Onset and Resolution of Chiari Malformations and Hydrocephalus in Syndromic Craniosynostosis following Posterior Vault Distraction.

BACKGROUND: Patients with syndromic craniosynostosis have an increased incidence of progressive hydrocephalus and Chiari malformations, with few data on the relative benefit of various surgical interventions. The authors compare the incidence and resolution of Chiari malformations and hydrocephalus between patients undergoing posterior vault distraction osteogenesis (PVDO) and patients undergoing conventional cranial vault remodeling. METHODS: Patients with syndromic craniosynostosis who underwent cranial vault surgery from 2004 to 2016 at a single academic hospital, with adequate radiographic assessments, were reviewed. Demographics, interventions, the presence of a Chiari malformation on radiographic studies and hydrocephalus requiring shunt placement were recorded. Mann-Whitney U and Fisher's exact tests were used as appropriate. RESULTS: Forty-nine patients underwent PVDO, and 23 patients underwent cranial vault remodeling during the study period. Median age at surgery (p = 0.880), sex (p = 0.123), and types of syndrome (p = 0.583) were well matched. Patients who underwent PVDO had a decreased incidence of developing Chiari malformations postoperatively compared with the cranial vault remodeling cohort (2.0 percent versus 17.4 percent; p = 0.033). Not surprisingly, no significant difference was found between the groups with regard to the incidence of postoperative hydrocephalus requiring shunt placement (PVDO, 4.1 percent; cranial vault remodeling, 4.3 percent; p = 0.999). CONCLUSIONS: As expected, PVDO did not significantly affect intracranial hydrodynamics to the extent that hydrocephalus shunting rates were different for patients with syndromic craniosynostosis. However, PVDO was associated with a reduced risk of developing a Chiari malformation; however, prospective evaluation is needed to determine causality. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

International Adoptees With Cleft Lip and/or Palate: Lessons Learned From a Growing Population.

BACKGROUND: International adoptees with cleft lip and palate (CLP) are a growing population in the United States. They represent a clinical challenge, presenting at various ages and stages of cleft repair. METHODS: A retrospective review of patients seen at the CLP Program at the Children's Hospital of Philadelphia (CHOP) between 1998 and 2012 with a history of international adoption was performed. Demographics, surgical histories, and long-term speech outcomes were reviewed. RESULTS: Seventy-four female and 77 male patients were evaluated. Patients were adopted at an average age of 2.3 years (range, 0.4-8.6 years); 80.8% (n = 122) of patients were adopted from China. The rate of international cleft adoption increased by approximately 1.5 patients per year (r = 0.7739, P < 0.001); 13.2% (n = 19) of all subjects with cleft palates had oronasal fistulas (ONFs) that required repair. The ONF rates for primary palatoplasties at CHOP were significantly lower compared to both preadoption repairs (P = 0.002) and postadoption repairs at outside hospitals (P = 0.01); 14.8% (n = 21) of all patients had secondary surgeries for velopharyngeal incompetence (VPI). Rates of secondary surgery for VPI were also significantly lower for primary palatoplasties at CHOP compared to both preadoption repairs (P = 0.0018) and postadoption repairs at outside hospitals (P = 0.0033). CONCLUSIONS: International adoptees with CLP are a growing population and are clinically challenging with high ONF rates and high secondary surgery rates for VPI. We recommend expedited repair of unoperated cleft palates in adoptees older than 18 months. Adopted patients with CLP should be rigorously evaluated for the need for speech therapy and secondary surgeries to correct for VPI.

Economic Analysis of Cleft Palate Repair in International Adoptees.

