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Birk-Barel intellectual disability dimorphism syndrome, also referred to as KCNK9 imprinting syndrome, is an exceedingly rare condition described in under 20 cases that presents with intellectual disability, hypotonia, scoliosis, dysphonia, dysphagia, and craniofacial dysmorphic features. The condition follows an autosomal dominant pattern of inheritance in the maternally expressed KCNK9 gene on chromosome 8. Due to the complexity of presentation, patients with Birk-Barel syndrome are optimally managed by a multidisciplinary team including a craniofacial surgeon. Previously described craniofacial dysmorphic features include micrognathia, cleft palate, dolichocephaly, broad nasal tip, and broad philtrum, among others. Here the authors describe a genetically confirmed case that has been managed in our institution's multidisciplinary cleft and craniofacial clinic. The authors aim to discuss Birk-Barel syndrome for a surgical and craniofacial audience with considerations for operative management in the context of a multidisciplinary team.

The purpose of this study was to evaluate the epidemiology and perioperative complications of different reconstructive strategies to correct cleft nasal deformity, with particular attention paid to type and timing of cartilage grafting.Retrospective cohort study was conducted of cleft rhinoplasty performed between 2012 and 2017 in North America utilizing the American College of Surgeons National Surgical Quality Improvement Program- Pediatric hospital network. Medical/surgical complications, reoperations, and readmissions within 30 days postoperatively were analyzed with appropriate statistics.During the study interval, 3317 pediatric patients underwent cleft rhinoplasty, with 8.0% involving the use of cartilage grafts. Ear cartilage was significantly more commonly used for intermediate repair, whereas rib cartilage was more commonly used for late repair (P=0.006). Overall, rhinoplasties with ear cartilage grafts had shorter procedure durations than those without cartilage grafts (P=0.005), whereas those with rib cartilage grafts had increased procedure duration (P0.001). The use of cartilage grafts was not associated with increased complications in either intermediate or late cleft rhinoplasty. Patients with bilateral clefts were more likely to undergo rhinoplasty with cartilage grafts overall (P=0.047) and with cartilage grafts for late reconstruction (P=0.039).Ear cartilage is most frequently utilized for intermediate repair, whereas rib cartilage is most frequently utilized for late repair during cleft rhinoplasty. Ear cartilage grafts are associated with significantly decreased procedure duration, whereas rib cartilage grafts are associated with significantly increased procedure duration. Not surprisingly, cleft rhinoplasty is relatively safe, with a 2% overall short-term complication rate.

Facial proportionality and symmetry are positively associated with perceived levels of facial attractiveness.The aims of this study were to confirm and extend the association of proportionality with perceived levels of attractiveness and character traits and determine differences in attractiveness and character ratings between "anomalous" and "typical" faces using a large dataset.Ratings of 597 unique individuals from the Chicago Face Database were used. A formula was developed as a proxy of relative horizontal proportionality, where a proportionality score of "0" indicated perfect proportionality and more negative scores indicated less proportionality. Faces were categorized as "anomalous" or "typical" by 2 independent reviewers based on physical features.Across the ratings for all faces, Spearman correlations revealed greater proportionality was associated with attractiveness ( ρ = 0.292, P0.001) and trustworthiness ( ρ = 0.193, P0.001), while lesser proportionality was associated with impressions of anger (ρ = 0.132, P = 0.001), dominance (ρ = 0.259, P0.001), and threateningness ( ρ = 0.234, P0.001). Mann-Whitney U tests revealed the typical cohort had significantly higher levels of proportionality (-13.98 versus -15.14, P = 0.030) and ratings of attractiveness (3.39 versus 2.99, P0.001) and trustworthiness (3.48 versus 3.35, P0.001).This study demonstrated that facial proportionality is not only significantly associated with higher ratings of attractiveness, but also associated with judgements of trustworthiness. Proportionality plays a role in evoking negative attributions of personality characteristics to people with facial anomalies.

PURPOSE The goal of this study was to investigate patient specific factors and surgical techniques that affect occipital step-off deformity in children undergoing posterior vault distraction osteogenesis (PVDO). METHODS Patients who underwent PVDO were retrospectively reviewed and included if a high resolution three-dimensional computed tomography scan was available 1-year post-distractor removal. Two craniofacial surgeons were blinded to individual subjects and presented with 3 still images of three-dimensional bone reconstructions and asked to rate the degree of step off: none, mild, moderate, or severe. The data were analyzed with multinomial logistic regressions and other appropriate statistics. RESULTS Forty-one patients met inclusion criteria. All patients had multisuture or bicoronal craniosynostosis. Multinomial logistic regression identified age at PVDO (Cox and Snell = 0.487, P

INTRODUCTION Children who require submandibular gland excision for ptyalism often have multiple associated comorbidities, including neurodevelopmental disorders and respiratory risk factors. The purpose of this study is to utilize a large multicenter database to elucidate the perioperative profile of submandibular gland excision in children, with particular focus on children who require submandibular gland excision for ptyalism. METHODS The American College of Surgeons National Surgical Quality Improvement Program Pediatric dataset was queried for submandibular gland excision performed from 2012 through 2018. Indications were subclassified based on International Classification of Disease (ICD)-9 and ICD-10 codes. Complications, readmissions, and reoperations were analyzed with appropriate statistics. RESULTS During the study interval, 304 pediatric patients underwent submandibular gland excision, which was mostly performed for ptyalism (56.9%), followed by inflammatory conditions (20.7%). Patients requiring submandibular gland excision for ptyalism were significantly younger (P

The purpose of this study was to compare perioperative safety and efficacy of posterior vault distraction osteogenesis (PVDO) in patients with primary nonsynostotic cephalo-cranial disproportion, namely slit ventricle syndrome and idiopathic intracranial hypertension (IIH), to a cohort of patients with craniosynostosis (CS). A retrospective review of patients undergoing PVDO from 2009 to 2019 at our institution was performed. Craniosynostosis patients were matched by sex and age at PVDO to the nonsynostotic cohort. Operative details, perioperative outcomes, and distraction patterns were analyzed with appropriate statistics. Nine patients met inclusion criteria for the non-CS cohort. Six patients (67%) underwent PVDO for slit ventricle and the remaining 3 patients (33%) underwent PVDO for IIH. The majority of CS patients were syndromic (n = 6, 67%) and had multisuture synostosis (n = 7, 78%). The non-CS cohort underwent PVDO at a median 56.1 months old [Q1 41.1, Q3 86.6] versus the CS cohort at 55.7 months [Q1 39.6, Q3 76.0] (P = 0.931). Total hospital length of stay was longer in the non-CS patients (median days 5 [Q1 4, Q3 6] versus 3 [Q1 3, Q3 4], P = 0.021). Non-CS patients with ventriculoperitoneal shunts had significantly less shunt operations for ICP concerns post-PVDO (median rate: 1.74/year [Q1 1.30, Q3 3.00] versus median: 0.18/year [Q1 0.0, Q3 0.7]; P = 0.046). In this pilot study using PVDO to treat slit ventricle syndrome and IIH, safety appears similar to PVDO in the synostotic setting. The cohort lacks adequate follow-up to assess long term efficacy, although short-midterm follow-up demonstrates promising results with less need for shunt revision and symptomatic relief. Future studies are warranted to identify the preferred surgical approach in these complex patients.

