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Vertical expandable prosthetic titanium rib (VEPTR) devices were designed to treat childhood scoliosis and thoracic insufficiency syndrome. Although they have drastically reduced patient mortality, they present a profound subcutaneous hardware burden. The authors examined the surgical and nonsurgical factors associated with salvage of VEPTR hardware exposures.Between 2014 and 2020, a prospective institutional database was queried for patients with VEPTR hardware complications who required soft-tissue reconstruction. Hardware salvage was considered successful if reconstruction allowed the hardware to be retained until the next VEPTR expansion.Fifty-eight patients required VEPTR hardware salvage. Hardware complications were successfully salvaged in 62.1 percent of patients at 60.0 percent of operative sites. Neuromuscular scoliosis (p = 0.041) and nonambulatory status (p = 0.018) were factors associated with VEPTR hardware salvage failure, whereas congenital scoliosis was associated with successful hardware salvage (p = 0.012) and preventing need for immediate hardware removal (p = 0.049). Exposed hardware, as compared to threatened exposure, was more likely to require immediate removal (p = 0.045) and result in unsuccessful hardware salvage (p = 0.015).Local and regional muscle flaps were able to prevent VEPTR hardware removal in the majority of patients, even in the setting of infection, immobility, incontinence, and multiple systemic comorbidities. Patients with neuromuscular scoliosis and nonambulatory status were at increased risk for failure, whereas those with incontinence and low body mass index trended toward increased risk of failure. Threatened exposure was associated with higher rates of salvage than exposed hardware, and thus earlier referral to plastic surgeons for soft-tissue salvage may be advised.Risk, III.

Previous literature has documented craniometric changes of the bony calvaria, increases in intracranial volume, and resolution of Chiari malformations following posterior vault distraction osteogenesis. No studies have analyzed changes to the soft-tissue envelope after posterior vault distraction osteogenesis. In this study, the authors aimed to provide objective measurements of scalp thickness in patients undergoing posterior vault distraction osteogenesis, utilizing facial soft-tissue thickness as a proxy to control for growth. The authors hypothesized that the soft tissues of the scalp are not made thinner by the distraction process, either in the region of distraction or in neighboring areas. Subjects who underwent posterior vault distraction osteogenesis for a craniosynostosis diagnosis who had high-resolution predistraction and post-distractor removal computed tomographic scans within 100 days of each operation were included. The scans were analyzed on Materialise Mimics version 21 software (Materialise, Ghent, Belgium). Six key craniometric landmarks (glabella, pogonion, zygion, vertex, euryon, and opisthocranium) were identified on the three-dimensional bone masks in the Frankfort horizontal plane. The points were overlaid onto soft-tissue thickness masks and thicknesses were recorded. Percent change in postoperative facial soft-tissue thickness was used to control for growth of the craniofacial soft-tissue envelope during the study interval. The preoperative and postoperative posterior vault distraction osteogenesis cohorts did not differ significantly at the glabella, pogonion, opisthocranium, vertex, and zygion. The corrected median thickness at the euryon was significantly increased in the post-posterior vault distraction osteogenesis cohort [2.67 mm (IQR, 2.49 mm to 4.02 mm) versus 5.26 mm (IQR, 3.83 mm to 7.82 mm), p = 0.002]. This is the first study to quantify changes in soft-tissue thicknesses preoperatively and postoperatively in patients undergoing posterior vault distraction osteogenesis, demonstrating maintenance of scalp thickness. .Therapeutic, IV.

Craniosynostosis may lead to elevated intracranial pressure, which may be implicated with impaired neurocognitive development. However, accurately measuring intracranial pressure is challenging, and patterns in craniosynostosis patients are poorly characterized. Spectral-domain optical coherence tomography may enable noninvasive assessment of intracranial pressure in pediatric patients with craniosynostosis.Pediatric patients with craniosynostosis undergoing surgical intervention between 2014 and 2019 prospectively underwent optical coherence tomographic evaluation. Intracranial pressure was directly measured intraoperatively in a subset of cases. Optical coherence tomographic parameters were compared to directly measured intracranial pressure and used for pattern assessment.Optical coherence tomography was performed in 158 subjects, among which 42 underwent direct intracranial pressure measurement during an initial cranial procedure. Maximal retinal nerve fiber layer thickness, maximal retinal thickness, and maximal anterior projection optical coherence tomographic parameters were positively correlated with intracranial pressure (p ≤ 0.001), with all parameters showing significantly higher values in patients with intracranial pressure thresholds of 15 mmHg (p0.001) and 20 mmHg (p ≤ 0.007). Patients with maximal retinal nerve fiber layer thickness and maximal anterior projection exceeding set thresholds in optical coherence tomography of either eye demonstrated 77.3 percent sensitivity and 95.0 percent specificity for detecting intracranial pressure above 15 mmHg, and 90.0 percent sensitivity and 81.3 percent specificity for detecting intracranial pressure above 20 mmHg. Patients with associated syndromes or multiple suture involvement and patients aged 9 months or older were significantly more likely to have elevated intracranial pressure above 15 mmHg (p ≤ 0.030) and above 20 mmHg (p ≤ 0.035).Spectral-domain optical coherence tomography can noninvasively detect elevated intracranial pressure in patients with craniosynostosis with reliable sensitivity and specificity. This technology may help guide decisions about the appropriate type and timing of surgical treatment.Diagnostic, I.

The relationship between poverty and incidence of cleft lip and cleft palate remains unclear. The authors investigated the association between socioeconomic status and cleft lip with or without cleft palate and cleft palate only in the United States after controlling for demographic and environmental risk factors.The U.S. 2016 and 2017 natality data were utilized. Proxies for socioeconomic status included maternal education, use of the Special Supplemental Nutrition Program for Women, Infants, and Children, and payment source for delivery. Multiple logistic regression controlled for household demographics, prenatal care, maternal health, and infant characteristics.Of 6,251,308 live births included, 2984 (0.05 percent) had cleft lip with or without cleft palate and 1180 (0.02 percent) had cleft palate only. Maternal education of bachelor's degree or higher was protective against, and delayed prenatal care associated with, cleft lip with or without cleft palate (adjusted ORs = 0.73 and 1.14 to 1.23, respectively; p0.02). Receiving assistance under the Special Supplemental Nutrition Program for Women, Infants, and Children was associated with cleft palate only (adjusted OR = 1.25; p = 0.003). Male sex, first-trimester tobacco smoking, and maternal gestational diabetes were also associated with cleft lip with or without cleft palate (adjusted ORs = 1.60, 1.01, and 1.19, respectively; p0.05). Female sex, prepregnancy tobacco smoking, and maternal infections during pregnancy were associated with cleft palate only (adjusted ORs = 0.74, 1.02, and 1.60, respectively; p0.05).Increased incidence of orofacial clefts was associated with indicators of lower socioeconomic status, with different indicators associated with different cleft phenotypes. Notably, early prenatal care was protective against the development of cleft lip with or without cleft palate.Risk, III.

