative analgesic strategies lead to comparable postoperative consumption of narcotic and time to resume oral intake. The authors advise careful consideration of NCA due to the potential for increased narcotic utilization that we found in our institution. Based on our promising findings, further studies are warranted to assess risks, benefits, and costs of performing peripheral nerve blocks at the time of palatoplasty.

(1) University of Washington, Seattle, WA, (2) Seattle Children’s Hospital, Seattle, WA, (3) Shriners Hospitals for Children Chicago, IL, (4) Shriners Hospitals for Children Chicago and University of Illinois at Chicago, IL, (5) Fred Hutchinson Cancer Research Center, Seattle, WA, (6) Shriners Hospitals for Children, University of Illinois, Chicago, IL, (7) University of Illinois-Chicago, IL

Background/Purpose: Infants and young children with cleft lip with and without cleft palate (CL±P) are candidates for multiple medical interventions. There is a shortage of well-conducted large randomized controlled trials of interventions however (Heike et al., 2020). Since children ≤6 years of age may not reliably report their own symptoms, US FDA recommends parent report of signs substitute for child report of symptoms, known as observer-reported outcomes (ObsROs) (United States Food and Drug Administration, 2012). To our knowledge, no ObsRO specific to clefts exists to be used in clinical trials or clinical practice.

Methods/Description: We tested 8 iCOO domains relevant for primary lip repair specifically: Behavior & Emotion, Breathing, Comfort, Communication, Feeding, Skin, Sleep, Vocalization. We conducted psychometric testing, including scale structure, reliability, validity, and responsiveness. We collected data from family caregivers in a multicenter study that included observation items and global impressions of function. We evaluated 65 items across 8 domains considered important to patients and providers that were previously developed and cognitively tested (Heike et al., 2020; Rosenberg, et al; 2018). All study procedures were approved by the participating site Institutional Reviews Boards.

Results: Five of 8 iCOO domains tested had acceptable internal consistencies, ranging from Cronbach alpha .67 to .86 (Behavior & Emotion, Comfort, Communication, Skin, Vocalization). Four of 5 iCOO scales tested had acceptable test–retest reliabilities with intraclass correlation coefficients (ICCs) ranging from 0.76 to 0.84 (Behavior & Emotion, Comfort, Communication, Vocalization). Of the individual ObsRO items in the other domains, all but 4 had acceptable ICCs (range 0.66-0.96). All global impression items had acceptable ICCs (range 0.76-0.91). iCOO scale standardized effect sizes assessed with Cohen’s d at 2-days following primary lip repair ranged from small to large: −0.79 (Behavior & Emotion), −1.23 (Comfort), 0.13 (Communication), −0.30 (Skin), and 0.25 (Vocalization). At 2-month follow-up, effect sizes ranged from small to large, respectively: 0.05, 0.12, 0.78, 0.26, and 1.19.

Conclusions: The iCOO meets accepted measurement standards for test–retest reliability, construct validity, and responsiveness to changes associated with primary lip repair. Because of high test–retest reliability portions of it may also be useful in clinical care with individual patients. It takes approximately 10 minutes to complete.

References

1. Heike CL, Albert M, Aspinall CL, et al. Development of an outcome measure of observable signs of health and well-being in infants with orofacial clefts [E-pub ahead of print]. Cleft Palate Craniofac J. 2020

2. United States Food and Drug Administration. Guidance for Industry: Acute Bacterial Otitis Media—Developing Drugs for Treatment. 2012.

3. Rosenberg J, Albert M, Aspinall C, et al. Parent observations of the health status of infants with clefts of the lip: results from qualitative interviews. Cleft Palate Craniofac J. 2018: 56; 646-657.

(1) Nationwide Children’s Hospital, Columbus, OH

Background/Purpose: While it is well known that children with cleft palate (with or without cleft lip) are at higher risk for speech and language difficulties than the general population (Riski & Delong, 1984; Scherer & D’Antonio, 1995), little is known about the speech-language profiles of children with cleft lip with or without cleft alveolus (CL/A) without a cleft palate. The risk of communication disorders in the general population of children without clefts is approximately 5% for articulation disorders and 3% for language disorders (Black et al., 2015). Vallino et al. (2008) reported 13% of children with nonsyndromic CL/A demonstrated articulation disorders and 18% demonstrated language delays. Deelder et al. (2011) reported similar findings; thus, these reports suggest children with nonsyndromic CL/A have an elevated risk of speech-language disorders. The purpose of this study was to determine the relative rates of occurrence of speech-language disorders in a consecutively evaluated group of children with CL/A, as well as examine the types of speech and language difficulties and impact of co-occurring syndromes and other diagnoses (eg, otitis media, submucous cleft palate), on risk for communication disorders.

Methods/Description: A retrospective chart review was completed with children from a single pediatric academic medical center, ages 12 to 72 months, with a cleft lip or cleft lip and alveolus, without an overt cleft palate. Participants included 86 children (58 male, 28 female). Forty children had cleft lip and alveolus; 46 had isolated cleft lip only. Five children also had a submucous cleft palate. Eight children had multiple congenital anomalies including 1 with CHARGE association and 1 with craniofacial microsomia.

Results: Fifty-three percent of children with CL/A had received at least one speech-language evaluation and 51% had received at least one team visit (including at least a surgeon, SLP, and orthodontist). The average age at first speech evaluation was 26.7 months. Overall, 37% (n = 17) of children assessed presented with some type of communication disorder. Of this group, 26% (n = 12) were diagnosed with a language disorder and 21% (n = 9) were diagnosed with an articulation disorder. The presence of ear tubes or submucous cleft palate was not significantly associated with the diagnosis of an articulation disorder (P = .22 and P = .19 respectively).

Conclusions: The results of this study are congruent with past reports suggesting children with CL/A have a higher risk of communication disorders relative to the general pediatric population. The risk rates in this cohort of children were higher than expected based on past reports which may be related to methodologic differences across studies; however, all continue to point to an increased risk of speech-language disorders in this patient population. Team care for children with CL/A should include routine and ongoing speech-language assessment starting at an early age to ensure management needs are identified and addressed in a timely manner.

(1) Children’s Healthcare of Atlanta, GA, (2) University of Rochester, Rochester, NY

Background/Purpose: Hydrocephalus is an increased volume of cerebrospinal fluid in the central nervous system due to disturbance in the flow, absorption, or formation of CSF. It affects 3 in 1000 live births in the United States. The mainstay of treatment is ventricular shunting. While this has increased survival, shunts can fail or lead to conditions such as slit ventricle syndrome or post-shunt craniosynostosis. If shunting fails, treatment options are limited. Patients may experience headaches, papilledema, and developmental delays. The same principles of cranial vault expansion that are used in treatment of craniosynostosis have been implemented in the treatment of patients with refractory hydrocephalus. However, this technique is invasive and can be limited by the amount of soft tissue coverage available. Cranial vault distraction osteogenesis allows for similar expansion but with less dissection of dura and more controlled expansion of soft tissues. This has not yet been described for treatment of refractory hydrocephalus. We predict that cranial vault distraction is a safe and effective treatment.

Methods/Description: This is a case series of consecutive patients at our institution who underwent posterior cranial vault distraction for hydrocephalus refractory to shunting. Objective outcome measures included lumbar puncture opening pressure, papilledema, head circumference, and emergency department visits or hospital admissions for shunt-related issues. Subjective outcome measures included patient- and parent-reported headaches and improvement in developmental milestones.

Results: Three patients were identified. All patients were 2 or 3 years old at the time of distraction. All patients presented with headaches. Two had elevated LP opening pressures and the third had not been tested. None had papilledema. They all had 3 to 5 ED visits/hospital admissions for headaches or shunt-related problems. One patient had severe developmental delays and was nonverbal. All 3 patients underwent successful craniotomy with placement of 2 or 3 uniplanar distractors. Distraction was completed and devices were subsequently removed 3-months later. All have had resolution of their headaches. The nonverbal patient has had drastic improvements in communication. One patient was readmitted for a pin site infection and one had wound breakdown treated with outpatient wound care. There have been no other ED visits or hospital admissions.

Conclusions: In our series, cranial vault distraction has preliminarily proven to be a safe and effective treatment for refractory and symptomatic hydrocephalus. It uses principles of cranial vault expansion but with a more favorable safety profile in terms of dissection and soft tissue management. Though it does require a second operation for device removal, we feel it is a beneficial technique and plan to continue this series.

(1) Ministry of Health, Alshamiyah, Kuwait, (2) Kuwait University, Alkhaldeya, Alasema, Kuwait

Background/Purpose: BACKGROUND: Non-syndromic cleft lip and palate (NCLP) is one of the most common craniofacial anomalies with the occurrence of 1.48/1000 live births in Kuwait. The etiology of OFC (Orofacial Clefts) is still not fully understood. However, environment and genetics play major roles in cleft lip and palate development. Several GWAS studies have implicated numerous genetic variants at different loci to increase risk of NCLP. Reproducibility and population based studies have suggested the association of the Bone Morphogenetic Protein 4 (BMP4 rs17563) with the increased development of NCLP. The protein product of this gene is important in the regulation of gene expression needed for bone and cartilage development. Some studies have also reported a potential role of BMP4 expression in the signal pathways for lip and palate fusion. The genetic variant rs17563 results from a functional nonsynonymous that alters the protein sequence and thereby may alter the protein function. OBJECTIVE: Based on the suggested role of BMP4 and its functional variant, this study aimed to investigate the genetic association of BMP4 rs17563 and if it may contribute to the increased risk of NCLP in Kuwait.

Methods/Description: METHODS: The subjects included are individuals with familial or sporadic NCLP (n = 65) and controls who are family members (n = 111) attending the Oral Clefts Clinic, Alamiri Dental Center. Venous blood was withdrawn, and clinical examination was conducted to confirm diagnosis. Genetic pedigrees were constructed for cases with positive family history using Progeny Genetics software. Total genomic DNA was extracted at Kuwait University Genetics Laboratories, Faculty of Sciences, and processed for genotyping by Realtime PCR. The genotype and minor allele frequencies (MAF) were estimated for rs17563 and compared between cases and controls using χ ² test.

Results: The genotypes for rs17563 were found to be in Hardy-Weinberg equilibrium for the total population (n = 176) and in cases and control (P > .05). The lowest observed genotype frequency was homozygous for the minor allele in cases (0.138) and controls (0.171). Although no significant difference nor association was observed for rs17563 MAF with NCLP, there was a difference in the genotype frequency distribution suggesting the need to increase the sample size.

Conclusions: Although the results did not show a significant association with rs17563, it may still be worthwhile to examine other variants at this gene loci (BMP4). Previous reports have indicated a functional role of BMP4 with the development of NSCLP and the contradictory results here may be ethnic related.

Acknowledgment: This research is funded by the Kuwait Foundation for the Advancement of Sciences Grant #PR17-13DS-01.

(1) Johns Hopkins Hospital, Baltimore, MD, (2) Washington University School of Medicine, St. Louis, MO

Background/Purpose: The aim of this systematic review and meta-analysis was to investigate treatment effects of nasoalveolar molding (NAM) in children with unliteral cleft lip and palate (UCLP).

Methods/Description: Included manuscripts met the following criteria: (1) involved children with UCLP who received NAM; (2) included a comparison group who either received non-NAM passive presurgical infant orthopedic appliances (PSIO) or who did not receive any PSIO; (3) reported at least one objective or validated measure of nasolabial, craniofacial, or palatal form; and (4) had patient follow-up beyond 4 years of age.

Results: A total of 12 studies were included in this review. Meta-analyses were possible for Asher-McDade parameters and cephalometric measurements. Compared to children who did not receive any PSIO, those who underwent NAM therapy were more likely to have good to excellent frontal nasal form (RR 2.4, 95% CI 1.24 to 3.68) and vermillion border (RR 1.8, 95% CI 1.19 to 2.71). However, there were no statistically significant differences in cephalometric measurements between these groups. Additionally, there were no statistically significant differences between children receiving NAM versus non-NAM PSIO. There was insufficient evidence to determine the impact of NAM on dental arch development.

Conclusions: The preponderance of evidence in this review suggests that NAM produces benefits in nasolabial aesthetic form when compared to no appliance-based presurgical treatment. However, there is insufficient evidence to conclude if NAM produces such benefits when compared to other passive PSIOs.

(1) Washington University in St. Louis, MO, (2) Washington University School of Medicine, St. Louis, MO

Background/Purpose: Opioid overprescribing has been reported in pediatric surgery, but is not well-documented for craniofacial procedures. New persistent opioid use after surgery is a major concern of the opioid crisis, and this phenomenon has been demonstrated in patients undergoing cleft-related surgeries. Accidental ingestion and drug diversion are additional risks of pediatric opioid prescriptions. Critical analysis of current prescribing patterns is essential for curbing excess opioid prescriptions and achieving safe prescribing practices.

Methods/Description: We retrospectively reviewed records for all patients undergoing surgery for cleft lip and/or palate or craniosynostosis at a single institution over 12 months (July 1, 2018, to July 1, 2019). Prescribed opioid doses at hospital discharge were analyzed by patient age and procedure with nonparametric univariate analysis. Correlations between patient age and number of prescribed doses were analyzed with Spearman’s Rho. Actual home use of prescribed opioids was analyzed with prospective in-person patient surveys.

Results: Prescriptions of 90 patients with cleft lip and/or palate and 43 patients with craniosynostosis were included. Median prescribed opioid doses were 10.3 for cleft lip and/or palate procedures (range 0-75), and 14.3 for craniosynostosis repairs (range 0-50). In patients with cleft lip and/or palate, there was a negative correlation between age at surgery and prescribed opioid doses (rs = −0.228, P = .031). Forty-five patients completed surveys reporting actual home opioid use. No patients used more than 10 doses. Forty percent reported no opioid use at home, 33% used 1 to 2 doses, 18% used 3 to 5 doses, and 9% used 6 to 10 doses.

Conclusions: Postoperative opioid prescriptions vary widely after common cleft and craniosynostosis procedures, and younger patients with cleft lip and/or palate may be more likely to be prescribed more doses. Actual home opioid use is less than prescribed amounts, with most patients using 5 or fewer doses.

(1) Meikai University School of Dentistry, Sakado, Saitama, Japan

Background/Purpose: Recently, intraoral scanners are utilized in many dental treatments. It is known that digital dentistry can provide more accurate treatment and better appliances, and also simplify the classical intervention. Moreover, digital dentistry can reduce the treatment time and the burden of patients. We have been performing presurgical infant orthopedics for children with CLP using passive plates. During the fabrication of the plates, alginate impressions have to be taken from infants. Since infants with CLP have high risk of vomiting and accidental swallowing during the alginate impression, it is essential to manage these risks strictly. To overcome these problems, intraoral scanners were used for infants with CLP and the accuracy of scanned images was evaluated.

Methods/Description: Recently, intraoral scanners are utilized in many dental treatments. It is known that digital dentistry can provide more accurate treatment and better appliances, and also simplify the classical intervention. Moreover, digital dentistry can reduce the treatment time and the burden of patients. We have been performing presurgical infant orthopedics for children with CLP using passive plates. During the fabrication of the plates, alginate impressions have to be taken from infants Since infants with CLP have high risk of vomiting and accidental swallowing during the alginate impression, it is essential to manage these risks strictly. To overcome these problems, intraoral scanners were used for infants with CLP and the accuracy of scanned images was evaluated.

Results: The used intraoral scanner didn’t have difficulty in taking STL data of 2 infants with CLP. The insertion of the silicone impression material to the clefts helped scanning. The shape difference between the STL data obtained from the intraoral scanner and the plaster models fabricated after the alginate impression was less than 100 nm. Finally, the passive plates which were fabricated from the STL data showed an acceptable fit to the infants.

Conclusions: Use of the intraoral scanner can dramatically reduce the risk of vomiting and accidental swallowing in infants with CLP and provide accurate passive plates for presurgical infant orthopedics.

(1) The Royal Children’s Hospital Melbourne, Parkville, Victoria, Australia

Background/Purpose: Individuals diagnosed with orofacial clefts are at higher risk for developing dental caries and have been reported to demonstrate poorer oral hygiene compared to individuals without cleft diagnoses. Early establishment of a dental home is a key part of preventive care. To encourage this, the hospital Cleft service sends a letter to families of children aged 18 months advising them to establish a dental home, providing details of both public and private sector services. Ongoing specialist multidisciplinary team assessments for these children then continues within the hospital environment at set points throughout their development with the first team visit occurring at age 3. Aims: The primary aim of this clinical audit was to determine whether this early dental advice does lead to establishment of a dental home prior to the first multidisciplinary cleft team visit. The secondary aims were to report on oral hygiene status and dental caries experience of this 3-year-old cohort. Recommendations from this audit will be used to inform future service development, in particular changes to early preventive dental care for children with cleft lip and/or palate.

Methods/Description: Design: Data were collected retrospectively from January 2018 to December 2019. Clinical records of 152 children scheduled to attend their 3-year-old cleft team visit were audited. A total of 126 children were seen by the dentist for an oral health assessment and the following information was documented: whether the child had previously seen a dentist, oral hygiene status, and absence/presence of dental caries. Findings: Analysis revealed that 68% (n = 86) of patients had seen a dentist prior to their 3-year-old visit. The majority (70%, n = 88) of all patients were found to have good oral hygiene; however, nearly half (43%, n = 16) the children with a cleft of the lip and palate had poor oral hygiene. The presence of dental caries was recorded in 21% (n = 27) of the children with a similar distribution across all cleft types.

Conclusion: While the majority of children had an established dental home by their 3-year old cleft team visit, this audit demonstrated there is room for improvement in delivery of timely preventive advice and access to oral health care. Objectives: (1) Learners will be able to recognize the importance of encouraging families to establish a dental home at a young age. (2) Learners will be able to identify 4 areas where delivery of early oral health care to families may be improved: (a) Sending a dental-introduction letter between 6 and 12 months of age. (b) Coordinating visits to the dentist with other specialist appointments prior to initial multidisciplinary team visits. (c) Encouraging non-dental team members to reinforce simple anticipatory guidance. (d) Providing local dentists with basic knowledge to support families. (3) Learners will be able benchmark their own cleft service practices to assess the effectiveness and delivery of early oral health information.

(1) McMaster University, Hamilton, Ontario, Canada, (2) Hospital for Sick Children, University of Toronto, Ontario, Canada, (3) Oxford University Hospitals, Oxford, United Kingdom, (4) The Spires Cleft Centre, Oxford, England, (5) University of Pittsburgh School of Medicine, Pittsburgh, PA, (6) Ron Joyce Children’s Health Centre, Hamilton, Ontario, Canada

Background/Purpose: CLEFT-Q is a condition-specific patient reported outcome instrument (PROM) for patients with CLP. The aim of this study was to examine cross-sectional construct validity of the CLEFT-Q scales. This form of construct validity examines the extent to which a PROM works as intended by testing pre-defined hypotheses about correlations of scores with other PROMS that measure related constructs, at one point in time.

Methods/Description: Patients aged 8 and 29 years with CLP were recruited from 6 cleft centers in Canada, USA and UK. Before undergoing rhinoplasty, orthognathic, cleft lip scar revision, and alveolar bone graft (ABG), participants were asked to complete the following: 9 CLEFT-Q scales, COHIP and a CHASQ subscale. Hypotheses were predefined for 53 expected correlations within and between scales that measured appearance and health-related quality of life (HR-QOL). Spearman correlations were calculated and interpreted as follows: <0.3 weak, 0.30-0.50 moderate, ≥0.50 strong.

Results: Participants (n = 177) were mostly male (61%) and aged between 8 and 11 years (42%). Overall, 39/53 (74%) hypotheses were supported. More specifically, 20/26 (77%) appearance scale correlation hypotheses, 2/3 (67%) HR-QOL correlation hypotheses, and 17/24 (71%) hypotheses about correlations between appearance and HR-QOL scales were supported.

Conclusions: The cross-sectional construct validity evidence for the CLEFT-Q scales presented in this study further supports their psychometric validity.

(1) Children’s Hospital of Pittsburgh, PA, (2) UPMC Childrens Hospital of Pittsburgh, PA, (3) Baystate Health System, Division of Plastic Surgery, Springfield, MA, (4) University of Pittsburgh, PA, (5) University of Pittsburgh School of Medicine, Pittsburgh, PA

Background/Purpose: The study of nasal changes in cleft lip and palate (CLP) orthognathics is limited. This study aimed to determine differences in 3-dimensional (3D) nasal changes for unilateral cleft lip and palate (UCLP) and bilateral cleft lip and palate (BCLP) patients after Le Fort I advancement.

Methods/Description: This study was a retrospective cohort evaluation of 18 subjects (9 male, 9 female) treated at a single institution over a 5-year period who had class III skeletal and dentoalveolar malocclusion related to non-syndromic cleft lip and/or palate and underwent surgical correction via Le Fort I osteotomy. Patients underwent pre- and postoperative cephalometric measurements and 3-dimensional (3D) volumetric assessment using 3D imaging software VECTRA® (Canfield Scientific) and 3DMD (3dMD LLC) for comparison to analyze nasal changes. Statistical analysis was performed to determine changes in nasal parameters using Student t test and multivariate analysis. Differences were considered significant for P values ≤ .05.

Results: All nasal parameters except for nasolabial angle and nasal length changed significantly postoperatively in the entire cohort. Alar base and flare width both increased significantly (2.1 mm, P = .0002, 1.4 mm, P = .0005), while both relative and total tip projection decreased (−3.4 mm, P = .000004, −2.2 mm, P = .0008). An advancement of the entire nasal complex was seen postoperatively (1.0 mm, P = .0005). UCLP patients had a significantly larger decrease in total nasal tip projection following surgery relative to BCLP patients (−-3.5 ± 1.2 mm, −0.8 ± 2.1 mm, P = .008). BCLP morphology increases the degree of 3D nasal surface area advancement relative to UCLP (1.2 mm ± 0.3, 0.8 mm ± 0.6, P = .028). Alar cinch stitch decreases nasolabial angle (P = .024) but increases nasal length (P = .004).