BACKGROUND Orthognathic surgery has traditionally been performed after skeletal maturity. Although these procedures are also being performed in children, the implications of earlier intervention and specific risk factors in this younger population remain unknown. METHODS The American College of Surgeons National Surgical Quality Improvement Program Pediatric dataset was queried for orthognathic procedures performed in 2018. Complications, readmissions, and reoperations were analyzed with appropriate statistics. RESULTS Overall adverse event rate after orthognathic surgery in pediatric patients was 7.8% (n = 22 of 281), which were associated with having any comorbidity (P

PURPOSE The purpose of this study is to quantitatively establish the volumetric changes observed with posterior vault distraction osteogenesis in the anterior, middle, and posterior thirds of the cranial vault; characterize change in cranial length, width, and height, correlating these changes to demographic variables that may help identify why younger kids gain more volume; and describe the short-term position of the transport segment. METHODS Multisuture craniosynostosis patients who underwent posterior vault distraction osteogenesis were retrospectively reviewed. Pediatric, dose-reduced, thin cut helically acquired head computed tomography scans were analyzed on Materialise Mimics v22 (Materialise, Ghent, Belgium). Pre and post-PVDO and "old" and "young" cohort were compared. RESULTS Twenty-one patients met inclusion criteria. The change in cranial length was significantly less at a median of 14.9 mm [Interquartile range (IQR) 11.8, 31.6] compared to the X-ray measured AP distraction distance (30 mm [IQR 24, 33]; P

In the pediatric general surgery literature, it has been shown that prenatal diagnosis of a congenital anomaly is an independent predictor of parental psychological distress. Surgical prenatal counseling can decrease parental anxiety by helping families understand the surgical needs and potential outcomes of their infant. In this retrospective analysis (n = 440), the authors sought to present our care pathway for prenatally diagnosed cleft lip and palate (CL/P) and explore the impact of cleft lip and palate-specific prenatal counseling on patient care by comparing the timing of clinical and surgical care between a cohort of patients who received prenatal CL/P consultation and a cohort of patients only seen postnatally. The authors hypothesize that our multidisciplinary prenatal care intervention is associated with earlier postnatal clinic visits and surgical repair. The care of all patients whose mother's presented for prenatal CL/P consultation (prenatal cohort, n = 118) was compared to all new CL/P patients without prenatal consultation at our institution (postnatal cohort, n = 322) from January 2015 through August 2019. 81.4% (n = 96) of the prenatal cohort returned for care postnatally while 2 pregnancies were interrupted, four neonates died, and 15 patients did not return for care. Prenatal consultation was associated with earlier postnatal clinic appointments (P

Previous literature has documented changes in nasal obstruction after acute LeFort I osteotomy. However, there is a paucity of studies that evaluate distraction-mediated LeFort I (DO-LFI) without concomitant intranasal interventions using the nasal obstruction symptom evaluation (NOSE) scale in Class III patients. The purpose of this study is to objectively evaluate nasal obstruction quality of life through the NOSE scale in patients undergoing DO-LFI. Inclusion into the study required both a preoperative (1 year ≤ date of service) and postoperative (≥6 months and ≤2 years) NOSE scale administration. Nasal obstruction symptom evaluation scales were compared using Wilcoxon signed rank test. There was a significant difference in composite NOSE scales, x̃ = 8.0 (interquartile range: 4.0-11.0), x̃ = 1.0 (interquartile range: 1.0-3.0), P 0.017, preoperatively and postoperatively respectfully. Additionally, when looking at individual components of the NOSE scale, nasal congestion or stuffiness, and trouble breathing through nose were significantly improved after DO-LFI (P 0.017). Nasal blockage or obstruction (P 0.084) and trouble breathing when exercising (P 0.076) trended towards significant improvement, as well. Trouble sleeping did not differ, P 0.611. We elucidate, in this pilot study, that there is an association between DO-LFI and patient reported nasal obstructive symptoms. Future prospective studies utilizing the NOSE scale are needed to determine causality.

INTRODUCTION While the use of virtual surgical planning (VSP) has been well described in the adult craniofacial literature, there has been little written about pediatric uses or trends. The purpose of this study is to evaluate the evolving utilization of VSP for pediatric craniofacial procedures. METHODS The authors' prospective institutional review board-approved craniofacial registry was queried for index craniofacial procedures from January 2011 through December 2018. Data was collected regarding utilization of traditional surgical planning versus VSP, as well as the extent of VSP's influence on the operative procedure. These data were analyzed for trends over time and compared using appropriate statistics. RESULTS During the study period, a total of 1131 index craniofacial cases were performed, of which 160 cases (14.1%) utilized VSP. Utilization of VSP collectively increased over time, from 2.0% in 2011 to 18.6% in 2018 (P

PURPOSE How different from "normal" are the cranial base and vault of infants with nonsyndromic, single-suture sagittal synostosis (NSSS)? This study quantitatively addresses this question utilizing computed tomography (CT) analytic technology. METHOD Head CT scans of infants with NSSS and normocephalic controls were analyzed using Mimics to calculate craniometric angles, distances, and segmented volumes. Craniometric measurements and asymmetry indices were compared between NSSS and control groups using linear regressions controlling for age. Ratios of anterior-, middle-, and posterior-to-total cranial vault volume were compared between groups using beta regressions controlling for age. RESULTS Seventeen patients with NSSS and 19 controls were identified. Cranial index and interoccipital angle were significantly smaller in NSSS compared with controls (P = 0.003 and

BACKGROUND Despite surgical correction of unilateral craniosynostosis (ULC), complex cranial base angulation can result in partial reversion to preoperative deformity with growth and time. Using 3-dimensional imaging, dysmorphic facial features of ULC in school-age patients were quantified and related to how they contribute to overall facial asymmetry and patient-reported outcomes. METHODS Children who underwent surgical correction of ULC were recruited from Yale University and Children's Hospital of Philadelphia. The 3D photographs were analyzed utilizing a Procrustes analysis of shape. Pearson's correlation was used to determine dysmorphic features' impact on overall asymmetry. Patients were stratified into "moderate" and "severe" asymmetry. Finally, asymmetry was correlated to patient-reported outcome scores. Statistical analysis was performed with SPSS-25 with P