OBJECTIVE Endoscopic strip craniectomy (ESC) and spring-mediated cranioplasty (SMC) are two minimally invasive techniques for treating sagittal craniosynostosis in early infancy. Data comparing the perioperative outcomes of these two techniques are sparse. Here, the authors hypothesized that outcomes would be similar between patients undergoing SMC and those undergoing ESC and conducted a study using the multicenter Pediatric Craniofacial Surgery Perioperative Registry (PCSPR). METHODS The PCSPR was queried for infants under the age of 6 months who had undergone SMC or ESC for sagittal synostosis. SMC patients were propensity score matched 1:2 with ESC patients on age and weight. Primary outcomes were transfusion-free hospital course, intensive care unit (ICU) admission, ICU length of stay (LOS), and hospital length of stay (HLOS). The authors also obtained data points regarding spring removal. Comparisons of outcomes between matched groups were performed with multivariable regression models. RESULTS The query returned data from 676 infants who had undergone procedures from June 2012 through September 2019, comprising 580 ESC infants from 32 centers and 96 SMC infants from 5 centers. Ninety-six SMC patients were matched to 192 ESC patients. There was no difference in transfusion-free hospital course between the two groups (adjusted odds ratio [aOR] 0.78, 95% CI 0.45–1.35). SMC patients were more likely to be admitted to the ICU (aOR 7.50, 95% CI 3.75–14.99) and had longer ICU LOSs (incident rate ratio [IRR] 1.42, 95% CI 1.37–1.48) and HLOSs (IRR 1.28, 95% CI 1.17–1.39). CONCLUSIONS In this multicenter study of ESC and SMC, the authors found similar transfusion-free hospital courses; however, SMC infants had longer ICU LOSs and HLOSs. A trial comparing longer-term outcomes in SMC versus ESC would further define the roles of these two approaches in the management of sagittal craniosynostosis.

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

Unilateral coronal nonsyndromic craniosynostosis is associated with asymmetric skull growth, which may influence cerebral long-term function. Twenty affected adolescents who underwent cranial vault remodeling at a mean age of 8.2 months (12 from the Yale Craniofacial Clinic and eight from the Children's Hospital of Philadelphia) all completed a double-blinded neurodevelopmental assessment at an average age of 12.1 years. The study cohort included 55 percent female and 50 percent right-sided craniosynostosis. Mean verbal intelligence quotient was highest at 117.3, and mean performance intelligence quotient was 106.4, for a mean full-scale intelligence quotient of 112.5. Patients performed above the national average on all academic achievements except for numerical operations, which was significantly lower than word reading (p = 0.022). Patients performed below average on all Beery-Buktenica visual motor tests; motor-coordination was poorer than both visual motor integration and visual perception (p = 0.027 and p = 0.005). Significant positive correlations existed between paternal education/visual perception (r = 0.450; p = 0.046) and household income/verbal intelligence quotient (r = 0.628; p = 0.004). Patients with right unilateral coronal nonsyndromic craniosynostosis had improved spelling compared with left-sided patients on multivariate regression (p = 0.033). Female patients had higher motor coordination (p = 0.024). Breast-fed patients had better performance intelligence quotient (p = 0.024), visual motor integration (p = 0.014), and visual perception (p = 0.031). Adolescents who underwent cranial vault remodeling at two institutions had above average intelligence quotient scores, but worse mathematical and visual motor achievement compared with control subjects. Left-side craniosynostosis patients performed worse in spelling than right-side patients. Breast-feeding was an independent predictor for improved performance intelligence quotient, visual motor achievement, and visual perception performance. Study findings are limited by the cohort size. A larger population study is required, which could validate or modify the study conclusions.

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

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

BACKGROUND: Macroglossia, a cardinal feature of the (epi)genetic disorder Beckwith-Wiedemann syndrome, is associated with obstructive sleep apnea, speech and/or feeding difficulties, and dental or jaw malalignment. These sequelae may be treated and/or prevented with tongue reduction surgery; the authors sought to determine whether certain Beckwith-Wiedemann syndrome patients may benefit from early surgical intervention before age 12 months. METHODS: The authors conducted a retrospective review of patients with Beckwith-Wiedemann syndrome who underwent tongue reduction from 2014 to 2019. The authors assessed primary outcomes of change in obstructive sleep apnea by polysomnography, respiratory support required, and feeding route before and after tongue reduction, and reviewed postoperative complications and the need for repeated tongue reduction. RESULTS: Of the 36 patients included, the median age at tongue reduction was 9.5 months (interquartile range, 3.8 to 22.8 months). For those with severe obstructive sleep apnea, there was a significant reduction in the obstructive apnea hypopnea index from 30.9 ± 21.8 per hour to 10.0 ± 18.3 per hour (p = 0.019) and improvement in nadir oxyhemoglobin saturation from 72 ± 10 percent to 83 ± 6 percent (p = 0.008). Although there was no significant change in overall supplemental feeding tube or respiratory support, there were specific patients who experienced clinically meaningful improvement. Of note, these positive outcomes applied equally to those who underwent surgery at a younger age (

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.

Both congenital cystic adenomatoid malformation (CCAM) and lambdoid craniosynostosis are exceedingly rare, with estimated incidences of around 1 in 30,000 live births for each. Herein, we present a case of a patient with prenatally diagnosed CCAM requiring 4 doses of prenatal steroids and ultimately thoracotomy with right lower lobectomy. The operation was uncomplicated, and genome-wide analysis of SNPs revealed no pathologic abnormalities. Development was normal until head shape changes were noted at 5 months of age at which point evaluation by a craniofacial surgeon and 3D CT imaging revealed left-sided lambdoid craniosynostosis. The patient underwent uncomplicated posterior and middle vault expansion and remodeling of his lambdoid synostosis at 11 months of age. This paper explores the etiopathogenesis of CCAM and lambdoid craniosynostosis in an attempt to identify common pathways involved in the development of these exceedingly rare diseases which, to date, have not been described in the peer-reviewed literature.

The authors' group characterized the cranio-maxillo-facial workforce 10 years ago, revealing high levels of career satisfaction but significant gender disparity. This study provides an updated profile of the international cranio-maxillo-facial workforce.A 30-question electronic survey was distributed to 387 cranio-maxillo-facial surgeons with membership in the American Society of Craniofacial Surgeons, American Society of Maxillofacial Surgeons, and international Society of Craniofacial Surgery. Questions related to demographics, training background, practice setting, surgical volume, career satisfaction, and perceived discrimination. Independent samples t test was used to compare continuous variables.The authors received 91 responses (response rate = 23.5%). The majority of respondents were White (n = 73, 80.2%), non-Hispanic (n = 85, 93.4%), heterosexual (n = 72, 79.1%), cisgender males (n = 74, 81.3%). Practice setting was primarily academic, 65.9% (n = 60) and group/hospital-based 88.3% (n = 68), with two-thirds of respondents practicing in the United States. Female surgeons reported earlier planned retirement (66 versus 70 years, P = 0.012) and more personal encounters with discrimination of any kind (69% versus 29%, P = 0.033). US craniofacial surgeons reported more racial and sexual orientation-based discrimination compared with non-US surgeons ( P = 0.049 and P = 0.048, respectively). Older surgeons (55 years old) reported less perceived gender discrimination ( P = 0.041). There was no difference between subgroups in career satisfaction or likelihood of repeating/recommending a cranio-maxillo-facial fellowship.Female representation in cranio-maxillo-facial surgery on an international scale has increased over the past decade, but this study demonstrates persistent, disparate perception of workplace discrimination by gender, practice region, and age.