DESIGN: Retrospective cohort study. SETTING: Major international tertiary care referral center for cleft palate repair. PATIENTS: One hundred thirty-eight patients at the Children's Hospital of Philadelphia who had palate repair performed between 2010 and 2013, excluding syndromic patients, patients undergoing palate revision, and patients with incomplete payment information. INTERVENTIONS: None. MAIN OUTCOME MEASURE: Fees and charges for procedures. RESULTS: Surgeon payment was significantly higher for international adoptees (Δ = $2047.51 [$128.35 to $3966.66], P = .038). Medicaid-adjusted surgeon payments averaged $1006 more for adoptees ([-$394.19 to $2406.98], P = .158). CONCLUSIONS: Hospital and anesthesiology costs for adoptee palate repair were highly variable but did not differ significantly from those for nonadoptees. Partly due to payer mix, surgeon reimbursement was somewhat higher for international adoptees. No difference in total payment was found.

An Assessment of 30-Day Complications in Primary Cleft Palate Repair: A Review of the 2012 ACS NSQIP Pediatric.

OBJECTIVE: 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. DESIGN: 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. RESULTS: 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%. CONCLUSION: 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.

A Comparative Cost Analysis of Cleft Lip Adhesion and Nasoalveolar Molding before Formal Cleft Lip Repair.

BACKGROUND: Patients with complete cleft lip and palate may benefit from cleft lip adhesion or nasoalveolar molding before formal cleft lip repair. The authors compared the relative costs to insurers of these two treatment modalities and the burden of care to families. METHODS: A retrospective analysis was performed of cleft lip and palate patients treated with nasoalveolar molding or cleft lip adhesion at The Children's Hospital of Philadelphia between January of 2007 and June of 2012. Demographic, appointment, and surgical data were reviewed; surgical, inpatient hospital, and orthodontic charges and costs were obtained. Multivariate linear regression and two-sample, two-tailed independent t tests were performed to compare cost and appointment data between groups. RESULTS: Forty-two cleft adhesion and 35 nasoalveolar molding patients met inclusion criteria. Mean costs for nasoalveolar molding were $3550.24 ± $667.27. Cleft adhesion costs, consisting of both hospital and surgical costs, were $9370.55 ± $1691.79. Analysis of log costs demonstrated a significant difference between the groups, with the mean total cost for nasoalveolar molding significantly lower than that for adhesion (p < 0.0001). Nasoalveolar molding patients had significantly more made, cancelled, no-show, and missed visits and a higher missed percentage than adhesion patients (p < 0.0001) for all except no-show appointments, (p = 0.0199), indicating a higher burden of care to families. CONCLUSIONS: Nasoalveolar molding may cost less before formal cleft lip repair treatment than cleft lip adhesion. Third-party payers who cover adhesion and not nasoalveolar molding may not be acting in their own best interest. Nasoalveolar molding places a higher burden of care on families, and this fact should be considered in planning treatment.

Mandibular distraction osteogenesis for the treatment of neonatal tongue-based airway obstruction.

Early postnatal tracheostomy for airway compromise is associated with high morbidity and cost. In certain patients with tongue-base airway obstruction (TBAO), mandibular distraction osteogenesis may be preferred. We present a comprehensive analysis of surgical, airway, and cephalometric outcomes in a large series of neonatal patients with TBAO. A retrospective review was performed of patients with laryngoscopically proven TBAO who underwent mandibular distraction osteogenesis before 1 year of age at our institution. Demographic, operative, postoperative, polysomnographic, and radiographic data were analyzed with the appropriate statistical test. Between 2010 and 2013, 28 patients younger than 1 year underwent mandibular distraction for TBAO. Distraction was performed for documented TBAO and failure to thrive at an average age of 58 days (range, 11-312) days with distractor removal after an average of 90 days. Preoperative polysomnograms were obtained on 20 patients with an average apnea-hypopnea index of 39.3 ± 22.0/h; the apnea-hypopnea index on postoperative polysomnograms obtained after distraction completion was significantly reduced in all 14 patients in whom it was measured (mean, 3.0 ± 1.5/h; P < 0.0001). Twenty patients transitioned to oral feeding, and cephalometric and airway diameters were improved (P < 0.0001). Distraction was successful in all but 4 patients including all patients with GILLS scores of 2 or less and 66% of patients with GILLS scores of 3 or greater. Neonatal mandibular distraction is a powerful tool to treat critical obstructive apnea in patients with TBAO. Appropriate patient selection remains a challenge; however, mandibular distraction represents a compelling treatment modality.