Otoplasty remains an overlapping clinical domain of plastic surgery and otolaryngology. The purpose of this study is to objectively analyze the safety of otoplasty and determine if there are any risk factors, such as surgical training, associated with increased patient morbidity.Retrospective cohort study was conducted of otoplasty procedures performed in North America by plastic surgeons and otolaryngologists between 2012 and 2017 using the American College of Surgeons National Surgical Quality Improvement Program Pediatric dataset. Statistical analysis was performed to analyze the relationships between comorbidities, congenital malformations, and postoperative complications.There were 777 otoplasty procedures performed during the study period. Median age at time of surgery was 8.3 years (95% CI 7.9-8.7 years). Plastic surgeons performed 75.8% (n = 589) procedures and otolaryngologists performed 23.4% (n = 182). No significant (P = 0.952) difference in the occurrence of postoperative complications between surgical specialties was appreciated despite the fact that operative time was significantly longer in procedures performed by otolaryngologists (121 minutes versus 94 minutes, P 0.001). Overall, 1.3% (n = 10 of 777) children experienced a complication, with the most common complication being superficial surgical site infection, occurring in 0.9% (n = 7 of 777) patients. There was no association of comorbidities (P all 0.324) or congenital malformations (P all 0.382) contributing to postoperative complications. Reoperation (0.8%, n = 6 of 777) and readmission (0.4%, n = 3 of 777) were uncommon; nevertheless, these adverse events were significantly associated with multiple inherent patient risk factors on multivariate regression.Otoplasty is a relatively safe surgical procedure with similarly low complication and readmission rates when performed by plastic surgeons and otolaryngologists. Surgical site infection remains the most prevalent complication after otoplasty. Readmission and reoperation after otoplasty were significantly correlated to prematurity, structural pulmonary abnormality, alimentary tract disease, and seizure disorder.

BACKGROUND Among congenital ear deformities, helical rim deformities are most common. Non-surgical ear molding has emerged as an effective option to treat helical rim deformities and could reduce the need for surgery later in a child's life. Despite this, there has never been a study examining how the general public rates corrections after ear molding. METHODS A retrospective chart review was conducted of all consecutive patients with helical rim deformities treated with InfantEar TM Molding System. Amazon Mechanical Turk (MTurk) was used to survey blinded respondents using photographs of patients' ears to determine the degree of normalcy on a scale of 1 (not normal) to 10 (completely normal) before and after molding. Ratings of ears were compared using a paired t test and percent improvement from baseline. RESULTS A total of 59 ears met criteria for evaluation by 497 MTurk respondents. Average age of patients at the time of treatment was 34.2 ± 16.8 days and mean therapy duration was 31.3 ± 13.1 days. A paired t test analysis found that MTurk respondents identified significant improvement in 91.5% of ears (n = 54) (P

Purpose Postoperative hip pain is commonly reported after anterior iliac crest harvest for alveolar bone grafting. The goal of this study is to describe our institution's experience and examine the efficacy of our pain management protocols. Methods A retrospective review was performed by abstracting demographic, operative, and pain management data from January 2011 to April 2013. Paired t-tests and Fisher exact tests were used to examine differences when comparing 2 groups, while ANOVA was used to examine difference between the 3 protocols for harvest and pain management: trapdoor technique and local anesthetic injection (TD+LAI), TD and pain catheter (TD+PC), and split crest and LAI. Results Eighty-four patients, 52 males (61.9%), averaging 8.8 years old (±2.9) were included. Postoperatively, 17 (71%) patients in the PC group received IV narcotics compared to 27 (45%) in those without a PC (P = .03). When comparing all 3 protocols, no significant difference was found in IV morphine usage or duration of IV morphine treatment. In subgroup analysis, when patients in the groups TD+PC versus TD+LAI were examined, those in the TD+PC group had significantly shorter hospital stays and were more likely to go home postoperative day 1 (P = .03; P = .04). Conclusions Overall, patients tolerated alveolar bone grafting well regardless of harvest technique or pain management approach. While indwelling PCs did not significantly decrease IV morphine usage, these patients had significantly shorter lengths of stays.

Background Craniosynostosis is the premature fusion of cranial sutures in pediatric patients, which may lead to elevated intracranial pressure due to cerebro-cephalic disproportion between a growing brain and constricted skull. It is unknown whether this increased pressure is distributed equally throughout the cranial vault, or whether certain areas of the brain experience greater pressure at these regions of premature osseous fusion. Methods Optical coherence tomography (OCT) is a noninvasive modality for detecting elevated intracranial pressure. Optical coherence tomography was utilized to measure the peripapillary retinal nerve fiber layer (RNFL) thickness in patients undergoing surgical correction of craniosynostosis. Retinal nerve fiber layer in the eye ipsilateral to the unicoronal suture fusion was compared to the RNFL in the eye contralateral to the unicoronal suture fusion. Results During the study interval, 21 patients met inclusion criteria. Median age at operative intervention was 8.0 months, and 28.6% patients presented with left-sided unicoronal craniosynostosis, whereas 71.4% of patients presented with right-sided unicoronal craniosynostosis. Rather than universal increase on the affected side of coronal suture fusion, retinal nerve fiber layer thickness parameters showed a rotation phenomenon, such that the patterns of elevation had a 45° circumferential rotation in the direction of intorsion. Conclusions The explanation for these results remains elusive, but they likely indicate either intracranial changes transmitted differentially to the peripapillary retina, or differing retinal morphology, between the ipsilateral and contralateral eyes in unicoronal craniosynostosis.

Nasal dermoid cysts represent a spectrum of complexity from dermal sacs to multiloculated sinus tracts with intracranial extension with treatments ranging from outpatient excision to transcranial procedures involving dissection of the dermoid from the dura mater. In this study, the authors examined national outcomes across the spectrum utilizing the NSQIP database. Interestingly, complication rates were uniformly low at 1.2% even though those requiring transcranial excision required significantly longer surgical procedures (P = 0.001), and were significantly more likely to be admitted as inpatients (P

Background Posterior vault distraction osteogenesis (PVDO) has been demonstrated to effectively increase intracranial volume, treat increased intracranial pressure, and improve head shape in syndromic patients. The purpose of this study is to compare changes along the posterior cranial base before and after distraction. Methods A retrospective review was completed of subjects who underwent PVDO with computed tomography scans at 2 time-points: within 3 months preoperatively and 1 to 6 months postoperatively. Using Mimics software, craniometric landmarks were identified and surface area of the foramen magnum was calculated. A comparison of pre- to postoperative measurements was completed using Wilcoxon matched-paired signed rank tests and linear regression. Results A total of 65 PVDO subjects were identified, 12 subjects met inclusion criteria. Mean operative age was 3.0 ± 4.0 years. The cranial vault was distracted on average 25.0 ± 6.0 mm, with those 12 months of age distracted 22.0 ± 4.9 mm (P = 0.0543). There was a significant increase in pre- to postoperative foramen magnum surface area (52.1 ± 63.2 mm, P = 0.002), length (0.9 ± 1.4 mm, P = 0.050), and width (0.6 ± 1.0 mm, P = 0.050). Similarly, linear distances between nasion and posterior cranial base landmarks such as foramen magnum (3.4 ± 4.2 mm, P = 0.010), and occipital protuberance (9.1 ± 9.6 mm, P = 0.003) were increased. Subjects under 12 months had a greater percentage increases in posterior vault length than those over 12 months. Conclusion Posterior vault distraction osteogenesis is associated with an increase in size of the foramen magnum, and lengthening of the posterior cranial base, both of which may be beneficial in patients with turribrachycephaly.

