The Cleft Palate Craniofacial Journal

To determine whether judgment of nasolabial esthetics in cleft lip and palate (CLP) is influenced by overall facial attractiveness.

Experimental study.

University of Bern, Switzerland.

Seventy-two fused images (36 of boys, 36 of girls) were constructed. Each image comprised (1) the nasolabial region of a treated child with complete unilateral CLP (UCLP) and (2) the external facial features, i.e., the face with masked nasolabial region, of a noncleft child. Photographs of the nasolabial region of six boys and six girls with UCLP representing a wide range of esthetic outcomes, i.e., from very good to very poor appearance, were randomly chosen from a sample of 60 consecutively treated patients in whom nasolabial esthetics had been rated in a previous study. Photographs of external facial features of six boys and six girls without UCLP with various esthetics were randomly selected from patients' files. Eight lay raters evaluated the fused images using a 100-mm visual analogue scale. Method reliability was assessed by reevaluation of fused images after >1 month. A regression model was used to analyze which elements of facial esthetics influenced the perception of nasolabial appearance.

Method reliability was good. A regression analysis demonstrated that only the appearance of the nasolabial area affected the esthetic scores of fused images (coefficient −11.44; P < .001; R² = 0.464). The appearance of the external facial features did not influence perceptions of fused images.

Cropping facial images for assessment of nasolabial appearance in CLP seems unnecessary. Instead, esthetic evaluation can be performed on images of full faces.

To compare the frequency and severity of obstructive sleep apnea (OSA) in middle-aged adults who underwent pharyngeal flap surgery for velopharyngeal insufficiency (VPI) with matched subjects who did not undergo pharyngeal flap surgery and to verify the relationship between OSA severity and internal pharyngeal dimensions.

National referral care center for cleft lip and palate.

Prospective study on 42 nonsyndromic subjects with repaired cleft palate with flap (F group, n = 22) and without flap (NF group, n = 20), aged 40 to 58 years. The main outcome measure was the apnea-hypopnea index (AHI), measured by in-lab nocturnal polysomnography (PSG). The OSA-related symptoms were investigated by Pittsburgh, Epworth, and Berlin questionnaires. The nasopharyngeal or velopharyngeal (NP/VP) cross-sectional area was measured by modified anterior rhinomanometry in subgroups of the F (n = 14) and NF (n = 10) groups at rest and during speech. Differences were considered significant at P < .05.

Questionnaire scores and frequency of self-reported symptoms of snoring, nasal obstruction, and breathing interruptions during sleep did not differ between groups. OSA was diagnosed by PSG in 60% and 77% of the NF and F subjects, respectively. The difference was not significant. No cases of severe OSA were observed. There was no correlation between AHI and NP/VP area.

A significant number of middle-aged adults with repaired cleft palate had OSA and related symptoms, regardless of the presence of a pharyngeal flap. Results suggest that VPI treatment with a flap may not cause sleep-disordered breathing in the cleft population. Besides advancing age, congenital upper airway abnormalities may be involved.

Due to the multidimensional nature of resonance disorders, multivariate diagnostic assessment is advisable. The nasality severity index (NSI) is based on this point of view. Because of the influence of personal and environmental variables on the current NSI, this study aims to refine this index.

Prospective case-control study.

Tertiary university hospital.

Forty-two patients with cleft lip and palate and 50 children without resonance disorders were tested.

Resonance was investigated by perceptual as well as objective measurements. A Nasometer was used to score nasalance, and spectral speech characteristics of a sustained sound /i:/ were determined, among which the voice low tone to high tone ratio (VLHR). Binary logistic regression analysis was performed to calculate the optimal index to discriminate patients from control children. Additionally, the validity of the index was determined based on data from an independent patient and control group.

The NSI 2.0, a weighted linear combination of three variables, can be obtained using the equation NSI 2.0 = 13.20 - (.0824 x nasalance /u:/ [%]) - (.260 x nasalance oral text [%]) - (.242 x VLHR 4.47*F0 [dB]). The NSI has a sensitivity of 92% and a specificity of 100%. Moreover, it has excellent validity (sensitivity 88%, specificity 89%).

