Pediatric Plastic Surgery in the COVID-19 Era.

ABSTRACT: The COVID-19 pandemic has forced the healthcare system to adopt novel strategies to treat patients. Pediatric plastic surgeons are uniquely exposed to high rates of infections during examinations and surgeries via aerosol-generating procedures, in part because of the predilection of viral particles for the nasal cavities and pharynx. Telemedicine has emerged as a useful virtual medium for encouraging prolonged patient follow-up while maintaining physical distance. It has proven beneficial in mitigating infection risks while decreasing the financial burden on patients, their families, and healthcare teams. New trends driven by the pandemic added multiple elements to the patient-physician relationship and have left a lasting impact on the field of pediatric plastic surgery in clinical guidelines, surgical care, and patient outcomes. Lessons learned help inform pediatric plastic surgeons on how to reduce future viral infection risk and lead a more appropriately efficient surgical team depending on early triage.

LeFort III Versus Monobloc Frontofacial Advancement: A Comparative Analysis of Soft Tissue Changes.

INTRODUCTION: The LeFort III and monobloc are commonly used midface advancement procedures for patients with syndromic craniosynostosis with well characterized postoperative skeletal changes. However, the differential effects of these procedures on facial soft tissues are less understood. The purpose of this study was to critically analyze and compare the effects of these 2 procedures on the overlying soft tissues of the face. METHODS: Frontal and lateral preoperative and postoperative photographs of patients undergoing monobloc or LeFort III were retrospectively analyzed using ImageJ to measure soft tissue landmarks. Measurements included height of facial thirds, nasal length and width, intercanthal distance, and palpebral fissure height and width. Facial convexity was quantified by calculating the angle between sellion (radix), subnasale, and pogonion on lateral photographs. RESULTS: Twenty-five patients with an average age of 6.7 years (range 4.8-14.5) undergoing monobloc (n=12) and LeFort III (n=13) were identified retrospectively and analyzed preoperatively and 6.4±3.6 months postoperatively. Patients undergoing LeFort III had a greater average postoperative increase in facial convexity angle acuity (28.2°) than patients undergoing monobloc (17.8°, P =0.021). Patients in both groups experience postoperative increases in nasal width ( P <0.001) and decreases in palpebral fissure height ( P <0.001). CONCLUSIONS: Both subcranial LeFort III advancements and monobloc frontofacial advancements resulted in significant changes in the soft tissues. Patients undergoing LeFort III procedures achieved greater acuity of the facial convexity angle, likely because the nasion is not advanced with the LeFort III segment.

Clinical Research Fellowship Fosters Mentorship, Teamwork, and Productivity: Our 11-Year Experience With a Craniofacial Research Fellowship.

INTRODUCTION: This study describes the development and explores the academic impact of a cleft and craniofacial research fellowship. MATERIALS AND METHODS: Research and career outcomes from 3 cleft and craniofacial surgeons, 14 clinical fellows, and 25 research fellows between 2010 and 2023 were examined. Academic productivity was measured by the number of peer-reviewed publications indexed in PubMed and podium presentations at national/international meetings. Residency match statistics were recorded for eligible research fellows. RESULTS: Over this 14-year period (11 with research fellows), the team produced 500 publications in 96 peer-reviewed journals, with 153 (31%) in Plastic and Reconstructive Surgery, 117 (23%) in the Journal of Craniofacial Surgery, and 32 (6%) in The Cleft Palate-Craniofacial Journal. Yearly publications increased from 15.3±7.6 per year (before fellowship) to 23.0±5.3 (with 1 fellow) to 38.3±12.9 (2 fellows) to 81.0±5.7 (3 fellows; P<0.001). There was a strong annual linear growth in publications since the beginning the research fellowship position (r=0.88, P<0.001). All (100%) clinical research fellows developed strong relationships with senior surgeons, and all who applied to plastic surgery residency matched a significantly higher success rate than the national average (P<0.05). CONCLUSION: Implementing a structured cleft and craniofacial clinical research fellowship was associated with a broad impact across all cleft and craniofacial team members, as reflected by increased academic output and high match rates among fellows. The fellowship also strengthens the talent pipeline into plastic surgery by fostering meaningful mentor/mentee relationships and provides a model that can be adopted in both surgical and nonsurgical fields.