Conclusions: Discrepancies exist in nasal changes after Le Fort I advancement between UCLP and BCLP patients. Patients in both groups exhibit increased alar base width and flaring, decreased total and relative nasal tip projection, and anterior advancement of the entire nasal surface complex as measured by 3D analysis. UCLP morphology is associated with a larger decrease in total tip projection than BCLP after Le Fort I, and a larger degree of total nasal complex advancement is seen after Le Fort I in the BCLP group. Increased understanding of outcomes for cleft patients undergoing Le Fort I can help improve the predictability of nasal aesthetic changes in this patient population.

(1) East Carolina University, Greenville, NC

Background/Purpose: Velopharyngeal (VP) ratios are continually used to evaluate normal VP anatomy and normal VP function. VP ratios are calculated by dividing the entire velar length by the pharyngeal depth. Individuals with normal anatomy present with VP ratios ranging from 1.2 to 1.43 (Subtelny, 1957; Tian et al., 2010). Individuals presenting with a VP ratio below the average range have previously been thought to have a disadvantageous VP mechanism which could lead to VP incompetence (VPI). However, Perry et al. (2018) reported individuals with a repaired cleft palate and normal resonance with a VP ratio of 0.7 to 1.21. This suggests that VP ratios alone cannot explain acceptable VP function for speech. Tian and Redett (2009) introduced the idea that an effective VP ratio may be a more appropriate indicator of normal parameters for speech. This ratio is calculated with the effective velar length, the distance from the posterior nasal spine to the velar eminence and includes the region of the velum that is activated during speech. The aims of this study are to examine if the VP ratio is preserved across the age span or if it varies with changes in the VP portal and to analyze if the effective VP ratio is more stable across the age span.

Methods/Description: Magnetic resonance imaging (MRI) was used to analyze velopharyngeal anatomy of 270 participants. Based on cranial growth rate, the participants were divided into the groups infants, children, adolescents, and adults for statistical analysis. The VP ratio and effective VP ratio were calculated using previously described methods. ANOVAs and a Games Howell Post Hoc Test were used to compare variables between groups.

Results: There was a statistically significant difference (P < .05) in all measurements between the 4 age groups. Pairwise comparisons reported statistically significant adjacent group differences (P <.05) for velar length, VP ratio, effective velar length, adenoid depth, and pharyngeal depth. No statistically significant differences between adjacent age groups was reported for effective VP ratio. Visually, velar length was noted to increase significantly from infancy to adulthood, while effective velar length grows at a slower rate, with minimal changes from adolescents to adults.

Conclusions: This study provides insights into differences in effective VP ratio and VP ratio across the age span. Effective VP ratio was not statistically significant between adjacent age groups, while VP ratio was statistically significant between adjacent age groups from the infant to child group and adolescent to adult. Therefore, the VP ratio changes significantly more across the age span in comparison to effective VP ratio. This may be due to velar length increasing in length throughout life, while the effective velar length does not experience a large increase in growth following adolescents. This study suggests that effective VP ratio is more correlated to VP function than VP ratios.

(1) Fundación de Anomalías Craneofaciales Fernando Ortiz Monasterio, Mexico City, Mexico, (2) Universidad Nacional Autónoma de México, Mexico City, Mexico, (3) Fundación de Anomalías Craneofaciales Fernando Ortiz Monasterio, Mexico City, Mexico, (4) Orthodontist of Rush Craniofacial Center, Rush University Medical Center, Chicago, IL, (5) Miami Children’s Hospital, Miami, FL, (6) Smile Train, New York, NY, (7) Instituto Nacional de Pediatría, Mexico City, Mexico, (8) Dentnuvó, Mexico City, Mexico, (9) Universidad del Ejercito y Fuerza Aérea de México, Mexico City, Mexico, (10) Hospital Infantil de las Californias, Tijuana, Mexico

Background/Purpose: One of the main stigmas of the cleft lip and palate (CLP) is a nasal deformity. Presurgical infant orthopedics (PSIO) focuses on reducing nasal deformity and cleft lip before the first lip surgery. However, due to the heterogeneity of outcomes registrations, its effectiveness has not been evaluated.

Methods/Description: A content validity study of an instrument with Delphi panel. The content validation of the instrument with the Delphi panel consisted of 3 stages: First stage: Development of the instrument—A systematic search of the literature, and an ad hoc questionnaire was conducted that included representative anthropometric measures to evaluate the effectiveness of presurgical infant orthopedics. All the material available in the questionnaire was divided into 3 domains; nasal, lip, and alveolar. Second stage: Pilot test and expert panel—The questionnaire was subjected to a pilot test Expert panel: Orthodontists and plastic surgeons with at least 10 years of experience in the treatment of cleft lip and palate, speaking languages English and Spanish were invited. Third stage. Rounds of choice—The anthropometric measures questionnaire circulated in 3 rounds with only 2 options to choose: Necessary and Not necessary. The first round: Validity of the grouping of anthropometric measures with the similarity of operational definitions and schematic location of anthropometric points. The integration into the measuring instrument of the anthropometric measurements were considered as necessary with agreement ≥80%. The second round is the feedback of the anthropometric measures with agreements between <80% and > 60% and the integration of new anthropometric measures proposed by experts. The third round is the anthropometric measures that reached the agreement between <80% and >60%, and the new anthropometric measurements proposed by experts are resubmitted. Objective: Experts should reconsider previous responses based on the feedback information. Only anthropometric measurements with agreement ≥80% considered necessary are included into the measurement instrument.

Results: During the first round 149 anthropometric measures were presented. Of which in the first round 15 anthropometric measures had an agreement ≥80%. In the second round, feedback, 8 new anthropometric measures proposed by the experts were incorporated. For the third round, 9 anthropometric measures were incorporated with an agreement ≥80% between the experts. The instrument to evaluate the effectiveness of PSIO contains 26 anthropometric measures considered necessary.

Conclusions: This research was able to identify and reduce the number of potentially useful anthropometric measurements to evaluate the effectiveness of interventions with PSIO. This instrument to evaluate the effectiveness of presurgical infant orthopedics could help to homogenize the outcome variables, it could also be used to initiate multicenter studies and analyze the different types of protocols in PSIO.

(1) Osaka University Cleft Palate Team, Suita, Osaka, Japan, (2) Graduate School of Dentistry, Osaka University, Suita, Osaka

Background/Purpose: Our department, Division of Oral-Facial Disorders, is responsible for pharyngeal flap operations to improve velopharyngeal function and also to evaluate pre- and post-operative speech function. We have newly adopted a unified velopharyngeal plasty combined with Furlow’s method for soft palate treatment since 2008. The purpose of this study was to examine the postoperative results on velopharyngeal function after utilizing our new operation methods.

Methods/Description: Forty-three speakers with cleft palate underwent newly modified velopharyngeal plasty in our department between 2008 and 2018. Two subjects who would not receive the evaluation were excluded. Pre- and post-velopharyngeal motion during speech and blowing were evaluated using a nasopharyngoscope and classified into 3 states: incompetence, borderline incompetence, and competence. Nasometry was also performed to evaluate the nasalance score of a standard sentence, a low-pressure sentence, and a high-pressure sentence pre- and post-operation. We compared velopharyngeal motion and nasalance score between pre- and post-operation.

Results: On preoperative endoscopic findings, 75% of subjects showed velopharyngeal incompetence in all tasks, and 25% of subjects demonstrated incompetence in speech but borderline incompetence or competence in blowing. On postoperative findings, 73% of subjects showed velopharyngeal competence in all tasks. The remaining subjects did not demonstrate incompetence but borderline closure with fair speech intelligibility. The averages of the pre- and post-operative nasalance score were 47.7% and 17.0% for the standard sentence, 40.9% and 17.1% for the low-pressure sentence, and 52.6% and 18.4% for the high-pressure sentence, respectively. There was a significant difference between the pre- and post-operative nasalance scores for all 3 sentences (Mann-Whitney U test, P < .001).

Conclusions: These results indicated that our new operation method, unified velopharyngeal plasty combined with Furlow’s method, could achieve fair to excellent velopharyngeal function postoperatively.

(1) Nationwide Children’s Hospital, Columbus, OH, (2) The Ohio State University Medical Center, Columbus, OH

Background/Purpose: Pierre Robin Sequence (PRS) is characterized by micrognathia, glossoptosis, upper airway obstruction, and frequently, cleft palate. Children with PRS are at risk for feeding and breathing difficulties, including failure to thrive and obstructive sleep apnea. However, few studies have documented developmental and psychosocial risks associated with PRS or receipt of related services. In order to address these gaps, a retrospective chart review study of patients with PRS treated between 2005 and 2018 was conducted at a single pediatric academic medical center’s craniofacial center.

Methods/Description: Charts of 108 patients (62% female; mean age at chart review: 5.97 ± 4.10 years) with PRS were reviewed. Demographic (eg, sex, race, age, insurance type, distance to care) and clinical characteristics (eg, associated syndromes or anomalies; type of treatment; history of developmental delays, evaluations and therapies; learning concerns; behavioral health services and diagnoses) were abstracted. Descriptive statistics and χ ² or Fisher exact tests were used to evaluate differences in developmental and psychosocial outcomes by sex, treatment (nonsurgical, mandibular distraction, tongue–lip adhesion), insurance type, and distance to care.

Results: Children were predominantly Caucasian (88%), resided outside of the hospital’s county lines (71%), and had Medicaid insurance (63%). Nearly all (96%) had cleft palate; 41% had associated anomalies or syndromes. Regarding treatment, 44% were treated nonsurgically, 32% with mandibular distraction, and 24% with tongue-lip adhesion. History of developmental delay was documented in 74%, most commonly speech/language delays, with no significant differences observed by sex, presence of a syndrome, or treatment type. Of those with developmental delays, 52.5% were referred for a hospital-based developmental evaluation, 80% received state-sponsored early intervention services, and 81% engaged in hospital-based developmental therapies. There were no significant differences in receipt of services by distance to care or insurance type; however, those who received surgical treatment were significantly (P = .04) more likely to receive developmental therapies than non-surgically treated children. Across the sample, 24% required an IEP or 504 plan in school. Ten percent were referred for hospital-based behavioral health services, 13% had global developmental delay, 2.8% ADHD, and 0.9% autism.

Conclusions: Developmental delays were common in this sample of children with PRS, with a minority having documented learning or behavioral health concerns. These findings, while descriptive and limited by use of available data recorded in medical charts, provide a greater understanding of the developmental and psychosocial risks for children with PRS, and can be used to offer anticipatory guidance for clinical care providers and parents. Findings also underscore the need for interdisciplinary care approaches including developmental and psychosocial risk screening.

(1) Division of General Surgery, University of Southern California, Los Angeles, CA, (2) Division of Plastic and Maxillofacial Surgery, Children’s Hospital Los Angeles, CA, (3) Division of Plastic and Reconstructive Surgery, University of Southern California, Los Angeles, CA, (4) University of Southern California, Keck School of Medicine, Los Angeles, CA, (5) Division of Anesthesiology, Children’s Hospital Los Angeles, CA, (6) Divison of Plastic and Maxillofacial Surgery, Children’s Hospital Los Angeles, CA, (7) Division of Plastic and Maxillofacial Surgery, Children’s Hospital Los Angeles, CA

Background/Purpose: Dogma established by Millard in 1976 regarding the ideal timing of elective surgery in infants has long centered on the “rule of 10s” (>10 weeks of age, >10 pounds, and has >10 g hemoglobin, and <10 white cell count). These guidelines were built upon archaic anesthetic protocols and do not accurately represent the safety profile of modern day agents. Opposition to neonatal anesthesia cite concerns regarding GABA-agonists and NMDA-receptor antagonists being associated with a degree of neural apoptosis. Opioids and low-dose dexmedetomidine, a highly selective alpha-2 agonist, remain among the few agents used for anesthesia that have not shown pro-apoptotic activity. As such, our institution has created a neonatal anesthetic protocol (NAP) designed with dexmedetomidine as the dominant agent in early cleft lip repair (ECLR).

Methods/Description: Patients who underwent ECLR (repair before 2.5 months of age) within the last 4.3 years were identified. These patients were separated into those receiving the NAP and those who did not. Retrospective review of their records included preoperative, perioperative, and postoperative data regarding major and minor complications, and medication side effects. Total anesthetic time was defined as time from induction to extubation. Major complication was defined as a code event, aborted surgery, or intraoperative death. Minor complication was defined as a sustained alteration in heart rate, apnea event, or prolonged emergence time. Medication side effect was defined as a transient alteration in heart rate or hypopnea. Unpaired t tests were utilized to assess for statistical differences between the NAP and Non-NAP groups. Stepwise binary logistical regression analysis assessed for differences in outcomes and predictors of complications and side effects in the total cohort, the NAP group, and the Non-NAP group.

Results: In all, 101 patients underwent ECLR during our study period. All patients were either ASA class 1 or 2. 65% (n = 65) received the NAP. The NAP group had a lower weight (4.01 ± 0.61 vs 4.38 ± 0.72, P = .007) and required less IV morphine equivalents in the PACU (10.8% vs 30.5%, P = .013). There were no other statistically significant intraoperative or preoperative variables. There were no major anesthetic complications for either group and the minor anesthesia complication and medication side effect rate for the NAP group versus the non-NAP group was 6.2% versus 0%, P = .166 and 4.6% versus 2.7%, P = .650, respectively.

Conclusions: Our novel neonatal anesthetic protocol demonstrated no major complications and an acceptable minor complication rate when administered to otherwise healthy patients undergoing ECLR. As safer alternative anesthetic agents and protocols are introduced, Millard’s rule of 10’s governing the ideal timing of elective surgery in infants may not apply to ASA 1 and 2 class patients.

(1) Division of General Surgery, University of Southern California, Los Angeles, CA, (2) University of Southern California, Keck School of Medicine, Los Angeles, CA, (3) Division of Plastic and Maxillofacial Surgery, Children’s Hospital Los Angeles, Los Angeles, CA, (4) Division of Plastic and Reconstructive Surgery, University of Southern California, Los Angeles, CA

Background/Purpose: Over the last 3 years, an institutional shift has taken place in which patients originally designated for nasoalveolar molding (NAM) as an adjunct to traditionally timed cleft lip repair (repair after 3 months) have instead undergone early cleft lip repair (ECLR) at 2 to 5 weeks of life without NAM. After implementing the ECLR program at our institution, only a small subset of patients still undergo NAM with the standard surgical timing of repair. The financial and social impact of this potential paradigm shift has not been studied. We sought to examine the financial cost-effectiveness of the ECLR protocol.

Methods/Description: We reviewed records for all patients who underwent NAM as an adjunct to cleft lip repair from November 2011 to June 2018. From November 2011 to February 2014, NAM with standard timing of lip repair was the only intervention offered to patients with wide cleft lip defects. From February 2014 to June 2018, ECLR without adjunctive NAM was offered as an alternative. Retrospective chart review of the 2 groups was conducted with emphasis on the following variables: NAM and ECLR cleft classifications, NAM dental visits, ECLR length of hospital stay, ECLR patients’ cleft width ratio (CWR), and operative dates. ECLR patients who had a CWR of > 0.5 and unilateral complete cleft lip (UCL) were identified as patients who would have originally been offered NAM as an adjunct to their cleft lip repair.

Results: NAM patients required an average of 11 dental visits, accounting for $2132 in lost income per family. Average direct charges for NAM totaled $12 290 for the hospital, physician, and device costs. Over the entire study period, the cumulative direct cost of NAM separate from the surgical repair of the lip was $970.910. ECLR patients underwent lip repair 80 days earlier than NAM patients (mean of 33 vs 113 days respectively, P < .001). Following the introduction of ECLR as an alternative to NAM with standard lip repair, NAM usage decreased by 48% (52 to 27 patients) and unilateral cleft lip patients undergoing NAM decreased by 86% (35 to 5 patients). In the study period offering ECLR, 26 patients were diverted from NAM to ECLR resulting in a health care cost burden of $319 540 less ($96 830 per year).

Conclusions: ECLR without NAM is more cost effective and results in excellent surgical and aesthetic outcomes. NAM as an adjunct to wide cleft lip and nasal repair is no longer the most cost effective option at our institution. We believe that ECLR has the potential to decrease the burden of health care costs in the United States.

(1) University of Wyoming, Laramie, WY, (2) New Hannover Regional Medical Center, Wilmington, NC, (3) Children’s Healthcare of Atlanta, GA, (4) East Carolina University, Greenville, NC

Background/Purpose: Velar stretch is defined as an increase of the velum’s length from the rest position to the elevated position, which is necessary to produce intelligible speech (Pruzansky et al., 1969). When the velum cannot completely separate the nasal and oral cavities during speech, velopharyngeal dysfunction (VPD) occurs. The literature has documented varying (and sometimes conflicting) results regarding which factors have the greatest influence on velar stretch, some of which include scarring from cleft repair surgery, muscle mass, range of motion, synergy of other muscles, and postsurgical dimensions of the region. The purpose of this study is to examine velar stretch between children with and without repaired cleft palate and determine if velar stretch is associated with adenoid depth. It was hypothesized that children with repaired cleft palate would exhibit decreased velar stretch compared to normative participants.

Methods/Description: In accordance with the IRB, 22 English-speaking children with typical resonance between 3 and 8 years of age were recruited to participate in this study. Half of the participants had repaired cleft palate. All participants underwent nonsedated magnetic resonance imaging (MRI). The MRI protocol, processing methods, and analysis are consistent with that used in previous investigations. During the MRI, each participant was instructed to sustain vowel phonation (/i/ and /s/) for 7.8 seconds to view the velum in a fully elevated position, and the best elevation was utilized for analysis.

Results: Inter- and intra-rater reliability was obtained. Nonparametric statistical analyses were utilized to compare measures between the participant groups due to the small sample size and nonnormal distribution of data. Independent sample Mann-Whitney U tests (α < .05) were used to compare differences between groups regarding the measures of interest. Significant differences were noted between participant groups for total velar length (U = 3.000, P < .001), effective velar length at rest (U = 30.000, P = .047), pharyngeal depth from the velar knee to the posterior pharyngeal wall (U = 29.000, P = .040), and total velar length during phonation (U = 20.000, P = .007). Remaining measures were not statistically significant, including velar stretch. A Spearman’s partial rank-order correlation was run to assess the relationship between velar stretch and adenoid depth while controlling for participant group. An increase in velar stretch was moderately associated with a decrease in adenoid depth, rs(20) = −.432, P = .045.

Conclusions: Velar stretch was not significant between groups. This finding is likely because all participants with repaired cleft palate presented with typical speech. Adenoid depth proved to be moderately associated with velar stretch. This is logical given the demand on the velum to stretch depends on the distance it must travel to achieve velopharyngeal closure, which is decreased by large adenoids. Limitations include a small sample size and varying surgical technique.

(1) Washington University in St. Louis, MO, (2) Washington University School of Medicine, St. Louis

Background/Purpose: Patients with craniosynostosis undergoing open cranial vault remodeling require adequate postoperative analgesia, but approaches for opioid use reduction are warranted to reduce adverse effects of opioid administration. Regional anesthesia with scalp peripheral nerve blocks is an important adjunct to traditional methods of perioperative analgesia. We aim to determine the effect of a preoperative scalp block on inpatient postoperative opioid use and patient pain scores, relative to standard analgesic protocols.

Methods/Description: We retrospectively reviewed all patients undergoing open cranial vault remodeling or fronto-orbital advancement for single-suture craniosynostosis between February 2012 and June 2019 at a single institution. Patients were grouped by whether or not a scalp block was administered for regional anesthesia. Scalp peripheral nerve blocks, added to the protocol beginning in 2017, were administered by the anesthesia team after induction of general anesthesia and prior to surgical incision. The primary outcome was total opioid consumption in oral morphine milligram equivalents per kg (MME/kg) on postoperative days (POD) 0 and 1. The secondary outcome was patient pain as recorded on the revised FLACC (rFLACC) scale by nursing staff on each postoperative day. Differences between patients with and without scalp blocks were analyzed with Fisher exact test, Mann-Whitney U test, or repeated measures ANOVA as appropriate.

Results: Of 50 patients included in the analysis, 31 received scalp blocks and 19 did not. The proportions of patients undergoing cranial vault remodeling (65% and 53%) and fronto-orbital advancement (35% and 47%) were similar in the 2 groups (P = .553). Median total opioid consumption was significantly lower in the scalp block group (2.2 vs 3.6 MME/kg, Mann-Whitney U = 120.5, P = .001). Mean pain scores were lower in patients with scalp blocks than those without on each postoperative day (POD 0, 1.9 vs 3.0; POD 1, 2.1 vs 2.4; POD 2, 0.4 vs 1.1; POD 3, 0.1 vs 0.9), but these differences were not statistically significant (P = .060). Median length of stay was 2 days in the scalp block group, and 3 days in the group without blocks (P = .145).

Conclusions: Scalp peripheral nerve blocks reduced total opioid consumption in the first 48 hours in patients undergoing open cranial vault remodeling and fronto-orbital advancement for single-suture craniosynostosis.

(1) McMaster University, Hamilton, Ontario, Canada, (2) Hospital for Sick Children, University of Toronto, Ontario, Canada, (3) Oxford University Hospitals, Oxford, United Kingdom, (4) The Spires Cleft Centre, Oxford, England, (5) University of Pittsburgh School of Medicine, Pittsburgh, PA, (6) Ron Joyce Children’s Health Centre, Hamilton, Ontario

Background/Purpose: CLEFT-Q is a condition-specific patient reported outcome instrument (PROM) for patients with CLP. The aim of this study was to examine internal responsiveness of the CLEFT-Q scales for 3 cleft-specific surgeries, that is, rhinoplasty, orthognathic, and lip scar revision surgery. Internal responsiveness refers to the ability of a PROM to detect change over time. Minimally important difference (MID) is the smallest difference in scores that patients perceive as an important deterioration or improvement.