BACKGROUND Fusion of cranial-base sutures/synchondroses presents a clinical conundrum, given their often unclear "normal" timing of closure. This study investigates the physiologic fusion timelines of cranial-base sutures/synchondroses. METHODS Twenty-three age intervals were analyzed in subjects aged 0 to 18 years. For each age interval, 10 head computed tomographic scans of healthy subjects were assessed. Thirteen cranial-base sutures/synchondroses were evaluated for patency. Partial closure in greater than or equal to 50 percent of subjects and complete bilateral closure in less than 50 percent of subjects defined the fusion "midpoint." Factor analysis identified clusters of related fusion patterns. RESULTS Two hundred thirty scans met inclusion criteria. The sutures' fusion midpoints and completion ages, respectively, were as follows: frontoethmoidal, 0 to 2 months and 4 years; frontosphenoidal, 6 to 8 months and 12 years; and sphenoparietal, 6 to 8 months and 4 years. Sphenosquamosal, sphenopetrosal, parietosquamosal, and parietomastoid sutures reached the midpoint at 6 to 8 months, 8 years, 9 to 11 months, and 12 years, respectively, but rarely completed fusion. The occipitomastoid suture partially closed in less than or equal to 30 percent of subjects. The synchondroses' fusion midpoints and completion ages, respectively, were as follows: sphenoethmoidal, 3 to 5 months and 5 years; spheno-occipital, 9 years and 17 years; anterior intraoccipital, 4 years and 10 years; and posterior intraoccipital, 18 to 23 months and 4 years. The petro-occipital synchondrosis reached the midpoint at 11 years and completely fused in less than 50 percent of subjects. Order of fusion of the sutures, but not the synchondroses, followed the anterior-to-posterior direction. Factor analysis suggested three separate fusion patterns. CONCLUSIONS The fusion timelines of cranial-base sutures/synchondroses may help providers interpret computed tomographic data of patients with head-shape abnormalities. Future work should elucidate the mechanisms and sequelae of cranial-base suture fusion that deviates from normal timelines.

BACKGROUND Resorbable hardware allows adequate strength for maintaining the relative position of the craniofacial skeleton during osseous healing, and allowing subsequent growth in pediatric patients. The purpose of this study was to determine the complication profile of the latest copolymer of resorbable plates for craniofacial reconstruction in pediatric patients. METHODS Retrospective query of the operative billing record was performed for use of the DePuy Synthes Rapidsorb Fixation System at the authors' tertiary children's hospital from 2015 to 2019. Three hundred twenty-five patients were randomly selected for analysis of complications within the first postoperative year. RESULTS Median age at craniofacial reconstruction was 3.0 years, with 127 procedures (39.9 percent) performed for an intracranial mass and 141 procedures (44.3 percent) performed for craniosynostosis. Overall, 7.9 percent patients had a postoperative wound complication. The most common complications were dehiscence (3.8 percent), hematoma/seroma (3.5 percent), and infection (2.5 percent). There were no instances of extrusion, plate fracture, or screw loosening. Hardware complications in the temporoparietal region were more likely to occur at incision sites (p = 0.001), whereas wound complications at the frontal region were more likely to occur away from incision sites (p < 0.001). There were no differences in complications between plates secured with resorbable screws or with the resorbable injectable polymer system (p ≥ 0.161 for all). Radiotherapy was significantly implicated in development of clinical infection (p = 0.001), culture-positive infection (p < 0.001), readmission (p = 0.007), reoperation (p = 0.003), and plate removal (p = 0.007). CONCLUSION Resorbable cranial hardware has an overall favorable complication profile for craniofacial reconstruction in pediatric patients undergoing surgical intervention for craniosynostosis or intracranial mass resection.

Study Design Single-center retrospective analysis of a prospectively collected registry. Objectives Identify factors predictive of rib-based distraction (RBD) instrumentation wound complication. Create a risk stratification model for RBD instrumentation wound complication. Summary of Background Data RBD instrumentation procedures have a high rate of wound complications, often requiring unplanned operative treatment. Currently, there is a relative lack of understanding of RBD complication risk factors compared with the comprehensive understanding of complication risk factors for other spine surgeries. Methods Between January 2011 and September 2015, patients treated with RBD instrumentation at a single institution were analyzed for risk factors associated with surgical wound complications that resulted in unplanned operative treatment. Univariate logistic regression determined predictors of wound complication and multivariate regression determined independent predictive factors; α = 0.10. Results A total of 122 patients aged 0–18 years underwent 140 implant surgeries in which 22 resulted in complications: 18 (82%) infectious and 4 (18%) noninfectious. Mean age at surgery was 5.2 years. Univariate analysis showed a correlation between wound complication rates and the following: male gender (p = .097), diapered patient with lower back incision (p = .004), bilateral procedure (p = .008), more than three incisions (p = .011), left iliac incision (p = .097), right iliac incision (p = .009), patient age ≤4 years (p = .10), and operative time >150 minutes (p = .079). Multivariate analysis identified the following independent predictors: age ≤ 4 years (p = .002), male gender (p = .04), number of skin incisions (p = .001), left iliac incision (p = .018), and nutritionally challenged (p = .044). The multivariate model predicted wound complications with an area under the receiver operating characteristic curve of 0.88. Conclusions Knowledge of risk factors for RBD instrumentation wound complications can be used to construct patient risk models. This can identify patients at higher risk for complications and influence clinical decision making. Level of Evidence Level II.

Beckwith-Wiedemann syndrome (BWS) is the most common epigenetic overgrowth and cancer predisposition disorder. Due to both varying molecular defects involving chromosome 11p15 and tissue mosaicism, patients can present with a variety of clinical features, leading to the newly defined Beckwith-Wiedemann spectrum (BWSp). The BWSp can be further divided into three subsets of patients: those presenting with classic features, those presenting with isolated lateralized overgrowth (ILO) and those not fitting into the previous two categories, termed atypical BWSp. Previous reports of patients with BWS have focused on those with the more recognizable, classic features, and limited information is available on those who fit into the atypical and ILO categories. Here, we present the first cohort of patients recruited across the entire BWSp, describe clinical features and molecular diagnostic characteristics, and provide insight into practical diagnosis and management recommendations that we have gained from this cohort.

