Pediatric Nasoorbitoethmoid Fractures: A Single Institution's 15-Year Experience.

BACKGROUND: Nasoorbitoethmoid (NOE) fractures impact growth of the craniofacial skeleton in children, which may necessitate differentiated management from adult injuries. This study describes characteristics, management, and outcomes of NOE fractures in children seen at a single institution. METHODS: A retrospective review of patients under 18 years who presented to our institution from 2006 to 2021 with facial fractures was conducted; patients with NOE fractures were included. Data collected included demographics, mechanism of injury, fracture type, management, and outcomes. RESULTS: Fifty-eight patients met inclusion criteria; 77.6% presented with Manson-Marcowitz Type I fractures, 17.2% with Type II, and 5.2% with Type III. The most common cause of injury was motor vehicle accidents (MVAs, 39.7%) and sports (31%). Glasgow Coma Scale and injury mechanism were not predictive of injury severity in the pediatric population ( P =0.353, P =0.493). Orbital fractures were the most common associated fractures (n=55, 94.8%); parietal bone fractures were more likely in Type III fractures ( P =0.047). LeFort III fractures were more likely in type II fractures ( P =0.011). Soft tissue and neurological injuries were the most common associated injuries regardless of NOE fracture type (81% and 58.6%, respectively). There was no significant difference in type of operative management or in the rates of adverse outcomes between types of NOE fractures. CONCLUSIONS: These findings suggest that pediatric NOE fractures, although rare, present differently from adult NOE fractures and that revisiting predictive heuristics and treatment strategies is warranted in this population.

3D Photography to Quantify the Severity of Metopic Craniosynostosis.

This study aims to determine the utility of 3D photography for evaluating the severity of metopic craniosynostosis (MCS) using a validated, supervised machine learning (ML) algorithm.This single-center retrospective cohort study included patients who were evaluated at our tertiary care center for MCS from 2016 to 2020 and underwent both head CT and 3D photography within a 2-month period.The analysis method builds on our previously established ML algorithm for evaluating MCS severity using skull shape from CT scans. In this study, we regress the model to analyze 3D photographs and correlate the severity scores from both imaging modalities.14 patients met inclusion criteria, 64.3% male (n = 9). The mean age in years at 3D photography and CT imaging was 0.97 and 0.94, respectively. Ten patient images were obtained preoperatively, and 4 patients did not require surgery. The severity prediction of the ML algorithm correlates closely when comparing the 3D photographs to CT bone data (Spearman correlation coefficient [SCC] r = 0.75; Pearson correlation coefficient [PCC] r = 0.82).The results of this study show that 3D photography is a valid alternative to CT for evaluation of head shape in MCS. Its use will provide an objective, quantifiable means of assessing outcomes in a rigorous manner while decreasing radiation exposure in this patient population.

"Validation of Artificial Intelligence Severity Assessment in Metopic Craniosynostosis".

OBJECTIVE: Several severity metrics have been developed for metopic craniosynostosis, including a recent machine learning-derived algorithm. This study assessed the diagnostic concordance between machine learning and previously published severity indices. DESIGN: Preoperative computed tomography (CT) scans of patients who underwent surgical correction of metopic craniosynostosis were quantitatively analyzed for severity. Each scan was manually measured to derive manual severity scores and also received a scaled metopic severity score (MSS) assigned by the machine learning algorithm. Regression analysis was used to correlate manually captured measurements to MSS. ROC analysis was performed for each severity metric and were compared to how accurately they distinguished cases of metopic synostosis from controls. RESULTS: In total, 194 CT scans were analyzed, 167 with metopic synostosis and 27 controls. The mean scaled MSS for the patients with metopic was 6.18 ± 2.53 compared to 0.60 ± 1.25 for controls. Multivariable regression analyses yielded an R-square of 0.66, with significant manual measurements of endocranial bifrontal angle (EBA) (P = 0.023), posterior angle of the anterior cranial fossa (p < 0.001), temporal depression angle (P = 0.042), age (P < 0.001), biparietal distance (P < 0.001), interdacryon distance (P = 0.033), and orbital width (P < 0.001). ROC analysis demonstrated a high diagnostic value of the MSS (AUC = 0.96, P < 0.001), which was comparable to other validated indices including the adjusted EBA (AUC = 0.98), EBA (AUC = 0.97), and biparietal/bitemporal ratio (AUC = 0.95). CONCLUSIONS: The machine learning algorithm offers an objective assessment of morphologic severity that provides a reliable composite impression of severity. The generated score is comparable to other severity indices in ability to distinguish cases of metopic synostosis from controls.