Background Posterior cranial vault distraction osteogenesis (PVDO) has gained popularity as the initial intervention in patients with syndromic craniosynostosis. Patients may require secondary frontal orbital advancement (FOA) following PVDO, but little is known about the perioperative risks associated with this staged management. The purpose of this study is to compare the perioperative morbidity profile of secondary FOA (study) to that of primary FOA (control). Methods A retrospective review was conducted for patients with syndromic or complex craniosynostosis undergoing FOA between 2004 and 2017. Univariate and multivariate analysis of demographic and perioperative data were performed. Results Forty-three subjects met inclusion criteria, 17 in the study cohort and 26 in the control cohort. The 2 cohorts were similar with regards to diagnosis and suture involvement, as well as weight-adjusted estimated blood loss, blood transfusion volume, and length of hospital stay (P > 0.050). Secondary FOA procedures required longer operating time (231 ± 58 versus 264 ± 62 min, P = 0.031) and anesthesia time (341 ± 60 versus 403 ± 56 min, P = 0.002). The secondary FOA cohort had a significantly greater proportion of procedures with difficult wound closure (19% versus 59%, P = 0.008). Two subjects in the study cohort developed a wound dehiscence, compared with 1 subject in the control cohort (P = 0.552). Frontal orbital advancement as a secondary procedure after PVDO was a predictor variable in multivariate analysis for wound difficulties (odds ratio 8.6, P = 0.038). Conclusion Syndromic and complex craniosynostosis may safely be managed with initial PVDO followed by FOA, with some increased wound closure difficulty.

Background In orthognathic surgery, virtual surgical planning (VSP) is gaining popularity over traditional surgical planning (TSP); however, concerns about cost of VSP have slowed adoption of this technology. This study investigates the clinical value of VSP versus TSP over the entire clinical care continuum. Methods Retrospective cohort study was conducted for patients undergoing maxillomandibular surgery between 2005 and 2016 at a tertiary pediatric hospital. Clinical value, defined as patient outcomes per unit cost, was analyzed between the 2 groups with appropriate statistics. Results The VSP (n = 19) and TSP (n = 10) cohorts had statistically similar hospital lengths of stay, rates of complications, readmissions, and duration of postoperative orthodontic treatment (P = 0.518, P > 0.999, P > 0.999, P = 0.812, respectively). VSP maxillomandibular procedures trended towards shorter operative times (P = 0.052). Total hospital charges were statistically similar between the TSP and VSP cohorts (P = 0.160). Medication, laboratory and testing, and room charges were also statistically similar between the TSP and VSP cohorts (P = 0.169, P = 0.953, and P = 0.196 respectively). Conclusions Indexed patient outcomes and costs incurred for maxillomandibular procedures were statistically similar between those utilizing TSP or VSP leading us to conclude that these 2 methods are associated with similar clinical value. This retrospective analysis should be followed with prospective data to give patients and insurers the best estimate of clinical value utilizing TSP and VSP.

PURPOSE The aim of this study is to evaluate the effect of timing of surgery and spring characteristics on correction of scaphocephalic deformity in patients undergoing spring-mediated cranioplasty (SMC) for sagittal craniosynostosis. METHODS The authors conducted a review of patients with sagittal craniosynostosis who underwent SMC at a tertiary referral center between July 2011 and March 2017, with a primary outcome measure of head shape, both preoperatively and postoperatively, determined by cephalic index (CI). Patient demographics and operative details including timing of surgery and spring characteristics were collected. Differences in CI preoperation and postoperation were compared using Wilcoxon signed-rank test. Ordinary least-squares linear regression was used to assess the impact of timing, number of springs, maximum single spring force, and total spring force on postoperative change in CI. RESULTS Thirty-six subjects (12 males and 24 females) were included in the study. Mean age at spring placement was 3.9 months (range: 1.9-9.2) with a mean follow-up of 1.4 years (range: 0.3-5.2). The mean number of springs used was 3 (range: 2-4). The mean maximum single spring force was 9.9 Newtons (N) (range: 6.9-13.0) and the mean total spring force was 24.6 N (range: 12.7-37.0). Mean CI increased from 70 ± 0.9 preoperatively to 77 ± 1.0 postoperatively (P

Background Pediatric complex cranial vault reconstruction (CCVR) surgery is often associated with significant blood loss and transfusion. The authors recently changed our transfusion practice during CCVR to using whole blood (WB) instead of reconstituted blood (RB). The aim of this study was to assess the impact of this practice change. Our hypothesis was that replacement with WB would be as effective as RB for the outcomes of total perioperative blood donor exposures (BDEs) and the incidence of laboratory evidence of postoperative coagulopathy. Methods The authors queried the Pediatric Craniofacial Surgery Perioperative Registry for children ages ≤48 months from our institution who underwent CCVR and received either RB or WB. The primary outcomes of total perioperative BDEs and the incidence of laboratory evidence of postoperative coagulopathy were compared between the 2 cohorts. Results The query returned 59 subjects in the RB cohort and 52 subjects in the WB cohort. There were no significant differences in demographic variables between the 2 groups. Patients in the WB cohort were more likely to have ≤1 BDEs when compared to the RB cohort (62% versus 39%, respectively, P = 0.02). There was no significant difference in the incidence of postoperative coagulation laboratory test abnormalities between the WB and RB cohorts (0% versus 3.4%, respectively, P = 0.50). Conclusion There was no postoperative coagulopathy in the WB cohort. Whole blood was also associated with significantly fewer perioperative BDEs. Whole blood appears to be as effective as RB for replacement of blood loss in craniofacial surgery.

There is no clear consensus for the optimal treatment of sagittal craniosynostosis; however, recent studies suggest that improved neurocognitive outcomes may be obtained when surgical intervention imparts active cranial expansion or remodeling and is performed before 6 months of age. The authors consider spring-mediated cranioplasty (SMC) to optimally address these imperatives, and this is an investigation of how helmet orthoses before or after SMC affect aesthetic outcomes.The authors retrospectively evaluated patients treated with SMC and adjunct helmeting for sagittal synostosis. Patients were stratified into 4 cohorts based on helmet usage: preop, postop, both, and neither. The cephalic index (CI) was used to assess head shape changes and outcomes. Twenty-six patients met inclusion criteria: 6 (23%) had preop, 11 (42%) had postop, 4 (15%) had preop and postop, and 5 (19%) had no helmeting. Average age at surgery was 3.6 months. Overall, CI improved from a mean 69.8 to 77.9 during an average 7-month course of care. Mean preoperative change in CI showed greater improvement with preop helmet (1.3) versus not (0.0), (P = 0.029), despite similar initial CI in these cohorts (70.4 and 69.6 respectively, P = 0.69). Nonetheless, all patient cohorts regardless of helmeting status achieved similar final CIs (range 76.4-80.4; P = 0.72).In summary, preoperative molding helmet therapy leads to improved CI at the time of spring-mediated cranioplasty. However, this benefit does not necessarily translate into overall improved CI after surgery and in follow-up, calling into question the benefits of molding helmet therapy in this setting.