Orbital hypertelorism represents lateralization of the orbits, meaning increased interorbital and outer orbital distances. Interorbital hypertelorism represents a failure of medial orbital wall medialization in the setting of normally positioned lateral orbital walls. The etiology and type of hypertelorism influence selection of an operative procedure, whereas the severity of deformity dictates surgical need. Choice of surgical procedure is dictated by anatomic considerations, such as degree of orbital hypertelorism, midfacial proportions, and occlusal status.

Conjoined twins who are classified as craniopagus (joined at the cranium) have a rare congenital anomaly. Despite advances in surgical techniques and critical care, the rate of complications and death is still high among twins with total fusion in which the superior sagittal sinus is shared. Here, we describe total-fusion craniopagus twins who at 10 months of age underwent successful surgical separation performed by a multidisciplinary team. Computer-aided design and modeling with a three-dimensional printer, custom-designed cranial distraction and constriction devices, and intraoperative navigation techniques were used. These techniques allowed for separation of the twins at an early age and harnessed the regenerative capacity of their young brains.

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.

Posterior cranial vault distraction osteogenesis is a powerful, reliable, low-morbidity method to achieve intracranial expansion. It is particularly useful in treating turribrachycephaly seen in syndromic craniosynostosis, allowing for gradual expansion of the bone while stretching the soft tissues over several weeks allowing greater volumetric expansion than conventional techniques. Posterior cranial vault distraction osteogenesis constitutes a more gradual remodeling modality, with infrequent complications. As a first step in intracranial expansion, it preserves the frontal cranium for future frontofacial procedures. A drawback is the need for a second surgery to remove the device, and this must be taken into account during counseling.

BACKGROUND Patients undergoing orthognathic skeletal correction present with a variety of comorbidities that may affect surgical outcomes. The purpose of this study was to determine how patient risk factors and operative technique contribute to complication rates after orthognathic surgery in the era of patient-specific implants. METHODS Retrospective cohort analysis was conducted of pediatric patients undergoing Le Fort I osteotomy, bilateral sagittal split osteotomy, and/or genioplasty from 2014 to 2018. Patient risk factors, operative characteristics, and postoperative outcomes were gathered and compared with appropriate statistics. RESULTS Ninety-four patients met inclusion criteria, with an overall 1-year complication rate of 11.7 percent (11 of 94). Patient-specific mandibular plates are significantly associated with infection (p = 0.009; OR, 8.8), occurrence of any complication (p = 0.003; OR, 8.3), readmission (p < 0.001; OR, 11.1), and reoperation (p < 0.001; OR, 11.4). In patients with syndromes or history of cleft lip/palate, patient-specific mandibular plates are associated with infection (p = 0.006; OR, 10.3), readmission (p < 0.001; OR, 21.6), and reoperation (p < 0.001; OR, 22.9). In multivariate regression controlling for age, sex, syndrome status, and orofacial cleft history, use of patient-specific mandibular plates was associated with infection (p = 0.017; adjusted OR, 12.5), any complication (p = 0.007; adjusted OR, 11.8), readmission (p = 0.001; adjusted OR, 17.9), and reoperation (p = 0.001; adjusted OR, 18.9). CONCLUSIONS In the era of patient-specific orthognathic surgery, syndromic status and use of patient-specific mandibular plates are associated with increased infection, readmission, and reoperation because of hardware-related complications. The authors' data support increased caution and counseling with use of patient-specific mandibular implants in patients with syndromic status, history of orofacial cleft, and history of previous maxillomandibular surgery given increased risk of hardware-related complications. CLINICAL QUESTION/LEVEL OF EVIDENCE Therapeutic, III.

Spring-mediated cranial vault expansion (SMC) has become a primary treatment modality at our institution to correct scaphocephalic head shape in the setting of isolated sagittal craniosynostosis (CS). Spring-mediated cranioplasty is associated with minimal procedural morbidity and reliable clinical efficacy, although long-term outcomes are not well elucidated. Herein we describe our institutional experience and lessons learned with SMC. We hypothesize that SMC performed in young infants offers durable scaphocephalic correction as measured by cephalic index (CI) at the 1, 3, and 5-year postoperative timepoints.Patients with isolated sagittal CS who underwent SMC at our institution during an 8-year period were retrospectively studied. The primary outcome measure was long-term head shape determined by CI at the 1, 3, and 5-year postoperative timepoints. Secondary outcomes included patient and spring factors associated with change in CI, including age and spring force. All statistical tests were 2-tailed with P < 0.05 denoting significance.In total, 88 patients underwent SMC at a median age of 3.3 months with a median preoperative CI 69 (interquartile range: [66, 71]). The postoperative CI increased to 73 [71, 76] at postoperative day 1. At 1 month, the CI increased by 8.6 to 77 (P < 0.0001) and appeared to reach a plateau at 3 months (76, [74, 78]) without further improvement (P < 0.10). At 5 years, CI remained stable without relapse (76, [75, 81], demonstrating an 8.9 increase from preoperative CI. Age at time of spring placement and change in CI were inversely related (P < 0.005). Total spring force directly correlated with increased change in postoperative CI at the 6-month postoperative timepoint (P < 0.02).In summary, SMC offers durable correction of scaphocephaly as measured by CI for patients with isolated sagittal CS at the 5-year postoperative timepoint. The cranial expansion observed 1-month post-spring implantation may serve as a proxy for long-term CI.

Midface advancement by distraction osteogenesis (DO) is commonly performed in patients with craniosynostosis for indications including midface hypoplasia, exorbitism, obstructive sleep apnea, class III malocclusion, and overall aesthetic facial deficiency. There is evidence to suggest that maxillary LeFort I advancement increases the risk of velopharyngeal dysfunction in the cleft palate population, yet few studies have investigated changes in speech following LeFort III or monobloc midface advancement in patients with syndromic craniosynostosis. The purpose of this study was to examine the effect of midface DO on speech as indicated by the Pittsburgh Weighted Speech Score in patients with Apert, Crouzon, and Pfeiffer Syndrome. Among 73 midface advancement cases performed during the study period, 19 cases met inclusion criteria. Overall, the highest post-advancement Pittsburgh Weighted Speech Score (PWSS) was significantly higher than the pre-advancement PWSS (0.52 versus 2.42, P = 0.01), indicating an acute worsening of VPI post-advancement. Specifically, the PWSS components nasal emission and nasality were significantly higher post-advancement than pre-advancement (nasal emission: 1.16 versus 0.21, P = 0.02) (nasality: 0.68 versus 0.05, P = 0.04). However, there was no significant difference between pre-advancement PWSS and the latest post-advancement PWSS (P = 0.31). Midface distraction is associated with an acute worsening of VPI post-operatively that is followed by improvement, and often resolution over time. Future work with additional patient accrual is needed to determine the effect of different advancement procedures and syndromes on VPI rates and profundity.