The NSI 2.0 discriminates patients from control children with high sensitivity, specificity, and validity. This multiparametric method can offer a more powerful approach in the assessment and treatment planning of individuals with hypernasality.

To prevent the occurrence of postoperative long lip, longitudinal postoperative changes in nasolabial forms of patients with unilateral cleft lip who underwent primary lip repair with or without upward advancement of the nasolabial components were compared.

Forty-three subjects (24 unilateral cleft lip and palate [UCLP] and 19 unilateral cleft lip solely, and cleft lip and alveolus [UCL/UCLA]) who underwent primary lip repair with upward advancement of the nasolabial components (NA group) and 30 subjects (16 UCLP and 14 UCL/UCLA) without upward advancement (LA group) were enrolled.

Postoperative photos taken at 1 and 6 months and at 1, 2, and 3 years were used for measuring the heights of the nasal alar base (NBH), the columellar base (CBH), Cupid's peak (CPH), and the upper lip (ULH). The ratios of these measurements between the affected and unaffected sides were calculated in both groups.

In the LA group, the 3-year postoperative all-items ratios of UCLP were significantly larger than those at 1 month postoperatively, demonstrating drooping of the nasolabial tissues in the affected side (all P < .01). Furthermore, the 3-year postoperative CPH and ULH ratio of UCL/UCLA was significantly larger than that at 1 month postoperatively, demonstrating the long lip (P < .01). In the NA group, the NBH, CBH, and CPH ratios of both UCLP and UCL/UCLA did not show significant differences between 1 month and 3 years postoperatively.

Upward advancement of the nasolabial components prevents postoperative long lip.

With the gradual replacement of the ICD-9 coding system by the newer ICD-10 system, there is a need to critically evaluate how the system improves—or fails to improve—the coding of specific cleft and craniofacial anomalies. The following review examines the new system and the key components for practitioners who treat patients with such anomalies.

While seemingly beneficial in certain respects, the newer ICD-10 system remains far from ideal for cleft and craniofacial anomalies. With future iterations of the system, a more concerted effort to precisely code such anomalies is warranted, which will likely require input from key practitioners.

To study the growth of length-for-age (L/A), weight-for-age (W/A), and body mass index (BMI) of children with cleft lip and palate receiving a normal diet; to establish specific growth curves for children with cleft palate with or without cleft lip (CLP/ICP) who had not undergone palatoplasty and for children with isolated cleft lip (ICL); and to assess if CLP/ICP growth differed from ICL growth and if CLP/ICP and ICL growth differed from growth for typical children.

Prospective and cross-sectional study.

Hospital for Rehabilitation of Craniofacial Anomalies, Bauru, São Paulo, Brazil.

Weight and length of 381 children with cleft lip and palate and who were younger than 2 years were recorded and used to calculate W/A, L/A, and BMI growth curves. The 2006 World Health Organization growth charts were used as a reference for typical children. All children received a normal diet for age.

Children with CLP/ICP had median W/A and BMI growth curves below growth curves for typical children but showed spontaneous recovery starting at approximately 5 months of age, even with nonoperated cleft palate. Children with ICL had growth similar to that of typical children. Children with CLP/ICP, who initially had W/A and BMI values less than those of the ICL group, had W/A and BMI equal to or higher than the ICL group after 9 months of age.

Children with CLP/ICP had impaired W/A and BMI growth with spontaneous recovery starting early in childhood. This study established specific W/A, BMI, and L/A growth curves for children with cleft lip and palate.

The transverse maxillary deficiency frequently observed in patients with cleft lip and palate (CLP) is usually treated by rapid maxillary expansion (RME). Considering that RME causes a significant increase of the internal nasal dimensions in children with unilateral CLP (UCLP), this study aimed to characterize the internal nasal geometry of children with bilateral CLP (BCLP) and transverse maxillary deficiency using acoustic rhinometry. The study also aimed to analyze changes caused by RME.