Crowdsourcing the Impact of Illustration in Cleft Surgery Education.

OBJECTIVE: This study aimed to (1) assess layperson preferences for how surgical information is presented; (2) evaluate how the format of visual information relates to layperson comfort with undergoing surgery, perceptions of surgeon character traits, and beliefs about artistic skill impacting plastic surgery practice; and (3) identify sociodemographic characteristics associated with these outcomes. DESIGN: A survey was developed in which one of five standardized sets of information depicting a unilateral cleft lip repair was presented as (1) text alone, (2) quick sketches, (3) simple drawings, (4) detailed illustrations, or (5) photographs. SETTING: Online crowdsourcing platform. PARTICIPANTS: Raters aged 18 years and older from the United States. INTERVENTIONS: None. MAIN OUTCOME MEASURE(S): After viewing the surgical information, participants answered three sets of Likert scale questions. Ratings were averaged to produce three composite scores assessing (1) comfort with undergoing surgery (2) perceptions of surgeon character traits, and (3) beliefs about plastic surgery and artistry. RESULTS: Four hundred seventy-nine participants were included. Surgeon character traits score was highest among participants who viewed detailed illustrations at 4.46 ± 0.59, followed by photographs at 4.43 ± 0.54, text alone at 4.28 ± 0.59, simple drawings at 4.17 ± 0.67, and quick sketches at 4.17 ± 0.71 (p = 0.0014). Participants who viewed detailed illustrations rated surgical comfort score and plastic surgery and artistry score highest, although differences did not achieve statistical significance. CONCLUSIONS: Viewing detailed cleft lip repair illustrations was significantly associated with positive perceptions of surgeon character traits. Our data help to contextualize methods of communication and education valued by the public when seeking cleft care.

Long-term aesthetic and photogrammetric outcomes in non-syndromic unicoronal synostosis: comparison of fronto-orbital distraction osteogenesis and fronto-orbital advancement and remodeling.

BACKGROUND: Fronto-orbital advancement and remodeling (FOAR) is among the most common surgical approaches for unicoronal craniosynostosis (UCS), although some data demonstrate failure to achieve long-term aesthetic normalcy, leading some to seek alternative treatment paradigms such as fronto-orbital distraction osteogenesis (FODO). This study compares long-term aesthetic outcomes of patients with UCS treated with FOAR and FODO. METHODS: Twenty patients (four males) with non-syndromic UCS presenting to our institution and undergoing distraction were compared to a matched cohort of 20 patients (six males) undergoing FOAR. Clinical photographs and ImageJ were used to quantify periorbital anatomy including palpebral fissures, pupil-to-brow distance (PTB), and margin-reflex distance (MRD1) in pixels. Whitaker classification was blindly assigned by craniofacial surgeons. RESULTS: Photogrammetric analysis and Mann-Whitney U tests demonstrated significantly improved postoperative symmetry in distraction patients for palpebral width (p = 0.020), MRD1 (p = 0.045), and canthal tilt (p = 0.010). Average Whitaker classification scores between FOAR (1.94) and distraction (1.79) cohorts were similar (p = 0.374). CONCLUSIONS: UCS patients demonstrated significant postoperative improvements in periorbital symmetry, with distraction patients demonstrating superior results in palpebral width and canthal tilt. FOAR and FODO patients achieved similar Whitaker classification scores. These cohorts will be followed until craniofacial maturity prior to making any definitive conclusions.

Spring forces and calvarial thickness predict cephalic index changes following spring-mediated cranioplasty for sagittal craniosynostosis.