Mandibular Measurements at the 20-Week Anatomy Ultrasound as a Prenatal Diagnostic Predictor of Pierre Robin Sequence.

BACKGROUND: Pierre Robin Sequence (PRS) is characterized by micrognathia, glossoptosis, and upper airway obstruction. Early recognition and appropriate perinatal management is crucial for optimizing outcomes. This study aimed to evaluate 20-week fetal ultrasounds to determine if specific mandibular measurements could predict PRS diagnosis and disease severity. METHODS: A retrospective case-control study of 48 patients with PRS and gender-matched controls was performed. Medical records were reviewed for respiratory and surgical interventions. Three parameters to assess micrognathia were measured on mid-sagittal profile ultrasound images: frontal nasal-mental angle (FNMA), facial-maxillary angle (FMA), and alveolar overjet. Student's t-test and univariate logistic regression was performed. P ≤ 0.05 was considered statistically significant. RESULTS: Patients with PRS demonstrated a significantly smaller mean FNMA compared to the control group, 129.3 ± 8.6° and 137.4 ± 3.2°, respectively (p < 0.0001), as well as significantly smaller mean FMA, 63.2 ± 9.2° and 74.8 ± 6.1°, respectively (p < 0.0001). The PRS group also demonstrated significantly larger mean alveolar overjet compared to the control group, 3.9 ± 1.4 mm and 2.1 ± 0.9 mm, respectively (p < 0.0001). The odds of respiratory intervention increased among cases when FMA was <68°. Additionally, there was a significant difference in median overjet between patients with PRS who did and did not require respiratory intervention. CONCLUSIONS: Mandibular features on the 20-week ultrasound can be measured to predict diagnosis and severity of PRS. This is an important first step to prepare for potential respiratory intervention at delivery to minimize perinatal hypoxia. Alveolar overjet, previously not described in prenatal ultrasound literature, is measurable and has utility in prenatal screening for PRS, as do FMA and FNMA.

Squamosal Suture Synostosis: An Under-Recognized Phenomenon.

INTRODUCTION: The squamosal suture (SQS) joins the temporal to the parietal bones bilaterally and is a poorly described site of craniosynostosis. SQS fusion is thought to occur as late as the fourth decade of life and beyond; however, we have incidentally noted its presence among our pediatric patients and hypothesize that it may occur earlier in life and more frequently than previously believed. METHODS: A retrospective review of imaging performed on pediatric patients was completed to identify patients with SQS synostosis. This included a review of clinical notes as well as computed tomography (CT) images obtained by our craniofacial clinic. Relevant patient data and imaging were reviewed. RESULTS: Forty-seven patients were identified with SQS synostosis, 21 were female (45%). Age at the time of radiographic diagnosis was 10.1 ± 8.4 years (range 17 days to 27 years). A majority of patients had bilateral SQS synostosis (57%), with a relatively even distribution of unilateral right (23%) versus left (19%). SQS was an isolated finding (no other suture involvement) in 15 patients (32%), all of whom were normocephalic and did not require surgical intervention. Thirty-two patients (68%) had concomitant craniosynostosis of other sutures, most commonly sagittal and coronal. Nine patients (19%) underwent surgery to correct cranial malformations-all these patients had multi-suture synostosis (P = 0.012). Twenty-seven patients (57%) had SQS synostosis diagnosed incidentally compared to 20 (43%) who were imaged with suspicion for synostosis. In those who were symptomatic, common findings included developmental delay, elevated intracranial pressure, hydrocephalus, seizures, and visual/hearing impairments. Ten patients (21%) were syndromic, the most frequent of which was Crouzon syndrome. No single pattern of calvarial malformation could be definitively described for SQS synostosis. CONCLUSION: Given that most isolated SQS synostosis cases were normocephalic, asymptomatic, and discovered incidentally, it is likely that there are many cases of unidentified SQS synostosis. The significance of SQS synostosis is currently unclear, and warrants further investigation into this phenomenon, its natural course, and its potential presence in the spectrum of normal development.