Costochondral grafting in craniofacial microsomia.

BACKGROUND: Craniofacial microsomia encompasses a spectrum of diagnoses associated with variable degrees of mandibular hypoplasia, facial asymmetry, chin deviation, occlusal abnormalities, and potential airway compromise. This study presents one surgeon's experience with costochondral rib grafting for mandibular reconstruction in children with Pruzansky/Kaban type 2B and type 3 mandibular hypoplasia. METHODS: An institutional review board-approved retrospective chart review was performed of all patients with craniofacial microsomia who underwent costochondral rib grafting for mandibular reconstruction performed by the senior author (S.P.B.) at The Children's Hospital of Philadelphia from January of 1998 to September of 2013. Demographic information, surgical history, operative details, postoperative complications, and outcomes were recorded. Plain radiographs and preoperative and postoperative three-dimensional computed tomographic scans were reviewed. RESULTS: Two hundred fifty-five patients were diagnosed with craniofacial microsomia, and 22 patients met inclusion criteria. Twelve boys and 10 girls underwent grafting at an average age of 7.2 years. Thirty-three costochondral rib grafts were performed, 11 unilateral reconstructions and 11 bilateral reconstructions. Twelve hemimandibles were type 2B and 21 were type 3. No intraoperative complications were reported, and no incidence of graft resorption was noted. No additional procedures were required in 27 reconstructed hemimandibles (81.8 percent), whereas six (18.2 percent) required secondary distraction osteogenesis. Only one patient developed postoperative ankylosis. No malunion or nonunion was noted. CONCLUSION: The approach described in this article allowed the authors to obtain reliably good results with costochondral rib grafting for type 2B and type 3 mandibular hypoplasia associated with craniofacial microsomia. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Analysis of risk factors associated with 30-day readmissions following pediatric plastic surgery: a review of 5376 procedures.

BACKGROUND: Unplanned surgical readmissions represent a benchmark outcome and pose a considerable cost burden for health care systems. The authors evaluated risk factors associated with readmission following pediatric plastic surgery using a prospective, validated, national database. METHODS: Patients younger than 18 years who underwent primary pediatric plastic surgery procedures were identified from the 2013 pediatric American College of Surgeons National Surgical Quality Improvement Program database.Two cohorts were compared: patients who experienced readmission and those who did not. Patient characteristics, comorbidities, intraoperative details,and 30-day postoperative outcomes, including complications and readmissions,were analyzed. Multivariate logistic regression analysis was used to identify factors associated with readmission. RESULTS: A total of 5376 patients were included, for an overall 2.40 percent readmission rate. The study cohort was, on average, 5.47 ± 5.21 years old,51.60 percent (n = 2774) were male, and 65.92 percent of cases (n = 3544)were outpatient procedures. The average number of relative value units per case was 10.15 ± 8.01. Patients with medical comorbidities (p < 0.001) and those with a preoperatively contaminated or infected wound were at higher risk for readmission (p < 0.001). Patients with higher American Society of Anesthesiologists scores (p < 0.001), longer operative times (p < 0.001), and longer hospitalizations (p < 0.0171) were also independently at greater risk for readmission. The most significant independent predictors of readmission were postoperative surgical and medical complications (OR, 6.94 and 11.92,respectively; p < 0.001). CONCLUSION: These results help target patients at greater risk for readmission and afford an opportunity to provide evidence-based interventions to mitigate risk and minimize cost burden for health care systems. (Plast. Reconstr. Surg.135: 521, 2015.) CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

The prevalence of strabismus in unilateral coronal synostosis.

