The Cleft Palate Craniofacial Journal

To assess the relationship between high intake of folic acid and positional plagiocephaly.

Retrospective case-control study with questionnaires administered in the clinic to biological mothers of children with positional plagiocephaly (PP group) and mothers of children without it (CO group).

Tertiary, institutional clinic. University Hospital of Maastricht, The Netherlands.

Exclusion criteria: mothers of children with syndromic disease or bone deformation and of children with clearly continuous forces on the skull (torticollis). A total of 94 mothers were included in the PP group (response rate: 85%), and 74 were included in the CO group (response rate: 75%). Final sample sizes were 75 in the PP group and 54 in the CO group.

The recommended daily dosage of folic acid in The Netherlands is 400 μg. The primary outcome was folic acid dosage in relation to the recommended dosage. A secondary outcome was duration of folic acid intake in relation to the recommended period of use during pregnancy.

In the PP group, 20% used double the recommended dosage of folic acid, compared with 6% in the CO group (p < .05). In the CO group, 59% used folic acid longer than recommended compared with 56% in the PP group (not significant). There were no differences in baseline characteristics except for gestational period: 39.5 weeks in the PP group and 37.9 in the CO group (p < .05).

Excessive daily intake of folic acid during pregnancy is seen among mothers of positional plagiocephaly patients.

Evaluation of the effect of presurgical orthopedics on maxillary archform up to 6 months of age.

Retrospective, single-blinded, case-control study.

Study model sets of 75 infants with consecutive, nonsyndromic, complete unilateral cleft lip and palate (excluding Simonart bands) from 1995 to 2005.

All patients (PSO group, n = 14; non-PSO group, n = 61) received lip repair/vomer flap at 3 months and soft palate repair at 6 months by the same consultant surgeon. The two groups were comparable at birth (p > .01) in all archform variables.

Sixteen variables were computed, following single-blinded analysis using the Reflex Microscope to describe the archform in the transverse, anteroposterior, and vertical dimensions and the arch circumference. Data were analyzed using a repeated-measures hierarchical analysis of variance with a significance level of 1%.

Repeatability studies showed good measurement precision. Presurgical orthopedics produced no statistically significant mean change in any archform variable when compared with the non-PSO group. The difference in the mean reduction in the alveolar cleft width between the groups was 0.69 mm (95% confidence interval, −0.89 to 2.28 mm, p = .52). Lip repair produced greater change in archform than did presurgical orthopedics, reducing the mean alveolar cleft width by 4.45 mm (95% confidence interval, 3.53 to 5.37 mm; p < .001).

There was no evidence that presurgical orthopedics produced any significant effect on archform, raising questions for its continued use in this context. Lip repair had a greater impact on arch dimensions than did presurgical orthopedics.

The purpose of this study was to evaluate subperiosteal release of the floor of the mouth (SRFM) musculature to correct neonatal airway obstruction in children with Pierre Robin sequence and to compare this procedure with other surgical options available.

A retrospective chart review of patients who underwent a SRFM for neonatal severe airway obstruction unresponsive to conservative management was performed. Preoperative and postoperative oxygenation parameters, sleep studies, feeding difficulties, and long-term evolution were documented.

Thirty-one consecutive patients between 1995 and 2009, 19 boys and 12 girls, had an SRFM procedure to alleviate severe neonatal airway obstruction. Eight children were syndromic, 15 had associated malformations, and eight had an isolated Pierre Robin sequence. The airway obstruction was corrected with SRFM alone in 26 children (84%), three patients remained dependent on nasopharyngeal airway, and two required subsequent mandibular osteogenic distraction procedures following the SRFM to manage persistent obstruction. No patient required a tracheostomy. The average age at SRFM was 33 days, and the patients were kept intubated for an average of 6 days postoperatively. Average hospital stay after the surgery was 45 days, and 10 patients went home with gavage feeding.

The SRFM is effective for the treatment of severe airway obstruction in patients with Pierre Robin sequence. The procedure is simple and has fewer complications and secondary procedures than other surgical options for correction of the airway obstruction.

To characterize airway obstruction before and after tongue-lip adhesion in children with micrognathia using polysomnography.

Retrospective pilot case series.

Evaluation of all children with micrognathia who underwent tongue-lip adhesion and polysomnography before and after surgery from 2002 to 2007 (N = 8).