BACKGROUND: Variables interacting to predict outcomes following spring-mediated cranioplasty (SMC) for non-syndromic craniosynostosis, including spring parameters and calvarial thickness, are poorly understood. This study assessed interactions between spring parameters and calvarial thickness to predict changes in cephalic index (CI) following SMC. METHODS: Patients undergoing SMC for non-syndromic sagittal craniosynostosis at our institution between 2014 and 2021 were included. Calvarial thickness was determined from patient preoperative CTs using Materalise Mimics at 27 points in relation to the sagittal suture. Linear mixed effects models were used to determine interactions between anterior, middle, and posterior calvarial thickness with spring force and length. RESULTS: Sixty-nine patients undergoing surgery at mean age 3.7 months were included in this study. Stronger posterior spring force interacted with thinner posterior calvarial thickness to predict greater changes in CI at 3 months postoperatively (p = 0.022). When evaluating spring force and calvarial thickness set distances from the sagittal suture, stronger posterior spring force interacted with thinner posterior calvarial thickness 5 mm (p = 0.043) and 10 mm (p = 0.036) from the sagittal suture to predict changes in CI. Interactions between spring parameters and calvarial thickness in the anterior and middle positions did not significantly predict changes in CI. CONCLUSIONS: Stronger posterior spring force interacted with thinner posterior calvaria to predict greater changes in CI 3 months following SMC for non-syndromic sagittal craniosynostosis. These results suggest dynamic interactions between several variables may impact outcomes following SMC.

Real Face Analysis of Masculinity-Femininity, Facial Ratios, and Attractiveness.

BACKGROUND: Previous studies have evaluated the influence of facial features in determining male and female sex using prototypical renderings or artificially altered faces in relatively small sample sizes. Using a large set of human photographs and raters, this study hypothesized that certain anatomic facial ratios are associated with perceptions of masculinity/femininity, can interact to predict sex, and are associated with ratings of attractiveness differently in males and females. METHODS: Ratings of masculinity-femininity and binary self-identification (male or female) were compared with facial anatomic ratios from 827 frontal facial photographs. Ratios were used to characterize facial feature relativity, where higher ratio scores indicated relatively more facial feature representation. RESULTS: Femininity was associated with prominent middle third ratio, nose length, lip vermillion height, eye height, and eye width ratios; masculinity was associated with prominence of the upper and lower facial thirds, nose width, chin height, and philtrum height ratios (all P < 0.01). Subgroup analysis demonstrated many of these relationships persisted when evaluating masculinity in females and femininity in males. Misgendering in males was associated with greater middle third ratio and upper lip ratio, whereas misgendering in females was associated with increased nose width ratio. CONCLUSIONS: This study demonstrates associations of femininity with increased horizontal middle third representation, and masculinity was associated with increased upper and lower horizontal representation. These facial ratios interact to predict male and female sex, which could have implications for optimizing facial feminization/masculinization outcomes and building algorithms for artificial intelligence analysis of faces.

Visual Attention, Bias, and Social Dispositions Toward People With Facial Anomalies: A Prospective Study With Eye-Tracking Technology.

BACKGROUND: Facial attractiveness influences our perceptions of others, with beautiful faces reaping societal rewards and anomalous faces encountering penalties. The purpose of this study was to determine associations of visual attention with bias and social dispositions toward people with facial anomalies. METHODS: Sixty subjects completed tests evaluating implicit bias, explicit bias, and social dispositions before viewing publicly available images of preoperative and postoperative patients with hemifacial microsomia. Eye-tracking was used to register visual fixations. RESULTS: Participants with higher implicit bias scores fixated significantly less on the cheek and ear region preoperatively (P = 0.004). Participants with higher scores in empathic concern and perspective taking fixated more on the forehead and orbit preoperatively (P = 0.045) and nose and lips (P = 0.027) preoperativel. CONCLUSIONS: Participants with higher levels of implicit bias spent less visual attention on anomalous facial anatomy, whereas participants with higher levels of empathic concern and perspective taking spent more visual attention on normal facial anatomy. Levels of bias and social dispositions such as empathy may predict layperson gaze patterns toward those with facial anomalies and provide insights to neural mechanisms underlying the "anomalous is bad" paradigm.