Cross-sectional prospective study.

Laboratory of Physiology, Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo, Bauru, SP, Brazil.

Fifteen children with repaired BCLP of both genders, aged 8 to 15 years, referred for RME, were prospectively analyzed.

Subjects underwent acoustic rhinometry before the expander installation and after the active phase of expansion. Cross-sectional areas (CSA) and volumes (V) of the nasal valve regions (CSA₁ and V₁) and turbinates (CSA₂, CSA₃, and V₂), were measured after nasal decongestion.

In the majority of the subjects, an increase of internal nasal dimensions was observed.

Percent changes of CSA₁, CSA₂, CSA₃, V₁, and V₂ were: +25%, +11%, +9%, 20%, and +12%, respectively. Differences were significant for all variables studied, except CSA₃ (P < .05).

RME promotes an increase in the internal nasal dimensions of children with BCLP, suggesting that RME is capable of substantially improving nasal patency in this population.

To analyze surgical complications after primary cleft palate repair in a setting with limited resources.

A total of 1608 consecutive cleft palate repairs with 1408 follow-ups, operated upon between 2011 and 2013, were reviewed retrospectively through medical records. Patients were 10 months to 50 years old at the time of surgery, with a median age of 9 years.

Guwahati Comprehensive Cleft Care Center, Guwahati, India.

Primary cleft palate repair.

Postoperative complications in terms of necrosis, dehiscence, fistula, infection, and “hanging palate” were assessed, as was perioperative bleeding. Logistic regression was used with complication (yes/no) as the binary dependent variable and with age, cleft type, and surgeon (visiting/long-term) as covariates.

The overall incidence of postoperative complications was 16.9% with a fistula rate of 13.6%. The incidence of perioperative bleeding was 1.8%. Logistic regression analysis identified cleft severity (P ≤ .001) and visiting surgeon (P ≤ .01) as factors related to the incidence of postoperative complications. Age at surgery was related to both the incidence of postoperative complications (P ≤ .001) and perioperative bleeding (P < .05).

Due to increased risks of surgical complications, older patients with complete clefts should only be operated upon after careful consideration. In addition, these patients should be assigned to surgeons experienced with this cleft type.

The aim of this study is to identify risk factors associated with complications and readmissions following cleft lip repair using the multicenter American College of Surgeons National Surgical Quality Improvement Program (ACS NSQIP) Pediatric.

Patients undergoing CPT codes 40700, 40701, and 40702 were extracted from the ACS NSQIP Pediatric. Fisher exact, χ², and rank-sum tests were used to evaluate risk factors.

Of the 525 patients included, 4.2% had complications, with respiratory complications being the most common. Risk factors included congenital malformations (P = .001), ventilator dependence (P = .002), oxygen support (P = .016), tracheostomy (P = .005), esophageal/gastric/intestinal disease (P = .007), impaired cognitive status (P = .034), acquired central nervous system abnormality (P = .040), nutritional support (P = .001), major and severe cardiac risk factors (P = .011 and P = .005), and an American Society of Anesthesiologists score of 3 (P = .002). In addition, complications were associated with undergoing a one-stage bilateral repair (P = .045) or concomitant ear, nose, and throat procedure (P = .045). The readmission rates for ambulatory patients and inpatients were 2.6% and 4.9% (P = .556), with an overall readmission rate of 4.6%. Ambulatory patients were older (P = .005) and had shorter operative times (P < .001).

Perioperative complications are low following cleft lip repair, with respiratory complications being the most common. Readmission rates of 4.6% are higher than expected, and insight into predictors of complications will allow surgeons to identify patients who could benefit from additional resources.

The study was carried out to learn if the columellar-frenulum angle could be used as one of the most important anthropometric measurements to predict the clinical severity in unilateral cleft lip patients.

This is a prospective case series conducted at Banaras Hindu University, Varanasi, India.