While in-hospital outcomes and long-term results of craniosynostosis surgery have been described, no large studies have reported on postoperative readmission and emergency department (ED) visits. The authors conducted this study to describe the incidence, associated diagnoses, and risk factors for these encounters within 30 days of craniosynostosis surgery.Using 4 state-level databases, the authors conducted a retrospective cohort study of patients

There is a growing literature on the advantages of posterior cranial vault distraction osteogenesis (PVDO) in infants, particularly those with syndromic and multisuture craniosynostosis. This study aims to compare perioperative outcomes of PVDO in older patients to those of infants. A prospective craniofacial database was queried for patients aged 5 and older undergoing PVDO; controls were diagnosis-matched infants. Demographic, perioperative, and distraction data was compared using a 2-sample t test and Fisher exact test.Twenty patients met inclusion criteria, and all had syndromic craniosynostosis. Mean age was 9.2 years for the older group, and 0.7 years for the younger. Older children had less weight-based blood loss (mean 58.6 ± 38.8 versus 14.6 ± 7.0 cc/kg, control versus older, P = 0.0092) and weight-based transfusion (mean 70.1 ± 37.2 versus 21.2 ± 9.9 cc/kg, control versus older, P = 0.0023); other perioperative variables were similar including duration of surgery, length of stay, distance distracted, time in consolidation, and length of follow-up. All patients in both groups successfully completed PVDO, and all older patients had resolution of papilledema and/or headaches. One from each group developed a wound infection that was treated with oral antibiotics.Posterior cranial vault distraction osteogenesis is a safe and efficacious for cranial vault expansion in syndromic craniosynostosis, with similar perioperative outcomes in older children as compared to infants. Posterior cranial vault distraction osteogenesis may be a reasonable alternative in older syndromic patients with acceptable frontal morphology and concerns for increased intracranial pressure.

Goldenhar syndrome is characterized by the typical features of craniofacial microsomia (CFM) with the addition of epibulbar dermoids and vertebral anomalies. The aim of this study is to examine the objective differences between patients carrying a diagnosis of Goldenhar syndrome to those diagnosed with CFM. Thus, we performed an Institutional Review Board-approved retrospective chart review on all patients who presented with a diagnosis of CFM or Goldenhar syndrome from January 1990 to December 2012. Demographic, diagnosis, OMENS+ classification, accompanying diagnoses, and radiographic data were collected. For subjective analysis, subgroups were designed based on the diagnosis Goldenhar syndrome or CFM per history. For objective analysis, subgroups were designed based on the presence of epibulbar dermoids and/or vertebral anomalies. The cohorts were compared with respect to associated medical abnormalities and severity of CFM features. One hundred thirty eight patients met inclusion criteria. Epibulbar dermoids and vertebral anomalies were seen in 17% and 34% of the patients, respectively. Only 10 patients (7.2%) had both epibulbar dermoids and vertebral anomalies. The subjective "Goldenhar" group (N = 44, 32%) was found to have a higher percentage of bilaterally affected patients (P = 0.001), a more severe mandibular deformity (P =

Fronto-orbital advancement and remodeling (FOAR) remains the most widely practiced treatment of unicoronal craniosynostosis (UCS) despite recent studies of ocular dysfunction and aesthetic shortcomings in the long-term. The aim of the study was to compare perioperative morbidity and short-term outcomes of a recently developed, nondevascularizing, distraction-based treatment of UCS with conventional FOAR. To do so, the authors compared the first 6 patients who were treated with a new osteotomy/distraction approach to the last 6 patients who underwent traditional FOAR for the treatment of UCS with regards to demographics, operative details, perioperative morbidity, and short-term outcomes.Between July 2012 and June 2014, 6 patients underwent each procedure. Duration of surgery and length of hospital stay in the distraction group were on average 2 hours 7 minutes and 3.4 days, respectively, significantly less than in the traditional FOAR group (P = 0.039, P = 0.032, respectively). Perioperative blood loss averaged 169 mL, which trended toward less than in the traditional group (mean of 400 mL, P = 0.065). Patients undergoing conventional compared with distraction osteogenesis-mediated FOAR were significantly more likely to develop new-onset strabismus postoperatively (odds ratio 15.4; P = 0.0384). All 12 patients completed therapy without complications and with Whitaker grade I results at latest follow-up.In the perioperative period, distraction-mediated cranial vault remodeling provides similar correction of the aesthetic deformity associated with UCS and an improved morbidity profile. Longer follow-up is needed to determine how distraction compares with FOAR with respect to neuropsychologic and long-term aesthetic outcomes.

The timing and surgical technique for the treatment of sagittal synostosis remain controversial. Calvarial vault remodeling (CVR), strip craniectomy (SC), and spring-mediated cranioplasty (SMC) are currently in use. We perform a meta-analysis of the literature to compare these 3 techniques.A literature search identified articles involving operative management of nonsyndromic sagittal synostosis. Comparison of 2 operative techniques was required, and methodology was assessed via the American Society of Plastic Surgeons' Levels of Evidence. Three techniques were considered: CVR, SC, and SMC. Meta-analysis was conducted for change in cephalic index (CI), reported as weighted mean difference (WMD). Pooled subgroup comparisons were performed for operative time, length of stay, blood loss, and cost.Twelve studies providing level 2 or 3 evidence were included. All studies involved CVR (n = 187), 8 involved SC (n = 299), and 7 involved SMC (n = 158). Head-to-head comparison of change in CI demonstrated a greater, yet statistically insignificant change for CVR versus SMC, WMD = 0.94 (-0.23 to 2.11) (P = 0.12, I(2) = 55%). Calvarial vault remodeling showed a statistically greater change in CI versus SC, WMD = 1.47 (0.47-2.48) (P = 0.004, I(2) = 66%). Compared with SMC/SC, CVR had longer operative length (170 vs 97 minutes), higher blood loss (238 vs 47 mL), longer length of stay (5.1 vs 2.9 days), and higher costs ($35,280 vs $13,147), all with P0.0001.This study, the first meta-analysis comparing 3 primary operations for correcting nonsyndromic sagittal synostosis, demonstrates no difference in CI for CVR versus SMC and a small but statistically greater improvement in CI favoring CVR over SC. Secondary outcomes favored SC/SMC procedures over CVR. However, long-term studies are still needed to adequately assess the risk-benefit ratios.