BACKGROUND Uncertain clinical evidence for treating positional plagiocephaly, especially with helmet therapy, creates difficulties in counseling parents of patients. This study investigates layperson perceptions and treatment preferences for positional plagiocephaly to provide patient-oriented evidence for management. METHODS Adult laypersons were recruited through crowdsourcing to view digitally-modified images of normal, mildly, moderately, or severely plagiocephalic infant heads. Participants provided demographic information and rated the infant's head shape and potential related social difficulties, likelihood of consulting a physician for treatment options, and likelihood of seeking helmeting treatment for the infant. RESULTS Nine hundred forty-five individuals participated in the study. Perception of head shape, prediction of future embarrassment and social difficulties, likelihood of seeking physician evaluation, likelihood of choosing helmet therapy, and willingness-to-pay for helmet therapy were pairwise-different between 4 plagiocephaly severities (corrected-P

BACKGROUND The purpose of this study was to determine what craniometric changes occur to both orbits of unicoronal craniosynostosis patients undergoing fronto-orbital advancement and remodeling, and which of these changes are associated with new onset of postoperative strabismus. METHODS A retrospective analysis was performed of the preoperative and postoperative orbits of 24 unicoronal craniosynostosis patients and the orbits of 24 control subjects, totaling 144 orbits. Eight parameters were evaluated using multivariate logistic regression analysis. One of the parameters was modified orbital index, an indicator of severity of harlequin deformity. RESULTS Significant differences in orbital dimensions and angles were present bilaterally in unicoronal craniosynostosis orbits when compared to controls. Fronto-orbital advancement and remodeling increased the ipsilateral unicoronal craniosynostosis orbital volume from 13,184 ± 2003 mm to 16,220 ± 2323 mm (p < 0.001). Ipsilateral horizontal cone angles were increased from 48 ± 5 degrees to 54 ± 7 degrees (p = 0.004). Ipsilateral vertical cone angles were decreased from 73 ± 8 degrees to 66 ± 10 degrees (p = 0.003). Ipsilateral modified orbital index improved from 0.83 ± 0.06 to 0.88 ± 0.06 (p = 0.003). Three of the 19 unicoronal craniosynostosis patients developed transient postoperative strabismus. Logistic regression analysis displayed a strong significant association between new-onset strabismus and a change in modified orbital index with a coefficient of 30.84 ± 14.51 (p < 0.05). CONCLUSIONS The orbital dysmorphology in unicoronal craniosynostosis is bilateral in nature, and it is not wholly treated with conventional fronto-orbital advancement and remodeling. The severity of ipsilateral orbital dysmorphology is correlated with the incidence of postoperative strabismus following conventional fronto-orbital advancement and remodeling. Future research is needed to develop strategies to mitigate the risk of development of strabismus in this group of patients. CLINICAL QUESTION/LEVEL OF EVIDENCE Risk, III.

Background The purpose of this Web-based survey was to elucidate the current perspectives of plastic surgery residency program directors on training residents to perform gender-affirming surgery. Methods Web-based surveys were distributed to 79 plastic surgery program directors. Demographic information and perspectives on training of gender-affirming surgery in plastic surgery residency were queried. Results Of 79 distributed surveys, there were 43 responses (54 percent). Overall, program directors reported that their trainees were prepared to address plastic surgery-related transgender concerns (67 percent), and believe plastic surgeons are the most appropriate specialty referral for each type of gender-affirming surgery (top/chest, 98 percent; facial, 95 percent; and bottom/genital, 79 percent). Ninety-three percent of program directors noted that transgender surgery is becoming more accepted and/or practiced in their referral area, with 26 percent reporting a dedicated clinic experience. There was a mixed response on the need for additional fellowship training for gender-affirming surgery. Residents are exposed to significantly more bottom (p = 0.0018), top (p = 0.0013), and facial operations (p = 0.00005) if they rotate through a "gender" clinic. Conclusions Of the queried program directors, the majority feel their residents are well-trained in gender-affirming surgery. However, residents have more clinical exposure in facial and top (chest) gender-affirming surgery as compared to bottom (genital) surgery. Although most program directors agree that plastic surgeons are the most important referral for top, bottom, and facial operations, there is less consensus over the role of fellowship training. Most program directors reported a desire to devote additional CME time to the topic in the coming years.

OBJECTIVEEvaluation of increased intracranial pressure (ICP) in the pediatric craniosynostosis population based solely on ophthalmological, clinical, and radiographic data is subjective, insensitive, and inconsistent. The aim of this study was to examine the intraoperative ICP before and after craniectomy in this patient population.METHODSThe authors measured the ICP before and after craniectomy using a subdural ICP monitor in 45 children. They regulated end-tidal carbon dioxide and the monitoring site under general anesthesia to record consistent ICP readings.RESULTSThe average age of the patient population was 29 months (range 3.8–180.5 months). Thirty-seven patients (82.2%) were undergoing initial craniosynostosis procedures. All craniosynostosis procedures were categorized as one of the following: frontoorbital advancement (n = 24), frontoorbital advancement with distraction osteogenesis (n = 1), posterior vault distraction osteogenesis (n = 10), and posterior vault reconstruction (n = 10). Nineteen of 45 patients (42.2%) had syndromic or multisuture craniosynostosis. The mean postcraniectomy ICP (8.8 mm Hg, range 2–18 mm Hg) was significantly lower than the precraniectomy ICP (16.5 mm Hg, range 6–35 mm Hg) (p < 0.001). Twenty-four patients (53%) had elevated ICP prior to craniectomy, defined as ≥ 15 mm Hg. Only 4 (8.9%) children had papilledema on preoperative funduscopic examination (sensitivity 17%, specificity 100%, negative predictive value 51%, and positive predictive value 100%). There were no significant differences in elevated precraniectomy ICP based on type of craniosynostosis (syndromic/multisuture or nonsyndromic) or age at the time of surgery. Patients undergoing initial surgery in the first 12 months of life were significantly less likely to have elevated precraniectomy ICP compared with patients older than 12 months (26.3% vs 73.1%, p = 0.005).CONCLUSIONSIn this study, the authors report the largest cohort of syndromic and nonsyndromic craniosynostosis patients (n = 45) who underwent precraniectomy and postcraniectomy ICP evaluation. A craniectomy or completed craniotomy cuts for distractors effectively reduced ICP in 43/45 patients. The authors’ findings support the notion that papilledema on funduscopy is a highly specific, however poorly sensitive, indicator of increased ICP, and thus is not a reliable screening method. These findings indicate that even nonsyndromic patients with craniosynostosis are at risk for increased ICP. Furthermore, patients who present prior to 12 months of age appear less likely to have elevated ICP on presentation. Further studies with other noninvasive imaging of the retina may be useful as an adjunct tool for determining elevated ICP.