The study was based on the Thomson and Reinders criteria (1995), and various anthropometric measurements, including the columellar-frenulum angle, were measured. A total of 30 patients (21 boys and 9 girls) were included in the study. The ages of the children varied from 4 months to 15 years. The left side was affected in 18 patients, and 2 gave a significant positive family history. All the patients were subjected uniformly to Tennison's cleft lip repair. Patients with strictly unilateral complete cleft lip and palate were included in the study. Microform clefts, incomplete cleft lips, and bilateral clefts were excluded from the study.

The data was analyzed using Pearson's correlation coefficient.

The CF angle correlated negatively with the soft tissue defect of the cleft, cutaneous lip length, and vermilion lip length discrepancies from normal and soft tissue width just behind the alveolus.

CF angle promises to offer a method by which prognosis of clefts can be predicted in terms of its severity in patients with unilateral cleft lip.

To analyze indications and outcomes for primary premaxillary setback.

Retrospective.

Academic children's hospital.

All children with bilateral complete cleft lip age ≤2 years of age who had premaxillary setback by one surgeon (1992 to 2011).

Twenty-five patients with bilateral complete cleft lip underwent primary premaxillary setback at an average age of 9 months; the mean follow-up was 47 months. There were three indications: failed dentofacial orthopedics (n = 9), delayed referral precluding manipulation (n = 10), and intact secondary alate (n = 6). Of 19 patients with bilateral complete cleft lip/palate, primary setback was combined with nasolabial repair (n = 11), adhesions (n = 2), or palatoplasty (n = 6). Patients who had nasolabial closure and setback were significantly younger than those who had combined palatal closure and setback (6.5 versus 16 months, P = .01). No patient exhibited postoperative premaxillary instability. Serial anthropometry showed similar growth of nasolabial features after both primary setback (n = 9) and active dentofacial orthopedics (n = 35).

Primary premaxillary ostectomy and setback permits synchronous bilateral nasolabial-alveolar closure or alveolar-palatal repair in a child with intact secondary palate. This procedure should be considered whenever dentofacial orthopedics cannot be accomplished. Speech is paramount in an older child; setback with palatal closure is scheduled before nasolabial repair. Disturbance of midfacial growth is likely following primary premaxillary ostectomy and setback in patients with bilateral complete cleft lip/palate; however, most already need maxillary advancement. Furthermore, premaxillary setback permits proper primary nasolabial design and construction in appreciation of expected changes with growth.

To determine whether there are differences in the anatomical cleft measurements between patients with isolated cleft palate (CP) with or without Pierre Robin sequence (PR) and those with cleft lip and palate (CLP).

Prospective case series.

Tertiary care university-based medical center.

From 1993 to 2012, CP and CLP patients were prospectively enrolled in a database.

Several anatomical cleft parameters were recorded with precise measurements at the time of palatoplasty under general anesthesia.

One hundred twenty-five patients were included in our study: 50 patients in the CP without PR group, 87 patients in the CP with or without PR (CPPR) group, and 38 patients in the CLP group. Patients in the CLP group had increased values for the following parameters compared to patients with CP and patients with CPPR: nasopharyngeal depth (P = .02 and P = .01, respectively); cleft width at posterior hard palate border (P < .001 and P < .001); width of the dental arch (P < .001 and P < .001); and total cleft length (P < 01 and P < .001). For cleft width at the soft palate level, there was a significant difference between the CP and CLP groups (P = .005). We found no statistically significant differences between the groups (CLP versus CP and CPPR, respectively) for the length of the soft palate (P = .74, P = .21) or for the length of the hard palate (P = .19, P = .08).

There are significant differences in cleft and palate measurements between CLP and CP patients that may be useful to predict the occurrence of velopharyngeal insufficiency or other complications.