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.

Patients with syndromic craniosynostosis manifest midfacial hypoplasia often treated by midfacial advancement. Benefits of midfacial advancement by distraction osteogenesis have been well studied; little is known about the perioperative morbidity of these procedures, specifically relating to device selection. This study compares the perioperative complications between semiburied- and halo-type distraction osteogenesis of the midface. A retrospective review was performed on all patients with syndromic craniosynostosis who underwent midface distraction with semiburied- or halo-type external distractors. Demographic information and operative/postoperative course were reviewed. Complications were categorized as hardware-related, infectious, and either as major (requiring additional intervention) or minor (requiring medication only). Chi-squared and Fisher exact test were used to compare variables.From 1999 to 2012, a total of 54 patients underwent midface distraction osteogenesis, including 23 patients with Apert syndrome, 19 patients with Crouzon syndrome, 10 patients with Pfeiffer syndrome, and 2 patients with other craniofacial syndromes. Thirty-three patients underwent a total of 34 subcranial Le Fort III distraction procedures and 21 underwent 21 monobloc distraction procedures. The mean age during surgery was 8.0 (range, 4.0-17.7) years, whereas the mean time between distractor placement and removal was 102.9 days. Thirty procedures were performed with external halo-type distractors (18 Le Fort III and 12 monobloc distractions), whereas 25 were performed with buried midface distractors (16 Le Fort III and 9 monobloc distractions). There were no significant differences in diagnoses or interventions between the distraction devices. Of the 19 distractor-related complications, there were a total of 10 (18.2%) in the halo group including 5 (9.1%) requiring separate operative intervention as well as 9 (16.4%) in the buried distractor group including 6 (10.1%) requiring separate operative intervention. Major infections were more common in the buried distractor group (n = 8) compared with the halo distractor group (n = 3) (P = 0.048). There were 4 (7.3%) patients in the halo group who had malposition or transcranial pin migration related to postoperative positioning or falls and required operative repositioning. Frontofacial distraction is an important technique in patients with syndromic craniosynostosis. Higher rates of halo displacement requiring surgery are offset with lower rates of infections compared with buried distractors.

The aims of this study were to document the incidence of new-onset craniosynostosis (NOC) after posterior vault distraction osteogenesis (PVDO), to determine risk factors for the development of NOC, and to deduce the cranial ramifications of NOC. An institutional review board-approved retrospective review of all patients who underwent PVDO at the Children's Hospital of Philadelphia was performed. Demographics, perioperative data, as well as preoperative and postoperative three-dimensional computed tomographic scans were analyzed. Suture patency preoperatively and postoperatively was recorded.Thirty patients underwent PVDO for suspected increased intracranial pressure and/or severe turribrachicephaly from 2008 to 2013. Twenty-four patients had syndromic diagnoses. The average age at the time of PVDO was 2.03 years. Distraction distances ranged from 19 to 40 mm, with an average of 28.7 mm. Among the 19 patients who had patent lambdoid sutures before PVDO, new-onset lambdoid fusion was seen in 17 patients after PVDO (89.5%), whereas the suture remained open in 2 patients (10.5%). New-onset lambdoid fusion was not significantly associated with age at distraction (P = 0.28), sex (P = 0.47), length of distraction (P = 0.93), or diagnosis (P = 0.61). Similarly, new-onset sagittal synostosis was not associated with age at distraction (P = 0.06), sex (P = 0.64), length of distraction (P = 0.83), or diagnosis (P = 0.25). None of the patients who developed NOC had characteristic head shape changes such as mastoid bulges or scaphocephaly. New-onset lambdoid and sagittal synostoses occur frequently after PVDO. Although the diagnosis of NOC is obvious radiographically, the clinical importance of the diagnosis morphometrically, neurodevelopmentally, and in cranial growth has yet to be fully investigated.

Three-dimensional virtual surgical planning using computer-aided design and modeling (CAD/CAM) has gained popularity in planning complex orthognathic and osteocutaneous free flap reconstructions of the head and neck because of its ability to guide complex geometric planning in three-dimensional space and save time in the operating room. The purpose of this study was to review our experience using CAD/CAM concepts in periorbital osteotomies. Three complex periorbital osteotomies were planned and performed: 1 case of bilateral vertical and horizontal orbital dystopia, 1 case of a Monobloc-Le Fort II in an Apert patient, and 1 case of recurrent hypertelorism in a patient with craniofrontal nasal dysplasia. The patients' charts were reviewed, including photographs, medical records, and CAD/CAM plans. The CAD/CAM planning sessions were held 3 to 6 weeks preoperatively and lasted approximately 1 hour. Both cutting guides and positioning guides were used, translating to significant precision of both the osteotomy as well as the final position of the orbits. Qualitatively, the cutting and positioning guides were easy to use and improved operating room efficiency. To conclude, in our hands, CAD/CAM virtual surgical planning is safe and effective in the performance of complex periorbital osteotomies. More work is needed to more clearly define surgical indications for this costly, new technology.

Background Bone transport osteogenesis (BTO), distraction of a free portion of bone across a defect, offers an autologous solution to large cranial defects that may allow treatment without permanent hardware implantation. This study establishes a sheep model to evaluate the feasibility and distraction kinetics of BTO. Methods Subtotal cranial defects (3.5 × 3.5 cm) were created in 10 young adult sheep and a transport segment (3.5 × 2 cm) traversed the defect at varying distraction rates (0, 0.5, 1.0, and 1.5 mm/day) using semi-buried cranial distractors. After a 6-week consolidation period, sheep were euthanized and the resultant bone was analyzed by CT, histology, and mechanical testing. Results Gross examination, histology, and 3D CT revealed that control animals had fibrous nonunion whereas distraction animals had ossified defects with fibrous nonunion at the distal docking site. There was one premature consolidation in the 0.5 mm/day group. The volume of bony regenerate in the 0.5, 1.0, and 1.5 mm/day distraction rate groups was statistically indistinct (P = 0.16). The mean flexural moduli (MPa) of non-decalcified samples from the control cranium, transport segment, and bone regenerate were found to be 4.50 ± 4.9, 6.17 ± 2.1, and 4.14 ± 4.8, respectively (P = 0.24). Conclusions This experiment provides proof of concept for BTO for large calvarial defects in a sheep model. Distraction at a rate of 0.5 mm per day may place individuals at higher risk for premature consolidation, but distraction rates did not have significant effects on regenerate quantity or quality. Future work will include the use of curvilinear distraction devices for 3-dimensional contour.