BACKGROUND: While there is a clear correlation between unilateral coronal synostosis (UCS) and ocular motility abnormalities, the literature provides little information as to the true epidemiology of strabismus, or the underlying etiology of these paralleled pathologies. The purpose of this study is to investigate the rate of oculomotor abnormalities associated with UCS and its management. METHODS: A retrospective review of all patients identified to have single-suture, nonsyndromic UCS treated by fronto-orbital advancement at a tertiary craniofacial referral center from 1977 to 2013 was performed. Inclusion criteria mandated complete medical, surgical, and ophthalmological records. Patients were evaluated for strabismus both preoperatively and postoperatively, and as to whether eye muscle surgery was performed. RESULTS: A total of 181 patients underwent treatment for UCS at our institution during the study period, of which 79 met the inclusion criteria. Twenty-nine patients had strabismus prior to any craniofacial surgical intervention. Following fronto-orbital advancement, 23 patients (46 %) developed a new onset strabismus. Fifty-five patients had no change in their preoperative ocular examination, and one patient had resolution of preoperative strabismus. Of the 51 patients who had postoperative strabismus, 30 went on to have eye muscle surgery. There were no statistically significant differences in gender (p=0.477), race (p=0.395), sidedness of suture involvement (p=0.552), or age at intervention (p=0.66) in comparing the group with new postoperative strabismus and those without. CONCLUSIONS: This study sheds new light on the prevalence of strabismus in UCS, and more importantly, the risk of developing strabismus in the setting of conventional fronto-orbital advancement. This data will allow more accurate preoperative counseling and reinforces the important role of ophthalmologists as members of the multidisciplinary craniofacial team.

Simultaneous unicoronal and sagittal distraction osteogenesis for the treatment of nonsyndromic multisutural craniosynostosis.

We present a case of multiplanar distraction osteogenesis for the simultaneous treatment of sagittal and unicoronal craniosynostosis in a nonsyndromic 2-month-old boy. Unilateral fronto-orbital advancement and sagittal suturectomy were performed. Distracters were fixed orthogonally in the sagittal and coronal positions to distract the affected coronal and sagittal sutures. The devices achieved 20 and 22 mm of advancement in the coronal and sagittal locations. A total intracranial volume increase of 62% was noted at 6 months' follow-up. This preliminary report demonstrates the procedure's short-term safety; future investigation is needed over the long term to determine its efficacy.

A critical evaluation of long-term aesthetic outcomes of fronto-orbital advancement and cranial vault remodeling in nonsyndromic unicoronal craniosynostosis.

BACKGROUND: This study reports long-term aesthetic outcomes with fronto-orbital advancement and cranial vault remodeling in treating unicoronal synostosis over a 35-year period. METHODS: Retrospective review was performed on patients with isolated unicoronal synostosis from 1977 to 2012. Demographic, preoperative phenotypic, and long-term aesthetic outcomes data were analyzed with chi-squared and Fisher's exact test for categorical data and Wilcoxon rank-sum and Kruskal-Wallis rank for continuous data. RESULTS: A total of 238 patients were treated; 207 met inclusion criteria. None underwent secondary intervention for intracranial pressure. At definitive intervention, there 96 (55 percent) Whitaker class I patients, 11 (6 percent) class II, 62 (35 percent) class III, and six (3 percent) class IV. Nasal root deviation and occipital bossing each conferred an increased risk of Whitaker class III/IV [OR, 4.4 (1.4 to 13.9), p = 0.011; OR, 2.6 (1.0 to 6.8), p = 0.049]. Patients who underwent bilateral cranial vault remodeling with extended unilateral bandeau were less likely Whitaker class III/IV at latest follow-up compared with those undergoing strictly unilateral procedures [OR, 0.2 (0.1 to 0.7), p = 0.011]. Overcorrection resulted in decreased risk of temporal hollowing [OR, 0.3 (0.1 to 1.0), p = 0.05]. Patients with 5 years or more of follow-up were more likely to develop supraorbital retrusion [OR, 7.2 (2.2 to 23.4), p = 0.001] and temporal hollowing [OR, 3.7 (1.5 to 9.6), p = 0.006] and have Whitaker class III/IV outcomes [OR, 4.9 (1.8 to 12.8), p = 0.001]. CONCLUSION: Traditional fronto-orbital advancement and cranial vault remodeling appears to mitigate risk of intracranial pressure but may lead to aesthetic shortcomings as patients mature, namely fronto-orbital retrusion and temporal hollowing. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Clarifying the relationships among the different features of the OMENS+ classification in craniofacial microsomia.