Methods/Description: Patients aged 8 and 29 were recruited from 6 cleft centers in Canada, United States, and United Kingdom. Participants were asked to complete the CLEFT-Q appearance scales most relevant to their surgery and all health-related quality of life (HR-QOL) scales before and approximately 6 months after surgery. Responsiveness was examined by comparing pre- and postoperative mean scores using the paired sample t test and calculating Cohen’s d effect size estimator. Cohen’s d effect size was interpreted with Cohen’s criteria: 0.20 small, 0.50 moderate, and 0.80 or above large. MIDs were estimated using the 0.5 effect size approach.

Results: Participants underwent rhinoplasty (n = 31), orthognathic (n = 21), and cleft lip scar (n = 18) operations. Most participants were male (56%) and between 8 and 11 years (41%). As expected, for rhinoplasty and orthognathic surgery, changes on the appearance scales were larger for aspects of appearance addressed by surgery. In rhinoplasty, effect sizes were larger for the nose (0.92, P = .001) and nostrils (0.94, P < .001) scales than for the face scale (0.51, P = .003). For orthognathic surgery, a larger effect size was observed for the jaw scale (1.80, P < .001) compared with teeth (1.16, P < .001), face (1.15, P = .001), and lip (0.94, P < .001) scales. The pattern of results for lip scar revision differed, with the largest effect size for the nose scale (0.76, P .03), followed by the lips (0.58, P = .009), and the cleft lip scar (0.50, P = .043) scales. Calculated MIDs were approximately ½ standard deviation as predicted. Statistically significant effect sizes on the HR-QOL scales were not found.

Conclusions: The CLEFT-Q was able to detect change in key outcomes for 3 cleft surgeries, providing evidence of its responsiveness. MIDs for each scale and cleft-related operation will aid in the interpretation of this PROM. Given the small samples, research in a larger and more culturally diverse sample is warranted.

(1) University of Southern California, Keck School of Medicine, Los Angeles, CA, (2) Division of Plastic and Maxillofacial Surgery, Children’s Hospital Los Angeles, CA, (3) Division of General Surgery, University of Southern California, Los Angeles, CA

Background/Purpose: Cleft palate is a common birth defect, affecting approximately 1 in 1700 births worldwide. Patients with cleft palate ± lip involvement (CP±L) often require multiple surgeries, diagnostic procedures, and long-term follow-up with a multidisciplinary craniofacial team. Plan of care varies by the extent of cleft involvement, as most often described by Veau classification. We aim to estimate the total Medicare charges associated with management of CP±L, as classified by Veau type.

Methods/Description: We determined the overall financial burden of cleft care through a model-based approach using theoretical treatment protocols based on institutional standard of care for patients with nonsyndromic CP±L. These patients were divided into 4 groups (Veau-I-IV) according to cleft phenotype. The theoretical protocols consisted of craniofacial team visits, diagnostic tests, and cleft-related surgeries. Patient records from 1975 to 2008 were reviewed to identify additional cleft-related procedures required beyond the standard surgical correction of the cleft (lip/palate/alveolus) defect. These surgeries include, but were not limited to, surgery for velopharyngeal insufficiency (VPI) and/or orthognathic surgery. Provider reimbursement for CPT codes of cleft-related craniofacial visits, diagnostic evaluations, and operations was determined using the Medicare Physician Fee Schedule Search for the year 2020 with appropriate geographical adjustment. Hospital reimbursements for the corresponding cleft-related interventions were determined by institutional review of charges collected from Medicare. Provider and hospital charges were summed to determine total Medicare reimbursement from time of birth to 21 years of age.

Results: At baseline, all patients with CP±L (regardless of Veau type) received craniofacial team visits yearly until age 21. The summed values of hospital and provider reimbursement for craniofacial team visits were $39 410.73 for all Veau types. The total number of surgeries, types of surgeries, and diagnostic studies required varied by Veau type. Baseline total reimbursements from Medicare for patients with Veau-I, Veau-II, Veau-III, and Veau-IV were $41.449.64, $46 225.53, $51 698.01, and $53 379.41, respectively. Of all, 19% and 54% of patients with CP±L received surgery for VPI or orthognathic surgery, respectively. When accounting for the addition of these procedures, maximal total charges to Medicare for Veau-I, Veau-II, Veau-III, and Veau-IV were $42 663.73, $47 439.62, $55 695.64, and $57 377.04, respectively, reflecting reimbursements for the top 10% of the population.

Conclusions: Caring for the patient with cleft palate requires an interdisciplinary team of surgeons, speech therapists, audiologists, dentists, orthodontists, and social workers. While the true hospital and provider reimbursements associated with cleft treatment vary between individual patient and payor, our model provides an overview of the anticipated financial burden to the health care system for a single patient with CP±L.

(1) University of California, San Diego, CA, (2) San Diego State University, San Diego, CA, (3) Istanbul Kültür University, Istanbul, Turkey, (4) Rady Children’s Hospital San Diego, CA

Background/Purpose: Congenital craniofacial conditions (CFCs) span unique subdiagnoses that impact health-related quality of life (HRQoL). Existing measures of HRQoL are often not developed in patients with CFCs, or focus only on those with cleft lip and/or palate (CLP). Our study utilized the CFC-QoL(1), a parent- and patient-reported outcome measure (PROM) developed in English and Spanish specifically for pediatric patients with diverse CFCs, in order to better capture the differences in their HRQoL.

Methods/Description: This cross-sectional study (N = 278) assessed parent-reports of HRQoL of children with CFCs using the CFC-QoL, which rates psychological function (PSY), physical function (PF), social impact (SI), family impact (FI), and appearance (APP). Analyses examined subscale scores, CFC subdiagnosis, gender, and ethnicity.

Results: Mean patient age = 9.34 (SD = 5.28). Patient gender = 52.5% F, 47.5% M. Patient ethnicity = 66.2% Hispanic, 33.8% non-Hispanic. CFC subdiagnoses = 52.2% CLP (22.3% unilateral, 20.9% bilateral, 9.0% cleft lip); 47.8% non-CLP CFCs (23.0% craniosynostosis, 9.7% dermatological condition, 9.7% microtia, 5.4% microsomia). Mean parent reports reflected mild-to-moderate impact of CFC across subscales. An independent samples t test found better parent ratings on PSY for patients with CLP compared to non-CLP CFCs (P = .004). For non-CLP CFCs, parents rated worse APP for girls than for boys (P = .001). For CLP CFCs, parent ratings were worse for Hispanic patients than for non-Hispanic patients on SI (P = .02) and APP (P = .04). For non-CLP CFCs, parent ratings were worse for Hispanic patients compared to non-Hispanic patients on PSY (P = .02) and PF (P = .002) but better on FI (P = .03). A one-way ANOVA found parent ratings varied across subdiagnoses on SI (P = .04), PF (P = .01), and APP (P = .03). Tukey post hoc tests showed parent ratings for patients with microtia were worse on SI than patients with unilateral CLP (P = .03), and compared to patients with craniosynostosis, worse on SI (P = .01) and PF (P = .02).

Conclusions: Our study reveals key differences between HRQoL of diverse CFCs. For patients with CLP, parents reported better PSY, and Hispanic parents rated SI and APP worse than their non-Hispanic counterparts. For patients with non-CLP CFCs, parents rated APP worse for girls than boys; while Hispanic parents reported worse PSY and PF, but better FI, than their non-Hispanic counterparts. Among non-CLP CFCs, microtia had worse SI and PF. These patterns indicate varied impacts of subdiagnosis and demographics on HRQoL, and the importance of establishing a tool to capture these impacts, to deliver optimal care.

Reference

Tapia VJ, Epstein S, Tolmach OS, Hassan AS, Chung NN, Gosman AA. Health-related quality-of-life instruments for pediatric patients with diverse facial deformities. Plast Reconstr Surg. 2016:138(1);175-187. doi:10.1097/prs.0000000000002285

(1) Nationwide Children’s Hospital, Columbus, OH, (2) University of West of England, Bristol, England

Background/Purpose: Youth with craniofacial conditions often experience facial appearance and/or speech dissatisfaction and related negative social impacts including teasing. However, there are few evidence-based interventions to help youth address body image concerns and social skills. Recently, an online-delivered, cognitive behavioral intervention program called YP Face IT was developed and piloted in the United Kingdom for use with adolescents with a range of appearance differences. However, this program has yet to be tested in US youth with craniofacial conditions. In order to address this gap, we evaluated the acceptability of YP Face IT in a sample of US adolescents with craniofacial conditions.

Methods/Description: Adolescents ages 12 to 17 years with craniofacial conditions treated at a US pediatric academic medical center were eligible to participate. Participants reviewed YP Face IT modules and took part in an interview that assessed their thoughts about the program, its cultural suitability, ways that the program could be improved, potential barriers to using it, and level of support needed to complete the program. Participants also completed a demographic survey and answered additional questions about YP Face IT (eg, How likely would you be to recommend YP Face IT to other teens?). Descriptive statistics were used to summarize survey responses and demographics.

Results: To date, 8 participants completed the study (75% female; mean age 14.4 ± 2.1 years; 50% identified as Asian; 75% with cleft lip and palate). None were currently engaged in a support group related to their craniofacial condition, but most indicated they were “likely” (37.5%) or “very likely” (25%) to utilize an online program. Topics of interest included navigating negative social media experiences (75%), making friends (75%), and answering questions about their diagnosis (75%). Youth reported that they liked (37.5%), really liked (12.5%), or felt neutral (50%) about YP Face IT. The program’s topics were rated as “somewhat relevant” (37.5%) or “relevant” (37.5%). No barriers were identified for using YP Face IT; youth found it easy to use (62.5%) and preferred to use it independently (75%). Most youth (75%) reported they were “likely” or “very likely” to recommend YP Face IT to others. Adolescents identified areas for improvement including updates to graphics to suit older adolescents (50%) and delivery of the program via smartphone app (62.5%). Data collection is ongoing to achieve our planned sample size of 20. Updated results will be presented.

Conclusions: US adolescents with craniofacial conditions had mostly favorable views of YP Face IT and were open to utilizing an online program to improve body image and social skills. These findings, while descriptive and limited by the small number of participants, provide a greater understanding of the acceptability of YP Face IT and can be used to further refine the program to engage American youth with craniofacial conditions.

(1) Division of General Surgery, University of Southern California, Los Angeles, CA, (2) Division of Plastic and Maxillofacial Surgery, Children’s Hospital Los Angeles, Los Angeles, CA, (3) Division of Plastic and Reconstructive Surgery, University of Southern California, Los Angeles, CA

Background/Purpose: The development of midface hypoplasia is common after palatoplasty and has been hypothesized to be influenced by the timing of hard palate repair. We perform a meta-analysis of the current literature to assess the risk of developing midface hypoplasia based on age at hard palate repair.

Methods/Description: A systematic review and meta-analysis was completed according to PRISMA guidelines. A Pubmed literature search using terms “palatoplasty” and “midface hypoplasia” was conducted and papers published between 1970 and 2019 were screened. Cephalometric data, including but not limited to SNA angle, was extracted and categorized by age at hard palate repair for 5 discrete age groups: <6, 7-12, 13-18, 19-24, and 25-83 months. A comparison of these age groups undergoing hard palate repair and a control group consisting of healthy patients without the cleft deformity was performed using independent T tests and Spearman correlation coefficients.

Results: SNA angles for groups undergoing palate repair were 77.9 ± 3.1º (<6 months), 77.7 ± 4.2º (7-12 months), 78.7 ± 4.2º (13-18 months), 75.1 ± 4.2º (19-24 months), and 75.5 ± 4.8º (25-83 months). The measurements were statistically different than the control group 82.4 ± 3.5º (P < .0001). Hard palate repair at 13 to 18 months had a statistically significant greater SNA angle than all other groups except for the <6 months group (P = .074). As age at hard palate closure increased beyond 18 months, the SNA angle decreased, corresponding to a more hypoplastic maxilla (Spearman’s correlation coefficient −0.381, P = .015).

Conclusions: Our analysis suggests that hard palate repair at a younger age decreases the likelihood of developing midface hypoplasia in adulthood. Minimizing midface hypoplasia in cleft palate patients by optimizing algorithms of care is a practical way to decrease the burden of disease on patients, families, and health care systems. Further studies are needed to evaluate the role of specific techniques on facial growth outcomes.

(1) UT Southwestern Medical Center, Dallas, TX, (2) Children’s Health, Dallas, TX, (3) UT Southwestern School of Medicine, Dallas, TX, (4) Analytical Imaging and Modeling Center, Children’s Health, Dallas, TX

Background/Purpose: Congenital auricular anomalies present along a spectrum of severity and if left untreated may affect quality of life. Ear molding has gained popularity as a nonsurgical alternative treatment for some congenital auricular deformities, taking advantage of the cartilage plasticity in the first weeks of life. Several molding techniques have been described in the literature, with variability in their complexity, outcome, and financial cost. We developed a convenient, and effective technique for neonatal ear molding, which has been taught in ACPA teaching courses for several years. Here, we describe the technique and evaluate outcomes in 283 ear treatments over the past 3 years.

Methods/Description: Treatment protocols were created for auricular deformities including Stahl’s ear, helical kinks, helical lidding/lop ear, prominent ear, and constricted ear. Therapy ideally begins before 14 days of age, and consists of continuous molding of the conformable auricule utilizing custom molded dental clay stents, which are secured to the ear using Mastisol adhesive liquid and Micropore tape. Additional steri-strips or micropore tapes are placed in strategic areas to support the ear in a more normal position. Ear molding is reviewed, adjusted, and reapplied every 3 weeks throughout treatment, performed under the care of Physician Assistants (PA) in clinic. Treatment typically consists of 2 sets of ear molds for a total of 6 weeks, but can extend to 9 weeks if necessary. Outcomes were evaluated by 3 experienced providers based on 2D photographs. The evaluation was conducted using a 5-point Likert scale ranging from 1-poor to 5-good.

Results: Our sample size consisted of 155 patients and a total of 283 ears. The 3 raters had mean ratings of 4.23 (± 0.75 SD), 4.0 (± 0.82 SD), and 3.81 (± 1.07 SD) respectively. Combined mean rating was 4.01 (± 0.77 SD).

Conclusions: Our results indicate substantial improvement in ear appearance. This technique provides a reproducible, and effective nonsurgical ear molding alternative to treat a variety of neonatal ear deformities.

(1) Children’s Memorial Hermann Hospital, Houston, TX, (2) University of Texas Health Science Center at Houston, Houston, TX, (3) University of Texas at Houston McGovern Medical School, Houston, TX

Background/Purpose: Patients with cleft palates can present with disordered resonance due to velopharyngeal insufficiency (VPI). Speech language pathologists (SLPs) can rate hypernasal resonance using the Universal Parameters for Reporting (UPR) speech outcomes which includes a 4 point scale for hypernasality. Velopharyngeal gap size can be quantitatively measured using nasopharyngoscopy or videofluoroscpoic palatal motion studies (VF-PMS). Our aim was to evaluate the correlation of UPR evaluation and the qualitative data gathered by VF-PMS and nasopharyngoscopy.

Methods/Description: We conducted a retrospective review of all patient’s with cleft palate at a single institution who were diagnosed with VPI from 1999 to 2014. All patients underwent perceptual speech evaluations using the UPR by a trained SLP and velopharyngeal imaging by either nasopharyngoscopy, VF-PMS, or both in order to evaluate for VPI at various in isolation, word, and phrase levels as deemed appropriate by the SLP. Speech sound errors consisting of compensatory misarticulations were avoided during imaging. Clinical evaluation of speech characteristics was completed using UPR. During VF-PMS, multiview imaging measured the distance between the velum and the posterior pharyngeal wall during sustained /i/ and /s/. The radiologist recorded images obtained at maximum velar movement to analyze maximum closure and document millimeter measurement of velopharyngeal gap findings. During nasopharyngoscopy, the SLP obtained quantitative measures based on en view findings. Correlation was assessed using a Spearman’s rank correlation coefficient.

Results: Of 113 patients who underwent speech evaluation by an SLP, 87% had a VF-PMS, 48% had nasopharyngoscopy, and 35% had both. Correlation of UPR hypernasality scores with gap size during sustained /i/ and /s/ during VF-PMS was 0.39 and 0.46, respectively. Correlation of UPR hypernasality scores with gap ratio from nasopharyngoscopy was 0.39. Correlation of UPR speech understandibility and gap size during sustained /i/ and /s/ was 0.24 and 0.32, respectively. Correlation of UPR speech understandibility and gap ratio from nasopharyngoscopy was 0.34.

Conclusions: Diagnosis of hypernasal resonance is dependent upon perceptual evaluation by systems such as the UPR and quantitative imaging findings. However, our study demonstrated low correlation between the physical gap size seen on VPI imaging. Given the ease of use and minimally invasive nature of these imaging studies, they remain a useful screening tool to identify patients who may require further evaluation of abnormal resonance to determine those patients who might require secondary speech surgery.

(1) University of Central Florida, Orlando, FL, (2) Nationwide Children’s Hospital, Columbus, OH, (3) East Carolina University, Greenville, NC

Background/Purpose: 22q11.2 deletion syndrome (22q11.2DS) is the most common genetic cause of velopharyngeal dysfunction (VPD) with a population prevalence of 1:2000 people. The primary muscle responsible for velar elevation during speech activities is the levator veli palatini (levator) muscle. MRI investigations on the velopharyngeal mechanism so far in the 22q population have focused on 2-dimensional measures and have not included information on 3-dimensional muscle shape and structure. No studies to date have the assessed muscle morphology, shape, and structure of the levator muscle in children with 22q11.2DS. The purpose of this study was to examine morphological variations of the levator muscle in children with 22q11.2DS in comparison to those in a nonsyndromic, noncleft cohort using MRI and advanced 3-dimensional computer technology.

Methods/Description: A total of 20 participants, 10 with a confirmed diagnosis of 22q11.2DS and 10 with normal velopharyngeal anatomy (age range: 4-12 years) completed the study. Children in the 22q11.2DS group were excluded if they had a history of prior palatal or velopharyngeal surgery. The control group was sex and age matched to the 22q11.2DS group to control for the effects of growth and sex on the variables. Participants were imaged using a high-resolution 3D anatomical scan called SPACE. All magnetic resonance images were transferred into Amira 6 Visualization Volume Modeling software. Quantitative and qualitative measures related to muscle volume and area were obtained. T-tests were conducted to assess differences between the 2 groups. A P value of < .05 was considered to be statistically significant.

Results: Preliminary results indicated significant variations among the 22q11.2DS group compared to the matched controls. The total volume of the levator muscle was found to be significantly smaller in the 22q11.2DS group compared to the control group. The levator muscle area was also found to be significantly smaller in the 22q11.2DS group compared to the control group. Significant differences were also noted for major and minor axes measures in the halfway point between origin and midline insertion within the velum. The 22q11.2DS group had greater variability across the measures, as demonstrated by larger standard deviations.

Conclusions: This study aimed to use a morphologic approach to provide 3-dimensional data on the levator muscle in children with and without 22q11.2DS. Preliminary results from this study support our hypothesis that children with 22q11.2DS present with significant differences in levator muscle morphology. Given that the levator muscle is the primary muscle responsible for velar elevation during speech, specific anatomical parameters of this muscle that shed insight on structural and functional features would be beneficial in understanding the complex underlying pathogenesis of VPD in 22q11.2DS.

(1) University of Miami Miller School of Medicine, Miami, FL, (2) UT Southwestern Medical Center, Dallas, TX, (3) Nicklaus Children’s Hospital, Miami, FL

Background/Purpose: Facial asymmetry caused by Vertical Orbital Dystopia (VOD) results in significant psychological distress and poor quality of life in affected patients. Traditionally, surgical correction of VOD has entailed a transcranial approach which includes standard frontal craniotomy, circumferential orbital osteotomy, vertical translocation of the orbit, and bone grafting to the lower maxilla. The traditional approach (TA) achieves the desired aesthetic result; however, caution has been expressed regarding the invasive nature of the technique and increased risk of complications. Paul Tessier described the Semi Open Skull (SOS) procedure for a simplified Le Fort III, wherein he made a burr hole at the top of the osteotomy to protect the dura. In this report, we describe a novel technique that is an extension of Tessier’s thinking elsewhere: the limited approach (LA) for simplified surgical correction of VOD.

Methods/Description: The LA differs from the TA in that it obviates the need for an invasive standard frontal craniotomy and entails a limited frontal craniotomy. A burr hole is made at the lateral edge of the osteotomy above the orbit prior to the osteotomy, and allows for adequate protection of the dura as bone is removed. The height of the section of bone that is to be removed is exactly equal to the amount of upward vertical movement of the orbit that is required for symmetry. A 45-year retrospective review was conducted of all patients who underwent surgical correction of VOD, as performed by a single surgeon between 1975 and 2020. Inclusion criteria for surgical correction of VOD included correction of a vertical discrepancy between the orbits via translocation of one or both orbits using a transcranial approach. Exclusion criteria included extracranial procedures such as bone grafting the floor of the orbit. Demographics, procedural characteristics, and complications were compared between 2 cohorts of patients: those who underwent the TA and those who underwent the LA for surgical correction of VOD. Complications were defined as infection of the frontal bone, permanent diplopia, permanent ptosis, and sudden-onset vision loss.

Results: Three-hundred and 2 patients were identified via retrospective review. Forty patients met inclusion criteria for correction of true VOD. There were 17 patients in the TA cohort and 23 patients in the LA cohort. Mean follow-up time was 3.0 ± 2.8 years (range: 0-10.1 years) for the TA and a mean of 4.0 ± 6.9 years (range: 0-32.3 years) for the LA. There were no complications reported for either approach.

Conclusions: The results of our retrospective review demonstrated no significant difference in complications between the TA and the LA. While both techniques are effective for surgical correction for VOD, the LA achieves the same result while reducing exposure of intracranial structures, potentially decreasing the risk of infection.