Current Treatment Landscape for Dystrophic Epidermolysis Bullosa: From Surgical Management to Emerging Gene Therapies and Novel Skin Grafts.

Epidermolysis bullosa is a genetic skin disorder characterized by blister formation from mechanical trauma. Dystrophic epidermolysis bullosa (DEB) is caused by mutations in the COL7A1 gene presenting as generalized blisters from birth, which can result in extensive scarring, alopecia, esophageal stenosis, corneal erosions, and nail dystrophy. This disease also often leads to pseudosyndactyly of the digits from the closure of webspaces, progressing to a "mitten hand" deformity. Although traditional and current treatment for DEB is largely supportive with wound care and iterative surgical pseudosyndactyly release, emerging gene therapies and novel skin grafts may offer promising treatment. Studies published in the early 2020s have used HSV-1 vectors expressing missing COL7A1 genes to restore collagen function. One of these treatments, B-VEC, is an HSV-1-based topical gene therapy designed to restore collagen 7 by delivering the COL7A1 gene, leveraging a differentiated HSV-1 vector platform that evades the patient's immune system response. Other work has been performed to retrovirally modify autologous keratinocytes, but limitations of this process include increased labor in harvesting and engineering autologous cells. This article provides an overview of DEB treatment with an emphasis on emerging gene therapies and novel skin grafts, especially as they pertain to pseudosyndactyly treatment.

Birk-Barel Intellectual Disability Dimorphism and KCNK9 Imprinting Syndrome: Craniofacial Surgery Considerations for an Exceedingly Rare Syndrome.

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.

Genetic Subtypes of Apert Syndrome Are Associated With Differences in Airway Morphology and Early Upper Airway Obstruction.

BACKGROUND: Apert syndrome is predominantly caused by 2 paternally inherited gain-of-function mutations in the FGFR2 gene, Pro253Arg, and Ser252Trp. Studies comparing phenotypic features between these 2 mutations have established differences in syndactyly severity and incidence of cleft palate. Obstructive sleep apnea can be debilitating in a subset of patients with Apert syndrome, yet is not well understood. This study aims to determine whether FGFR2 mutations impart differential effects on airway physiology and morphology. METHODS: Patients with Apert syndrome and confirmatory molecular testing were reviewed for polysomnography, nasal endoscopy, microlaryngoscopy and bronchoscopy, and computed tomography imaging. Obstructive apnea-hypopnea index and oxygen saturation nadir, nasal airway volumes, choanal cross-sectional area, and midfacial cephalometric dimensions were compared across mutation types. RESULTS: Twenty-four patients (13 Ser252Trp, 11 Pro253Arg) were included. Severe obstructive sleep apnea (obstructive apnea-hypopnea index>10) occurred in 8 (62%) patients with Ser252Trp mutations compared with 1 (9%) patient with Pro253Arg mutations ( P =0.009). Computed tomography imaging at 1 year of age demonstrated that nasopharyngeal airway volumes were 5302±1076 mm 3 in the Ser252Trp group and 6832±1414 mm 3 in the Pro253Arg group ( P =0.041). Maxillary length (anterior nasal spine-posterior nasal spine, P =0.026) and basion-anterior nasal spine ( P =0.007) were shorter in patients with Ser252Trp mutations. CONCLUSIONS: The findings suggest that the Ser252Trp mutation in Apert syndrome is associated with higher severity obstructive sleep apnea and decreased nasopharyngeal airway volume. Heightened clinical awareness of these associations may inform treatment planning and family counseling.

Machine Learning in Metopic Craniosynostosis: Does Phenotypic Severity Predict Long-Term Esthetic Outcome?