A Three-Dimensional-Based Morphometric Analysis of a Standardized Overcorrection Technique for Fronto-Orbital Advancement in Metopic Craniosynostosis.

INTRODUCTION: The concept of "overcorrection" for trigonocephaly has been reported to achieve both anterior cranial fossa expansion and normalization of craniofacial form. The purpose of this study is to describe in detail a standardized technique to fronto-orbital advancement utilizing the concept of "overcorrection" and objectively evaluate intermediate results. METHODS: This retrospective study included patients with isolated metopic synostosis who underwent surgery via the proposed surgical technique and age and sex-matched unaffected controls. Craniofacial morphometric analysis was performed on pre-, immediate post-, and intermediate postoperative (>2 years) three-dimensional (3D)-rendered computed tomographic (CT) scans and photographs. Key CT-based measurements included interzygomaticofrontal suture distance (IZFS), endocranial bifrontal angle (ECA), and temporal expansion. 3D photogrammetry was performed using established measurements and associated Z-scores converted. A Paired t-test and analysis of variance were performed when appropriate. RESULTS: Forty-one patients were included. A comparison of pre- and immediate postoperative CT scans demonstrated statistically significant increases in all measurements. Subset analysis of 12 patients with intermediate follow-up (age: 39.6 ± 3.6 months) demonstrated significant differences from preoperative values except for IZFS, which decreased from immediate postoperative values and was smaller than age- and sex-matched controls. 3D photogrammetry demonstrated a mean Z-score above the norm for frontal breath. 3D photogrammetry is also positively correlated with CT-based measurements. CONCLUSIONS: This standardized "overcorrection" approach for trigonocephaly can provide the appropriate changes to maintain a normal ECA despite a reduction in bifrontal width over time. 3D photogrammetry positively correlated with CT-based measurements and may provide useful information when following patients clinically. Long-term follow-up assessment to determine the necessary degree of overcorrection at skeletal mature is needed.

The Clinical Significance of Clinocephaly in Late-Presentation Sagittal Craniosynostosis.

The diagnosis of late-presentation sagittal suture craniosynostosis (SCS) can be challenging, especially in the setting of subtle physical exam findings. The clinical significance of clinocephaly-a retro-coronal concavity along the midvault-in this context remains unknown. The aim of this study is to evaluate the predictive value of clinocephaly in identifying late-presentation SCS.A retrospective chart review of all patients >1 year old presenting to the craniofacial clinic with a concern for SCS was performed. The presence or absence of SCS in the setting of clinocephaly was recorded following diagnostic imaging. Student's t test, Chi Square test, and multivariate logistic regression analysis were performed to determine predictors for SCS.75 patients met inclusion criteria. 32 patients (42.7%, 6% female) were diagnosed with SCS. No difference in age between patients with and without SCS was detected. Stratification of patients by age (1-2, 2-4, and >4 years) revealed a higher rate of SCS in younger patients (P = 0.04). The cephalic index (C.I.) of those with sagittal synostosis was significantly smaller but within the normal range, indicating a more scaphocephalic shape (P = 0.003). Logistic regression analysis revealed that C.I. was a strong predictor for SCS (P = 0.003). Of those with SCS, a mix of complete and partial fusion of the sagittal suture was appreciated.This study found that 42.7% of patients with clinocephaly had SCS. C.I. was the only predictor for SCS and unique suture fusion patterns were identified in those with SCS. This study suggests that clinocephaly should be considered a core component of the exam and work-up for SCS. Future studies aimed at evaluating the positive predictive value of this exam finding and identifying risk factors associated with late-presentation SCS are underway.

Transgender Mastectomy: Incidence of High-Risk Pathologic Findings and the Need for Postoperative Cancer Surveillance.