(1) University of Miami Miller School of Medicine, Miami, FL, (2) Nicklaus Children’s Hospital, Miami, FL

Background/Purpose: Craniofacial dermoid cysts are a common embryological deformity comprising 60% of all facial cysts. Their remarkably consistent distribution gives rise to the hypothesis that craniofacial dermoid cysts are developmentally related to craniofacial clefts. In 1976, Paul Tessier published his classification for craniofacial clefts based on anatomical and operative observations and experiences. Tessier cleft zones follow well-defined zones of the face and orbit, and correlate with embryological developmental fields. Embryonic developmental fields correspond to blocks of tissue that interact in a programmed fashion to produce the topology of the face. Tessier cleft zone 9 is complex, supplied by the confluence of 3 distinct developmental fields. During the embryonic fusion process, interposition of intracranial mesencephalic neural crest between developmental fields creates the conditions for dermoid cysts at specific anatomical locations. This theory is similar to theories underlying the embryological origins of craniofacial clefts, such as disorders in migration of neural crest cells or failures of embryological fusion processes. We hypothesize that lateral brow dermoids are a form fruste of Tessier No. 9 craniofacial clefts, and present a 20-year retrospective review evaluating the distribution of lateral brow dermoids along Tessier cleft zone 9.

Methods/Description: Patients who underwent dermoid cyst excision between January 1, 2000, to July 1, 2020, were reviewed retrospectively. Inclusion criteria included a diagnosis of lateral brow dermoid cyst, confirmed by pathology reports. Patients diagnosed with alternative types of cysts or nonlateral brow dermoid cysts were excluded. Photographs of patients were evaluated by 2 independent reviewers. The frequency of presentation of lateral brow dermoid cysts in different regions vertically along Tessier cleft zone 9 including the forehead (above brow and not in contact with the brow), suprabrow (directly above and in contact with the brow), subbrow (directly beneath and in contact with the brow), and infrabrow (beneath brow and not in contact with the brow) regions was evaluated, and proportions and percents were calculated and compared.

Results: In all, 176 patients were identified via retrospective review, and 65 patients met inclusion criteria. Of the lateral brow dermoid cysts (Tessier cleft zone 9), 12.3% of lateral brow dermoid cysts were located in the forehead region (8/65), 36.9% were located in the suprabrow region (24/65), 27.7% were located in the subbrow region (18/65), and 23.1% were located in the infrabrow region (15/65).

Conclusions: Lateral brow dermoid cysts follow a vertical distribution similar to the vertical orientation and location of Tessier cleft zone 9, presenting in various vertical locations including the forehead, suprabrow, subbrow and infrabrow regions. This finding supports the theory that there is a common embryological derivation between dermoid cysts and craniofacial clefts.

(1) Tufts University School of Medicine, Boston, MA, (2) Tufts Medical Center, Boston, MA

Background/Purpose: The Covid-19 pandemic has prompted an increase in use of telehealth visits across specialties. Here, we examine the strengths and weaknesses of a hybrid in-person and virtual multidisciplinary craniofacial team visit model following a 3 month trial period.

Methods/Description: Following reopening of an ambulatory, urban, Northeast, craniofacial clinic within an academic medical center, the decision was made to establish a hybrid in-person and telehealth model for subsequent team care. In this model, patients present in person for evaluation by an audiologist, otolaryngologist, and facial plastic surgeon. The remainder of the craniofacial team joins the visit virtually through the ZOOM platform. Standardized digital photographs of the face and dentition were provided to remote members to supplement live audio and video data from the in-person visit. After a 3 month trial of this hybrid format, survey data were collected from providers and patients. An anonymous, online survey was distributed to the multidisciplinary team; patient satisfaction surveys (administered by a nonaffiliated individual) were carried out over the phone. Questions were answered using 3 and 5-point Likert scales of agreement and quality.

Results: Patient ages ranged from 22 months to 39 years. Over 6 clinics, 24 patients were evaluated: pathologies included cleft lip and alveolus (3), complete cleft lip and palate (7), isolated cleft palate (9), and other craniofacial conditions (5). The response rate from 20 members of the craniofacial team was 65%. Results from 13 provider surveys showed that over 80% of providers felt that the hybrid visit format did not change their ability to obtain a history or formulate a treatment plan. Only 33% of providers felt that joining the visit virtually limited their ability to perform a specialty-specific exam. When asked if hybrid visits are an adequate alternative to in-person visits, 55% of responding providers agreed or strongly agreed. Response rate from 24 patient families was 56% (n = 13), 7 families were unable to be reached and 3 declined to participate. Of the 13 patient families who responded, 2 were at home for their team encounter (entirely virtual) and 11 presented in person for a hybrid visit. Comparing the hybrid telehealth visit to prior in-person encounters, 84% of responding families were neutral or positive toward the new system; 82% of surveyed families found the overall experience to be very good or excellent. 87.5% of families found the visit length to be excellent and 69% had confidence in the physical exam performed. When asked if they would use this format in the future, 60% agreed or strongly agreed.

Conclusions: With uncertainty surrounding the ability to return to traditional in-person cleft team visits, it is important to evaluate the potential benefits and shortcomings of telehealth interfaces for multidisciplinary craniofacial care and to identify potential ways to improve the patient and provider experience.

(1) The University of North Carolina at Chapel Hill, Chapel Hill, NC

Background/Purpose: Pressure-flow testing is an objective approach to assess velopharyngeal (VP) function during speech of individuals. Warren and colleagues reported extensive data, largely from adolescent and adult speakers, to suggest that VP areas less than 5 mm ² reflect adequate function while areas greater than 20 mm ² reflect inadequate function. It has been our experience, however, that some school-age children who present with clinically significant hypernasality and audible nasal air emission have VP areas well under 10 mm ² . This may be due to relative differences in vocal tract size between children and adults. The purpose of this study is to present preliminary data using a new speaker-centered aerodynamic metric that describes VP function of school-age children with repaired cleft palate.

Methods/Description: Sixteen children, 6 to 12 years of age (mean = 9.4 years), with repaired cleft palate with or without cleft lip underwent pressure-flow testing using a nasal mask approach. They produced series of /pi/, /bi/, and /mi/ syllables. VP area was calculated for the /p/, /b/, and /m/ segments. In addition, a palatal closure efficiency (PaCE) index was calculated for /p/ and /b/ based on each child’s VP area during /m/ using the formula: 100 – ([plosive area ÷ m area] × 100). This provided a speaker-centered percentage of VP closure relative to each child’s VP opening during the nasal consonant /m/. Seven graduate students used a 100 mm visual analog scale (VAS) to judge perceived hypernasality (1 = normal, 100 = severe) of audio recordings of the children counting from 1-10 and saying “papa plays baseball.”

Results: Mean VP areas for /p/ and /b/ were 9.8 mm ² (range 0-34.7) and 7.8 mm ² (range 0-31.1), respectively. Mean PaCE indices for /p/ and /b/ were 58% (range 0-100) and 65% (range 0-99), respectively. Mean perceived hypernasality for the speech sample was 42 mm (range 4-96). Pearson’s correlation coefficients were .828 (P = .001) between hypernasality and /p/ area; .718 (P = .002) between hypernasality and /b/ area; −.797 (P = .001) between hypernasality and PaCE /p/; and −.696 (P = .003) between hypernasality and PaCE /b/. Of 6 children who had VP areas less than 5 mm ² during /p/, PaCE indices ranged from 49% to 100% and perceived nasality ranged from 4 to 50 mm on the VAS.

Conclusions: The PaCE index is speaker-centered and, thus, a more meaningful way to report pressure-flow results to parents and clinicians. Our preliminary findings suggest that interpretation of VP closure as “adequate” using absolute area criteria may misrepresent some children who present with perceptually salient symptoms. Additional research is needed to determine the overall clinical utility of the PaCE index.

(1) College of Medicine, University of Lagos, Lagos, Nigeria, (2) University of Nigeria, Nsukka, Enugu, Nigeria, (3) University of Iowa/ Postdoctoral Research Fellow, Iowa, IA, (4) University of Iowa, Iowa City, IA, (5) University of PortHarcourt, PortHarcourt, Rivers, Nigeria, (6) University of Lagos, Lagos, Nigeria, (7) Department of Oral Pathology, Radiology and Medicine, College of Dentistry, University of Iowa, Iowa, IA

Background/Purpose: The etiology of orofacial cleft is considered to be multifactorial with polygenic, environmental, epigenetics, and interaction between genetics and environmental factors playing a role. There is no clear consensus on the effect of parental age regarding the risk of orofacial clefts though many studies have reported associations between advanced maternal or paternal age and risk of orofacial clefts. This study aims to investigate the relationship between paternal age, maternal age, and both on the severity of orofacial clefts.

Methods/Description: This was a retrospective study in which cases were subjects clinically diagnosed with nonsyndromic cleft lip and/or palate (CL/P). For the influence of parental age on cleft severity, the CL/P and isolated cleft palate were considered 2 separate populations because of their different embryological origins. Complete CL/P. In the CL ± P population, the data were grouped by the analysis of the influence of severity. For this purpose, the previously described subgroups were combined as follows: IC (incomplete/less severe clefts) vs CC (complete/ more severe clefts) as well as L vs R-sided cleft. The CP population comprised of: incomplete cleft palate (ICP) and complete cleft palate (CCP). The parental age was classified into young father, old father or young mother, old mother based on the median ages of the parents. The risk of orofacial clefts was analyzed based on these groups. Statistical analysis: For the primary analysis, a binary outcome variable was defined with 2 values (0 = IC, 1 = CC). Pearson’s χ ² test was applied to analyze the association between parental age and the severity of orofacial clefts. Based on logistic regression, the relative risk with confidence interval was calculated between severity of orofacial clefts and parental age.

Results: The total number of nonsyndromic orofacial cleft cases analyzed was 267: 202 CL ± P and 65 CP cases. Young fathers are categorized as <35 years while old fathers are ≥35 years old while young mothers are categorized as those >30 years while old mothers are ≥30 years old. There is no statistical significant association between type of CL ± P and parental age in young fathers (P = .93). When old fathers are considered, percentage of complete CL ± P cases increases especially in old mothers showing that risk of CL ± P is more associated with old father–old mother combination (P = .036). In old fathers, the risk of CL ± P is increased (OR: 2.66, CI: 1.04-6.80) and the risk is also increased for incomplete CL ± P (OR: 2.209, CI: 1.04-4.70). In old fathers, there is increased risk of developing right sided CL ± P (OR: 1.61, CI: 1.0-2.59). There is reduced risk of isolated cleft palate in young fathers (OR: 0.36, CI: 0.07-1.71) but the risk increases when considering complete cleft palates (OR: 1.63, CI: 0.71-3.7)

Conclusions: Risk of CL ± P is associated with increase fathers age but more with incomplete cases. The combination of old-father and old-mother is more associated with risk of OFC.

(1) CARe Medical College, Dhaka, Bangladesh

Background/Purpose: Lower midline facial cleft is a very rare craniofacial anomaly. This cleft was first described by Couronine in 1819 and since then less than 100 cases have been reported in literature with different severity. In 1976, Paul Tessier introduced the number system for craniofacial clefts, wherein the lower midline cleft was notified as no 30. This defect may present with varying degree of severity from isolated soft tissue of the lower lip to the tongue, alveolus, mandible, floor of the mouth, and neck structures. The anterior portion of the tongue may be bifid, which may be associated with ankyloglossia. Duplication of tongue, aglossia are also reported with lower midline cleft. Here, we want to share our experience.

Methods/Description: Design: Retrospective study with follow-up of 6 months to 5 years. Setting: Academic Hospital. Patients/Participants: 2 patients with lower mid-line facial cleft. Interventions: Lower mid-line facial cleft surgery. Main Outcome measures: Outcome measures were patients’ self satisfaction and comments of peers.

Results: Two primary lower mid line facial cleft operations were done in 1 male and 1 female patients. The age of the patients were 6 months and 37 years. One patient present with soft tissue cleft and other one with bifid tongue and ankyloglossia along with mid-line cleft of lower lip and mandible, Postoperative period was uneventful. No case of wound dehiscence or wound infection was observed.

Conclusions: Tessier 30 facial cleft and associated clinical anomalies are extremely rare congenital facial deformities. An early result of repair is encouraging. Long-term follow-up regarding mandibular development, dentition, and scar is to be observed.

(1) Montefiore Medical Center, Bronx, NY, (2) Princeton University, Princeton, NJ

Background/Purpose: Surgical procedures with loupe magnification, headlights, and microscopes expose surgeons to mechanical stress that can increase risk of long-term musculoskeletal pain and injury. Identifying the prevalence and cause of work-related musculoskeletal discomfort may guide preventative strategies to prolong well-being, job satisfaction, and greater duration of surgical careers. While surgical literature has addressed similar questions among other specialties, no group has evaluated ergonomics and work-related musculoskeletal injuries among craniofacial surgeons. In our study, we aimed to highlight the prevalence, incidence, and causes of work-related discomfort and injuries among craniofacial surgeons and help develop preventative strategies.

Methods/Description: After obtaining IRB approval, a 29-question online survey was distributed to the surgeon members of the American Cleft Palate-Craniofacial Association. In all, 873 surveys were distributed in total, and the anonymous responses were recorded using Google forms. Bivariate analysis was performed using Wilcoxon signed rank test with alpha set at .05.

Results: In all, 873 surgeons were surveyed with an initial response rate of 17% (n = 149). Eighty-seven surgeons (60.4%) reported having experienced musculoskeletal injury or symptoms during their craniofacial career. Of surgeons reporting musculoskeletal symptoms, 47 (53.4%) reported seeking medical treatment for and 85 (58.6%) reported self-treating their symptoms. Sixty-eight percent reported knowing someone with chronic work-related musculoskeletal discomfort, 53% reported knowing a colleague who underwent an operation for work-related musculoskeletal injury, and 28% reported a colleague on temporary or permanent disability for work-related musculoskeletal injuries. On a scale of 1-10, respondents endorsed significantly greater pain scores while using headlights (median pain = 3 [IQR: 2-5], P < .001), loupes (median pain = 3 [IQR: 3-5], P < .001), or operative microscope (median pain = 3 [IQR: 2-4], P = .01) as compared to operating without additional equipment (median pain = 2 [IQR: 2-3])

Conclusions: Craniofacial surgery is technically complex and often involves long procedures, use of adjuncts such as headlights and loupe magnification, and ergonomically straining surgical postures. Overtime, these can lead to musculoskeletal discomfort and injury. In our study, we found the majority of surgeons had experienced work-related musculoskeletal injuries, requiring a spectrum of medical and self-treatment. Significantly higher pain scores were reported with surgical adjuncts (loupes, headlights, and microscopes) than without. Over 50% of respondents knew a colleague who required treatment with surgical intervention, and over a quarter reported knowing someone on temporary or permanent disability from work-related injuries. While these are preliminary results, they point to an underreported and important phenomenon within craniofacial surgery, one meriting candid conversation and active preventative strategies.

(1) Hospital for Rehabilitation of Craniofacial Anomalies, University of Sao Paulo, Sao Paulo, Brazil, (2) University of Sao Paulo, Bauru, Sao Paulo, Brazil, (3) Department of Speech, Language and Hearing Disorders, Bauru School of Dentistry, University of Sao Paulo, Bauru, Sao Paulo

Background/Purpose: A pharyngeal bulb reduction program associated with intensive speech therapy (PBRP/IST) can increase the movement of the pharyngeal walls and therefore decrease velopharyngeal gap size in preparation to secondary surgery to correct velopharyngeal insufficiency (VPI). Purpose: To report the results of a PBRP/IST for a 19-year old girl presenting with VPI after surgical correction of cleft lip and palate.

Methods/Description: VPI was treated with a pharyngeal bulb prosthesis since the patient presented with a large velopharyngeal gap and poor movement of the pharyngeal walls during speech, compromising the success of a secondary surgery. Although she was able to achieve normal resonance with the prosthesis in place, she had a desire to undergo secondary surgery. The PBRP/IST lasted 3 weeks with a total of 35-daily speech therapy sessions of 45 minutes each. During this period, 2 bulb reductions were made under nasoendoscopy: the first reduction was made before the first therapy (5 mm reduction) and the second reduction was made after the 14th therapy (also a 5 mm reduction). Audio recordings were obtained before and after the bulb reductions and 3 experienced SLPs rated the recordings regarding presence or absence of hypernasality and analyzed the nasoendocopic images to estimate gap size.

Results: Before, after each reduction and after the last speech therapy session, the patient underwent perceptual speech assessment, nasoendoscopy, and nasometry, which results have shown the effect of PBRP/IST on velopharyngeal function leading the patient to remain with normal resonance using a much smaller bulb fulfilling a much smaller VP gap. Six months after the PBRP/IST the patient underwent a follow up with speech assessment, nasometry and nasoendoscopy, maintaining the results achieved.

Conclusions: After PBRP/IST, the patient achieved normal speech resonance with a pharyngeal bulb 3 times smaller fulfilling a much smaller VP gap. The patient became a better candidate for secondary surgery to correct VPI.

(1) Hospital for Rehabilitation of Craniofacial Anomalies, University of Sao Paulo, Sao Paulo, Brazil, (2) University of Sao Paulo, Bauru, Sao Paulo, Brazil, (3) Department of Speech, Language and Hearing Disorders, Bauru School of Dentistry, University of Sao Paulo, Bauru, Sao Paulo

Background/Purpose: Glottal stop (GS) is one of the most common type of atypical place of articulation used by individuals with operated cleft palate. GS is thought to occur as consequence of oral-nasal coupling, becoming incorporated into the child’s developing speech system. While GS can be particularly resistant to speech therapy, the selection of an adequate therapeutic approach to correct place of sound production along with the frequency of speech therapy might also be an important aspect related to the success of the intervention. As described in literature, a structured intensive speech therapy program meets the facilitating conditions for the correction of atypical place of articulation related to oral-nasal coupling. The purpose of this study is to investigate the influence of an intensive speech therapy program to correct GS in the speech of individuals with cleft palate.

Methods/Description: 37 Brazilian Portuguese speakers (21 males and 16 females, mean age 19 years) with operated cleft palate (± cleft lip) were selected as participants for this study. Due to velopharyngeal insufficiency (VPI) and hypodynamic velopharynx (HVP) a pharyngeal bulb (PB) combined with an intensive speech therapy program (IST) was indicated for all participants. With the PB in place all 37 participants received 35 sessions of IST over a period of 3 weeks (3 sessions daily). The therapy target was the correction of atypical place of articulation, particularly glottal stop (GS). Pre and post IST audio recordings were rated by 3 speech/language pathologists regarding presence or absence of GS. The speech samples were recorded during production of 6 sentences with plosive sounds of Brazilian Portuguese language.

Results: After IST, 5 (14%) participants corrected GS productions in all target consonants; 27 (75%) presented a significant reduction in the use of GS; 4 (11%) did not present any change. A significant change in the use of GS was observed after the IST program for the consonants “p, t, k, d” (Mc Nemar P < .05).

Conclusions: Intensive speech therapy program is an effective method to correct or reduce the occurrence of GS articulation in the speech of individuals with VPI+HVP.

(1) All India Institute of Medical Sciences, Rishikesh, India

Background/Purpose: A normal looking median tubercle is very important for the aesthetics of upper lip. But median tubercle reconstruction has not been highlighted in the usual techniques of cleft lip repair. Thus we adopted this modified bilateral cleft lip repair technique of Murthy which utilizes the prolabial tissue to form the median tubercle. The purpose of this study was to compare and analyze the anthropometric and 3-dimensional reconstruction outcomes of labial contour of the bilateral cleft lip repairs done by this new technique and those done by Mulliken technique.

Methods/Description: Children who underwent bilateral cleft lip repair by Mulliken or Murthy technique between March 2017 and October 2018 with 2 years follow-up were included. Anthropometric measurements were taken by a paper ruler. Three-dimensional scanning was done by Artec 3D scanner. Measurement was performed using Geomagic freeform software. The outcome parameters analyzed were labial volume, labial surface area, midline labial height, and lateral labial height.

Results: Twenty-six children were included: 15 repaired by Mulliken technique, 11 repaired by Murthy technique. Mean age of repair was 6.2 ± 1.3 months. Postoperative measurements were taken at 2 years follow-up. The groups were age matched. There was significant increase in labial volume (17.2 percent, P = .05) and labial surface area (12.5 percent, P = .05) by the Murthy technique in comparison to Mulliken technique.

Conclusions: The Murthy technique is a simple but elegant modification of bilateral lip repair which significantly increases the contour of the median tubercle.

(1) Montefiore Medical Center, Bronx, NY, (2) Jacobi Medical Center, Bronx, NY

Background/Purpose: Given anatomic proximity and common trauma etiologies, maxillofacial trauma can be associated with concomitant injuries to the cervical spine. The pediatric and adult facial skeleton differ in important ways, and while bony craniofacial trauma is less common among pediatric patients, it still causes significant morbidity and mortality. Contemporary adult literature describes epidemiology, fracture patterns, and factors associated with maxillofacial trauma and concomitant cervical injuries; however, to date, there have been no large series studies determining the same in the pediatric population. In this study, we use the National Trauma Data Bank (NTDB) to assess the incidence, risk factors, and outcomes of concomitant maxillofacial and cervical fractures within the pediatric population.

Methods/Description: Using the NTDB from 2007 to 2017, pediatric patients (<18 years) suffering from facial fractures were identified based on ICD 9 and ICD 10 diagnostic codes. Demographics, injury characteristics including presence of concomitant cervical fractures (CCF), and outcomes were analyzed using 2-tailed t test and multivariate binary logistic regression.

