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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.

The goal of this study was to describe the 10-year evolution of the authors' surgical technique and institutional perioperative outcomes using posterior vault distraction osteogenesis (PVDO) in patients with syndromic and multisuture craniosynostosis.The authors performed a retrospective cohort study of patients who underwent PVDO for treatment of syndromic and multisuture craniosynostosis at a single institution over a 10-year period. Demographic data, perioperative outcomes, distraction patterns, and complications were analyzed. Outcomes of patients in the first 5 years (early cohort) were compared with those of the latter 5 years (late cohort).One hundred ten patients underwent a total of 118 PVDO procedures. Patients with a syndromic diagnosis represented 83.6 percent of the cohort ( n = 92) and were significantly younger than patients with a nonsyndromic diagnosis at the time of first PVDO (median [25th percentile, 75th percentile] 14.1 [6.6, 40.1] versus 42.7 [15.2, 59.6] months; p = 0.014). Mean distraction distance in the anterior-posterior direction was 30.8 mm (SD, 7.4). Compared with the early cohort, PVDO in the late cohort had faster median operative times (144.0 [123.0, 189.0] minutes versus 161.0 [138.0, 199.0] minutes; p = 0.038), lower estimated blood loss as a percentage of blood volume (28.5 [20.6, 45.3] versus 50.0 [31.1, 95.8]; p0.001), and lower blood replacement as percentage of blood volume (39.5 [23.8, 59.1] versus 56.3 [37.8, 110.1]; p = 0.009).This 10-year experience with PVDO demonstrates continued overall safety and efficacy with improved perioperative outcomes over time. Although PVDO has become the authors' first line of expansion in syndromic craniosynostosis, shortcomings such as need for device removal, infection concerns, and potential for cerebrospinal fluid leak merit attention by the craniofacial community.Therapeutic, III.

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.

Although conventional posterior vault distraction osteogenesis is known to achieve the greatest improvement in intracranial volume, anteroposterior expansion of the cranial vault is not always ideal in certain head shapes. Transverse posterior vault distraction osteogenesis is an alternative reconstructive strategy in patients presenting with recalcitrant multisuture craniosynostosis resulting in scaphocephaly with posterior narrowing. The authors present a multimedia demonstration of placing the cranial hinge points at the superior anterior aspect of the bone segments in order to achieve increased intracranial volume and significant improvement in posterior head shape, while preserving normal anterior skull dimensions.

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.

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

Background Linton A. Whitaker is a pioneer of craniofacial surgery. He served as chief of plastic surgery at the Children's Hospital of Philadelphia and University of Pennsylvania and director of the craniofacial training program. Herein, the authors reflect on his legacy by studying the accomplishments of his trainees. Methods Dr Whitaker's trainees who completed (a) craniofacial fellowship training while he was director of the program or (b) residency training while he was chief were identified. Curricula vitae were reviewed. Variables analyzed included geographic locations, practice types, academic leadership positions, scholarly work, and bibliometric data. Results Between 1980 and 2011, 34 surgeons completed craniofacial fellowship training under Dr Whitaker, and 11 completed plastic surgery training under his chairmanship and subsequent craniofacial fellowship. The majority had active craniofacial practices after training (83.3%) and practice in an academic setting (78.0%). Most settled in the northeast (31.1%) and south (31.1%) but across 24 states nationally. Overall, the mean ± SD number of publications was 76 ± 81 (range, 2-339); book chapters, 23 ± 29 (0-135); H-index, 18 ± 12 (1-45); and grants, 13 ± 16 (0-66). Of those who pursued academia, 53.1% were promoted to full professor, 46.9% had a program director role, 75.0% directed a craniofacial program, and 53.1% achieved the rank of chief/chair. Conclusions Equally important to Dr Whitaker's clinical contributions in plastic and craniofacial surgery is the development and success of his trainees who will undoubtedly continue the legacy of training the next generation of craniofacial surgeon leaders.

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.

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.

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

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.

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