BACKGROUND: Chest masculinization surgeries are one of the most common gender-affirming procedures performed. There is a need for better understanding of the risk of breast cancer and postsurgical screening in female to male (FtM) individuals. This study aimed to evaluate the incidence of high-risk pathologic findings in FtM transgender patients undergoing gender-affirming chest reconstructive surgery. METHODS: Medical records were reviewed from all FtM patients undergoing gender-affirming chest reconstructive surgery from January 2010 to February 2021 by 3 plastic surgeons at the University of Pittsburgh Medical Center. Relative risk of malignant progression was used to stratify pathologic data. Subsequent management of atypical, in situ, and invasive pathology were recorded. RESULTS: A total of 318 patients were included in this study; the average age at surgery was 24.6 ± 8.1 years. Eighty-six patients (27%) had a family history of breast and/or ovarian cancer. Overall, 21 patients (6.6%) had some increased risk of breast cancer: 17 (5.3%) had proliferative lesions, mean age 38.2 ± 12.4 years; 2 had atypical ductal hyperplasia, ages 33.4 and 38.3 years; and 2 had invasive ductal carcinoma, ages 35.4 and 40.6 years. CONCLUSIONS: In this study, we found that 6.6% of FtM transgender patients undergoing top surgery had an elevated risk of breast cancer, with 1.2% of patients having a greater than 2 times risk of breast cancer. These results highlight the importance of appropriate preoperative screening as well as pathological analysis of surgical specimens to help guide clinical care. The authors advocate for a thorough breast cancer risk assessment before surgery for all patients, as well as using pathologic findings to guide postoperative cancer screening and follow-up.

Galactorrhea After Nipple-Sparing Mastectomy: Case Report, Review of the Literature, and Algorithmic Approach to Management.

ABSTRACT: Nipple discharge is a rare but possible occurrence after nipple-sparing mastectomy (NSM). This study presents the first case of galactorrhea in a female patient after NSM. Although milky discharge due to physiologic lactation related to pregnancy is more common, galactorrhea is still possible and should be worked up appropriately to ensure that all breast tissue has been removed and that there are no other more worrisome causes.

Revision Pharyngoplasty in Cleft Palate and Velopharyngeal Insufficiency: Management and Outcomes.

INTRODUCTION: Velopharyngeal insufficiency (VPI), a stigmatizing hallmark of palatal dysfunction, occurs in a wide spectrum of pediatric craniofacial conditions. The mainstays for surgical correction include palate repair and/or pharyngeal surgery. However, primary pharyngoplasty has a failure rate of 15% to 20%. Although revision pharyngoplasty may be necessary in those with persistent VPI, little is known regarding the indications for and outcomes after such procedures. The purpose of this study is to describe the authors' experience with indications for and outcomes after revision pharyngoplasty. METHODS: A single-center retrospective review was performed of all patients undergoing revision pharyngoplasty between 2002 and 2019. Demographic data and Pittsburgh Weighted Speech Scores, diagnoses, comorbidities, and complications were tabulated. Two-tailed Student t test was used, and a P value of 0.05 or less was considered statistically significant. RESULTS: Thirty-two patients (65.6% male) met inclusion criteria for this study. The most common diagnoses included cleft palate (68.8%), submucous cleft palate (SMCP, 18.8%), and congenital VPI (6.3%, likely occult SMCP). Most patients (84.4%) underwent palatoplasty before their initial pharyngoplasty. The primary indication for initial pharyngoplasty was VPI (mean age 7.1 ± 4.6 years). The most common indication for revision pharyngoplasty (mean age 11.2 ± 5.1 years) included persistent VPI (n = 22), followed by obstructive sleep apnea (OSA) (n = 11). Persistent VPI (n = 8) and OSA (n = 6) were the most common complications after secondary pharyngoplasty. Thirteen patients (40.6%) within the revision pharyngoplasty cohort required additional surgical intervention: 4 underwent tertiary pharyngoplasty, 4 underwent takedown for OSA (n = 3) or persistent VPI (n = 1), 3 underwent takedown and conversion Furlow for persistent VPI (n = 2), OSA (n = 2) and/or flap dehiscence (n = 1), and 2 underwent palatal lengthening with buccal myomucosal flaps for persistent VPI. Of the 4 patients who required a tertiary pharyngoplasty, the mean age at repair was 6.6 ± 1.1 years and their speech scores improved from 13.5 to 2.3 after tertiary pharyngoplasty (P = 0.11). The overall speech score after completion of all procedures improved significantly from 19 to 3.3. CONCLUSION: Patients who fail primary pharyngoplasty represent a challenging population. Of patients who underwent secondary pharyngoplasty, nearly half required a tertiary procedure to achieve acceptable speech scores or resolve complications.

Pediatric Plastic Surgery Operating Room Block-Time Utilization: A Casualty of Illness.