Results: In all, 8 726 496 records from the 2007 to 2017 NTDB were analyzed to identify 32 952 pediatric patients who had experienced isolated facial bony trauma, with 2695 of these suffering CCF. Of all, 83.4% of the CCF patients were aged 13 to 18 (P < .0001), 61.8% were male (P < .0001), 62.6% had suffered poly facial trauma, and overall had a higher median injury severity score (16-24 vs 1-8; P < .0001). Statistically significant differences were noted in mechanism of injury, and hospital complications—deep vein thrombosis, pulmonary embolism, unplanned intubation, and unplanned return to OR being more common in patients that had CCF (P < .0001); 6.8% of patients with mandible fractures had associated cervical fractures. Regression analyses showed nasal, maxillary, and poly facial trauma patients had higher odds ratios of having CCF when compared to mandible fractures. CCF patients had longer total length of stay compared to patients with facial fractures only (mean, [SD]) (9.4, [10.4] vs 3.6, [5.7]) (P < .0001), had higher rehab needs (27.1% vs 7%), and were more likely to have all-cause mortality (4.4% vs 0.9%; P < .0001).

Conclusions: Differences exist between pediatric and adult facial trauma patients, including the incidence of CCF. Specifically, our study found mandible fractures in the pediatric population had a lower association with CCF when compared to rates commonly cited from adult literature. We also found age, gender, injury severity score, facial region, and mechanism of injury to be associated with differences between pediatric patients with facial bony trauma only compared to those with CCF. CCF patients had higher rates of complication and worse outcomes, indicating a higher disease burden. Overall, the pediatric facial trauma patient is at risk for CCF, and clinicians should tailor management accordingly to minimize morbidity and mortality.

(1) Case Western Reserve University School of Medicine, Cleveland, OH, (2) University Hospitals, Case Western Reserve University, Cleveland, OH

Background/Purpose: Otological procedures are key components of cleft lip and palate management. However, the overall otological burden and utilization of these procedures in this patient population remain poorly understood. The purpose of this study was to examine national trends in patient utilization and hospitalization charges associated with otological procedures for cleft lip and palate patients over multiple years.

Methods/Description: The Healthcare Cost and Utilization Project National Inpatient Sample database was analyzed from January 2007 to December 2014. All patients who were diagnosed with cleft lip or palate who underwent otological procedures were included. Variables of interest included demographic data, hospital characteristics, hospitalization data, and total hospital charges. All charges were inflated to May 2019 value with the consumer price index. Univariate and generalized linear models were used to examine associations between various factors of interest and the final adjusted hospitalization charge as well as the change in these factors over the multiple years included.

Results: Of al, 62 471 patients were diagnosed cleft lip or palate during hospitalization between January 2007 and December 2014. Of these patients, 4178 (6.7%) received an otological procedure, including myringotomy (3479, 83.3%), auditory function testing (447, 10.7%), and tonsillectomy (110, 2.6%) and were included in the study. Regression analysis showed that total procedure volume did not change significantly over the years examined (P = .06). Higher procedure volume (>30 cases/year) was associated with large bedsize hospitals (P < .001), nonprofit private hospitals (P < .001), and urban teaching hospitals (P < .001). The mean total hospital charge for these patients was $28 108.87 (interquartile range: $12 529.55-$32 854.57). Hospitalization charges also increased significantly in this time period (P < .001), in conjunction with an average hospital length of stay of 1.91 days that did not change significantly during the study period (P = .149).

Conclusions: Otological procedures for patients with cleft lip and palate appear to be utilized mainly at larger, urban teaching and nonprofit private hospitals. While hospital length of stay and procedure volume appears be stable across the years studied, hospitalization charges associated with the procedures rose significantly. Further studies are necessary to examine other factors that could be driving the increased hospitalization charges to better target strategies for reducing health care burden and improve barriers to access to these important procedures for cleft lip and palate management.

(1) Spectrum Health Medical Group, Grand Rapids, MI, (2) Spectrum Health, Grand Rapids, MI, (3) Spectrum Health/Michigan State University, Grand Rapids, MI

Background/Purpose: Children born with cleft lip and palate need orthodontics to achieve the best dental facial relationship. However, some patients are highly sensitive to oral stimulation. Patients with autism for example may struggle with the oral sensory stimulation that accompanies traditional metal orthodontic braces. Children with sensory issues have torn their braces off, others could not tolerate having braces put on. As a result, many children with sensory issues receive no treatment at all and the final result is always less than satisfactory. We have found that through the use of Invisalign orthodontics, children born with a cleft lip and/or palate with increased sensitivity to oral stimulation can often achieve a successful orthodontic result. Invisalign therapy appears to be a good alternative for children whose oral facial sensitivity or medical conditions preclude the use of traditional metal orthodontics. Purpose: To demonstrate the effective use of nonmetallic orthodontic treatment for children who cannot tolerate traditional metal braces.

Methods/Description: Early case results using nonmetallic orthodontic treatment will be reviewed. Parental perception regarding the overall results of their child’s experience with Invisalign orthodontic treatment will be discussed as well. Additionally, a case will be reviewed for demonstration purposes.

Results: Invisalign orthodontics successfully aligned the dental arches in the 4 patients who underwent the treatment. There were no complications. Patients and families were happy with the results

Conclusions: Using Invisalign orthodontics can effectively treat the complex dental issues seen in the cleft lip and palate defects. Patients with oral aversion issues now have an opportunity to receive an alternative treatment avoiding standard metal braces while still achieving best results.

(1) Cleft Palate Center University Hospital Ruzinov; Comenius University, Bratislava, Slovakia, (2) Comenius University, Bratislava, Slovakia

Background/Purpose: This study compares application of “Real Time Listening” (RTL) to judgements of parents, RTL+ modification, and simple phonetic transcription (SPHT) for evaluation of early phonetic repertoires in 10 Slovak cleft children at age 16 to 34 months. It verifies 2 aspects of the underlying theory: (1) RTL will evaluate phonetic repertoires of children similarly as their parents and (2) Parents will be able to inform about the key phonetic and language features and by that to detect the need for early intervention (EI) for their children.

Methods/Description: Videotapes of spontaneous speech during natural play with mothers were collected for each child. Parents completed questionnaire (PQ) about key features of child’s speech and language. During the videotaping SLT performed in-the-session simple phonetic transcription (ISSPHT) of child’s productions, and after the recoding 2 SLT-s performed RTL, RTL+, and simple phonetic transcription (SPHT) of all video-samples. Each sample was retranscribed at least 2 weeks later and after transcribing at least 3 other samples to ensure that coders would not remember the same child. From each sample/child we obtained 8 phonetic repertoires and these were compared and analyzed for: (1) consonants in the repertoire; (2) absence of oral pressure sensitive sounds/consonants; (3) absence of alveolar sounds/consonants; (4) presence of compensatory and (5) palatal sounds; (6) stage of language development; and (7) need for early intervention.

Results: Results show that parent judgements were more similar to RTL+ and SPHT than to RTL but agreements with SLT judgments were substantial for all 3 methods (ranging from 74%-78%). Parent reports led to correct decision about the need of early intervention in 70% of children however only 3 out of 10 parents judged all crucial speech and language parameters similarly to SLT. Majority of parents (7/10) had difficulties to correctly report about compensatory sounds. Inter-coder agreement in all 3 methods between 2 coders was high: ranging from 90% to 96% when coding consonant types, and 80% to 100% when coding symptoms of cleft palate speech.

Conclusions: RTL, RTL+ but also SPHT were shown to be useful and reliable methods of evaluation of early phonetic repertoires of cleft children. Our results show that RTL+ and SPHT are somewhat better methods for evaluation of symptoms of cleft palate speech and emerging consonants which are parameters important for decision making of SLT-s in early intervention. The second important result is that parents are able to identify a need for early intervention in their children but they have difficulties in identifying compensatory and palatal sounds.

(1) Spectrum Health Medical Group, Grand Rapids, MI, (2) Spectrum Health, Grand Rapids, MI, (3) Spectrum Health/Michigan State University, Grand Rapids, MI

Background/Purpose: The Double Opposing Buccal Flap Procedure for Palatal Lengthening (DOBFP) has become a proven alternative to the nonphysiologic pharyngeal flap (PF) and sphincteroplasty (SP). Traditional PF and SP procedures tether the velum specifically, the levator veli palatini, rendering it essentially nonfunctional. Alternatively, the DOBFP maintains a functional levator palatini and the natural velar unit remains as the closing mechanism for the nasal pharyngeal space. While the DOBFP lengthens the anterior portion of the velum in patients with wider velar gaps, the lengthened palate invariably falls more inferiorly with each millimeter gained. As this progression continues the point of velar contact on the posterior pharyngeal wall also falls progressively downward and the closure may become less competent. Velar contact position is unique to each individual. The DOBFP can predictably lengthen the palate while maintaining a close to normal closure mechanism. However included in the missing tissue inherent in cleft defects is the musculus uvulae. It functions to raise the palate so it may contact at the proper level on the posterior pharyngeal wall. Without the musculus uvula, the closure can occur to low, leading to dysfunctional closure. In present day, medicine palate muscles are irreplaceable. This is one reason why some patients with wider nasal pharyngeal gaps fail to achieve effective closure with the DOBFP alone. The Functional Palate Suspension (FPS) expands the treatable velar gap range of the DOBFP. The FPS raises the palate upward to a more superior contact point on the posterior wall without damaging or tethering the natural closing mechanism. Purpose: To give caregivers more and safer ways to treat velopharyngeal dysfunction with techniques that maintain natural closure mechanisms. Reducing the complications associated with compensatory secondary speech surgeries such as PF or SP.

Methods/Description: The speech results and complications of 17 patients were retrospectively reviewed. The occurrence of obstructive sleep apnea (OSA) symptoms was also reviewed.

Results: Of the 17 patients who underwent the FPS, the complication rate was 0% for syndromic (N = 6) and nonsyndromic (N = 11) patient populations. The development of normal speech status post FPS was 66.7% for syndromic patients and 90.9% for nonsyndromic patients. There were no reports of OSA among either patient population.

Conclusions: The FPS in concert with the DOPBFP is an effective alternative reconstructive option to treat VPD. The FPS achieves velar closure by placing the natural levator sling at the appropriate point of closure without narrowing the nasopharyngeal space. This approach falls within the Anatomic Cleft Restoration Philosophy.

(1) Mayo Clinic, Rochester, MN

Background/Purpose: As each are relatively rare, the co-occurrence of childhood apraxia of speech (CAS) and velopharyngeal insufficiency (VPI) is truly exceptional and poses unique challenges to speech-language pathologists (SLPs) and otolaryngologists (ENTs) in the treatment of these children. CAS is a deficit of speech motor control and impacts timing of articulatory movements, including those of the velum. Such mistiming may cause children with CAS to exhibit intermittent or inconsistent hypernasality and aberrant closure of the velopharyngeal port during speech. Other features of CAS in the setting of VPI are restricted phonemic inventories that consist primarily of nasal sounds /m, n/ and substitution of nasal for nonnasal sounds (Baas et al, 2015). Co-occurring childhood CAS and VPI may occur in children with genetic phenotypes that include craniofacial anomalies and motor speech disorders. Baylis and Shriberg (2019) reported that 82% of children with 22q11.2 deletion syndrome demonstrated motor speech disorders (MSDs), including speech motor delay, childhood dysarthria, CAS, and some children with both childhood dysarthria and CAS. Although speech treatments for CAS and VPI as separate speech disorders are well-described in the literature, very little is known about treatment for children with concomitant CAS and VPI. The purpose of the current project is to report long-term outcomes of children with concomitant CAS and VPI.

Methods/Description: A retrospective review of multidisciplinary VPI clinic records identified 34 records of children who were diagnosed with both CAS and VPI at Mayo Clinic Rochester. Inclusion criteria included evaluation by SLP and ENT, surgical intervention, and at least one follow-up visit. The records were reviewed to extract communication and medical diagnoses, description of CAS and other communication disorders, velopharyngeal structure and function pre and post intervention, instrumental and perceptional measures of resonance pre and post intervention, type of surgical management, description of speech therapy, and length of follow-up.

Results: All patients had a diagnosis of CAS, with many children exhibiting other communication disorders. Velopharyngeal insufficiency was determined by results of nasendoscopy. Nasometry measures were completed. All children received surgical treatment for their VPI. Improved velopharyngeal function and/or improved speech and resonance were reported in all cases.

Conclusions: This retrospective review of patients with co-occurring VPI and CAS identified a subset of children whose speech sound production was meaningfully enhanced by management of their VPI. The success of the surgical intervention for VPI may have been facilitated by intensive speech therapy. We conclude that intensive speech therapy and ongoing reassessment of motor speech and articulation skills, and systematic reevaluation of velopharyngeal function is needed for children with CAS and VPI to ensure timely modifications to surgical and speech therapy plans.

(1) McGovern Medical School at The University of Texas Health Science Center at Houston (UTHealth), Houston, TX

Background/Purpose: Since the beginning of the COVID-19 pandemic in early March, there has been a push to expand virtual patient care visits instead of in-person clinic visits. Studies have found that telemedicine can provide efficient triaging, reduction in emergency room visits, and conservation of health care resources and personnel. While virtual patient care has been implicated in providing similar outcomes to traditional face-to-face care, there are a lack of studies on the effectiveness of virtual care visits for patients with abnormal head shapes. This study aims to develop an understanding of whether physicians can accurately diagnose pediatric patients with craniosynostosis or deformational plagiocephaly via virtual care visits, and whether they can determine if affected patients will benefit from helmet correction or if surgical treatment is required.

Methods/Description: An IRB-approved retrospective chart analysis over a 4-month period (March 1, 2020, to June 30, 2020) was performed analyzing all pediatric patients (<18 years old) who underwent virtual care calls for diagnosis and treatment of abnormal head shape. Patients were referred to UTPhysicians Pediatric Surgery clinic for evaluation by a member of the Texas Cleft-Craniofacial Team (2 surgeons or 1 physician’s assistant). Variables such as patient demographics, diagnosis, and need for confirmation were recorded.

Results: Thirty-five patients were identified who fit our search criteria. Out of these patients, 11 (31.43%) cases were diagnosed with craniosynostosis and 22 (62.86%) cases were diagnosed with deformational plagiocephaly. Median age at virtual care evaluation was 14.10 months (IQR 5.729, 27.542) for patients diagnosed with craniosynostosis and 6.51 months (IQR 4.669, 7.068) for patients diagnosed with deformational plagiocephaly. All 11 (100%) patients diagnosed with craniosynostosis were referred for a confirmatory CT scan prior to undergoing surgical intervention and saw an alleviation in head shape postoperatively. Eighteen (81.82%) of patients diagnosed with deformational plagiocephaly were recommended to undergo conservative treatment and the remaining 4 (18.18%) were recommended for helmet therapy. Two cases (5.71%) were unable to be diagnosed virtually. These patients needed a follow-up visit in person to establish a diagnosis and plan of treatment.

Conclusions: Virtual care visits are increasing in frequency and this includes consultations for abnormal head shapes. Our study underscores the feasibility of virtually diagnosing and recommending a plan for treatment in pediatric patients with abnormal head shapes, with only 5.71% requiring additional treatment or in-person visits to confirm the diagnosis. This information can be implemented to further our knowledge on the accuracy of diagnosis and treatment options for patients with craniosynostosis and deformational plagiocephaly. Further analyses are needed to quantify the financial and patient-reported outcomes of virtual care visits for these patients.

(1) UT Southwestern Medical Center, Dallas, TX, (2) Analytical Imaging and Modeling Center, Children’s Health, Dallas, TX, (3) University of Texas at Dallas, Richardson, TX, (4) UT Southwestern School of Medicine, Dallas, TX

Background/Purpose: The most common types of nonsyndromic craniosynostosis in infants are sagittal, metopic, and unicoronal. These forms of craniosynostosis have appreciable features that have predominantly been diagnosed using clinician physical exams and CT scans. Although effective, not all medical providers have adequate training on categorizing craniosynostosis disorders, and CT scans are expensive and require radiation exposure. Deep learning, a subtype of machine learning artificial intelligence, takes advantage of the machine’s innate computing power to self-extract features and learn novel algorithms without requiring taxing manual feature extraction by humans. In this report, we use deep learning convolutional neural networks (CNN) to provide an objective and generalizable method to diagnose and classify craniosynostosis in infants from 2D photographs.

Methods/Description: We created an annotated data set of labeled 2D photographs (normal, metopic, sagittal, and unicoronal) using standard clinical photography from the image repository at our center. We extended this data set set by adding photographic images of children with craniofacial conditions from the internet. A total of 1076 images were used in this study. We developed a deep learning CNN model using a pre-trained ResNet-50 model to classify the data as normal, metopic, sagittal, or unicoronal. Front and top view photographic images were used to train and test the model. Training and testing accuracies were obtained.

Results: The testing accuracy for the craniosynostosis ResNet50 model achieved an overall accuracy of 90.6%. The sensitivity and precision were 100% and 100% for metopic, 93.3% and 100% for sagittal, and 66.7% and 100% for unicoronal, respectively.

Conclusions: Our deep learning CNN model resulted in high prediction capability for correctly classifying common craniosynostosis disorders, outperforming the current state-of-the-art model for craniosynostosis classification. The results suggest that this model might be clinically useful for the objective diagnosis and differentiation of craniosynostosis and could be deployed as an aid to practicing primary care providers that do not have specialized training in craniofacial deformity.

(1) Spectrum Health Medical Group, Grand Rapids, MI, (2) Spectrum Health, Michigan State University, Grand Rapids, MI

Background/Purpose: The Anatomic Cleft Restoration Philosophy outlines that a complete restoration is only possible when all missing tissues are effectively replaced. In present day medicine, it is not possible to replace all tissue types. Palate muscles are not replaceable, they must be protected at all costs. Cleft palate malformations vary greatly. Some children present with very deficient and hypo plastic velar muscles. Hypo plastic soft palate muscles are small and often severely displaced anteriorly into the maxilla. This patient subgroup presents a reconstructive surgeon with difficult challenges. Scarring is a given with every dissection. Small, delicate muscles are particularly susceptible to scarring. Even moderate scarring can render them immobile. Velar muscle immobility can lead to lifetime vocal cripple. Patients with scarred soft palates are often treated with compromise reconstructions. The normal nasal pharyngeal closure driven by the fast twitch velar muscles is replaced by a compromise reconstruction driven by slow twitch swallow muscles. The complications associated with pharyngeal flaps (PF) and/or sphincteroplasties (SP) and other compromise operations are well documented. The No Touch Procedure is designed to protect the muscles. By minimizing scarring near the Velar muscles normal velar muscle function can be maintained, and a complete palate restoration can be achieved. Purpose: To present an alternative treatment option for patients born with small and/or anteriorly displaced velar muscles.

Methods/Description: The No Touch Cleft Palate Repair improves speech outcomes for patients born with small and/or severely displaced velar muscles. Additionally, the No Touch repair has low complication rate. A retrospective review of 23 patients born with small velar muscles treated with the No Touch technique was conducted. Postoperative speech results and complications were analyzed.

Results: By utilizing the No Touch Repair, this anatomic subgroup can achieve excellent speech results and low complication rates as demonstrated in this presentation containing 31 years of patient data. The No Touch Repair achieved a 0% complicate rate for both nonsyndromic and syndromic patient populations. The development of normal speech was 100% for nonsyndromic patients and 66.7% for syndromic patients. Additionally, no symptoms of obstructive sleep apnea were reported by either patient population.

Conclusions: The No Touch Palate Repair achieves successful velar function with less radical velar muscle dissection than traditional repairs. Traditional repairs typically dissect around the velar muscles either partially or completely. Most children with abundant muscles can tolerate this scarring. Children born with small muscles cannot tolerate this degree of scaring which can lead to reduced velar movement and permanent vocal compromise. By moving the velum as a unit, the No Touch Repair reduces scarring around the delicate muscles. Additionally, by replacing missing palate tissue, the velar sling is position.

(1) University of Iowa, Iowa, IA, (2) University of Iowa, Postdoctoral Research Fellow, Iowa, IA, (3) University of Iowa College of Public Health, Department of Biostatistics, Iowa, IA, (4) University of Iowa College of Dentistry, Iowa, IA, (5) Department of Orthodontics University of Dundee, Dundee, Scotland, (6) Addis Ababa University, Addis Ababa, Ethiopia, (7) College of Medicine University of Lagos, Lagos, Nigeria, (8) KwaZulu-Natal University, KwaZulu-Natal, Durban, South Africa, (9) NIH, Bethesda, DC, (10) University of Ferrara, Ferrara, Italy, (11) Kwame Nkurumah University of Science and Technology, Kumasi, Ghana, (12) Iowa Institute of Oral Health Research, Iowa, IA, (13) Knust-Kath, Kumasi, Ashanti, Ghana (14) University of Pittsburgh, Pittsburgh, PA, (15) Department of Oral Pathology, Radiology and Medicine, College of Dentistry, University of Iowa, Iowa, IA

Background/Purpose: Non syndromic orofacial clefts (NSOFCs) are the most common type of orofacial clefts accounting for 70% of cleft lip with or without palate (CL±P) and 50% of cleft palate only (CPO). The etiology of NSOFCs is polygenic and multifactorial, involving complex interaction between genetic and environmental factors. To date, over 60 risk loci have been identified through GWAS of common variants. However, these common variants only provide modest explanation for the heritability of NSOFCs. In order to identify the missing heritability for NSOFCs, we investigated the role of low frequency and rare coding variants in our African only GWAS data.

Methods/Description: Variants with MAF < 5% were genotyped in 1000 cases and 898 unrelated controls from Ghana, Ethiopia, and Nigeria. Of the cases, 799 had a cleft lip with or without palate (CL±P) and 201 had a cleft palate only (CPO). We conducted a Single-variant association tests using logistic regression while adjusting for population structure (the first 28 vectors of the genotypes).

