J. Thomas Paliga

A craniometric analysis of posterior cranial vault distraction osteogenesis.

Background: Posterior cranial vault distraction osteogenesis has replaced fronto-orbital advancement in some centers as the first-line treatment in patients with syndromic craniosynostosis. Despite this fact, little has been written about its craniometric effects on children with syndromic craniosynostosis. Methods: A retrospective review of all patients who underwent posterior distraction was performed. Patient demographic, perioperative data, and preoperative/postoperative computed tomographic scans were reviewed. Volumetric and craniometric indices were calculated and measured using commercial three-dimensional imaging software. Results: From 2008 to 2012, 22 patients underwent posterior vault distraction osteogenesis for suspected intracranial hypertension or severe turribrachicephaly. In 13 patients, this was the first cranial vault procedure performed, whereas eight had previous fronto-orbital advancement and one had parieto-occipital reshaping. Half of patients underwent posterior cranial vault distraction osteogenesis before age 1 year; the average age at surgery was 2.3 years (range, 0.3 to 14.1 years) and distraction length averaged 27.3 mm (range, 19 to 35 mm). Average length of surgery was 2.9 hours (range, 1.6 to 3.8 hours), and average blood loss was 400 ml (range, 200 to 600 ml). Total treatment length was 91 days (range, 48 to 147 days). Distraction length averaged 27.3 mm (range, 19 to 35 mm). Intracranial volume increase averaged 21.5 percent (range, 7.5 to 70.0 percent; p < 0.0001) and 28.4 percent (range, 10.8 to 66.0 percent; p = 0.01) in the subset of patients younger than 1 year. Posterior cranial height increased 12.2 percent (range, 0 to 35 percent; p = 0.002), and basofrontal angle decrease averaged 3.9 percent (range, 0 to 12 percent; p = 0.003), indicating a decrease in cranial height trajectory and improvement in frontal bossing. Conclusions: Posterior cranial vault distraction is a safe and effective operation that may lower risk of intracranial hypertension and abnormal skull morphology. Interestingly, cranial morphological benefits were seen both anteriorly and posteriorly. 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. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

The spheno-occipital synchondrosis fuses prematurely in patients with Crouzon syndrome and midface hypoplasia compared with age- and gender-matched controls.

PURPOSE Premature closure of the spheno-occipital synchondrosis (SOS) has been associated with midface hypoplasia in animal models and patients with specific forms of syndromic craniosynostosis. The present study aimed to characterize SOS fusion in patients with Crouzon syndrome. PATIENTS AND METHODS A case-control study was performed in patients with Crouzon syndrome treated at the Childrens Hospital of Philadelphia from 1984 to 2012. The cases included patients with Crouzon syndrome and at least 1 high-quality computed tomography (CT) scan in which SOS patency could be assessed. Age- and gender-matched control CT scans were identified for comparison. The patient age at the CT scan was evaluated as the predictor, with SOS patency identified as the outcome variable. Three independent reviewers with high inter-rater reliability graded the SOS patency as open, partially fused, or completely fused. The Wilcoxon rank sum test was used to compare the Crouzon group and the controls. RESULTS During the study period, 30 patients were identified with Crouzon syndrome. A total of 24 patients, all with midface hypoplasia and with 112 cranial CT scans, met the inclusion criteria. Accordingly, 112 age- and gender-matched control CT scans were assessed. No patient in the control group had midface hypoplasia. Within the Crouzon group, the average age at complete closure (14.0 ± 3.4 years) evident on the CT scan was significantly younger than that in the control group (16.6 ± 2.2 years; P = .0152). The average age when the scans showed complete patency of the SOS in the Crouzon group (1.3 ± 1.1 years) was significantly younger than that in the control group (3.2 ± 2.3 years; P = .0001). CONCLUSIONS The SOS closes significantly earlier in patients with Crouzon syndrome compared with age- and gender-matched controls. The strong statistical correlation supports premature closure of the SOS as a possible mechanistic contributor to midface hypoplasia.

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.

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

AbstractEarly 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.

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

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

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

Economic Analysis of Cleft Palate Repair in International Adoptees.

8,765,183 per year.

Closure of the Spheno-Occipital Synchondrosis in Patients with Crouzon Syndrome: A Link to Midface Hypoplasia

Abstract 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.

A Comparative Analysis of Complications in 55 Midface Distraction Procedures in Patients with Syndromic Craniosynostosis

Design Retrospective cohort study. Setting Major international tertiary care referral center for cleft palate repair. Patients One hundred thirty-eight patients at the Childrens 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 =