BACKGROUND: There have been few longitudinal studies assessing the effect of preoperative phenotypic severity on long-term esthetic outcomes in metopic craniosynostosis. This study evaluates the relationship between metopic severity and long-term esthetic outcomes using interfrontal angle (IFA) and CranioRate, a novel metopic synostosis severity measure. METHODS: Patients with metopic craniosynostosis who underwent bifrontal orbital advancement and remodeling between 2012 and 2017 were reviewed. Preoperative computed tomography head scans were analyzed for IFA and CranioRate, a machine learning algorithm which generates quantitative severity ratings including metopic severity score (MSS) and cranial morphology deviation (CMD). Long-term esthetic outcomes were assessed by craniofacial surgeons using blinded 3-rater esthetic grading of clinical photos. Raters assessed Whitaker score and the presence of temporal hollowing, lateral orbital retrusion, frontal bone irregularities and/or "any visible irregularities." RESULTS: Preoperative scans were performed at a mean age of 7.7±3.4 months, with average MSS of 6/10, CMD of 200/300, and IFA of 116.8±13.8 degrees. Patients underwent bifrontal orbital advancement and remodeling at mean 9.9±3.1 months. The average time from operation to esthetic assessment was 5.4±1.0 years. Pearson correlation revealed a significant negative correlation between MSS and age at computed tomography ( r =-0.451, P =0.004) and IFA ( r =-0.371, P =0.034) and between IFA and age at surgery ( r =-0.383, P =0.018). In multinomial logistic regression, preoperative MSS was the only independent predictor of visible irregularities (odds ratio=2.18, B =0.780, P =0.024) and preoperative IFA alone significantly predicted Whitaker score, with more acute IFA predicting worse Whitaker score (odds ratio=0.928, B =-0.074, P =0.928). CONCLUSIONS: More severe preoperative phenotypes of metopic craniosynostosis were associated with worse esthetic dysmorphology. Objective measures of preoperative metopic severity predicted long-term esthetic outcomes.

Geospatial and Socioeconomic Factors Interact to Predict Management and Outcomes in Cleft Lip and Palate Surgery: A Single Institution Study of 740 Patients.

OBJECTIVE: Determine interactions between geospatial and socioeconomic factors influencing cleft lip and/or cleft palate (CL/P) management and outcomes. DESIGN: Retrospective review and outcomes analysis (n = 740). SETTING: Urban academic tertiary care center. PATIENTS: 740 patients undergoing primary (CL/P) surgery from 2009 to 2019. MAIN OUTCOMES MEASURES: Prenatal evaluation by plastic surgery, nasoalveolar molding, cleft lip adhesion, and age at CL/P surgery. RESULTS: Prenatal evaluation by plastic surgery was predicted by the interaction between higher patient median block group income and shorter patient distance from the care center (OR = 1.07, p = 0.022). Nasoalveolar molding was also predicted by the interaction between higher patient median block group income and shorter distance from the care center (OR = 1.28, p = 0.016), whereas cleft lip adhesion was predicted by higher patient median block group income alone (OR = 0.41, p < 0.001). Lower patient median block group income predicted later age at cleft lip (ß = -67.25, p = 0.011) and cleft palate (ß = -46.35, p = 0.050) repair surgery. CONCLUSIONS: Distance from the care center and lower median income by block group interacted to significantly predict prenatal evaluation by plastic surgery and nasoalveolar molding for patients with CL/P at a large, urban, tertiary care center. Patients living farthest from the care center who received prenatal evaluation by plastic surgery or who underwent nasoalveolar molding had higher median block group income. Future work will determine mechanisms perpetuating these barriers to care.

Speech Outcomes Following Operative Management of Velopharyngeal Dysfunction (VPD) in Non-Syndromic Post-Palatoplasty Cleft Palate Patients.