BACKGROUND: The OMENS+ classification is commonly used to describe the phenotypically diverse craniofacial features of craniofacial microsomia. The purpose of this study was to evaluate associations among the individual components of the OMENS+ criteria. METHODS: An institutional review board-approved retrospective chart review was performed for patients who presented with a diagnosis of unilateral or bilateral craniofacial microsomia to the craniofacial clinic from January of 1990 to December of 2012. Demographic, diagnosis, classification, treatment, and radiographic data were abstracted for all patients who met inclusion criteria. Associations and correlations were evaluated using the Spearman rank test and a logistic regression model. RESULTS: One hundred five patients (61 male and 44 female) with craniofacial microsomia met inclusion criteria. Eighty-one patients (77.1 percent) had unilateral microsomia and 24 (22.9 percent) had bilateral microsomia. Twenty-eight patients (26.7 percent) had macrostomia. Correlations were all significantly interrelated (p = 0.000 to p = 00.018) between the degree of orbital, mandibular, and soft-tissue deformities. Moreover, the severity of ear deformity and facial nerve involvement were also significantly correlated (p = 0.008). Between these two groupings, there was a significant correlation between soft-tissue deficiency and nerve involvement (p = 0.010). Macrostomia was associated with the individual components of the group orbit (p = 0.008), mandible (p = 0.000), and soft tissue (p = 0.005). CONCLUSIONS: The association between structures using the OMENS+ classification may be caused by their branchial arch origin. Structures mainly developed from the first branchial arch (orbit, mandible, and soft tissue) are associated in degree of severity, as are the structures mainly derived from the second branchial arch (facial nerve and ear). CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

Incidence of positive screening for obstructive sleep apnea in patients with isolated cleft lip and/or palate.

OBJECTIVE: To determine the incidence of obstructive sleep apnea (OSA) in children with isolated cleft lip and/or palate (CL/P). METHODS: The present prospective study was performed at a pediatric tertiary care centre. Consecutive patients evaluated at the cleft clinic from January 2011 to August 2013 were identified. Patients' families prospectively completed the Pediatric Sleep Questionnaire (PSQ), a validated tool used to predict moderate to severe OSA. Patients with CL/P and an underlying syndrome or other craniofacial diagnosis were excluded. A positive OSA screen was recorded if the ratio of positive to total responses was >0.33. Risk factors associated with a positive screen were identified using the Student's t or ANOVA test. RESULTS: A total of 867 patients completed the PSQ, 489 of whom with isolated CL/P met inclusion criteria. The mean age was 8.4 years. The overall incidence of positive screening was 14.7%. The most commonly reported symptoms among positive screeners were 'fidgets with hands or feet' (73.6%), 'interrupts others' (69.4%) and 'mouth breather during the day' (69.4%). The most sensitive items were 'stops breathing during the night' and 'trouble breathing during sleep', with positive predictive values of 0.78 and 0.67, respectively. Sex, body mass index, ancestry and cleft type were not significantly associated with increased risk for positive screening. CONCLUSION: One in seven children with isolated CL/P screened positively for OSA according to the PSQ. This finding highlights the potential importance of routine screening in this at-risk group.