BACKGROUND: Optimizing operating room (OR) utilization is a critical component of health care system efficiency. The purpose of our study was to analyze the extent of OR cancellation and its effect on raw utilization of OR block time allotted to surgeons in the pediatric plastic surgery department. METHODS: The authors retrospectively reviewed the cases of 4 plastic surgeons at a tertiary pediatric hospital between 2018 and 2019. Data collected included patient demographics, type of surgery, time of cancellation, reason for cancellation, length of surgery, and minutes of block time allotted to each surgeon per year. Percent of cases canceled, scheduled times lost, and block times lost were calculated. RESULTS: Surgeons A, B, C, and D scheduled 170, 416, 305, and 474 cases, respectively. Overall, 7% of cases were canceled, 9.1% of scheduled time was lost, and a total of 5.1% of block time was lost due to cancellation. Patients of surgeon A and D were more likely to cancel due to reasons classified as ''other,'' including causes such as failure of nil per os or a family's last-minute decision to forego elective surgery (33.3% and 37.2%, respectively), whereas patients of surgeon B and C were more likely to cancel due to illness (67.9% and 36.4%, respectively). CONCLUSIONS: Cancellations negatively impact raw utilization times; if fewer cases are performed, allotted block times are redistributed. Communication with patients in the week prior to surgery may allow for earlier identification of likely cancellations. Future directions include exploring whether particular surgeon characteristics are linked to rate of cancellations.

Tissue Augmenting Palatoplasty for the Treatment of Velopharyngeal Insufficiency.

PURPOSE: Persistent velopharyngeal insufficiency (VPI) following primary palatoplasty remains a difficult problem to treat. This study evaluates speech outcomes following revision palatoplasty with tissue augmentation using buccal myomucosal flaps (BMF) as an alternative to pharyngoplasty for patients with VPI. METHODS: A retrospective single-center review of revision palatoplasty with tissue augmentation at a tertiary pediatric hospital Cleft-Craniofacial Center between January 2017 and March 2021 was conducted. Patients with a history of previous palatoplasty, a diagnosis of persistent or recurrent VPI, and comprehensive pre- and postoperative speech evaluations who underwent revision palatoplasty with BMF were included. RESULTS: Twenty patients met inclusion criteria (35% female, 20% syndromic). Mean age at the time of revision palatoplasty with BMF was 9.7 years. Preoperatively, all patients had stigmatizing speech and received the recommendation for speech surgery; the mean Pittsburgh Weighted Speech Score (PWSS) was 14.3 ± 4.9. The mean postoperative PWSS at the most recent assessment was 4.2 ± 2.3, representing a statistically significant improvement from preoperative scores (P < .001). Mean follow-up time was 8.9 months. Following revision palatoplasty with BMF, only one patient has received the recommendation for further speech surgery. No complications were noted. CONCLUSION: In patients with VPI following primary palatoplasty, revision palatoplasty with tissue augmentation offers an alternative to pharyngoplasty. This approach preserves dynamic velopharyngeal function, improves speech outcomes, and should be considered an option when treating patients with post-primary palatoplasty VPI.

Computed tomography associated radiation exposure in children with craniosynostosis.

BACKGROUND: The role of computed tomography (CT) for diagnosis and surgical planning for craniosynostosis (CS) is well-established. The aim of this study was to quantify the cumulative medical radiation exposure from CT in patients with CS at a tertiary care children's hospital. METHODS: Medical records of patients who presented at < 2 years of age and underwent surgical intervention for CS were examined for demographic information. Effective radiation dose (ERD) in mSv was calculated for each head CT. Descriptive statistics and ANOVA were performed. Mean ± SD is reported; p < 0.05 was considered significant. RESULTS: Two hundred seventy-two patients met inclusion criteria: 241 nonsyndromic and 31 with syndromic diagnoses. For nonsyndromic patients, mean age at first head CT was 6.0 ± 4.9 months, mean number of CT scans obtained was 2.1 ± 1.1, and the mean total combined ERD was 9.1 ± 4.8 mSv. CT scans obtained at < 6 months of age had a significantly greater ERD than those obtained at > 6 months, 5.3 ± 1.9 versus 4.3 ± 1.4 mSv, respectively (p = 0.001). CONCLUSIONS: Patients with nonsyndromic CS undergo 2 CT scans on average related to their diagnosis, with a mean total ERD of 9.1 mSv; this is equivalent to 1.5 years of the average annual background radiation dose a person living in the USA will encounter from environmental radiation, medical exposures, and consumer products. A CT obtained at < 6 months is associated with a higher ERD; thus, we recommend delaying imaging from the initial presentation to the time of pre-operative planning when possible.