Eight children met inclusion criteria; six were girls. The mean interval between polysomnography and tongue-lip adhesion was 6 days (range, 2 to 13 days) preoperatively and 17 days (range, 5 to 32 days) postoperatively. Severe obstructive sleep apnea was identified in seven of eight (88%) children, with a mean preoperative obstructive apnea hypopnea index of 52.6 events per hour (range, 7.1 to 85.7 events per hour). None had significant central sleep apneas (>5 per hour). Tongue-lip adhesion resulted in a mean decrease of 34.5 events per hour (range, −65.8 to 71.6 events per hour). After tongue-lip adhesion, seven of eight (87.5%) patients had an improved obstructive apnea hypopnea index, with resolution of obstructive sleep apnea in one child and improvement to mild (two) and moderate (two) obstructive sleep apnea in four others. Only one child had an obstructive apnea hypopnea index that increased after tongue-lip adhesion. Peak end-tidal pCO₂ measurements were elevated in all eight children before surgery at a mean of 60 mm Hg (range, 52 to 76 mm Hg) that improved to 51 mm Hg (range, 45 to 59 mm Hg), with normal peak levels in four children. Oxygen saturation nadir improved from 73% (range, 58% to 81%) to 82% (range, 65% to 94%).

Tongue-lip adhesion may be performed in micrognathic infants to alleviate airway obstruction. Polysomnographic evaluation in this pilot study before and after surgery suggests that tongue-lip adhesion usually improves obstructive sleep apnea, but only 38% had complete resolution. Future studies of tongue-lip adhesion efficacy should include Polysomnographic evaluation.

The tongue-lip adhesion has undergone several modifications in an attempt to reduce surgical complications and failure rates. Current techniques rely on the use of a button at the tongue base for suspension, which raises concerns about possible aspiration and interference with oral motor function and bottle-feeding. A new technique for tongue-lip adhesion is proposed that adds a tongue suspension to the standard surgical adhesion.

A total of 22 patients with Pierre Robin sequence who received a tongue-lip adhesion via a tongue suspension technique were reviewed. The surgical technique differs from the standard surgical approach by the use of a suture weave across the base of the tongue instead of a standard button to suspend the tongue anteriorly.

Average age at the time of tongue-lip adhesion was 13.9 days, with a mean operative time of 88.8 minutes. A marked improvement in postoperative oxygenation was seen in the majority of patients. One dehiscence occurred secondary to a traumatic postoperative extubation, eventually requiring a tracheostomy for subglottic stenosis.

A technical innovation for performing a tongue-lip adhesion using a tongue suspension in conjunction with a standard transverse adhesion of the lip is described. The advantage of the tongue-lip adhesion with suspension includes immediate postoperative extubation, as well as removal of concerns regarding button aspiration and possible interference in early developmental oral motor function and bottle-feeding. This technique is reproducible, expanding the craniofacial surgeon's armamentarium for the management of difficult airways in Pierre Robin sequence.

Surgical management for severe obstructive sleep apnea has been tracheostomy, which has significant morbidity.

To determine the efficacy of internal mandibular distraction in treating severe obstructive sleep apnea in infants and neonates.

Retrospective review of medical records of 29 patients who underwent internal mandibular distraction for obstructive sleep apnea secondary to micrognathia.

Nonprofit, academic, pediatric medical center.

A total of 29 infants with obstructive sleep apnea were studied. Nine were included in the respiratory failure group requiring intubation prior to distraction surgery. The other 20 were included in the respiratory distress group and underwent preoperative polysomnography that assessed the severity of obstructive sleep apnea as measured by the apnea-hypopnea index. One patient expired following surgery; the remaining 28 underwent postoperative polysomnography determining their postoperative apnea-hypopnea index.

Bilateral mandibular distraction with internal microdistractors.

Improvement in the apnea-hypopnea index or extubation.

The nine respiratory failure patients avoided tracheostomy and were successfully extubated postdistraction. Eight in this group had postoperative polysomnographies showing a mean apnea-hypopnea index of 3.13 (range, 0 to 13.9). All 20 patients in the respiratory distress group underwent polysomnography and showed improved apnea-hypopnea indices (p < .001). The mean pre-op apnea-hypopnea index was 39.7 (range, 4.5 to 177), and the mean post-op apnea-hypopnea index was 5.8 (range, 0 to 34). Average improvement in the apnea-hypopnea index was 33.9. The mean follow-up period was 18.7 months (1.6 to 45.2 months).

Infants with micrognathia and obstructive sleep apnea may avoid tracheostomy and its inherent risks and complications by undergoing internal mandibular distraction, which is a viable alternative to tracheostomy.