Results: We observed a suggestive significant association with rs149899012 (MTHFD1, Pcombined = 2.09e-05, PCL/P = 1.35e-04) and rs45602837 (HNF4A Pcombined = 2.77e-05, PCL/P = 4.54e-05) for all NSOFCs combined and with CL±P. A near significant association with rs74318559 (MFSD6 L, PCP = 1.83e-06), rs141126223 (GRAP2, PCP = 4.09e-06), and a suggestive significant association with rs17612861 (DNAH9, PCP = 1.28e-04) and rs566233643 (KRT34, PCP = 1.32e-04) in the CPO group. Furthermore, gene set analysis and burden tests are ongoing, and the results will be presented when completed.

Conclusions: The MTHFD1 is a widely studied cleft candidate gene involved in the folic acid metabolic pathway. HNF4A is a transcription factor implicated in diabetes mellitus. KRT34 and GRAP2 are involved in keratin and dental anomalies; respectively. The results from these preliminary analyses suggest important roles for low frequency variants in the identification of candidate genes for NSOFCs.

(1) Childrens Mercy Hospital, Kansas City, MO

Background/Purpose: Sagittal craniosynostosis has some variability in presentation. Some patients appear to be affected more anteriorly, while others are more affected posteriorly. Others are quite affected bidirectionally. The aims of this article are to describe a subtype of sagittal craniosynostosis which presents with a retrocoronal temporo-parietal transverse band-like constriction, to demonstrate enhanced anterior cranial vault dysmorphology in these patients, and to elucidate reconstructive solutions for this problem.

Methods/Description: For the years 2015 to 2018, 51 patients with nonsyndromic single-suture sagittal craniosynostosis were operated upon by the authors. All patients were followed in a multidisciplinary craniofacial program including genetics. Patients routinely underwent a preoperative CT scan and pre- and postoperative photographs. A retrospective chart review captured the results described below.

Results: Four of these patients had severe retrocoronal transverse bilateral temporoparietal band-like constrictions. Four more patients were affected moderately. These patients all had a worsening of their anterior cranial vault deformity including enhanced bitemporal narrowing and increased frontal bossing. Of the 4 severe deformities, 3 underwent a subtotal cranial vault remodeling at a mean age of 9 months (range, 8-12 months). These 3 patients had overcorrection of the transverse expansion intraoperatively as well as subtotal resection of the greater wing of the sphenoid bone. The fourth patient underwent strip craniectomy at 3 months of age with widened lateral wedge ostectomies taken anteriorly, followed by helmeting. Mean follow-up was 23 months (range, 13-37 months). All 4 had excellent results with 2 residual mild bitemporal constrictions. The 4 moderate deformities all did well with 2 treated with cranial vault remodeling both at 8 months of age, and 2 treated with strip craniectomy and postoperative helmeting at a mean age of 2.5 months. One of these patients had mild frontal bossing and mild bitemporal constriction at 16 months postoperatively. There were no perioperative complications in these 8 patients.

Conclusions: Sagittal craniosynostosis patients with a significant retrocoronal transverse temporoparietal transverse band-like constriction have a worsened anterior dysmorphology. Principles of operative correction should include overexpansion transversely and takedown of the greater wing of the sphenoid bone bilaterally when done as an open procedure. Surgical follow-up of almost 2 years in the more severe patients demonstrates satisfactory outcomes. When a strip craniectomy with bilateral wedge ostectomies and postoperative helmeting is performed, the width of the anterior wedge ostectomy should be increased.

(1) Hospital for Rehabilitation of Craniofacial Anomalies, University of Sao Paulo, Bauru, Sao Paulo, Brazil

Background/Purpose: Treacher Collins syndrome (TCS) is a congenital condition in which the main clinical features are zygomatic and mandibular hypoplasia associated or not with a cleft palate. A hyperdivergent skeletal pattern and a class II malocclusion are frequently observed as well. It has been shown that TCS negatively impacts sleep and breathing in this population. However, little is known about the impact of this craniofacial anomaly on mastication. The study aimed at evaluating the masticatory function of individuals with TCS through bite force and masticatory efficiency assessment. Additionally, the present study evaluated the nutritional status of this population through anthropometric measurements.

Methods/Description: The IRB approved this study. Forty-one individuals, divided into 2 groups, were evaluated: (1) Control Group (CON) (24 ± 8 years of age): 20 nonaffected adult individuals with class I skeletal pattern, (2) Treacher Collins Syndrome Group (TCS) (25 ± 9 years of age): 21 adult individuals with TCS. The masticatory function was assessed using 2 different methods: (1) Isometric bite force measurement using a gnathodynamometer (IDDK Kratos, Brasil). Measurements were taken in triplicate, bilaterally at the area of the molars, and the highest value (expressed in N) was considered for analysis, and (2) masticatory efficiency, by calculating the size of food particles after 20 standardized masticatory cycles (Image J—National Institutes of Health—NIH) using a standard piece of raw carrot, followed by the bolus’s disposal in a sterile falcon tube. After lightly drying the particles with an absorbent paper, it was placed in a transparent plastic pellicule and then scanned in a high-resolution table scanner (HP Deskjet Ink Advantage2540 all-in-one). The obtained images were transformed into binary figures, and the area of the particles was measured using Image J® Software (NIH open source software: ImageJ website, http://imagej.nih.gov/ij/). Nutritional status was assessed through body mass index (BMI). P ≤ 0.05 were considered significant.

Results: Mean values of BF for the CON and the TCS groups corresponded to 431.6 ± 134.1 N and 170.9 ± 109.4 N (right molar), and 427.4 ± 147.8 N and 171.2 ± 93.9 N (left molars), respectively. These differences were statistically significant. The mean particle size for the CON and TCS groups corresponded to 0.66 ± 0.52 mm ² and 1.58 ± 1.93 mm ² . Although more than 2 times greater for the TCS group, this difference was not considered statistically significant. A statistically similar BMI was observed in both groups and corresponded to 23.4 ± 4.7 (CON) and 23.4 ± 6.1 (TCS). Underweight individuals were only observed in the TCS group (24%). The 2 groups did not differ significantly by age.

(1) University of Wisconsin, Madison, WI

Background/Purpose: No consensus exists regarding the optimal methods or indications for measuring intracranial pressure (ICP) in children with craniosynostosis. Methods of ICP monitoring can range from clinical assessments to invasive measurement devices. The purpose of this literature review is to investigate the published methods and protocols for evaluating ICP, with particular emphasis on the pediatric craniosynostosis patient population.

Methods/Description: The PubMed database was searched for all studies involving pre-, intra- and postoperative measurement of ICP in patients with craniosynostosis published between 1960 and June 2020. Only English-language studies were included. Extracted data included patient population, study methodology, cohort size, and method(s) of ICP monitoring.

Results: A total of 108 studies met inclusion criteria for ICP monitoring in craniosynostosis patients. In all, 78% of these studies reported only on patients with craniosynostosis, while 17% included other etiologies of elevated intracranial pressure and 5% evaluated complications of ICP monitoring. Of the 108 studies identified, 27 (25%) were reviews, 10 (9%) were case reports, 68 (63%) were case series, and only 1 (1%) case–control study was identified. Two studies evaluated the use of novel technologies. Of the case series, only 27 studies included over 50 patients. The most commonly reported method of ICP monitoring was evaluation for papilledema in 18.5% of studies, followed by radiographic imaging (15.7%). Invasive methods of testing were evaluated in 22.2% of studies, including intraparenchymal devices (13.0%), subdural devices (7.4%), and epidural devices (1.9%). Noninvasive testing included visual evoked potentials (VEP) (7.4%), telemetric monitoring (3.7%), optical coherence tomography (OCT) (1.9%), and transcranial Doppler (1.9%). Other studies evaluated novel technologies, reviewed methods, or used multiple methods (23%) within their case series or reviews.

Conclusions: There are few published studies on ICP monitoring for pediatric craniosynostosis patients with high level evidence. Invasive methods such as intraparenchymal or subdural devices are considered to be most accurate, although more common noninvasive methods such as evaluation for papilledema, radiographic imaging studies, VEP, and OCT may be used. Recent innovations in telemetric and transcutaneous Doppler methods are emerging. More prospective studies with standardized protocols for diagnosis of elevated ICP, indications for invasive or noninvasive testing, selection of subsequent intervention, outcomes, and long-term follow-up are needed to determine optimal ICP monitoring practices and duration of follow-up for craniosynostosis patients.

(1) The University of Chicago, Pritzker School of Medicine, Chicago, IL, (2) The Pennsylvania State University, University Park, PA, (3) The University of Chicago, Department of Orthopedic Surgery and Rehabilitation Medicine, Chicago, IL, (4) The University of Chicago, Section of Plastic and Reconstructive Surgery, Chicago, IL

Background/Purpose: Craniosynostosis, the abnormal fusion of cranial sutures, occurs in 3.1 to 4.6 per 10 000 live births. Approximately 21% of cases can be attribute to an inherited mutation leaving the other 79% of cases are sporadic or from an unknown cause. These nonsyndromic cases are most likely the result of polygenetic and epigenetic influences that have not yet been elucidated. BMP9 is a protein from the Bone Morphogenetic Protein family that has been shown to be a potent osteogenic growth factor in many studies. Some studies have demonstrated that BMP9 plays a role in organizing the development of the cranial suture, most notably a study that showed that BMP9 induced osteogenesis in cells from the cranial sutures of a craniosynostosis patient. There is a notable lack of studies looking at the effect of BMP9 within the cranial suture in vivo. Our study used transgenic BMP9 knockout mice to investigate whether BMP9 effects the phenotype of cranial sutures in mice.

Methods/Description: Transgenic mice were created from commercially available mutant embryonic stem cells from KOMP (Knockout Mouse Project Repository, https://www.komp.org) and litters were obtained that contained heterozygote (het) BMP9 +/- and knockout (KO) BMP9 -/-. Mice were euthanized at 2 and 4 weeks. Skulls were harvested and fixed in 10% neutral buffered formalin. Skulls were then imaged by micro-CT. Micro-CT images were analyzed with morphometric analysis on Amira image analysis software using qualitative and quantitative analysis of suture patency as well as Euclidean Distance Matrix Analysis (EDMA). Skulls were also decalcified, paraffin embedded, and microsectioned for gross light microscopy. RNA was isolated using Trizol and qPCR analysis was performed to analyze RNA expression.

Results: Our preliminary results include 10 scans from mice aged 2 and 4 weeks. Our results show that there is a negligible difference in the patency of the sutures between het and KO mice by both qualitative and quantitative analysis on microCT. Histology similarly shows no difference in the structure of sutures. qPCR showed that BMP9, as well as other osteogenic markers are decreased in the KO mice sutures, although Runx2 levels were not decreased. Initial (n = 6) morphometric analysis using EDMA showed the cranial vault of the mutant mice were smaller on average by 3% (P = .001).

Conclusions: In conclusion, this is the first study to characterize the cranial phenotype of BMP9 KO mice. There is evidence that the mutation of other BMPs leads to craniosynostosis including BMP2. It appears the knockout of BMP9 does not affect the suture morphology in mice at 2 or 4 weeks although it may affect overall skull development. The normal Runx2 expression suggests that loss of BMP9 leads to the upregulation of other factors to compensate at the cranial suture. We hope that our findings add to the growing literature defining the underlying mechanism of craniosynostosis and we plan to further define and investigate the effect of BMP9 on cranial development.

(1) The University of Chicago, Pritzker School of Medicine, Chicago, IL, (2) University of California, Department of Surgery, San Francisco, CA, (3) The University of Chicago, Section of Plastic and Reconstructive Surgery, Chicago, IL

Background/Purpose: Opioids prescribed by surgeons account for 9.8% of the total opioid prescriptions in the United States and some postoperative patients go on to develop persistent opioid use after surgery. Therefore, it is important to evaluate predictors of opioid prescribing following surgical procedures in order to identify ways to optimize prescribing patterns. A recent study showed that there were multiple factors associated with prolonged opioid use after plastic surgery and a smaller study showed that plastic surgeons prescribe more opioids than patients are consuming. The goal of this study was to examine opioid prescribing among adults who underwent orthognathic surgery and to analyze factors associated with high-dose postoperative opioid prescribing and delayed opioid refills.

Methods/Description: Exempt status was granted by the University of Chicago IRB. Patients were identified using CPT codes for orthognathic surgery in the IBM Truven MarketScan database between 2003 and 2017. The following outcomes were reviewed: the presence of an outpatient opioid claim from 31 days before until 90 days after their operation; the total oral morphine milliequivalents (MMEs) that patients had in fulfilled claims in the time period and; the presence of an opioid prescription refill from 90 until 365 days after the operation. Independent variables included demographics, complications, and comorbidities, which were identified using ICD-9/10 codes. Univariate analysis was performed, and multiple logistic regression was used to determine associations between the variables listed above and the binomial outcomes: presence of an opioid prescription and; presence of an opioid refill. A gamma regression was used to evaluate the effect of the independent variables on total MMEs prescribed in the perioperative period.

Results: Our study yielded a cohort of 9926 opioid naïve adults. Only a minority of patients had a filled opioid claim in the perioperative period (46.3%). Among patients with an opioid prescription, the average prescribed MME in the perioperative period was 534 oral MMEs total, and 66 oral MMEs daily. Of patients with an opioid claim, 89 (1%) had a refill of their opioid prescription past 90 days postoperatively. Age was a significant predictor of having a postoperative opioid claim (P < .001), as was a history of substance abuse (P < .05). We found multiple factors associated with increasing opioid prescription amount, including age (P < .001) and days hospitalized (P < .001). One of the strongest predictors of refilling an opioid prescription was being prescribed more than 600 oral MMEs in the perioperative period (OR 3.76, P < .001), with a dose effect response showing an increasing likelihood for late refill with an increasing amount of opioids prescribed.

Conclusions: Our data point to age and the volume of perioperative opioid prescriptions being important factors in persistent opioid use. We hope that our study can help direct evidence-based guidelines that promote effective pain management and opioid prescribing.

(1) Baylor College of Medicine, Texas Children’s Hospital, Houston, TX, (2) Smile Train, New York, NY

Background/Purpose: Electronic health records (EHR) have become the standard medium for documenting all aspects of patients’ health information in high-income countries (HICs) due to their many benefits over paper charting. In contrast to HICs, health care institutions in low-and middle-income countries (LMICs) report difficulties with EHRs due to their prohibitive cost, dependence reliable electricity and internet, lack of training, or lack of adequate electronic equipment. Smile Train, the world’s largest cleft charity, created Smile Train Express (STX), an EHR designed to track cleft treatments outcomes performed by Smile Train partners. This EHR, with online and offline functionality, is available free of charge to Smile Train’s active partners around the globe. Smile Train administered a survey to analyze the usage of STX for identifying avenues to decrease the administrative burden of medical documentation for its partners. The purpose of this study is to identify trends in cleft treatment documentation from 42 countries to improve the implementation of EHR software in LMICs.

Methods/Description: A survey was administered in 7 languages to 884 active Smile Train partners to minimize the risk of erroneous responses. Survey questions were designed to determine each partner’s primary medium for preliminary entry of cleft treatment data, the reasoning behind using said medium, status of internet connectivity, and whether they used an EHR program in addition to STX.

Results: Of the 884 partners administered a survey, 155 partners (18%) provided full responses while 9 partners (1%) provided partial responses. Most partners utilized a single medium for preliminary data entry (89%) with paper charting (66%) far outweighing the next most common medium of institutional EHR software (27%). Improving clinical workflow and ensuring data reliability were the primary motivations for responders to use paper charting despite having access to offline and online EHR software (53% and 22%, respectively). The majority of responders reported intermittent (48%) or reliable (43%) internet connectivity.

Conclusions: Paper charting remains the most commonly employed medium of clinical and demographic data collection within LMICs. Our findings indicate that partner institutions preferred paper charts over EHR software primarily due to the negative effect that EHR software has on clinical workflow. Data reliability of paper record keeping was the second most common reason for paper charting. Further studies are needed to determine how the implementation of EHR software can be improved to facilitate clinical workflow within hospitals in LMICs.

(1) Columbia University and the NewYork-Presbyterian-Morgan Stanley Children’s Hospital, New York, NY, (2) Weill Cornell Medical College, New York, NY, (3) Columbia University Medical Center, New York, NY

Background/Purpose: Patients with a cleft lip and/or palate (CLP) present a complex clinical challenge requiring a multidisciplinary approach for optimal treatment. Increased psychosocial support has been shown to improve patient outcomes. This institution’s Craniofacial Team Day (CTD) is a community event that allows patients with CLP and their families to interact with the cleft care team in a family-centered setting. The event includes breakout groups, speeches from providers, and creative events to help families meet. In this study, we surveyed attendees of the 2nd annual CTD to determine if community events can improve engagement with the treatment team for psychosocial support.

Methods/Description: A pre- and post-event survey of the 108 family members that attended the 2019 CTD was conducted using Qualtrics. The survey assessed the event’s effect on parents’ perceptions of the cleft care team, understanding of resources available to them, and engagement with psychosocial support. Questions used a 5-point Likert scale. Responses were compared between the pre- and post-event surveys.

Results: There was a 43% response rate to the pre-event survey. Prior to the CTD, 52% of respondents didn’t know another family with a child who had a CLP. While 41% of families said they were not concerned at all about their child’s future career, 21% were either “very concerned” or “somewhat concerned.” CLP families turned to their extended family (53%) and CLP medical providers (58%) at similar rates for psychosocial support. The postevent survey response rate was 16%. Among this group, 90% said they were more optimistic about their child’s well-being after the CTD. Additionally, 76% of families indicated that they plan to spend more time talking to other CLP families and learning from their shared experiences. In the post-CTD survey, there was a significant increase in the Likert values families assigned to the importance of psychosocial support for CLP patients. The majority (54%) of families also said that the most helpful part of team day was the opportunity to meet other families who have children with CLP.

Conclusions: Community gatherings such as a CTD have a positive effect on patients and their families’ attitudes toward pediatric craniofacial care. Previous research has demonstrated that increased psychosocial support improves outcomes for CLP patients. This institution’s CTD significantly increased the desire of families with CLP children to engage with psychosocial support. The CTD specifically helped families identify resources available through the craniofacial care team and other CLP families. This increased awareness and identification of resources demonstrates how community-based craniofacial awareness events can improve outcomes for CLP patients.

(1) Oregon Health and Science University, Portland, OR

Background/Purpose: Palatoplasty carries an inherent risk of perioperative adverse airway events (AAE). Basta et al. recently developed a risk assessment tool to identify variables that corresponded with higher risk of AAEs in this patient population (Cleft Palate–Craniofacial J. 2018). We used these characteristics to identify at-risk patients and developed a protocol to improve safety after palate and pharyngeal surgery at our institution.

Methods/Description: Our prospective study is designed to evaluate for quality improvement after implementing a protocol to ensure airway safety in pediatric patients undergoing palatoplasty or pharyngoplasty with craniofacial surgeons at Doernbecher Children’s Hospital. We identified high risk patients for placement of a tongue stitch to assist in postoperative airway management. High risk patients are defined as those with laryngoscopy worse than grade 2b, >2 intubation attempts, structural airway abnormalities, obstructive sleep apnea, severe hypotonia or neuromuscular disorder, and intraoperative narcotic dose >0.35 mg/kg morphine equivalents. Data collected includes AAEs, oxygen nadir during hospitalization, frequency of tongue stitch use, calls to the anesthesia or surgical team with airway concerns, and unit of inpatient stay (ward or ICU).

Results: The protocol was instituted in January 2019, with 3 craniofacial surgeons involved in its development and implementation. A total of 63 palatoplasties and 6 pharyngoplasty were performed in January 2019 to June 2020. Of these cases, 40 patients (58%) were identified as high risk, with 69.4% receiving a tongue stitch per our protocol. In total, 9 patients (11.6% of total) experienced an AAE postoperatively including stridor, desaturations, and/or obstruction requiring tongue stitch use, supplemental oxygen, and/or periprocedural steroids. Seven of the 9 total patients who experienced an AAE had a tongue stitch in place. All of these patients were classified as high risk per our protocol. The airway was safely managed in all cases with only one requiring ICU care postoperatively.

Conclusions: Our history with variability in surgeon use of the tongue stitch and prior AAEs after palatoplasty inspired a change to our perioperative airway management. We used our historical performance together with the risk assessment tool developed by Basta et al. to inform a new protocol to standardize management for high risk patients. Preliminary results demonstrate airway safety conferred by tongue stitch placement. There is room for improvement in adherence to the protocol with the need for adjustment to stitch placement criteria as one patient experienced an AAE who was not identified as high risk. We are continually evaluating the protocol, collecting and reporting our data at 6 month intervals, and making adjustments as indicated by our experiences.

(1) Kaplan Medical Center, Tel-Aviv, Israel, (2) UT Southwestern School of Medicine, Dallas, TX, (3) Analytical Imaging and Modeling Center, Children’s Health, Dallas, TX

Background/Purpose: Metopic craniosynostosis is traditionally repaired with fronto-orbital advancement (FOA) or alternatively by limited short scar strip craniectomy (LSSSC) with postoperative helmet therapy. There is controversy among surgeons regarding resultant head shape outcomes between the 2 methods. This study aims to assess how surgeons perceive the postoperative aesthetic results of the 2 metopic craniosynostosis repair methods.

Methods/Description: Upon IRB approval, 10 board certified plastic surgeons from different institutions across the United States were enrolled in this study to evaluate the surgical outcomes of 13 (n = 6 LSSSC; n = 7 FOA) patients who presented for surgical correction of isolated metopic craniosynostosis. The rating surgeons were all fellowship trained in craniofacial surgery and had completed plastic surgery residency, had not participated in the care of the patients and were blinded to the technique used. Clinical photographs at 1 year postoperative were shown to the surgeons raters via WebEX videoconferencing (Cisco Systems). The surgeons rated the aesthetic outcomes on a Likert scale of 1 (poor) to 5 (excellent). In addition, the surgeons were asked to identify which surgical method was performed on each patient, LSSSC or FOA.