In the setting of recurrent infection and multiple failed reconstruction attempts, the choice of the ideal reconstructive material for salvage cranioplasty remains a source of controversy in the literature. The purpose of this study is to establish the safety and utility of antibiotic-impregnated polymethyl methacrylate (PMMA) for salvage cranioplasty.A prospectively maintained database of all patients who underwent salvage cranioplasty using vancomycin and tobramycin-impregnated methyl methacrylate from January 2011 to July 2013 was reviewed. Vancomycin and tobramycin were mixed in PMMA, which was then applied to a rigidly fixed titanium mesh for reconstruction. Patients' demographics, indications, and outcomes of this technique were evaluated.Nine patients (mean age: 47 years) underwent vancomycin and tobramycin-impregnated PMMA reconstruction with a mean follow-up of 9.3 months (range 3.5-23 months). On average, these patients underwent 4 procedures (range: 1-15), which included repeat craniotomy, debridement for infection, and failed reconstructions over the course of 3.6 years (range: 7 months to 14 years) before salvage cranioplasty. All patients required salvage cranioplasty due to infection, with the most common bacteria isolated in culture being Propionibacterium acnes (n = 3), multiresistant coagulase-negative Staphylococcus (n = 3), methicillin-resistant Staphylococcus aureus (n = 2), and Enterobacter (n = 2). The average size of the craniectomy defect was 130 cm(2), and there were no incidences of postoperative infection, postoperative complications, or need for revisions.To conclude, in short-term follow-up, vancomycin and tobramycin-impregnated PMMA reconstruction appears safe and effective in salvage cranioplasty. Our early report represents a proof of concept--the true test is whether these short-term successes translate to stable long-term results.

The spheno-occipital synchondrosis (SOS) is a critical component of midfacial and cranial base growth. Premature closure has been associated with midface hypoplasia in animal models and syndromic craniosynostosis subpopulations with Apert and Muenke syndromes. To link premature SOS closure and midface hypoplasia in patients with Pfeiffer syndrome, a retrospective case-control study was performed in patients treated at a large craniofacial center between 1982 and 2012 diagnosed with Pfeiffer syndrome. At least 1 computed tomography (CT) scan was required to assess SOS patency. Age-/sex-matched control CT scans were also assessed for SOS patency. Three independent reviewers with high interrater reliability (κ = 0.88) graded SOS patency as open, partially closed, or completely closed. Wilcoxon rank sum test compared the Pfeiffer patients with control subjects. A total of 63 CT scans in 16 patients with Pfeiffer syndrome, all with midface hypoplasia, and 63 age-/sex-matched control scans, none of whom had midface hypoplasia, met inclusion criteria. Earliest partial SOS closure in patients with Pfeiffer syndrome was seen at 5 days compared with control subjects at 7.07 years. Earliest age at complete fusion was 2.76 years in the Pfeiffer cohort and 12.74 years in control subjects. Average age at partial closure was significantly younger (4.99 ± 3.33 years; n = 31 scans) in patients with Pfeiffer syndrome compared with control subjects (10.92 ± 3.53 years) (P = 0.0005), whereas average age at complete closure (11.90 ± 7.04 years) was not significantly different than that in control subjects (16.07 ± 3.39 years). Although definitive causality cannot be concluded, a strong correlation exists between midface hypoplasia and premature SOS closure in Pfeiffer syndrome.

Multisuture craniosynostosis with a mild cloverleaf deformity is rare but associated with high morbidity and mortality. Conventional treatment to alleviate intracranial hypertension in a young infant involves multisuturectomy and relies on passive correction of the deformity followed by additional staged reconstruction later in infancy. Early regional craniectomy and rigid reconstruction have been described, but the cranial bone has limited stability to tolerate plate fixation and extensive dissection of the bone-dura interface may devascularize the cranial bone flap and limit its durability. The authors report an interesting technique to treat a mild form of cloverleaf skull deformity using early, nondevascularizing osteotomies followed by application of semiburied cranial distractors in multiple planes to increase intracranial volume and treat the deformity, and its attendant volumetric constriction, in a single stage.

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.

The psychosocial impact of craniofacial disfigurement affects both the developing child and his/her family. The Facial Reconstruction Center at the Children's Hospital of Philadelphia has employed a Parent Liaison (PL) to provide psychosocial support to families and has been an invaluable resource in this regard. We hypothesize that a PL impacts the overall outcome of the surgery by building trust between the parents and medical institution, and increasing satisfaction. An anonymous satisfaction survey was sent to families of craniofacial patients treated between January 1976 and June 2012. All patients who had undergone at least 1 craniofacial procedure had addresses on file and were included in this study. Statistical analyses were performed using the Mann-Whitney U test.During the study, 441 surveys were mailed to families meeting the inclusion criteria. A total of 151 families returned completed surveys (34.2%), and 121 surveys were included for analysis (27.4%). In rating overall satisfaction, families who met with the PL had statistically higher scores than those who had not (P = 0.0011). Parents who met with the PL preoperatively reported greater satisfaction in time spent answering questions (P = 0.0029) and the perception that questions were adequately answered (P = 0.0039). No statistical difference was observed in postoperative preparedness between families that did and did not meet the PL. The results demonstrate that the PL is beneficial in the education, experience, and satisfaction of families treated at a large Craniofacial Center. The PL complements the surgeon's treatment of the physical by adding psychosocial support.

BACKGROUND Craniofacial surgery is a diverse subspecialty of plastic surgery that focuses on a wide range of head and neck pathology in children and adults. The purpose of this study was to define the characteristics of this group of surgeons and to compare subgroups within the specialty. METHODS A 36-question, anonymous, electronic survey was sent to 403 craniofacial surgeons; the response rate was 30% (121). Distribution was to members of the International Society of Craniofacial Surgeons and to graduates of fellowships recognized by the American Society of Craniofacial Surgeons. Data were collected and analyzed for surgeon demographics, geography, practice setting (academic vs private), case mix and volume, and career satisfaction. Comparisons were made between US and international surgeons, males and females, and surgeons of different ages. RESULTS The craniofacial surgeons in this study ranged in age from 29 to 75 years (mean, 53 years); 92% were male, and 8% were female. They are largely academic (69%), high in academic rank (54% full professors), predominantly male (92%), and actively practicing craniofacial surgery. There are significant differences between international and domestic surgeons in terms of training background (64% vs 36% plastic surgery residency, P = 0.003) and volume of craniofacial surgery (56% vs 26% performing more than 5 complex craniofacial procedures per year, P = 0.002). CONCLUSIONS Craniofacial surgeons are a highly successful academic group with high career satisfaction. There are significant differences between US and international craniofacial surgeons in terms of demographics and practice, with more US surgeons performing fewer major craniofacial osteotomies. There is a significant gender disparity, which warrants further study.