To investigate whether sodium supplementation increases weight gain in babies with grade 3 Pierre Robin sequence if their urinary sodium is low.

Retrospective review of all medical, dietitian, and nursing notes of babies admitted from 2000 to 2007, inclusive.

Tertiary center for cleft care.

A total of 33 babies requiring nasogastric and nasopharangeal intubation for Pierre Robin sequence were identified. Those for whom weights could not be measured accurately (n = 4) were excluded from further study.

Urinary sodium was low and weight gain poor in 21 of the remaining 29 babies (72%), and oral sodium supplementation was given to establish and maintain a normal urinary sodium (10 to 20 mmol/L).

Mean daily weight gain.

The mean daily weight gain of the 18 babies with low urinary sodium admitted in the first few days of life improved from 20 to 34 g (p < .001) following oral sodium supplementation. This gave a mean difference of 14 g (lower quartile = 6 g, upper quartile = 25 g, 95% confidence interval = 20, 90% confidence interval = 10). The mean daily weight gain of the eight patients who did not require sodium supplementation was 33.6 g.

Urinary sodium tests are simple and noninvasive and allow monitoring of urinary sodium. If low, adequate supplementation improved weight gain (p < .001), reversing failure to thrive in babies with severe Pierre Robin sequence. These findings have been unreported previously.

To use finite element analysis animated simulations to investigate factors affecting velopharyngeal closure.

A coronal section multicomponent finite element analysis model of a human soft palate was created in Simulia Abaqus 6.5–1 from high resolution WIR I images of a single adult female subject, interpreted by reference to published anatomic dissections. Tissues were assigned hyperelastic property coefficients for neo-Hookean behavior, with gravity at 9.8 ms^–2 in the y-axis. Vector forces based on estimations in previous publications were applied throughout levator veli palatini and palatopharyngeus muscles, using a nonlinear analysis algorithm, to produce animated simulations of velopharyngeal space closure. Variation of levator veli palatini angle from 60° to 49°, the contribution of palatopharyngeus muscle, and the effect of submucous cleft were investigated for their effects on velopharyngeal closure.

The animated simulations showed anthropomorphic behavior and supported the previously suggested effects of the levator veli palatini angle, with reduced effectiveness of velopharyngeal closure as levator veli palatini angle decreases. Palatopharyngeus action reduced the efficiency of closure for a levator veli palatini angle of 60°, and a submucous cleft reduced this for both our normal subject and for a levator veli palatini angle of 60°, but both palatopharyngeus action and a submucous cleft enhanced closure for a levator veli palatini angle of 49°.

This study advances soft palate finite element analysis to a real-subject-based multicomponent hyperelastic model that demonstrates anthropomorphic behavior. Animated simulations using the model demonstrate the possible effects of levator veli palatini angle, a submucous cleft, and the contribution of the palatopharyngeus.

As an initial step to a complex reconstruction model for virtual surgery, the present study was carried out to provide data on the prenatal cleft lip and palate uvular muscle in eight specimens.

Serial sections of viscerocrania of 18 aborted embryos and fetuses were studied microscopically and segmented manually. Registration, three-dimensional reconstruction, and finite element analyses were conducted.

Incompletely clefted uvulae showed anterior fusion and dorsal fission of the bilateral uvular muscle bodies. A complete cleft lip and palate specimen evidenced single bilateral uvular muscle bodies lying medially and orally below the cleft shelf, its central longitudinal fibers running beneath the oral-median mucosa. In incompletely clefted uvulae, 10% to 50% of circular peripheral fibers crossed the midline within the central third of the anterioposterior muscle, behind the levator loop. Of the fibers, 30% to 60% crossed to the ipsilateral palatopharyngeus muscle. Fibers inserted into the uvular basal membrane in a 60% nasal and 40% oral distribution at the middle third of the macroscopically clefted uvula. The macroscopic uvula itself consisted of loose connective tissue and salivary glands. Deformation analysis did disclose local stress, suggesting the uvular muscle contributes to velopharyngeal closure in normal anatomy and extends the cleft edges in cleft palate.

Cleft lip and palate reconstruction should reasonably use the uvular muscle to augment the velar midline bulk. Uvular muscle deformation calculation was successful, permitting functional insight on the basis of microanatomical specimens, so far a bigger complete velar model can be ventured.

The objective of this study was to determine the developments, morphometric features, and shapes of the maxillary and mandibular arches during the prenatal period in a large series of fetal material.