Results: All 10 surgeons successfully rated all images. Mean surgeon scores of aesthetic outcomes were similar between groups: LSSSC, 3.27 ± 1.09 SD; FOA, 3.51 ± 0.95 SD (P = .171). When asked to identify which procedure each patient had received, only 63.8% of responses were correct. Mean blood loss was significantly lower with LSSSC versus FOA (202.0 ± 361.2 mL vs 371.43 ± 122.9 mL; P < .001). In addition, the mean blood transfusion was significantly lower in LSSC (92.5 ± 49.9 mL vs 151.3 ± 51.2 mL; P < .001). Furthermore, the operative duration (from anesthetic induction to extubation) was significantly shorter in the LSSSC group (mean: 3:06 ± 0:24 hours) versus the FOA group (mean: 7:53 ± 0:31 hours; P < .001).

Conclusions: Experienced, fellowship-trained craniofacial surgeons who were not involved in the care of the studied patients with metopic craniosynostosis could not reliably determine which technique was utilized, nor was there a better average outcome noted with either technique with respect to overall head shape. Additionally, decreased blood loss and operative times were documented with the LSSSC technique, corroborating previous data from other groups. LSSSC offers an important alternative to traditional open FOA, with less blood loss and operative time, and equal ratings of outcome by blinded craniofacial surgeons.

(1) Children’s Hospital of Philadelphia, Philadelphia, PA

Background/Purpose: Existing literature has assumed that there is an ideal distraction protocol for given osseous segments, and that distraction should proceed at a linear distance rate throughout the active distraction period. Rather than distracting at a constant distance rate, we hypothesize that distracting at a constant force rate may be more optimal for osteogenesis and soft tissue compliance. To establish the foundation for these investigations, we need to better understand the variable forces involved with current distraction protocols. The purpose of this study is to determine the forces imparted by distraction osteogenesis of the mandible and cranium over the timeline of an activation period.

Methods/Description: Seventeen patients undergoing distraction of the mandible or cranial vault with semi-buried KLS-Martin distractors were included in this prospective study. Distractors were activated each day by study personnel using a digital torque-measuring screwdriver. Torque measurements were then converted into linear force values and associated with patient outcomes.

Results: Cranial vault distraction (CVDO) was performed on 7 subjects (41.2%), and mandibular distraction (MDO) on 10 subjects (58.8%). Average age among subjects undergoing MDO was 43.1 ± 46.9 days, while average age of subjects undergoing CVDO was 2.3 ± 1.9 years. Mean latency period length was 2.4 ± 0.9 days, while that of the active distraction period was 18.2 ± 5.0 days. The average consolidation period was 72.0 ± 26.2 days. The average number of activations that were measured using the torque measuring tool was 17.3 ± 10.9 measurements per subject. Across the entire cohort, the maximum force per activation was 27.0 ± 23.5 N, and the elastic force (the rise in force over a single activation) was 10.7 ± 14.1 N. Maximum force (CVDO: 52.9 ± 20.2 N vs MDO: 12.9 ± 8.5 N; P < .0001), and elastic force (CVDO: 22.0 ± 15.6 N vs MDO: 4.5 ± 8.2 N; P < .0001) were significantly higher in the CVDO subgroup than in the MDO cohort. Paired t test showed that late peak activation force was significantly higher than early peak activation force in both CVDO (P < .0001) and MDO cohorts (P < .0001). On multivariate regression analysis, statistically significant associations were seen between maximum activation force and the following independent variables: active DO day number (B = 1.1; P < .001), DO rate (mm/day) (B = 8.9; P = .016), CVDO (relative to MDO) (B = 41.4; P < .001), and device failure (B = 10.3; P = .004).

Conclusions: In CVDO and MDO, both the magnitude and trend in forces follow relatively significantly unique and predictable patterns, and correlate significantly with distraction rates. Both subgroups demonstrated significant tissue elasticity, with forces rising from beginning to the end of an activation. Next, our study will move toward varying these distraction protocols in an attempt to achieve a more constant force rate and evaluate outcomes of patient comfort and resultant soft tissue morphology.

(1) Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China, (2) Department of Oral and Craniomaxillofacial Surgery, Shanghai Ninth People’s Hospital, Shanghai, China

Background/Purpose: Congenital defect of unilateral soft palate is a rare congenital oral and maxillofacial malformation. We reported a case of velopharyngeal insufficiency due to cleft palate and congenital soft palate defect on the left side.

Methods/Description: The patient was a 5-year-old female presented with congenital soft palate defect and cleft lip on the left side. The posterior pharyngeal lymphoid follicles could be seen through the defect. Her face appeared to be symmetrical, and there was no manifestation of facioplegia. Considering that the defect was relatively small and the patient’s pharyngeal cavity was large and deep, the patient received posterior pharyngeal flap for velopharyngeal insufficiency under general anesthesia. The patient’s velopharyngeal function was improved after surgery and speech training. In the repair of soft palate defect, attention should be paid to the recovery of palatal function. Mucoperiosteal island flap can be used for smaller defects, while larger defects may need to rely on vascularized free flap repair, which may be difficult for parents to accept. Therefore, tissue-engineered oral mucosa can be used for palatal reconstruction in the future.

(1) Boston Children’s Hospital, Boston, MA, (2) Connecticut Children’s, Hartford, CT, (3) Cincinnati Chidren’s Hospital Medical Center, Cincinnati, OH

Background/Purpose: Outcome assessment following palatoplasty varies significantly across institutions and there exists no widely applied standardized outcome metric. Use of a clear, consistent, and comparable outcome metric would help to provide quality benchmarks in cleft palate care. The purpose of this study is to perform an ongoing retrospective review in this same cohort of patients following primary palatoplasty from 2007 to 2013 to assess cleft palate repair OOR by age 8 years.

Methods/Description: Previously, we created a novel value-based quality metric termed “OOR” (Optimal Outcome Reporting) to assess cleft palate repair at age 5 years. An optimal outcome after cleft palate repair is defined as a single operation, velar competence and the lack of unintended palatal fistulae; and all 3 must be achieved for a patient to be deemed to have an “optimal outcome.” We will track our patient cohort between the ages of 5 and 8 to assess for potential changes in outcome, procedures, and associated costs.

Results: Of all, 94 patients were identified. Median age at operation was 10 months. In all, 55% were female and 54% were Caucasian. Syndromic diagnoses existed in 23/94 of patients, most commonly Robin (9/23). Veau I represented the most common cleft type (32%), followed by Veau III (28%) and Veau II/IV (20%). In this patient cohort, OOR at age 5 years was identified in 68/94 patients (72%). Eight patients had suboptimal outcomes for experiencing more than one operation, while 18 patients failed for velar incompetence. A significantly greater number of nonsyndromic patients demonstrated an “optimal” result compared to syndromic patients. Of the patients with suboptimal outcomes, patients requiring more than one operation by age 5 experienced significantly more clinic visits and accrued higher costs compared to “optimal” result patients. Ongoing data collection and analyses are underway to assess OOR, clinic encounters and costing data in this same cohort of patients at age 8 to assess outcome metric durability.

Conclusions: OOR in cleft palate care describes the outcome that patients and families would ideally like to experience following cleft palate repair: single operation, velar competence, and no postoperative fistulae. This simple and understandable metric for assessing surgical outcomes can be determined for individual surgeons and across various cleft palate centers. A standardized outcome assessment will allow for benchmarking and serve as a basis for tracking quality improvement initiatives. We are performing an ongoing OOR assessment in 94 patients at age 8 and beyond to track OOR metric durability in an effort to determine whether there exists a suitable single time point at which to assess and compare cleft palate repair OOR. We will also continue to evaluate cost of care for optimal and suboptimal patient outcomes to define the financial benefit of optimal patient outcomes in cleft palate repair.

(1) Boston Children’s Hospital, Boston, MA

Background/Purpose: Encephalocele is characterized as a cephalic hernia that protrudes through a congenital defect in the cranium. These protrusions contain cerebrospinal fluid, meninges, and sometimes segments of brain tissue. Little has been reported on the long-term outcomes of adaptive abilities of patients treated for encephalocele. This study aims to assess, follow, and gain a greater understanding of the long-term developmental prognosis, specifically adaptive abilities, of patients with repaired encephalocele.

Methods/Description: This is a long-term evaluation of the developmental outcomes of children born with encephalocele, subsequently repaired at Boston Children’s Hospital. Outcomes are reported biannually via the completion of Vineland-3, as well as follow-up interviews conducted biannually with research members. Adaptive abilities assessed include communication, daily living, and socialization skills. Secondary brain deformities at the time of encephalocele removal were also recorded.

Results: Every child evaluated reported signs of impairment in adaptive skills at the time of first questionnaire intake. Among n = 5 patients, 4 patients demonstrated significant delay in communication, daily living, and socialization skills, with Vineland-3 scores ranging from 1st to 14th percentile in these respective categories, compared to same-aged peers. Several patients exhibited secondary brain deformities at the time of encephalocele removal. It is likely that these deformities contributed to speech, language, vision, and motor impairment reported among patients.

Conclusions: Patients born and treated operatively for encephalocele remain at risk for significant impairment in adaptive skills. It is imperative to evaluate the likely long-term cognitive, adaptive, and social outcomes among patients when considering encephalocele removal. Plans must be taken to optimize quality of life among this patient population.

(1) Operation Smile Inc, Los Angeles, CA, (2) University of Southern California, Los Angeles, CA, (3) University of California, Los Angeles School of Dentistry, Los Angeles, CA, (4) Shriners Hospital for Children, Los Angeles, CA

Background/Purpose: Cleft palate is among the most common congenital disorders worldwide and is correctable through surgical intervention. However, suboptimal surgical results may cause velopharyngeal insufficiency (VPI). VPI, when symptomatic, can cause hypernasal or unintelligible speech. In severe cases, VPI requires functional reconstructive surgery. The postoperative risk of VPI varies significantly in the literature but may be attributed to differences in study size, cleft type, surgical technique, and operative age. To identify the potential impact of these factors, we examined the risk of VPI after primary palatoplasty, accounting for operative age and surgical technique.

Methods/Description: PubMed, Embase, and Web of Science were searched for original studies that examined speech outcomes after primary palatoplasty. This study was registered on PROSPERO (CRD42020193982). The search identified 4740 original articles. Forty studies that reported mean age at palatoplasty and VPI-related outcomes were included. VPI rate was calculated for the entire included population and for each operative age quartile (0-6 months [Q1], 6-12 months [Q2], 12-24 months [Q3], and over 24 months [Q4]). A subgroup analysis was done using data from the 23 studies that reported 4 common palatoplasty techniques (Von Lagenbeck, Veau-Wardill-Kilner, Furlow Double Opposing Z-plasty, and Bardach’s 2-Flap). Data was analyzed using descriptive statistics and χ ² tests.

Results: Mean operative age of the 11 124 patients included in this study was 14.3 months, and 20% developed VPI. Palatoplasty at 6 to 12 months (Q2) had the lowest occurrence of VPI (17.8%, P < .00001). Patients from the other 3 quartiles—Q1, Q3, and Q4—developed VPI in 27.6%, 28.4%, and 26.9% of cases, respectively. The palatoplasty technique subgroup analysis included 2281 patients. Veau-Wardill-Kilner had the highest association with VPI (30.3%, P < .01). Patients who underwent the other techniques had lower VPI rates that were not statistically different (Von Langenbeck 23.3%, Furlow Double Opposing Z-plasty 20.5%, and Bardach 2-Flap 22.1%).

Conclusions: An optimal time to perform primary palatoplasty to diminish risk of postoperative VPI may be between 6 and 12 months. The Veau-Wardill-Kilner technique may be associated with a higher chance of developing VPI compared to other standard techniques. Larger cohort longitudinal studies are needed to better determine risk factors for symptomatic VPI following primary palatoplasty to improve clinical outcomes.

(1) University of Pennsylvania School of Dental Medicine, Philadelphia, PA, (2) Division of Plastic and Reconstructive Surgery, The Children’s Hospital of Philadelphia, PA, (3) Department of Medicine and Genetics, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA

Background/Purpose: Epithelial Splicing Regulatory Protein (ESRP) is the major regulator of epithelial specific alternative RNA splicing in developing facial prominences. Accordingly, ESRP mutations are associated with cleft lip and palate (CL/P) in humans and Esrp1 knockout (KO) leads to complete penetrance of CL/P in mice along with changes in epithelial gene expression. We have shown that haploinsufficiency of SOX2 rescues cleft lip but not the cleft palate in Esrp1 KO embryos. It is unknown whether failed palatal fusion in Esrp1-/-: Sox2+/- embryos is due to epithelial fusion defect or deficient mesenchymal growth. The aim of this study is to further investigate the role of Sox2+/- in upper lip and palatal fusion in Esrp1 KO embryos.

Methods/Description: E18.5 embryos were harvested from Esrp+/- female mated with Esrp+/-: Sox2+/- male and genotyped. Esrp1+/+: Sox2+/+ (WT), Esrp1-/-;Sox2+/+ (KO), and Esrp1-/-: Sox2+/- (Rescued) were then processed for whole mount skeletal staining with Alzarin Red and Alcian Blue. Palatal organ culture was used to test fusion competence of the palate from Esrp1-/-: Sox2+/-. Palatal shelves dissected from E14.5 embryos were juxtaposed in the same orientation as in vivo and cultured for 3 days. They were processed for histology and immunostaining for cell apoptosis and proliferation.

Results: Skeletal staining shows that defective development of the facial bones is largely limited to maxilla, premaxilla, and palatal bone in Esrp1 KO mice. In Esrp1-/-: Sox2+/- mice, development of the maxilla and premaxilla is restored; the palatal bone however remains underdeveloped, consistent with failed palatal fusion. WT palatal shelves are able to undergo epithelial fusion and achieve mesenchymal confluence in organ culture, which is characterized by medial epithelial cell apoptosis/disappearance and mesenchymal cell proliferation. On the other hand, Esrp1 KO palatal shelves fail to fuse, showing the lack of medial edge epithelial apoptosis and reduced mesenchymal cell proliferation. Interestingly, Esrp1-/-: Sox2+/- palatal organ cultures showed epithelial fusion/disappearance and mesenchymal confluence.

Conclusions: Sox2 haploinsufficiency restores Esrp1 KO induced epithelial dysfunction in not only upper lip but also palatal shelves in mouse embryos. Thus, we conclude that failed palatal fusion in Esrp1-/-: Sox2+/- embryos may be due to deficient growth of the palatal mesenchyme. We are working on RNAseq analysis of WT, KO and Rescued embryonic facial prominences and the palate to identify ESRP1 targets that are critical for upper lip and palatal fusion.

(1) NIDCR, Bethesda, MD, (2) NIH/NIDCR, Bethesda, MD, (3) NIEHS, RTP, NC

Background/Purpose: Complete congenital Arhinia is a rare embryologic defect characterized by absence of the soft tissue and skeletal structures of the nose. It is often associated with ocular and reproductive defects (Bosma Arhinia Microphthalmia syndrome) and has been genetically associated with a rare SMCHD1 gene mutation. The exact pathogenesis mechanism remains unclear. Arhinia is compatible with life and the patients usually adapt to oral breathing. Surgical reconstruction is usually postponed until preschool age or later. In total, 88 cases have been reported, most of which describe neonates or include limited information regarding the craniofacial phenotype. The purpose of this study is a detailed craniofacial, oral, and dental phenotypic characterization of Arhinia.

Methods/Description: Eight arhinia probands (4 males-4 females), with a mean age of 28.4 (18-53) years, were enrolled in an IRB approved study (protocol: 16-D-0040) and were examined at the NIH Craniofacial and Dental Clinic. A clinical examination, 2D, 3D photos, and head CBCTs were used for their craniofacial analysis. 2D cephalometric and geometric (3D) morphometric analyses were performed. Age, gender, and ethnicity matched orthognathic control group was used for the needs of the 3D analysis.

Results: Clinically, a complete absence of natural soft and skeletal tissue nasal structures, ossification of the nasal aperture, absence of nasopharyngeal airway, midface hypoplasia, and anosmia were identified in all probands. Other significant clinical findings included longer facial third, mandibular prognathism mainly due to chin prominence, increased inner canthal distance, soft tissue asymmetry, whistle lip deformity, and a high arched and narrow palate. The dental phenotype included hypodontia, Class III malocclusion, anterior and posterior crossbite. CBCT examination revealed complete absence or severe hypoplasia of the paranasal sinuses, nasolacrimal duct stenosis or atresia, and ossification of the cribriform plate in all cases. Cephalometric analysis results supported the clinical findings and revealed a short anterior cranial base length and obtuse cranial base angle in most cases. Morphometric analysis indicated an overall difference in skull size, shape, and degree of asymmetry between arhinia and control subjects.

Conclusions: The complete absence of the nose has a significant impact on the development of the midface structures and the cranial base. However, facial width, total facial height, and vertical dental relationships were not as severely affected. Our future goal is to further understand the role of SMCHD1 gene in craniofacial development.

(1) Birmingham Children’s Hospital, Coventry University, Birmingham, United Kingdom, (2) The University of Nottingham, Nottingham, United Kingdom, (3) Great Ormond Street Hospital NHS Trust, London, United Kingdom, (4) Coventry University, Coventry, United Kingdom, (5) Oxford Brookes University, Oxford, United Kingdom

Background/Purpose: There is a lack of standardization in the speech samples used to assess speech at age 3-years in the CP ± L population. This reflects the developmental spectrum of 3-year-olds and the unique challenges this presents when assessing speech. Typically, 2 types of speech sample are used, single word naming assessments, for example, The Restricted Word List (RWL) and sentence repetition, for example, The Great Ormond Street Speech Assessment (GOS.SP.ASS). This study compares 2 different speech samples to determine if there is an ideal speech sample to use at this age.

Methods/Description: Two speech samples were specifically designed and adapted for this age-group. Sample A comprised 2-minutes of spontaneous speech and, a picture naming task utilizing the RWL ² and an additional 29 target words including additional phonemes /m, ŋ, l, z, ʃ, tʃ, dʒ, h/ in different word positions (59 pictures in total). Sample B consisted of the repetition of 24 short phrases represented by pictures. Each sample targeted the same phoneme 4 times. Twenty 3-year-olds with CP ± L and five 3-year-olds without CP ± L were recruited. The participants were randomized as to whether they completed Sample A or B first. All samples were video recorded by one SLT. Completion of the speech samples was rated as: not completed, partially completed, or fully completed.

Results: The average length of time to complete (partially or fully) Sample A was 16.5 minutes compared with an average of 6 minutes for Sample B, with a mean of 22.5 minutes for both samples. In the CP ± L group, one participant did not complete either speech sample. Of all, 70% of the CP ± L group fully completed Sample A and Sample B. Participants who only partially completed Sample A did not complete Sample B. Those fully completing the first sample also fully completed the second, irrespective of which sample. The group without CP ± L completed both samples.

Conclusions: The majority children in the CP ± L group completed both speech samples, suggesting that most 3-year-old children can complete comprehensive speech samples in a single appointment. Short phrase repetition is however a more challenging task at this age than single word repetition. Comprehensive assessments at age 3-years facilitates more accurate intervention planning and comparison with 5-year outcomes. This study demonstrates how the RWL ² can be integrated into a speech sample, confirming that data collection for cross-linguistic comparisons can be part of more comprehensive, language specific speech sampling tasks.

(1) NIDCR, Bethesda, MD, (2) NIH/NIDCR, Bethesda, MD, (3) University of Washington, Seattle, WA, (4) NIAID, Bethesda, MD

Background/Purpose: Elevated transforming growth factor (TGF)-β signaling has been implicated in the pathogenesis of 2 rare diseases, Loeys-Dietz syndrome (LDS) and Shprintzen-Goldberg syndrome (SGS). This study is the first to quantitatively examine the craniofacial deformities associated with the different genetic LDS types and SGS, and to correlate the craniofacial phenotype to genotype using a multimodality approach.

Methods/Description: We explore the variability within and across a cohort of N = 44 patients, with the use of clinical phenotyping, 3D photo surface analysis, cephalometric analysis, and geometric morphometric analysis.

Results: The most characteristic clinical findings were the presence of mandibular retrognathism (84%), low set ears (73%), down-slanting palpebral fissures (70%), flat midface projection (68%), blepharoptosis (65.9%), abnormal lip shape (64%), scleral show (61.4%), skin translucency (59.1%), broad forehead (54.5%), hypertelorism (40.9%), cleft palate/bifid uvula (38.6%), blue sclera (34%), telecanthus (27.3%), and craniosynostosis (23%). Functional abnormalities included snoring (38.6%), temporomandibular joint disorder (34.1%), nystagmus (25%), strabismus (22.7%), obstructive sleep apnea (13.6%), and hearing loss (11.4%). 3D photo analysis confirmed the clinical findings and indicated some additional subclinical trends. Cephalometric findings supported the clinical data and confirmed the presence of alterations in the cranial base. The mean cohort cranial base angle value was 3SDs above the norm, indicating a flattened cranial base angle, with the most significant alterations in LDS2 (z = 4.20). There was also significant shortening of the anterior cranial base (z = −2.35), with clinical significance in LDS1, LDS2, and LDS4. According to the geometric morphometric analysis, upon comparison of the overall shape of LDS1 and LDS2 with skeletal Class I, II, and III matched control groups, LDS2 overlaps more with Class II/retrognathic patients, whereas LDS1 overlaps with both Class I and II patients.

Conclusions: LDS2 and SGS patients were the most severely affected overall. Based on these results, both facial structures and the cranial base are affected by misregulations in the TGF-β pathway. This systematic phenotypic methodology could become a valuable tool in clinical research and differential clinical diagnosis of these conditions.