OBJECTIF: Déterminer l'incidence d'apnée obstructive du sommeil (AOS) chez les enfants ayant une fente labiale ou palatine isolée (F/LP). MÉTHODOLOGIE: La présente étude prospective a été effectuée dans un centre de soins tertiaires. Des patients consécutifs évalués à la clinique de fente labiale ou palatine entre janvier 2011 et août 2013 ont été repérés. Les familles des patients ont rempli prospectivement un questionnaire sur le sommeil pédiatrique (PSQ), un outil validé utilisé pour prédire l'AOS modérée à grave. Les patients ayant une F/LP et un syndrome sous-jacent ou un autre diagnostic crâniofacial étaient exclus. Le dépistage d'AOS était positif si le ratio entre la réponse positive et les réponses totales était supérieur à 0,33. Les facteurs de risque de dépistage positif ont été déterminés à l'aide du test t de Student ou du test ANOVA. RÉSULTATS: Au total, 867 patients ont rempli le PSQ, dont 489 avaient une F/LP qui respectait les critères d'inclusion. Ils avaient un âge moyen de 8,4 ans. L'incidence globale de dépistage positif s'élevait à 14,7 %. Les symptômes les plus déclarés chez les personnes dépistées comme positives étaient « agite les mains ou les pieds ¼ (73,6 %), « interrompt ¼ (69,4 %) et « respire par la bouche pendant la journée ¼ (69,4 %). Les faits les plus inquiétants étaient « arrête de respirer pendant la nuit et « a de la difficulté à respirer pendant qu'il dort ¼, dont les valeurs prédictives positives étaient respectivement de 0,78 et de 0,67. Le sexe, l'indice de masse corporelle, l'ascendance et le type de fente ne s'associaient pas de manière significative à un risque accru de dépistage positif. CONCLUSION: Un enfant sur sept ayant une F/LP isolée était positif à l'AOS selon le PSQ. Cette observation fait ressortir l'importance potentielle du dépistage systématique dans ce groupe à haut risque.

Earlier evidence of spheno-occipital synchondrosis fusion correlates with severity of midface hypoplasia in patients with syndromic craniosynostosis.

BACKGROUND: The spheno-occipital synchondrosis is an important driver of facial and cranial base growth. The current study characterizes its fusion in patients with Apert, Crouzon, and Pfeiffer syndromes and correlates early fusion with the presence, and degree, of midface hypoplasia. METHODS: A retrospective case-control study was performed of all syndromic patients treated between 1996 and 2012. Case computed tomographic scans and age- and sex-matched control scans were analyzed as demonstrating either open, partially fused, or completely fused synchondroses, and patient age at each scan was recorded. Midface hypoplasia as determined by sella-nasion-A point angle measurement at the time of midface surgery was correlated to fusion status. RESULTS: Fifty-four patients with 206 computed tomographic scans met inclusion criteria. Two hundred six age- and sex-matched control scans were also identified. Average age at computed tomographic scanning was 6.1 years. The earliest ages of partial and complete fusion were 1.1 and 7.0 years, respectively, among cases; and 6.2 and 12.7 years, respectively, among controls. The odds of synchondrosis fusion in case computed tomographic scans was 66.0 times that of controls (95 percent CI, 9.2 to 475.5 times that of controls; p < 0.000001). Average age of synchondrosis fusion was 3.5 years (range, 0.5 to 6.0 years). Average sella-nasion-A point angle at the time of midface surgery was 67.5 degrees (range, 58 to 76 degrees), with a positive correlation between earlier age of fusion and more severe midface hypoplasia (p = 0.028). CONCLUSIONS: The spheno-occipital synchondrosis fuses earlier in syndromic patients compared with age-matched controls. Moreover, there is a positive correlation between earlier fusion and degree of midface hypoplasia, although definitive causality cannot be concluded. This is the first study to demonstrate such a correlation in human subjects. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Utility of screening for obstructive sleep apnea syndrome in children with craniofacial disorders.