Cleft lip and/or palate (CL/P) is one of the most common birth conditions in the world, but little is known about its causes. Professional opinion remains divided as to which treatments may be the most beneficial for patients with CL/P, and the factors that contribute to psychological adjustment are poorly understood. The use of different methodological approaches and tools plays a key role in hampering efforts to address discrepancies within the evidence base. A new UK-wide program of research, The Cleft Collective, was established to combat many of these methodological challenges and to address some of the key research questions important to all CL/P stakeholders.

To describe the establishment of CL/P cohort studies in the United Kingdom and to consider the many opportunities this resource will generate.

To date, protocols have been developed and implemented within most UK cleft teams. Biological samples, environmental information, and data pertaining to parental psychological well-being and child development are being collected successfully. Recruitment is currently on track to meet the ambitious target of approximately 9800 individuals from just more than 3000 families.

The Cleft Collective cohort studies represent a significant step forward for research in the field of CL/P. The data collected will form a comprehensive resource of information about individuals with CL/P and their families. This resource will provide the basis for many future projects and collaborations, both in the United Kingdom and around the world.

This study aimed to assess the safety and feasibility of transoral robotic surgery for the reconstruction of soft palatal clefts.

The application of transoral robotic surgery for soft palate muscle reconstruction was investigated. The da Vinci Surgical Robot was first used on a cadaver to assess the optimal positioning of the patient and the robot. The robot was then used for the dissection and reconstruction of palatal muscles in 10 consecutive patients with palatal clefts. The procedures were documented using video and still photography. A group of 30 control patients were subjected to surgery with manual instruments. Surgical and clinical outcomes were evaluated with at least 6 months of follow-up (8 ± 1 months).

The use of the surgical robot on a cadaver provided great dexterity and excellent 3D depth perception. The transoral access was efficient and safe for the precise dissection, reorientation, and suturing of palatal muscles. In our series, the surgical duration was longer for the robotic approach than for the manual approach (87 ± 6 minutes versus 122 ± 8 minutes, P < .0001). No intraoperative or postoperative complications occurred in either group.

A robotic surgical approach can be used safely for palatal surgery. We believe that the precise dissection of the palatal muscles provided by the robotic system might reduce damage to the vascularization and innervation of these muscles, as well as damage to the mucosal surfaces that could cause fistula formation. In addition, this technique might improve palatal function and Eustachian tube function in cleft palate patients.

To identify the oral and nasal microbial profile of cleft palate children and control children and to reveal interrelationships between the microbiome and the high prevalence of infectious diseases.

Saliva and nasal samples of 10 cleft palate children and 10 age-matched control children were analyzed. Total microbial genomic DNA was isolated, polymerase chain reaction-denaturing gradient gel electrophoresis was applied to obtain fingerprints, and selected bands on fingerprints were sequenced.

The results revealed a significantly lower saliva microbial diversity in cleft children and a different microbial component in both saliva and nares in children with cleft palate. A higher component similarity between the oral and nasal samples was found in the cleft group than in the control group. Lautropia species and Bacillus species were significantly less present among the saliva samples of cleft group. Dolosigranulum species and Bacillus species were significantly fewer in the nasal cavity of cleft group. Streptococcus species became much more predominant in the nasal cavity of the cleft group than in that of the control group.

A disturbed ecological ecosystem is found in oral and nasal microbiome of children with cleft palate as a consequence of the abnormal communication between the two cavities. Further studies are needed to explore the relationship between the disturbed microbiome and diseases.

Parry-Romberg syndrome (PRS) is a rare craniofacial disease that causes progressive hemifacial atrophy of the soft tissue before spontaneously entering remission. Autologous fat grafting may provide a less invasive alternative, producing aesthetically pleasing results while avoiding the need for traditional microsurgical free flap coverage.

A systematic review of the literature was conducted. Inclusion and exclusion criteria were applied. The case report highlights the technique using two-dimensional and three-dimensional photography.