(1) Vanderbilt University School of Medicine, Nashville, TN, (2) Vanderbilt University Medical Center, Nashville, TN, (3) Vanderbilt University, Department of Biostatistics, Nashville, TN, (4) Vanderbilt University Medical Center, Vanderbilt Children’s Hospital, Nashville, TN, (5) Vanderbilt University, Nashville, TN

Background/Purpose: After infants undergo primary palatoplasty, serial speech evaluations ensure identification of problems with normal speech development. The purpose of this study was to examine early post palatoplasty speech outcomes in children, distinguished by cleft type, comorbidities, ear tube placement, language exposure, and adoption status.

Methods/Description: After IRB approval, patients who underwent primary palatoplasty between 2013 and 2018 and who received a subsequent speech language pathology (SLP) evaluation at a tertiary care medical center were included. Patient demographics, comorbidities, home language, cleft type, surgical information, and speech evaluation characteristics were documented. Statistical significance was set a priori at P < .05.

Results: Of the 196 patients identified (43% female, mean age of initial speech evaluation 30.6 ± 17.9 months), type of palatoplasty performed was not associated with hypernasality on initial speech evaluation (P = .189) or speech delay diagnosis (P = .605). Patients with ear tubes were more likely to be diagnosed with speech delays (P = .032); however, there was no difference in speech delay type (expressive, mixed; (P = 1.0) or whether they received therapy (P = .861). Postoperative hypernasality was not significantly differentiated by ear tube placement (P = .08). Although English-only speaking patients were evaluated at an earlier age (19.3 ± 17.7 vs 21.1 ± 14.3 months, P < .001) postoperatively, patients with multiple languages at home were not more likely to be diagnosed with speech delay (P = .3) or receive therapy (P = .116). Adopted patients (n = 25) were significantly older at time of palatoplasty (26.0 ± 15.3 vs 18.8 ± 17.1 months, P < .001). Initial hypernasality assessment was poorer in adopted patients when hypernasality was examined as a continuous variable from no (1) to severe (6) hypernasality (3.5 ± 1.7 vs 2.5 ± 1.7, P = .031); they were not more likely to be diagnosed with speech delay (P = .233). They were more likely to receive therapy (P = .002) and hypernasality improvement on follow-up was comparable to nonadopted patients (P = 1.0).

Conclusions: Overall, no difference in early speech outcome hypernasality was found for children of varying cleft types, ear tube placement or languages spoken at home. Adopted patients, while undergoing later palatoplasty, were not more likely to be diagnosed with speech delay. Children who received ear tubes were more likely to be diagnosed with speech delays, an outcome that could potentially be explained by closer follow-up of patients with ear tubes by ENT and SLP. This data also suggests that earlier hearing intervention may play an important role in speech delay mitigation. Understandably, early speech evaluations may not be adequate in identifying speech outcome differences and evaluations must be repeated throughout childhood development. Future studies exploring early speech and hearing intervention are warranted.

(1) Case Western Reserve University, Cleveland, OH, (2) Case Western Reserve University School of Dental Medicine, Cleveland, OH

Background/Purpose: Patients with Cleidocranial Dysplasia (CCD) typically exhibit class III facial pattern with delayed eruption of permanent teeth, multiple supernumerary teeth, and multiple impacted teeth. Orthodontic traction of the impacted teeth could be reinforced using temporary anchorage devices (TADs). Considering the lack of development of the alveolus, TADs are standardly placed palatally in this cohort of patients. The aim of this study is to utilize Cone-Beam Computed Tomography (CBCT) to compare the palatal bone thickness between individuals with CCD and control group (CON).

Methods/Description: This retrospective study included 7 individuals with CCD and 59 controls. Pretreatment CBCT volumes were imported into Dolphin software, which was utilized to measure the palatal bone thickness. The incisive foramen and midpalatal suture were set to be the reference landmarks and were located in the sagittal and coronal views of the CBCT. The palatal bone thickness was measured at 4 to 24 mm with 4-mm intervals posterior to the level of the distal wall of the incisive foramen on the sagittal plane, and at 0, 2, 4, 6 mm bilateral to the midpalatal suture on the coronal plane, yielding a total of 42 measurement points distributed symmetrically along the palatal region. Palatal bone thickness was measured perpendicular to the horizontal plane at each designated point.

Results: Overall, the palatal bone thickness in individuals with CCD tended to show higher values when compared to the controls. The greatest differences were shown in points measured at 4 mm posterior to the incisive foramen with 6 mm bilateral to the midpalatal suture (R6/4 = 4.30 mm, L6/4 = 4.06 mm; P < .001), followed by points measured at 8 mm posterior to the incisive foramen with 6 mm bilateral to the midpalatal suture (R6/8 = 3.98 mm, L6/8 = 3.19 mm; P < .001). No statistical significances were evident in the midpalatal suture area and 2 mm bilateral to it in all anteroposterior intervals (P > .05).

Conclusions: Among patients with CCD, palatal bone thickness was illustrated to be greater than that in the controls. The differences are emphasized at 6 mm and 4 mm bilateral to the midpalatal suture in 4 mm and 8 mm posterior to the incisive foramen. Findings from the current study should be accounted for during surgical and orthodontic planning for this cohort of patients, demonstrating that palatal TADs may be utilized safely, but still, customized analysis of each individual is required.

(1) Vanderbilt University School of Medicine, Surgical Outcomes Center for Kids, VUMC, Nashville, TN, (2) Vanderbilt University Medical Center, Surgical Outcomes Center for Kids, Nashville, TN, (3) Vanderbilt University, Department of Biostatistics, Nashville, TN, (4) Vanderbilt University Medical Center, Department of Otolaryngology-Head and Neck Surgery, Nashville, TN, (5) Vanderbilt University Medical Center, Department of Plastic Surgery, Nashville, TN

Background/Purpose: Both otolaryngologists and plastic surgeons treat patients with cleft lip and palate. Due to differences in training background, they may have different approaches to palatoplasty and speech surgeries that could impact outcomes. The purpose of this study was to evaluate any differences between otolaryngologists and plastic surgeons in approach to initial cleft palate repair, postoperative complications, and early speech outcomes.

Methods/Description: After IRB approval, patients who underwent primary palatoplasty between 2013 and 2018 at a tertiary care medical center were included in the study. Patient demographics, cleft type, palatoplasty type, postoperative complications (eg, fistula, dehiscence, VPI, hemorrhage, airway swelling, need for oxygen support, and/or reintubation), and speech evaluations were recorded. Statistical significance was set a priori at P < .05.

Results: Of the 188 patients identified, 66% (n = 125) had their initial palatoplasty performed by an otolaryngologist. Plastic surgeons performed primary palatoplasties earlier in life than otolaryngologists (P < .001), but there was no difference in the types of repair performed for each type of cleft palate. There were no differences in postoperative complications (P = .507). Patients whose primary surgeon was an otolaryngologist were more likely to receive ear tubes (P < .001). There were no differences between the 2 types of surgeons in terms of speech delay diagnoses (P = .388), receiving speech therapy (P = .721), total number of speech surgeries per patient (P = .309), severity of hypernasality (P = .330), or improvement between speech evaluations (P = .639).

Conclusions: While there was a difference in age at initial repair, there were no significant differences in types of repairs, complications, or early speech outcomes between otolaryngologists and plastic surgeons. This data reinforces the collaborative and multidisciplinary nature of craniofacial teams for patients with cleft palate; otolaryngologists and plastic surgeons may have different initial approaches, but there are no significant differences in early outcomes. Future studies that evaluate other outcomes between the different types of surgeons, such as hearing outcomes and aesthetic satisfaction, could prove valuable.

(1) Hospital for Rehabilitation of Craniofacial Anomalies, Sao Paulo, Brazil, (2) Hospital for Rehabilitation of Craniofacial Anomalies, University of Sao Paulo, Sao Paulo, Brazil, (3) Department of Orthodontics, Hospital for Rehabilitation of Craniofacial Anomalies, University of Sao Paulo, Sao Paulo, Brazil

Background/Purpose: The aim of this study was to determine the accuracy of 3 different methods for rating the dental arch relationship comparing physical plaster models, 2-dimensional (2D) imaging and 3-dimensional (3D) video of digitized models.

Methods/Description: Seventy-eight plaster models of patients with complete unilateral cleft lip and palate (CUCLP), submitted to lip and palate repair at the Hospital for Rehabilitation of Craniofacial Anomalies–University of São Paulo (HRAC-USP). Three experienced and calibrated orthodontic examiners were selected to rate the dental arch relationship of plaster models (Group 1), as well as 2D images (Group 2) and 3D video (Group 3) achieved using the Scanner 3Shape’s R700TM. Rating was performed using the Goslon Yardstick index, a specific analysis to evaluate the severity of interarch relationship of patients with CUCLP. The total sample was reevaluated after a 30-day period to verify the intra- and interexaminer agreement.

Results: Weighted Kappa statistics indicated excellent intraexaminer reliability (0.87 to 1). The interexaminer reliability was considered very good between the 3 examiners (0.8 to 0.91) and the 3 groups (0.86 to 0.97).

Conclusions: Physical plaster models, 2-dimensional (2D) images, and 3-dimensional (3D) video of digitized models showed an excellent accuracy for assessing the severity of dental arch relationships using the Goslon yardstick. The use of 2D and 3D images seems to be a good alternative to replace the physical models and facilitate future multicenter studies for comparison of growth and different treatment protocols in CLP.

(1) New York Medical College, Valhalla, NY

Background/Purpose: In craniosynostosis, diagnostic imaging is crucial for identifying skull abnormalities, planning for surgical correction, and postoperative and follow-up evaluations. However, classifying postoperative corrected skull morphology is a highly subjective task for all craniofacial surgeons. Initially, many surgeons utilized the Whittaker categorization test as the postoperative outcome measure, which is based on the subjective aesthetic appearance as determined by the surgeon, patient, and patient’s family. While the advancement of 3D imaging and anthropometric measurements techniques has allowed various research groups to produce normative skull models to serve as treatment guides, it is still difficult to determine the objective morphological standard. The purpose of this study is to review the current standards for classifying normal skull morphology used by medical modeling and virtual surgical planning industries in craniosynostosis cases.

Methods/Description: This paper is a review of the current surgical industrial standards of normal cranial morphology and the primary comparative methods used by major medical modeling companies. The morphometric measurements that are used in postoperative evaluation of craniosynostosis patients utilized by Stryker 3D systems, KLS Martin Group, Hanger Clinic, and DePuy Synthes were included in the study.

Results: A review of the strategies utilized by some of the major medical device manufacturers in craniosynostosis correction surgery is presented. Many of these companies have progressed to using 3D normative skull construction and 3D surface analysis to measure cranial morphology. However, many variations remain among industry standards.

Conclusions: Normative head shapes and cohort data matched to similar population groups have a crucial function in the diagnosis, preoperative planning, and postoperative evaluations in craniofacial surgery. However, industry standards for “normal head shape” utilized in surgical analysis and planning remain diverse and can be somewhat opaque to the craniofacial community. Despite the advancement of 3D imaging techniques, most reported outcomes following craniofacial surgical interventions are still subjectively defined.

(1) University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, (2) Division of Plastic and Reconstructive Surgery, University of Pennsylvania, Philadelphia, PA, (3) Division of General Pediatrics, Children’s Hospital of Philadelphia, PA, (4) Division of Plastic and Reconstructive Surgery, Children’s Hospital of Philadelphia, PA

Background/Purpose: Studies investigating disparities in the care of patients with clefts increasingly show delays in surgical and comprehensive care on the basis of racial identity, primary spoken language, and other socioeconomic factors. However there is little literature on how to effectively counteract these inequities. In 2016, our institution created a Cleft Nurse Navigator (CNN) role with the goals of improved diagnosis, referral, and communication among families and providers. We hypothesize that this program has improved the timing of care and reduced disparities between patient populations, thus enhancing the equity in the distribution of cleft care services at our institution.

Methods/Description: An institutional database was queried to identify patients born between May 2009 and November 2019 who presented for initial cleft care. Patients were separated into 2 cohorts, Pre-CNN and Post-CNN, on the basis of birth date relative to CNN implementation. Patients with atypical clefts and submucosal cleft palates, international adoption, and presentation for care after 250 days of life were excluded. Patient demographics, cleft diagnosis, appointment and surgical timing, information pertaining to feeding issues, and patient-cleft team communications were abstracted. Timing of care, feeding issues, and communications were assessed by racial group and insurance coverage before and after implementation of the CNN by univariate and multivariate analysis.

Results: In all, 739 patients were included in this analysis, 454 patients in the Pre-CNN cohort and 285 patients in the Post-CNN cohort. After the implementation of the CNN, there was a reduction in median age at first outpatient appointment (P = .021), an increase in patient-cleft team communications (P < .001), and a decrease in reported feeding concerns (P < .001). In the Pre-CNN cohort, nonwhite patients experienced delays in first outpatient appointment (P < .001), cleft lip repair (P = .011), and cleft palate repair (P = .019) compared to white patients. Publicly insured patients experienced delays in first outpatient appointment (P < .001), cleft lip repair (P < .001), and cleft palate (P = .025) repair compared to privately insured patients. In the Post-CNN cohort there were no significant differences in surgical timing on the basis or racial identity nor insurance type. Multivariate linear regression demonstrated that the decrease in age at first appointment was attributable to increases in patient-cleft team communications (P < .001) and an increased frequency of prenatal surgical consultations (P < .001).

Conclusions: A variety of factors lead to delays in cleft care for marginalized patient populations. These findings suggest that a cleft nurse navigator can reduce disparities of access and communication, and improve early feeding in at-risk cohorts. Future investigations may assess the CNN’s impact on non-English speaking patients and patients who are undocumented.

(1) Hospital for Rehabilitation of Craniofacial Anomalies, University of Sao Paulo, Sao Paulo, Brazil, (2) Department of Orthodontics, Hospital for Rehabilitation of Craniofacial Anomalies, University of Sao Paulo, Sao Paulo, Brazil, (3) University of Sao Paulo, Sao Paulo, Brazil

Background/Purpose: This presentation will describe the management of palatal fistula after primary repair of UCLP.

Methods/Description: Data regarding outcome of primary palatal repair for all patients with UCLP that were treated consecutively at an institution (within a period of 8 years) was reviewed. Medical records and photographic images from 153 patients with palatal fistula occurring after the primary palatal repair were analyzed in order to identify how fistula were managed. Possibility of fistula management approaches included surgical correction, prosthetic obturation, and follow-up with no intervention. Percentage occurrence of each management approach was identified for this presentation.

Results: From a group of 466 consecutively treated patients with UCLP, 313 patients (67%) had no palatal fistula. The remaining 153 patients (33%) presented palatal fistula, including 79 (17%) had fistula anterior to the Incisive Foramen (IF); 41 (9%) had fistula posterior to the IF; and 33 (7%) had fistula in a region that included area anterior and posterior to the IF. In the group of 153 individuals studied, 75 patients received surgical correction of the fistula; 4 patients had fistula obturated with palatal prosthesis, and 3 received unsuccessful surgery and were later treated with obturation. Clinical follow-up was done with the remaining group.

Conclusions: Palatal fistula was not managed surgically for about half of the population studied (46%). Implications for speech production will be discussed during the presentation.

(1) Hospital for Rehabilitation of Craniofacial Anomalies, University of Sao Paulo, Sao Paulo, Brazil, (2) Department of Orthodontics, Hospital for Rehabilitation of Craniofacial Anomalies, University of Sao Paulo, Sao Paulo, Brazil, (3) University of Sao Paulo, Sao Paulo, Brazil

Background/Purpose: This presentation presents a protocol for classification of palatal fistula based on morphological, embryological, and symptomatological criteria.

Methods/Description: The proposed protocol includes: (1) a definition of oronasal fistula (ONF); (2) the identification of anatomical land marks as reference points for the classification system; (3) the description of strategies for identification of the incisive foramen (IF) in the operated palate and for identification of the transition area between hard and soft palate; (4) the description of the embryological and morphological criteria for fistula classification; (5) the inclusion of symptomatology as an important aspect for fistula management decisions.

Results: Anatomical landmarks included incisive foramen (IF), upper alveolar arch, and transition area between the hard and soft palate. Using the embryological criteria, a fistula may be classified as PREIF (located in the region anterior to the IF), POSIF (located in the region posterior to the IF), and PREPO (fistula affecting both the regions: anterior and posterior to the IF). The morphological criteria included 5 regions: Region-1: prealveolar and/or at the alveolar arch; Region-2: hard palate anterior to IF; Region-3: hard palate posterior of IF; Region-4: the transition between hard and soft palate; Region-5: soft palate. The symptoms associated with the fistula may include hypernasality and other speech symptoms; otitis media and other otological symptoms; nasal regurgitation of food, and the possibility of asymptomatic fistula.

Conclusions: The new classification system for palatal fistula has named BROSCO-DUTKA and may be used during live intraoral evaluation as well as during the analysis of photographic images. Professionals involved in the management of the cleft lip and palate, including plastic surgeons, orthodontists, and speech pathologists, still need to validate the protocol in future research.

(1) University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, (2) Children’s Hospital of Philadelphia, Philadelphia, PA, (3) Children’s Hospital of Philadelphia, Division of Plastic Surgery, Philadelphia, PA

Background/Purpose: Patients with hemifacial microsomia (HFM) may undergo unilateral mandibular distraction (MDO) prior to skeletal maturity in an effort to improve facial symmetry. Some of these patients have mild obstructive sleep apnea, and MDO’s effect on airway volumes has not been quantified. This study aimed to tackle this shortcoming, using imaging to analyze the upper airway of patients with HFM before and after surgical intervention with MDO.

Methods/Description: We retrospectively reviewed patients with a diagnosis of unilateral HFM whom underwent unilateral MDO with a vertical vector at age 4 to 14 years at a single institution from 2004 to 2019. Patients with pre- and post-MDO 3-dimensional computed tomography (3D-CT) scans of the upper airway within 12 months of distractor placement and removal, respectively, were included. Head and neck postures were determined by cervical lordosis, pitch, roll, and yaw angles. Pre- and post-operative pharyngeal airway volumes (PAV), minimum retropalatal cross-sectional areas (RP CSA), and retroglossal cross-sectional areas (RG CSA) and associated anteroposterior (AP) distances were measured using Mimics 22.0 (Materialise). Comparison was done using paired t tests and linear mixed-effects models controlling for head and neck postures.

Results: Ten patients met inclusion criteria. Mean age at surgery was 8.5 ± 3.3 years, and mean duration between pre- and post-surgery scans was 230 ± 85.7 days. No statistically significant differences were found between pre- and post-operative PAV (t(10) = 0.88, P = .40), minimum RP CSA (t(10) = −0.35, P = .73), RP AP distance (t(10) = −1.28, P = .23), or RG AP distance (t(10) = 0.15, P = .88) when head posture was not corrected for. However, uncorrected preoperative minimum RG CSA was found to be significantly greater than postoperative measurements (t(10) = 2.40, P = .04). After adjusting for head posture, airway volume (χ₁ ² = 1.02, P = .31), RG CSA (χ₁ ² = 3.64, P = .06), RP CSA (χ₁ ² = 0.16, P = .69), RG AP distance (χ₁ ² = 0.04, P = .85), and RP AP distance (χ₁ ² = 0.09, P = .76) did not differ significantly between pre- and postsurgical time points.

Conclusions: Vertical vector MDO in patients with HFM is not associated with statistically significant changes in the dimensions of the upper airway. This is an important finding, as it may guide discussions surrounding risk/benefit ratio for MDO in childhood.

(1) University of Miami Miller School of Medicine, Miami, FL, (2) Nicklaus Children’s Hospital, Miami, FL

Background/Purpose: One-stage bilateral cleft lip repair is frequently espoused as the gold standard for bilateral cleft lip repair procedures. Two-stage bilateral cleft lip repair is an alternative method that, while not as universally accepted, may result in acceptable functional and aesthetic long-term outcomes. We propose and provide long-term follow-up for a different approach to bilateral cleft lip repair following the principles used for the unilateral cleft deformity. Although the downside of this surgical technique is that it involves an additional operation, in the long-term it may be an acceptable alternative to one-stage repairs.

Methods/Description: This is a retrospective review of all patients that underwent 2-stage repair for complete bilateral cleft lip deformity, performed by a single surgeon. Patients meeting inclusion criteria were in mixed dentition and were treated with the following protocol: (1) Pre-surgical naso-alveolar molding and approximation of alveolar segments, (2) Staged rotation advancements with gingivoperiosteoplasty and closure of alveolar defect extending back to closure of the anterior palate, (3) McComb nasal correction, (4) Rotation advancement lip repair just as is done in a unilateral cleft, (5) Repetition of the procedure on the contralateral side after 3 months, (6) Closure of the remaining hard palate and soft palate with levator muscle retroposition occurred at 18 months. Cephalometric and anthropometric evaluation at mixed dentition was conducted in order to evaluate maxillary growth. Mean anthropometric measurements for bilateral cleft lip patients treated with staged rotation advancements were compared to mean Farkas anthropometric values for noncleft patients (nasolabial angle, cutaneous/total upper lip height, and nasal tip protrusion/nose height). Mean cephalometric measurements for our cohort were also compared to cephalometric values for noncleft patients.

Results: Thirty-two patients were identified via retrospective review who met inclusion criteria. There was no significant difference between anthropometric values for normal versus cleft patients for nasolabial angle, cutaneous/total upper lip height, and nasal tip protrusion/nose height (P > .05). Anthropometric measurements for nasolabial angles, columella length, the lobule and philtral width fell within 1 to 2 standard deviations of the norm. Mean cephalometric values fell within 1 to 2 standard deviations for age-specific values for SNA (80.0 ± 3.7°), SNB (74.0 ± 3.4°), ANB (4.0 ± 1.4°), and facial axis (90 ± 3.5 mm) for noncleft patients.

Conclusions: Using the staged method, noses are normal with a normal nasolabial angle and a normal columella. Lips are full and pouting. There is no ventroflexion of the premaxilla. The results that can be obtained with the use of the staged rotation advancement procedure justify continuing its use. The long-term follow-up of this patient population has proven to have results that resemble the dimensions and ratios of the lip and nose of unaffected children.