OBJECTIVE: Approximately 30% of patients with a history of repaired cleft palate (CP) go on to suffer from velopharyngeal dysfunction (VPD). This study discusses the operative management of VPD and postoperative speech outcomes in a cohort of CP patients. SETTING: An academic tertiary pediatric care center. METHODS: Retrospective cohort study. PATIENTS: Patients with history of repaired CP (Veau I-IV) who underwent operative management of VPD between January 1st, 2010 and December 31st, 2020. Operative modalities were posterior pharyngeal flap (PPF), sphincter pharyngoplasty (SPP), Furlow palate re-repair, and buccal myomucosal flap palate lengthening (PL). OUTCOME MEASURES: The primary outcome measure is postoperative speech improvement evaluated by the Pittsburgh Weighted Speech Scale (PWSS). RESULTS: 97 patients met inclusion criteria. 38 patients with previous straight-line primary palatoplasty underwent Furlow re-repair; these patients were significantly younger (7.62 vs 11.14, P < .001) and were more likely to have severe VPD per PWSS (OR 4.28, P < .01, 95% CI 1.46-12.56) when compared to VPD patients with previous Furlow repair. 21.1% of these patients required an additional non-revisional VPD procedure. The remaining patients underwent a non-revision procedure (26 PPF, 22 SPP, 11 PL); all experienced significant (P < .001 on paired t-test) reductions in PWSS total and subgroup VPD severity scores without difference in improvement between operation types. SPP was statistically associated with all-cause complication (OR 2.79, 95% CI 1.03-7.59, P < .05) and hyponasality (OR 3.27, 95% CI 1.112-9.630, P < .05). CONCLUSION: Furlow re-repair reduced need for additional VPD operations. Speech outcomes between non-revisional operations are comparable, but increased complications were seen in SPP.

Disability Caused by Cleft Lip and Palate: A Systematic Review and Critical Valuation Appraisal.

OBJECTIVE: Measuring disability as a concept of impaired global function enables beneficiaries of treatment, the impact of treatment, and targets of health system investment to be rigorously assessed. Measures of disability are not well established for cleft lip and palate. This study aims to systematically review disability weight (DW) studies pertaining to orofacial clefts (OFCs) and identify methodological strengths and shortcomings of each approach. DESIGN: Systematic literature review of studies that met the following criteria: (1) peer-reviewed publication, (2) focus on disability valuation, (3) mention orofacial clefts, and (4) publication January 2001-December 2021. SETTING: None. PATIENTS/PARTICIPANTS: None. INTERVENTIONS: None. MAIN OUTCOME MEASURE(S): Disability weight method of valuation and the value itself. RESULTS: The final search strategy yielded 1,067 studies. Seven manuscripts were ultimately included for data extraction. The disability weights used in our studies, including those newly generated or taken from the Global Burden of Disease Studies (GBD), ranged widely for isolated cleft lip (0.0-0.100) and cleft palate with or without cleft lip (0.0-0.269). The GBD studies limited their consideration of cleft sequelae informing disability weights to impact on appearance and speech-related concerns, while other studies accounted for comorbidities such as pain and social stigma. CONCLUSIONS: Current measures of cleft disability are sparse, inadequately reflect the comprehensive impact of an OFC on function and socialization, and are limited in detail or supporting evidence. Use of a comprehensive health state description in evaluating disability weights offers a realistic means of accurately representing the diverse sequelae of an OFC.

Pharyngeal Airway Changes Following Mandibular Distraction Osteogenesis as Evaluated with Laryngoscopy.