BACKGROUND: Children with craniofacial disorders are at increased risk for obstructive sleep apnea syndrome. Methods for diagnosing obstructive sleep apnea syndrome in this population remain controversial. Sleep studies are the criterion standard but are impractical for all patients. The utility of obstructive sleep apnea syndrome questionnaires such as the Pediatric Sleep Questionnaire is unknown in children with craniofacial disorders. The authors hypothesized that the Pediatric Sleep Questionnaire would be a sensitive tool for detecting obstructive sleep apnea syndrome in children with craniofacial abnormalities. METHODS: A retrospective review of consecutive children with diagnosed craniofacial disorders who both completed the Pediatric Sleep Questionnaire and underwent polysomnography was performed. Demographics, Pediatric Sleep Questionnaire score, and polysomnographic data were recorded. Statistical analysis included calculation of sensitivity, specificity, positive predictive value, and negative predictive value for the Pediatric Sleep Questionnaire. RESULTS: Eighty-three children aged 2 to 18 years were included in the study. Of these, 44 (53.0 percent) screened positive on the Pediatric Sleep Questionnaire and 23 (27.7 percent) had polysomnographic evidence of obstructive sleep apnea syndrome, but the sensitivity of the Pediatric Sleep Questionnaire for detecting obstructive sleep apnea syndrome in this sample was only 0.57 and the specificity was 0.48. Positive predictive value and negative predictive value were 0.30 and 0.74, respectively. The correlation between the apnea hypopnea index and Pediatric Sleep Questionnaire score was 0.152 (p = 0.17). CONCLUSIONS: A substantial portion of craniofacial patients referred for polysomnography was found to have obstructive sleep apnea syndrome. However, the Pediatric Sleep Questionnaire is not a good screening tool for obstructive sleep apnea syndrome in children with craniofacial conditions. More research is needed to determine which patients with craniofacial disorders should be evaluated for obstructive sleep apnea syndrome by polysomnography or other means. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, II.

Screening for obstructive sleep apnea in children with syndromic cleft lip and/or palate.

BACKGROUND: Craniofacial malformations including cleft lip and/or palate (CL/P) increase risk for obstructive sleep apnea (OSA). While 30% of CL/P occurs in the context of underlying genetic syndromes, few studies have investigated the prevalence of OSA in this high-risk group. This study aims to determine the incidence and risk factors of positive screening for OSA in this complex patient population. METHODS: The Pediatric Sleep Questionnaire (PSQ) was prospectively administered to all patients cared for by the cleft lip and palate clinic at the Children's Hospital of Philadelphia between January 2011 and August 2013. The PSQ is a 22-item, validated screening tool for OSA with a sensitivity and specificity of 0.83 and 0.87 in detecting an apnea-hypopnea index (AHI) >5/hour in healthy children. The Fisher exact and Chi-square tests were used for purposes of comparison. RESULTS: 178 patients with syndromic CL/P completed the PSQ. Mean cohort age was 8.1 ± 4.4 years. Patients were predominately female (53.9%), Caucasian (78.1%), and had Veau Class II cleft (50.6%). Craniofacial syndromes included isolated Pierre Robin Sequence (PRS) (29.8%), 22q11.2 deletion syndrome (14.6%), Van der Woude syndrome (6.7%), and other rare genetic abnormalities (28.8%). The overall incidence of positive OSA screening was 32.0%. Males were at increased risk for positive OSA screening (P = 0.030), as were non-Caucasians (P = 0.044). Symptoms with the highest positive predictive value for OSA were "others comment on child appearing sleepy" (76.2%) and "stops breathing during the night" (75.0%). Notably, patients with 22q11.2 deletion syndrome were at highest risk for positive screens (50.0%, P = 0.042). CONCLUSIONS: Nearly a third of our patients with syndromic CL/P screened positively for OSA (32.0%), highlighting the importance of screening in this at-risk population. Future work will correlate screening results with polysomnograms to help validate these findings. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, III.

Screening for obstructive sleep apnea in children treated at a major craniofacial center.