Background Controversy exists regarding the treatment of mandibular hypoplasia in craniofacial microsomia patients, notably the role of mandibular distraction osteogenesis. The authors compared the need for orthognathic surgery in skeletally mature craniofacial microsomia subjects who either did (study group) or did not (control group) undergo early mandibular distraction osteogenesis. Methods A retrospective review was conducted of all craniofacial microsomia patients evaluated between January of 1993 and March of 2017. This study included patients with a Kaban-Pruzansky grade I to III mandible, and who were at least 14 years old at the time of the latest follow-up. Results Thirty-eight subjects met inclusion criteria: 17 who underwent mandibular distraction osteogenesis and 21 who did not (mean age, 18.95 ± 2.82 years versus 17.95 ± 2.14 years, respectively; p = 0.246). The degree of mandibular deformity was matched (distraction, 29.4 percent Kaban-Pruzansky grade IIb and 5.9 percent grade III; no distraction, 23.8 percent grade IIb and 9.5 percent grade III; p = 0.788). No significant difference was noted between the distraction and no-distraction cohorts with regard to need for orthognathic surgery [distraction, n = 10 (58.8 percent); no distraction, n = 8 (38.1 percent); p = 0.203]. Conclusions The results seem to suggest that there is no significant difference in orthognathic surgery rates at skeletal maturity between craniofacial microsomia subjects who underwent early mandibular distraction osteogenesis and those who did not. Subjects who undergo distraction may still ultimately require orthognathic surgery to correct facial asymmetry. Additional studies are required to determine the optimal timing and technique of distraction, the importance of orthodontic management during and after distraction, and the early psychosocial benefits of improved facial symmetry. Clinical question/level of evidence Therapeutic, III.

BACKGROUND Sphenoid wing dysplasia in patients with neurofibromatosis type 1 may result in challenging and significant changes, including ultimately vision loss. The authors describe the radiographic patterns of sphenoid dysmorphology with time and age, and the impact of surgical intervention on preservation of vision. METHODS A retrospective study was performed at a single pediatric hospital, identifying subjects with neurofibromatosis type 1. Records were reviewed in their entirety for each subject, with attention to ophthalmologic evaluation. Radiographic images were digitally analyzed and scored for sphenoid transformations on a grade of 0 to 3. RESULTS Fifty-two subjects were identified. On initial imaging, 42.31 percent had a normal sphenoid (grade 0), 32.69 percent had an abnormal contour (grade 1), 11.54 percent had thinning (grade 2), and 13.46 percent had a gross defect (grade 3). Among the 45 subjects with serial imaging, 55.56 percent demonstrated progression of the deformity of at least one grade. Deformity progression correlated with length of imaging interval. Enucleation was noted to occur more often in subjects with a gross sphenoid defect (p < 0.0001). Of subjects identified as having a gross sphenoid defect, 26.7 percent were managed using orbitosphenoid reconstruction with titanium mesh and cranial bone graft, and 33.3 percent were managed with soft-tissue debulking and canthopexy only. Vision was more likely to be preserved with early orbitosphenoid reconstruction (p < 0.05). CONCLUSIONS Sphenoid dysplasia is a progressive disease. Loss of vision is associated with a gross defect, and appears to be better preserved with early orbitosphenoid reconstruction with titanium mesh and cranial bone graft.

BACKGROUND How hospital case-volume affects operative outcomes and cost continues to grow in importance. The purpose of this study was to examine the relationship of case volume with operative outcomes and cost in cleft palate repair. METHODS Subjects undergoing cleft palate repair between 2004 and 2015 were identified in the Pediatric Health Information System. Outcomes were compared between two groups: those undergoing treatment at a high-volume institution, and those undergoing treatment at a low-volume institution. Primary outcomes were as follows: any complication, prolonged length of stay, and increased total cost. RESULTS Over 20,000 patients (n = 20,320) from 49 institutions met inclusion criteria. On univariate analysis, those subjects who underwent treatment at a high-volume institution had a lower rate of overall complications (3.4 percent versus 5.1 percent; p < 0.001), and lower rates of prolonged length of stay (4.5 percent versus 5.8 percent; p < 0.001) and increased total cost (48.6 percent versus 50.9 percent; p = 0.002). In multivariate regression analyses, subjects treated in high-volume centers were less likely to experience any complication (OR, 0.678; p < 0.001) and were less likely to have an extended length of stay (OR, 0.82; p = 0.005). Subjects undergoing palate repair at a high-volume institution were no less likely to incur increased total cost (OR, 1.01; p = 0.805). CONCLUSION In institutions performing a high volume of cleft palate repairs, subjects had significantly decreased odds of experiencing a complication or prolonged length of stay. CLINICAL QUESTION/LEVEL OF EVIDENCE Therapeutic, III.

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 The relationships between hospital/surgeon characteristics and operative outcomes and cost are being scrutinized increasingly. In patients with craniosynostosis specifically, the relationship between hospital volume and outcomes has yet to be characterized. METHODS Subjects undergoing craniosynostosis surgery between 2004 and 2015 were identified in the Pediatric Health Information System. Outcomes were compared between two exposure groups, those undergoing treatment at a high-volume institution (>40 cases per year), and those undergoing treatment at a low-volume institution (40 cases per year). Primary outcomes were any complication, prolonged length of stay, and increased total cost. RESULTS Over 13,000 patients (n = 13,112) from 49 institutions met inclusion criteria. In multivariate regression analyses, subjects treated in high-volume centers were less likely to experience any complication (OR, 0.764; p < 0.001), were less likely to have an extended length of stay (OR, 0.624; p < 0.001), and were less likely to have increased total cost (OR, 0.596; p < 0.001). Subjects undergoing strip craniectomy in high-volume centers were also less likely to have any complication (OR, 0.708; p = 0.018) or increased total cost (OR, 0.51; p < 0.001). Subjects undergoing midvault reconstruction in high-volume centers were less likely to experience any complications (OR, 0.696; p = 0.002), have an extended length of stay (OR, 0.542; p < 0.001), or have increased total cost (OR, 0.495; p < 0.001). CONCLUSION In hospitals performing a high volume of craniosynostosis surgery, subjects had significantly decreased odds of experiencing a complication, prolonged length of stay, or increased total cost compared with those undergoing treatment in low-volume institutions. CLINICAL QUESTION/LEVEL OF EVIDENCE Therapeutic, III.

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.