PURPOSE: Mandibular distraction osteogenesis (MDO) may improve airway grade in patients with Robin Sequence (RS), but little is known about the response of the oropharyngeal airway to the distraction process in cases of tongue base obstruction (TBAO). This study used drug-induced sleep endoscopy (DISE) to evaluate the impact of MDO on the oropharynx. METHODS: RS patients with severe obstructive sleep apnea (OSA) were prospectively enrolled, and underwent DISE prior to MDO, and at the time of distractor removal. Laryngoscopy views, glossoptosis degree, polysomnography (PSG) results, oxygen saturations and airway measurements were compared pre- and post-MDO. RESULTS: Twenty patients met inclusion criteria. At the time of distractor placement, a grade II laryngoscopic view was most frequently observed (63%), and one patient (5%) had a grade I view. Median obstructive apnea hypopnea index (OAHI) improved after MDO (49.1 [30.2-74.0] to 9.1, [3.9-18.0], p ≤ .001). Median oxygen saturation nadir also improved (preoperative 69% [60-76] to 85% [82-91], p ≤ .001). At distractor removal, mean laryngoscopic view improved (p ≤ .002) with no views that were grade 3 or higher. Median intraoperative oropharyngeal width improved, (3.1 mm [2.8-4.4] to 6.0 mm [4.4-6.8], p ≤ .021), as did median cephalometric anteroposterior oropharyngeal width (3.5 mm [2.7-4.1] to 6.3 mm [5.6-8.2], p ≤ .002). CONCLUSION: Following MDO, RS patients with TBAO have an approximate doubling of oropharyngeal width and an improvement in laryngoscopic grade. These findings likely contribute to improved oxygenation, OAHI and ease of intubation.

Making the Diagnosis in Sagittal Craniosynostosis-It's Height, Not Length, That Matters.

INTRODUCTION: This study assesses the diagnostic reliability of a novel photogrammetric measurement to distinguish sagittal craniosynostosis (SS) from control and false positive cases (SNS). METHODS: Head CTs from 2014-2020 were reviewed for patients with sagittal synostosis (SS, n = 177), presumed sagittal synostosis with normal imaging (SNS, n = 30), and controls (n = 100). Using preoperative clinical photographs and CTs, a measurement reflecting the anterior-posterior location of the vertex was measured using an angle drawn between the cranial vertex, nasion, and opisthocranion (VNO) in profile view, with the head in a neutral position. RESULTS: Mean age at pre-operative head CT was 9.5 months for the SS cohort, 4.2 months for the SNS cohort, and 8.9 months for controls (p = .327). Mean age at pre-operative clinical photograph was 9.5 months for the SS cohort and 4.2 months for the SNS cohort (p = .149). Pearson correlations revealed no significant association between age and VNO angle. The average VNO angle measured on clinical photographs was 54.7° ± 3.8° for the SS group, 43.1° ± 2.2° for the SNS group, and 41.1° ± 3.7° for controls (p < .001). Receiver operating characteristic (ROC) analysis yielded a cut-off of ≥ 50° to identify SS. Diagnostic sensitivity and specificity were 96.6% and 99.2%, respectively. Three-rater analysis yielded an average ICC of 0.742 (p = .004). CONCLUSIONS: Measurement of the VNO angle is a reliable screening tool to diagnose sagittal craniosynostosis, with an angle of 50° or more suggesting suture synostosis. This method relies on the relationship between the anterior displacement of the vertex and occipital bulleting to approach the diagnostic accuracy of CT imaging.

Post-operative vision loss: analysis of 587 patients undergoing endoscopic surgery for pituitary macroadenoma.