Timely diagnosis of obstructive sleep apnea (OSA) in patients with craniofacial disorders may help prevent long-term adverse sequelae of upper airway obstruction, namely pulmonary hypertension, failure to thrive, and impaired neurocognitive development. Currently, little is known about the incidence of OSA in this high-risk population. A prospective study examining the incidence of positive screening for OSA in patients cared for by the craniofacial team at a large, urban referral center was performed. From January 2011 to August 2013, all patient families were asked to complete the Pediatric Sleep Questionnaire. This validated tool has a sensitivity of 85% and specificity of 87% in predicting a positive sleep study when the ratio of positive-total responses is 0.33 or greater. Screening results were evaluated via Chi-squared and Fisher tests according to demographic and clinical variables. A total of 234 children seen in our craniofacial clinic completed the Pediatric Sleep Questionnaire. The mean screening age was 8.38 years, and 47% were male (110/234). Total incidence of positive OSA screening was 28.2% (66/234). Of the total population, 128 patients had an underlying syndrome (54.7%), whereas 106 patients were nonsyndromic (45.3%). Both groups were at equivalent risk for screening positive for OSA (28.1% versus 28.3%, P = 1.0). Among children with a craniofacial diagnosis, patients with a cleft lip and/or palate were at equivalent risk for screening positive for OSA as patients without a cleft (25.5% versus 32.6%, P = 0.24). The OSA symptoms affect almost one third of patients seen by our craniofacial team. Syndromic and nonsyndromic patients seem to be at equivalent risk as those patients with and without an oropharyngeal cleft. Future work will correlate these findings with formal polysomnography and may serve to heighten awareness of OSA in this at-risk population.

Patient risk factors for ambulatory cleft lip repair: an outcome and cost analysis.

BACKGROUND: The purpose of this study was to identify variables that may preoperatively predict successful outcomes in ambulatory cleft lip repair and to quantify the potential cost savings. METHODS: A retrospective chart review of all patients who underwent cleft lip repair at a tertiary cleft care center from January of 2010 to May of 2013 was performed. Because inpatient stay is the authors' current practice, overnight desaturations, poor oral intake, and failure to stop intravenous narcotics during the first day were considered indicators of a poor candidate. Charge data were also collected. RESULTS: Twenty-nine of 111 patients were deemed likely to fail ambulatory surgery. American Society of Anesthesiologists score greater than 2 (p=0.019), low birth weight (p=0.022), bilateral cleft lip-cleft palate (p=0.003), central nervous system or neurologic diagnosis (p=0.046), syndromic and/or multiple congenital abnormalities (p=0.024), prior emergency room visits (p=0.021), failure to thrive (p=0.046), and age older than 7 months (p=0.028) were associated with poor candidacy. A diagnosis of cleft lip was protective (p=0.015). Fifty-five patients had no risk factors for a poor ambulatory outcome and did not stay more than 1 day. The average savings per patient who met the authors' criteria was $4261. CONCLUSIONS: The authors found that 49.5 percent of children may be safely eligible for ambulatory cleft lip repair. Patients with risk factors may be best served by a short hospital admission. This would lead to a national savings of $8,765,183 per year.

Maxillary involvement in hemifacial microsomia: an objective three-dimensional analysis of the craniofacial skeleton.

The aim of our study is to better understand the maxillary involvement in relation to the mandibular deformation in hemifacial microsomia (HFM). To do so, consecutive patients with HFM treated at The Children's Hospital of Philadelphia from 2000 to 2012 were included in our study. Both two-dimensional and three-dimensional analyses of the bony and sinus structures of the midface and mandible were performed using three-dimensional segmentation software. Patients were stratified into groups based on the Kaban-Pruzansky classification--mild (0-1), moderate (2A), and severe (2B-3)--as well as rank ordering based on overall severity. Analyses involved paired t-tests within severity groups, 1-way analysis of variance when assessing across groups (ipsilateral/contralateral ratio), and regression to assess for trends. Thirty patients were included (4 mild, 12 moderate, and 14 severe). The mandibular volume ratio differed across all patient groups (P < 0.001) and trended with rank order (P < 0.001). No significant difference in maxillary bony volume ratio was found across all patient groups (P = 0.16). In patients with severe disease, the maxillary bone volume was found to be significantly decreased on the ipsilateral side as compared with the contralateral side (P = 0.0123). There was no difference in maxillary sinus volume between ipsilateral and contralateral sides within any patient groups or in comparing across groups (P = 0.10). No significant trend was found in the volume ratio of mandible and maxilla (P = 0.41). To conclude, the maxillary sinus seems to show no difference in volume when comparing between laterality and severity groupings. These findings suggest that there may be alternative influences other than the vascular insult acting as the driving force behind the mandibular deformity and the additional classic clinical findings of HFM.