BACKGROUND Frontal sinus pneumatization plays an important role in brow protrusion, and absence of frontal sinus development may be associated with brow retrusion. Using unicoronal craniosynostosis as a model, the authors studied the relationship among frontal sinus volume, supraorbital retrusion, and brow position. METHODS The authors conducted a retrospective review of unicoronal craniosynostosis patients with head computed tomographic scans and photographs taken from ages 5 to 18 years. Frontal sinus volume and supraorbital retrusion were calculated using three-dimensional computed tomographic reconstructions. A "brow score" from 0 to 3 was assigned to each patient's photographs by a consensus of two craniofacial surgeons; sinus morphology and brow scores were compared. RESULTS The study included 20 unicoronal craniosynostosis patients. The affected side demonstrated increased retrusion (7.1 ± 3.7 mm versus 3.0 ± 3.1 mm; p < 0.001) and decreased frontal sinus volume (0.8 ± 0.9 cc versus 2.6 ± 1.8 cc; p < 0.001). Qualitative brow retrusion scores differed between affected and unaffected sides (score of 0, 17 percent affected versus 78 percent unaffected; score of 1, 28 percent versus 22 percent; score of 2, 39 percent versus 0 percent; and score of 3, 17 percent versus 0 percent; p < 0.001). Brow scores trended with retrusion measurements (score of 0, 2.7 ± 2.9 mm retrusion; score of 1, 6.1 ± 3.5 mm; score of 2, 8.9 ± 3.2 mm; and score of 3, 9.7 ± 2.8 mm; p < 0.001). Subjects with brow scores of 0 or 1 had larger frontal sinus volumes than subjects with brow scores of 2 or 3 (p = 0.018). CONCLUSIONS Qualitative brow retrusion correlates strongly with both quantitative supraorbital retrusion and frontal sinus volume. Although this retrospective study cannot prove causality, which is likely multifactorial, its strong correlation with frontal sinus pneumatization highlights the importance of the frontal sinus in brow position in unicoronal craniosynostosis. CLINICAL QUESTION/LEVEL OF EVIDENCE Therapeutic, III.

In North America, the number of craniofacial surgery fellowship graduates is increasing, yet an analysis of practice settings upon graduation is lacking. We characterize the practice types of recent graduates of craniofacial fellowship programs in the United States and Canada.A 6-year cohort of craniofacial fellows in the United States and Canada (2010-2016) were obtained from craniofacial programs recognized by the American Society of Craniofacial Surgery. Practice setting was determined at 1 and 3 years of postgraduation, and predictors of practice setting were determined.A total of 175 craniofacial surgeons were trained at 35 fellowship programs. At 1 year of postgraduation, 33.6% had an academic craniofacial position and 27.1% were in private practice (p = 0.361). A minority of graduates pursued additional fellowships (16.4%), nonacademic craniofacial positions (10.0%), academic noncraniofacial positions (5.7%), and international practices (7.1%). At 3 years of postgraduation, the percentage of graduates in academic craniofacial positions was unchanged (34.5% vs 33.6%, p = 0.790). The strongest predictors of future academic craniofacial practice were completing plastic surgery residency at a program with a craniofacial fellowship program (odds ratio = 6.78, p0.001) and completing an academic craniofacial fellowship program (odds ratio = 4.48, p = 0.020).A minority of craniofacial fellowship graduates practice academic craniofacial surgery. A strong academic craniofacial surgery background during residency and fellowship is associated with a future career in academic craniofacial surgery. These data may assist trainees choose training programs that align with career goals and educators select future academic surgeons.

BACKGROUND This study investigates the associations between local anesthesia practice and perioperative complication, length of stay, and hospital cost for palatoplasty in the United States. METHODS Patients undergoing cleft palate repair between 2004 and 2015 were abstracted from the Pediatric Health Information System database. Perioperative complication, length of stay, and hospital cost were compared by local anesthesia status. Multiple logistic regressions controlled for patient demographics, comorbidities, and hospital characteristics. RESULTS Of 17,888 patients from 49 institutions who met selection criteria, 8631 (48 percent), 4447 (25 percent), and 2149 (12 percent) received epinephrine-containing lidocaine, epinephrine-containing bupivacaine, and bupivacaine or ropivacaine alone, respectively. The use of epinephrine-containing bupivacaine or bupivacaine or ropivacaine alone was associated with decreased perioperative complication [adjusted OR, 0.75 (95 percent CI, 0.61 to 0.91) and 0.63 (95 percent CI, 0.47 to 0.83); p = 0.004 and p = 0.001, respectively]. Only bupivacaine- or ropivacaine-alone recipients experienced a significantly reduced risk of prolonged length of stay on adjusted analysis [adjusted OR, 0.71 (95 percent CI, 0.55 to 0.90); p = 0.005]. Risk of increased cost was reduced in users of any local anesthetic (p < 0.001 for all). CONCLUSIONS Epinephrine-containing bupivacaine or bupivacaine or ropivacaine alone was associated with reduced perioperative complication following palatoplasty, while only the latter predicted a decreased postoperative length of stay. Uses of epinephrine-containing lidocaine, epinephrine-containing bupivacaine, and bupivacaine or ropivacaine alone were all associated with decreased hospital costs. Future prospective studies are warranted to further delineate the role of local anesthesia in palatal surgery. CLINICAL QUESTION/LEVEL OF EVIDENCE Therapeutic, III.

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.

Erosive pustular dermatosis of the scalp (EPDS) is an uncommon chronic inflammatory response to scalp trauma that usually resolves with cicatricial alopecia. It most commonly affects elderly patients with a history of actinic damage. Herein, we describe a 16-year-old girl with acrofacial dysostosis type 1 presenting after surgery with crusting purulent scalp lesions, whose clinical presentation and histopathologic findings were consistent with EPDS. A review of the literature on EPDS in children is also detailed.

BACKGROUND The frequency of respiratory events in the perioperative period, and optimal duration of intubation during early mandibular distraction osteogenesis activation, are poorly understood. This study assesses potential risk factors associated with perioperative respiratory events, particularly the need for reintubation, following mandibular distraction osteogenesis surgery. METHODS A retrospective review was conducted for infants (younger than 1 year) undergoing mandibular distraction osteogenesis for tongue-based airway obstruction between November of 2010 and December of 2017. Univariate and multivariate analyses of sentinel events and outcomes were performed. RESULTS Ninety infants (median age, 35 days) were included (50 percent were syndromic). Twenty-seven subjects (30 percent) experienced a respiratory event requiring intervention before discharge, including 14 subjects who failed initial extubation. Subjects extubated earlier than postoperative day 5 failed extubation more frequently (33%) compared to those extubated later (9%; p = 0.005). Respiratory events occurred more frequently when extubation was attempted at distraction lengths of 5 mm or less (42 percent) compared to greater than 5 mm (21 percent; p = 0.032). Logistic regression modeling showed that syndromic status (OR, 14.8) and secondary airway anomaly (OR, 6.1) were significant predictors for respiratory events, whereas greater length of distraction at the time of extubation was protective (OR, 0.8; p < 0.05). CONCLUSIONS Postoperative intubation of at least 5 days with associated mean distraction of 5 mm appears to be associated with successful extubation trial following mandibular distraction osteogenesis surgery. Patients with congenital syndromes and secondary airway anomalies are more likely to experience perioperative respiratory events. CLINICAL QUESTION/LEVEL OF EVIDENCE Risk, III.