Our review yielded 31 articles in addition to our case describing 147 cases of lipofilling to correct PRS soft-tissue defects. Patients underwent an average of 2.2 procedures, receiving on average 95 mL of grafted fat. Disease severity was classified into mild (41%), moderate (42%), and severe (17%) in the identified patients. Increasing disease severity correlated with an increasing number of procedures and fat-grafting volumes to achieve adequate aesthetic outcomes (mean, 1.5 and 38 mL; 2.3 and 81 mL; 3.7 and 129 mL, respectively). Reported benefits over flap-based reconstructions included reductions in cost (40%), operative time (50%), donor-site morbidity (52%), and rate of complications (33%). Aesthetic benefits cited included improved skin quality (65%), more natural contours (1%), and more natural facial expressions (10%).

Fat grafting for correction of PRS-associated soft-tissue defects is receiving heightened acceptance for its ability to restore natural facial contours. While additional fat-grafting procedures may be required with increased disease severity, autologous fat grafting may be a beneficial option as a sole modality to correct PRS-associated soft-tissue atrophy.

Oral cleft is a common craniofacial birth defect that leads to long-lasting adverse outcomes. In Thailand, there have been two studies of the prevalence of oral clefts using data from university hospitals during 1969 through 1978 and 1988 through 1999, which found prevalence rates of 1.23 and 1.22 per 1000 live births, respectively.

The primary outcome was to assess the prevalence of oral clefts from the birth defects registry during 2009 through 2013 in three provinces in southern Thailand. The secondary outcomes were to correlate the risk of oral cleft and maternal age.

Population-based study.

Four hundred sixty-seven hospitals in three provinces in southern Thailand.

Oral cleft cases and maternal data—including live births, stillbirths, and termination of pregnancy following a prenatal diagnosis—were collected from the birth defects registry.

Of the total 186,393 births, there were 269 oral cleft cases, giving an average prevalence of 1.44 per 1000 births (95% CI, 1.22-1.63). The most common cleft type was cleft lip and palate (45.0%), followed by cleft palate (29.0%), with 15.6% syndromic cleft. The mean maternal age was 28.0 ± 6.4 years. There were no differences in prevalence of oral clefts among the different maternal age groups. However, advanced maternal age 35+ years was associated with syndromic cleft children.

The prevalence of oral clefts was 1.44 per 1000 live births, with 15% of cases having an associated congenital anomaly or a recognized syndrome. Increased maternal age was associated with a higher prevalence of syndromic cleft.

This study uses the American College of Surgeons Pediatric National Surgical Quality Improvement Program (ACS NSQIP Pediatric), a multicenter database, to identify risk factors for complications after cleft palate repair.

Patients undergoing Current Procedural Terminology (CPT) codes 42200 and 42205 were extracted from the 2012 ACS NSQIP Pediatric. Patients older than 36 months or those who had undergone an additional surgery that altered the risk were excluded. Outcome variables were combined to create a complication variable. Fisher's exact, Pearson chi-square, and Wilcoxon rank-sum tests were used for analysis.

Eligibility criteria were met by 751 patients. Of these, 192 (25.6%) had unilateral clefts, 146 (19.4%) bilateral, and 413 (55.0%) were unspecified. The average age at time of surgery for those without and with complications was 421.1 ± 184.8 and 433.6 ± 168.0 days, respectively (P = .76). Of the 21 (2.8%) patients with complications, respiratory complications were the most common. Risk factors associated with complications included American Society of Anesthesiologists classification of 3 (P = .003), nutritional support (P = .013), esophageal/gastric/intestinal disease (P = .016), oxygen support (P = .003), structural pulmonary/airway abnormality (P = .011), and impaired cognitive status (P = .009). Patients undergoing concurrent laryngoscopy (P = .048) or other surgeries (P = .047) were also found to be associated with increased complications. The 30-day fistula rate was 0.5%, and the readmission rate was 1.9%.

Perioperative complications for primary palatoplasty were 2.8% according to the ACS NSQIP Pediatric. Preoperative patient-related factors as well as concurrent surgeries may affect 30-day complication rates. These results help target those at greater risk for complications and allow for appropriate interventions to mitigate risks.