PURPOSE: Vision loss following surgery for pituitary adenoma is poorly described in the literature and cannot be reliably predicted with current prognostic models. Detailed characterization of this population is warranted to further understand the factors that predispose a minority of patients to post-operative vision loss. MATERIALS AND METHODS: The medical records of 587 patients who underwent endoscopic transsphenoidal surgery at the Mount Sinai Medical Centre between January 2013 and August 2018 were reviewed. Patients who experienced post-operative vision deterioration, defined by reduced visual acuity, worsened VFDs, or new onset of blurry vision, were identified and analysed. RESULTS: Eleven out of 587 patients who received endoscopic surgery for pituitary adenoma exhibited post-operative vision deterioration. All eleven patients presented with preoperative visual impairment (average duration of 13.1 months) and pre-operative optic chiasm compression. Seven patients experienced visual deterioration within 24 h of surgery. The remaining four patients experienced delayed vision loss within one month of surgery. Six patients had complete blindness in at least one eye, one patient had complete bilateral blindness. Four patients had reduced visual acuity compared with preoperative testing, and four patients reported new-onset blurriness that was not present before surgery. High rates of graft placement (10/11 patients) and opening of the diaphragma sellae (9/11 patients) were found in this series. Four patients had hematomas and four patients had another significant post-operative complication. CONCLUSIONS: While most patients with pituitary adenoma experience favourable ophthalmological outcomes following endoscopic transsphenoidal surgery, a subset of patients exhibit post-operative vision deterioration. The present study reports surgical and disease features of this population to further our understanding of factors that may underlie vision loss following pituitary adenoma surgery. Graft placement and opening of the diaphragma sellae may be important risk factors in vision loss following ETS and should be an area of future investigation.

Surgical outcomes in patients with endoscopic versus transcranial approach for skull base malignancies: a 10-year institutional experience.

OBJECT: The authors performed an extensive comparison between patients treated with open versus an endoscopic approach for skull base malignancy with emphasis on surgical outcomes. METHODS: A single-institution retrospective review of 60 patients who underwent surgery for skull base malignancy between 2009 and 2018 was performed. Disease features, surgical resection, post-operative morbidities, adjuvant treatment, recurrence, and survival rates were compared between 30 patients who received purely open surgery and 30 patients who underwent purely endoscopic resection for a skull base malignancy. RESULTS: Of the 60 patients with skull base malignancy, 30 underwent open resection and 30 underwent endoscopic resection. The most common hisotype for endoscopic resection was squamous cell carcinoma (26.7%), olfactory neuroblastoma (16.7%), and sarcoma (10.0%), and 43.3%, 13.3%, and 10.0% for the open resection cohort, respectively. There were no statistical differences in gross total resection, surgical-associated cranial neuropathy, or ability to achieve negative margins between the groups (p > 0.1, all comparisons). Patients who underwent endoscopic resection had shorter surgeries (320.3 ± 158.5 minutes vs. 495.3 ± 187.6 minutes (p = 0.0003), less intraoperative blood loss (282.2 ± 333.6 ml vs. 696.7 ± 500.2 ml (p < 0.0001), and shorter length of stay (3.5 ± 3.7 days vs. 8.8 ± 6.0 days (p < 0.0001). Additionally, patients treated endoscopically initiated adjuvant radiation treatment more quickly (48.0 ± 20.3 days vs. 72.0 ± 20.5 days (p = 0.01). CONCLUSIONS: An endoscopic endonasal approach facilitates a clinically meaningful improvement in surgical outcomes for skull base malignancies.

Cadaveric Bone Allografting for Severe Mandibular Hypoplasia With Alleviation of Upper Airway Obstruction in Goldenhar Syndrome.

ABSTRACT: Severe congenital mandibular hypoplasia may cause significant upper airway obstruction, often necessitating immediate tracheostomy. Recent surgical advances have demonstrated early intervention with mandibular distraction osteogenesis over tracheostomy may yield desirable aesthetic and functional outcomes. Here we present a case of bilateral mandibular hypoplasia, severe on the right and mild on the left, secondary to Goldenhar syndrome yielding significant tongue-based upper airway obstruction that was surgically corrected with mandibular bone allograft reconstruction of the right condyle, ramus, and posterior half of the mandibular body. Postoperative polysomnography revealed significantly improved obstructive apnea and computed tomography demonstrated adequate placement of the bone allograft to overcorrect the affected hemi-mandible. To the authors' knowledge, this is the first report of immediate mandibular allografting alleviating tongue-based upper airway obstruction secondary to mandibular hypoplasia in an infant. Mandibular bone allografting may be a favorable alternative to immediate tracheostomy with delayed surgical intervention, though long-term follow up is needed to assess graft durability and maintenance of airway patency.