Muenke syndrome (MIM #602849), the most common syndromic craniosynostosis, results from the recurrent pathogenic p.P250R variant in FGFR3. Affected patients exhibit wide phenotypic variability. Common features include coronal craniosynostosis, hearing loss, carpal and tarsal anomalies, and developmental/behavioral issues. Our study examined the phenotypic findings, medical management, and surgical outcomes in a cohort of 26 probands with Muenke syndrome identified at the Children’s Hospital of Philadelphia. All probands had craniosynostosis; 69.7% had bicoronal synostosis only, or bicoronal and additional suture synostosis. Three male patients had autism spectrum disorder. Recurrent ear infections were the most common comorbidity, and myringotomy tube placement the most common extracranial surgical procedure. Most patients (76%) required only one fronto-orbital advancement. de novo mutations were confirmed in 33% of the families in which proband and both parents were genetically tested, while in the remaining 66% one of the parents was a mutation carrier. In affected parents, 40% had craniosynostosis, including 71% of mothers and 13% of fathers. We additionally analyzed the medical resource utilization of probands with Muenke syndrome. To our knowledge, these data represent the first comprehensive examination of long-term management in a large cohort of patients with Muenke syndrome. Our study adds valuable information regarding neuropsychiatric and medical comorbidities, and highlights findings in affected relatives.

BACKGROUND AND PURPOSE Treatment of metopic craniosynostosis often involves bitemporal expansion of the anterior cranium. This report compares perioperative variables of a surgical technique in which the fronto-orbital bandeau is bent in situ to traditional fronto-orbital advancement and remodeling (FOAR). METHOD/DESCRIPTION Six patients undergoing the hinge technique were compared to the senior author's 6 most recent conventional FOARs. Study and control cohorts were reasonably well matched for age and sex; only those with nonsyndromic, single-suture metopic synostosis were included. Perioperative variables such as OR time, blood loss, hardware costs, length of stay, and perioperative complications were compared between groups using unpaired t tests. RESULTS Operative time was significantly decreased when the hinge technique was utilized, with a mean operative time of 159 ± 14.0 minutes for hinge patients and 193 ± 33.8 minutes for nonhinge patients (P = 0.049). Hardware was also significantly decreased from an average of 2.2 ± 1.0 plates and 35 ± 13.8 screws in nonhinge patients to 1 plate and 20 screws in each hinge patient (P

STUDY OBJECTIVES: Beckwith-Wiedemann syndrome (BWS) is a rare pediatric overgrowth disorder that includes a spectrum of clinical findings including macroglossia, especially in those with loss of methylation at the imprinting control region (IC2 LOM) on chromosome 11. Children with BWS can have very severe obstructive sleep apnea (OSA), but the prevalence of OSA in this population is poorly understood, as is the relationship between OSA and the BWS genotype/phenotype. We hypothesized that there would be a high prevalence of OSA in children with BWS, and that OSA would be more severe in children with IC2 LOM. METHODS: Medical records from children evaluated from March 2015 through January 2018 were reviewed for results from polysomnography, genetic testing, and clinical assessment. RESULTS: A total of 26 children with BWS not previously treated for OSA underwent polysomnography, genetic testing, and clinical assessment. Median (range) age was 5 months (3 days to 33 months). Most children, 20 (76.9%), had an obstructive apnea-hypopnea index (OAHI) > 2 events/h. Median (range) OAHI was 4.4 events/h (0 to 56.1 events/h). OAHI was significantly greater in participants younger than 6 months compared with those older than 6 months (P = .008). Those with IC2 LOM did not have a greater OAHI (P = .61) than those with other genetic causes of BWS, but OAHI had a strong positive correlation with BWS clinical score (Spearman rho = .54, P = .004). CONCLUSIONS: There is a high prevalence of OSA in children with BWS with macroglossia. Younger children with BWS and those with more phenotypic features may be at greatest risk of OSA. CITATION: Cielo CM, Duffy KA, Taylor JA, Marcus CL, Kalish JM. Obstructive sleep apnea in children with Beckwith-Wiedemann syndrome. J Clin Sleep Med. 2019;15(3):375–381.

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.

Background Posterior pharyngeal flap (PPF) surgery is effective for treating velopharyngeal insufficiency but has historically been associated with risk of airway compromise. This study aims to identify risk factors for complications from and readmission after PPF using a national database. Methods Patients who underwent PPF surgery were selected from the 2012 American College of Surgeons National Surgical Quality Improvement Program Pediatric (NSQIP-PEDS) database. Patient characteristics, comorbidities, and complication/readmission data were analyzed. Results Among 225 study patients, 12 (5.3%) suffered perioperative complications. The most common complications were pulmonary in nature (5 patients, 2.2%), including prolonged postoperative mechanical ventilation (3 patients, 1.3%). Underlying asthma ( P = .024) or any cardiac risk factor ( P = .047) conveyed significant complication risk. Further, severe cardiac risk factors were associated with postoperative bleeding ( P = .024). Readmission (4 patients, 1.7%) and reoperation (3 patients, 1.3%) occurred at mean intervals of 9 and 10 days after the original procedure. Seventy-nine patients (35%) were discharged postoperatively on an outpatient basis, and this subgroup included only one patient (1.2%) with a complication ( P = .038). Conclusions The overall perioperative complication rate for PPF surgery is low at 5.3%. Patients with underlying cardiac risk factors, severe American Society of Anesthesiologists Physical Status class, and asthma should prompt greater attention given their heightened risk profiles.

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

Children with craniofacial anomalies are a heterogeneous group at high risk for obstructive sleep apnea (OSA). However, the prevalence and structural predictors of OSA in this population are unknown. We hypothesized that infants with micrognathia would have more significant OSA than those with isolated cleft palate ± cleft lip (ICP), and those with ICP would have more significant OSA than controls. We postulated that OSA severity would correlate with reduced mandibular size, neurodevelopmental scores, and growth.Prospective cohort study. 15 infants with ICP, 19 with micrognathia, and 9 controls were recruited for polysomnograms, neurodevelopmental testing, cephalometrics (ICP and micrognathia groups) at baseline and a follow-up at 6 mo.Baseline apnea-hypopnea index (AHI) [median (range)] of the micrognathia group [20.1 events/h (0.8, 54.7)] was greater than ICP [3.2 (0.3, 30.7)] or controls [3.1 (0.5, 23.3)] (p = 0.001). Polysomnographic findings were similar between ICP and controls. Controls had a greater AHI than previously reported in the literature. Cephalometric measures of both midface hypoplasia and micrognathia correlated with OSA severity. Neurodevelopment was similar among groups. OSA improved with growth in participants with ICP and postoperatively in infants with micrognathia.Micrognathia, but not ICP, was associated with more significant OSA compared to controls. Both midface and mandibular hypoplasia contribute to OSA in these populations. OSA improved after surgical correction in most infants with micrognathia, and improved without intervention before palate repair in infants with ICP.

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.