The study was carried out on 225 human fetuses aged between 8 and 40 weeks of gestation. General, extraoral, and intraoral parameters were taken from each fetus. The width and depth measurements and the shapes of the maxillary and mandibular arches were achieved using the casts obtained. The arches were categorized according to the shapes of the anterior and posterior regions of the arches.

Palatal and mandibular indices did not change during the prenatal period. Maxillary arch measurements were greater than the mandibular measurements. Moreover, width measurements of both arches were greater than the depth measurements during fetal life. We found that the frequencies of triangular maxillary and mandibular arches were highest in the first and second trimesters; whereas, the square model became more frequent in the third trimester and at full term. Classification of the arches according to their posterior regions showed that the tips of the arches shifted medially toward the term. There were significant correlations between width and depth parameters of the arches and extraoral parameters.

Data obtained in the study would be useful for clinicians by presenting an objective scale related to the prenatal period as well as being helpful in assessing the development of this region, identifying the anomalies and pathologies, and assisting treatment.

To analyze the prevalence and severity of gingival recession in individuals with cleft lip and alveolus with or without cleft palate.

Cross-sectional.

Tertiary referral hospital.

A total of 400 individuals with cleft lip and alveolus with or without cleft palate, aged 15 to 49 years, without any previous periodontal treatment.

Gingival recession was evaluated by measuring the distance from the cementoenamel junction to the most coronal point on the gingival margin, analyzing the prevalence and scoring its severity as mild (<3 mm), moderate (3 to 4 mm), or severe (4 mm). The prevalence and severity of gingival recession was compared between sextant 2 (cleft sextant) and the mean of the other sextants (1, 3,4,5, and 6: noncleft sextants). The relationship between gingival recession in the cleft area and gender was evaluated and data were analyzed statistically. The correlation between gingival recession and age was assessed also.

There was no statistically significant gender difference in gingival recession. The prevalence and severity of gingival recession increased with age, and most of the affected teeth were premolars and molars. The cleft area did not present a higher prevalence and severity of gingival recession.

Individuals with cleft lip and alveolus with or without cleft palate presented the same prevalence and severity of gingival recession compared with other populations. The area adjacent to the cleft (sextant 2) did not show greater risk of gingival recession than other areas (sextants 1, 3, 4, 5, and 6).

The literature suggests that individuals with history of cleft lip and palate who present with midfacial growth deficiency are at higher risk of presenting lisping. The relationship between distortions during production of linguoalveolar fricative sounds and the severity of malocclusion, however, has not been established for the population with cleft.

To study the association between lisping and dental arch relationship.

Speech samples and dental arch casts were obtained from 106 children with operated unilateral cleft lip and palate (UCLP) during the stage of mixed dentition and before orthodontic treatment. Videotaped productions of the phrase lu saci saiw sedul were rated by speech-language pathologists for the identification of lisping during [s]. Dental arch casts were rated by orthodontists using the Goslon Yardstick and the Five-Year Index to establish dental arch relationship.

Multiple logistic regression showed no significant association between lisping and dento-occlusal index (p = .802) and age (p = .662). Substantial interjudge agreement during auditory-perceptual ratings was found (kappa = .63). Almost perfect agreement was found between orthodontists while establishing the dental arch relationship (kappa = .81).

This study failed to reveal an association between lisping and dental arch relationship in children with operated UCLP. Multiple variables may play a role in determining occurrence of lisping, warranting further investigation.

Antiretroviral prophylaxis has been found to be effective in preventing vertical HIV transmission to the offspring of infected mothers. Because medicine and the art of public health require benefits to outweigh any plausible risks, our study aimed to explore and quantify preliminary associations between antiretroviral medications and clefting.

We analyzed 5 years of available data from the Food and Drug Administration's Adverse Events Reporting System (Medwatch program) and calculated reporting odds ratios (RORs) and their associated 95% confidence intervals (CIs).

The medications with the highest effects were efavirenz with an ROR of 196 (95% CI, 86 to 447), lamivudine with an ROR of 60.2 (95% CI, 14.25 to 148), the combination abacavir sulfate/lamivudine/zidovudine with an ROR of 59.3, and nelfinavir with and ROR of 50.5, followed by nevirapine, lopinavir/ritonavir, and lamivudine/zidovudine.

Given the multifactorial etiology of cleft lip and palate, further studies are needed to assess the relative safety of antiretroviral prophylaxis and the specific conditions or potential synergies that might lead to the development of this defect.