The purpose of this study was to describe our technique of bilateral mandibular distraction for micrognathia and to highlight the ultrasonic scalpel as an alternative to conventional saws in performing osteotomies for mandibular distraction osteogenesis. To do so, we retrospectively reviewed all patients who underwent mandibular distraction with an ultrasonic scalpel for tongue-based upper airway obstruction due to micrognathia between 2010 and 2011. Study outcome measures include operative blood loss, length of surgery, postoperative complications, and avoidance of a tracheostomy. Excel (Microsoft) was used to calculate averages, P values (2-tailed Student t test), and SDs for operative data, sleep studies, and cephalometric analysis. Nine patients--7 females and 2 males--were distracted for a mean distance of 17 ± 6 mm. Mean blood loss was 15 ± 7 mL, and the average length of surgery was 111 ± 27 minutes. One patient returned to the operating room for debridement/washout of a wound infection, but distraction was continued without sequelae. There were no other postoperative complications. Resolution of airway obstruction was evidenced by clinical examination and avoidance of a tracheostomy in all cases. Based on these data, we feel that mandibular distraction with univector, internal distractors, and ultrasonic osteotomies at the mandibular angle is safe and efficacious at relieving tongue-based upper airway obstruction and avoiding a tracheostomy.

Background: The monobloc procedure has been criticized owing to its tendency for cerebrospinal fluid leak, relapse, infection, and incomplete ossification. Such risks have been decreased through gradual advancement of the monobloc via distraction osteogenesis. This cadaver study was undertaken to develop an endoscopic, ultrasonic monobloc osteotomy to further minimize risks and potentially improve outcomes. Methods: Three fresh, adult human cadavers were used in this study. Endoscopic ultrasonic monobloc osteotomy was completed in all cadavers with 3 incisions hidden in the hair-bearing scalp. The incisions afforded access for small craniotomies through which the dura was dissected from the frontal bones. An ultrasonic scalpel and endoscope then traveled extradurally to osteotomize the frontal bones, temporal bones, sphenoid wings, and superior aspects of the orbits intracranially. Pterygomaxillary dysjunction was performed with conventional osteotomes intraorally. Results: The endoscopic ultrasonic monobloc osteotomy was completed as a single fragment in all 3 cadavers. No additional incisions were required. Completeness of the osteotomy and integrity of the single fragment were evaluated by manual examination and endoscopic visualization of free movement at osteotomy sites. Osteotomy completion took less than 2.5 hours. Dura and periosteum surrounding all osteotomies remained intact, eliminating concern for injury to adjacent soft tissue. Careful placement of temporal incisions and craniotomies was critical to facilitate completion of osteotomies in a clinically safe manner. Conclusions: We have demonstrated the feasibility of an endoscopic ultrasonic monobloc advancement technique in cadavers. The technique can be completed without a bicoronal incision while completely protecting all vital structures. The preservation of vascularity and periosteum afforded by this technique may provide improved outcomes and reduced complications.

Drawbacks to conventional Le Fort III osteotomy include bleeding, infection, relapse, and scar at the coronal incision. We performed an endoscopically assisted Le Fort III osteotomy with an ultrasonic scalpel in cadavers to develop a new technique that minimizes such complications. Endoscopically assisted Le Fort III osteotomy was performed in 3 fresh, adult human cadavers. Access incisions included the transconjunctival lower lid with lateral canthotomy, the lateral upper gingivobuccal sulcus, and a stab incision in the medial aspect of the upper eyelid. Osteotomies at the zygomaticofrontal suture, the lateral orbital wall, the orbital floor, and the medial orbital wall were carried out with an ultrasonic scalpel under direct and endoscopic visualization from the trasconjunctival incision. The zygomatic arch and the pterygomaxillary region were osteotomized via the upper gingivobuccal sulcus incisions. The nasofrontal junction and the septum were accessed and cut via a stab incision in the medial upper eyelids. Disimpaction was completed with minor digital pressure inferiorly.Each of the 3 Le Fort III osteotomies was complete, and mobility was checked by manual examination. There was a steep learning curve to the operation, but the final cadaver dissection took 99 minutes to complete. The ultrasonic scalpel provided for maximal ease in cutting bone and minimal disruption to adjacent soft tissues as judged by postoperative direct examination. This cadaver study demonstrates the feasibility of a minimally invasive, endoscopically assisted Le Fort III osteotomy using an ultrasonic scalpel. Further experimental work combined with refinements in technique and equipment will help bring this advancement into clinical application.

Treatment of the Apert syndrome phenotype aims to correct airway obstruction, exorbitism, elevated intracranial pressure, midface hypoplasia, and malocclusion. Cranial vault expansion prevents elevated intracranial pressure, normalizes head shape, and protects the globes, but variation exists in surgical timing and osteotomy to treat the midface. We present the case of an 11-year-old female patient with Apert syndrome and no prior surgical interventions who presented with severe turribrachycephaly, exorbitism, severe midface retrusion, and apertognathia. A monobloc distraction with simultaneous Le Fort II distraction was planned using computer-aided design and modeling (CAD/CAM) techniques to provide for concurrent distraction of the segments in independent vectors without bony interferences. Monobloc minus Le Fort II distraction was performed without intraoperative complications. Surgical time was 340 minutes with an estimated blood loss of 1100 mL. Distraction began on postoperative day 5 at a rate of 1.5 mm/day for the Le Fort II via an external Halo distractor and 1 mm/day for the monobloc segment via internal distractors anchored bitemporally. The monobloc was distracted a total of 17 mm in a horizontal vector, while the Le Fort II segment was distracted 18 mm horizontally and 5 mm inferiorly. The Halo distractor was removed 3 months following the procedure and the internal distractors 1 month later. Monobloc minus Le Fort II distraction enables correction of the Apert phenotype with a single-stage approach, potentially decreasing the burden of care with improved results. Utilization of CAD/CAM modeling allows for accurate planning of multisegment distraction in independent vectors without concerns for bony interferences.

Melnick-Needles syndrome is an X-inked-dominant skeletal dysplasia in which there is deficient osteoblastic activity. Patients present with craniofacial anomalies consisting of a prominent forehead, exorbitism, mandibular hypoplasia, cheek fullness, and class II malocclusion. Severe mandibular hypoplasia leads to upper airway restriction, an increased incidence of sleep apnea and pneumonias, and occasionally respiratory failure. This is a report of a patient with Melnick-Needles syndrome who presented to our unit after multiple bouts of respiratory failure and with a tracheostomy in whom mandibular distraction osteogenesis was used to retire her tracheostomy and to cure her sleep apnea. The patient underwent bilateral, external, unidirectional mandibular distraction with a vector parallel to the occlusal plane. After a latency period of 5 days, distraction was initiated at a rate of 1 mm/day for 34 days. At this point, the patient was able to breathe with the tracheostomy plugged, and her occlusion had changed from a class II to a class III relationship. She no longer snored, and pulse oximetry on room air was normal while standing or supine. Interestingly, the patient's consolidation phase was prolonged--255 days--possibly attributable to altered bony metabolism. To our knowledge, this is the first reported case of mandibular distraction osteogenesis used to cure obstructive sleep apnea and eliminate the need for tracheostomy in a patient with Melnick-Needles syndrome. In the future, prophylactic mandibular distraction may prevent the need for tracheostomy in this group of patients.