Posterior Cranial Vault Distraction Osteogenesis Utilizing a Posterior-Superior Distraction Vector in the Treatment of Mercedes Benz Pattern Craniosynostosis.

ABSTRACT: Bilateral lambdoid and sagittal synostosis, or Mercedes Benz Syndrome, is a rare complex craniosynostosis resulting in frontal bossing, a tapered posterior fossa, and an anteriorly displaced cranial vertex. Its ideal surgical correction must result in posterior expansion, skull elongation, and caudal repositioning of the vertex. We present a craniometric analysis of skull changes with posterior-superior distraction and introduce a novel craniometric measure: vertex position. In this study, a retrospective review was performed to analyze outcomes of posterior cranial vault distraction osteogenesis (PVDO) using a posterior-superior distraction vector from 2016 to 2019. Cranial vertex position was measured as a fraction of the occipitofrontal diameter from rostral to caudal (0-1.0). Four patients underwent PVDO at mean age 10.61 ±â€Š3.16 months utilizing a posterior-superior distraction vector. Linear distraction distance averaged 30.30 ±â€Š0.90 mm with a mean consolidation period of 3.98 ±â€Š0.72 months. Mean corrected change in intra-cranial volume was 236.30 ±â€Š3.71 mL, at an average rate of 7.81 ±â€Š2.00 mL/mm of distraction. Increases in anterior cranial height (7.83 ±â€Š2.51 mm), middle cranial height (8.43 ±â€Š4.21 mm), posterior cranial height (13.15 ±â€Š7.45 mm), and posterior cranial fossa height (21.99 ±â€Š8.55 mm) were observed. Cranial vertex demonstrated a mean posterior movement of 0.18 ±â€Š0.13. PVDO utilizing a posterior-superior distraction vector for management of nonsyndromic bilateral lambdoid and sagittal synostosis effectively increases intracranial volume and height and provides an esthetic outcome with posterior movement of the cranial vertex.

A Road Map to Creating a High-Quality Clinical Database in Plastic Surgery.

BACKGROUND: Detailed in-house databases are a staple of surgical research and a crucial source of data for many studies from which clinical guidelines are built. Despite the importance of generating a clear and thorough developmental design, the literature on database creation and management is limited. In this article, the authors present their stepwise single-institution process of developing a clinical facial fracture database. METHODS: The authors outline the process of development of a large single-institution clinical pediatric facial fracture database. The authors highlight critical steps from conception, regulatory approval, data safety/integrity, human resource allocation, data collection, quality assurance, and error remediation. The authors recorded patient characteristics, comorbidities, details of the sustained fracture, associated injuries, hospitalization information, treatments, outcomes, and follow-up information on Research Electronic Data Capture. Protocols were created to ensure data quality assurance and control. Error identification analysis was subsequently performed on the database to evaluate the completeness and accuracy of the data. RESULTS: A total of 4451 records from 3334 patients between 2006 and 2021 were identified and evaluated to generate a clinical database. Overall, there were 259 incorrect entries of 120,177 total entries, yielding a 99.8% completion rate and a 0.216% error rate. CONCLUSIONS: The quality of clinical research is intrinsically linked to the quality and accuracy of the data collection. Close attention must be paid to quality control at every stage of a database setup. More studies outlining the process of database design are needed to promote transparent, accurate, and replicable research practices.

CranioRate: An Image-Based, Deep-Phenotyping Analysis Toolset and Online Clinician Interface for Metopic Craniosynostosis.

BACKGROUND: The diagnosis and management of metopic craniosynostosis involve subjective decision-making at the point of care. The purpose of this work was to describe a quantitative severity metric and point-of-care user interface to aid clinicians in the management of metopic craniosynostosis and to provide a platform for future research through deep phenotyping. METHODS: Two machine-learning algorithms were developed that quantify the severity of craniosynostosis-a supervised model specific to metopic craniosynostosis [Metopic Severity Score (MSS)] and an unsupervised model used for cranial morphology in general [Cranial Morphology Deviation (CMD)]. Computed tomographic (CT) images from multiple institutions were compiled to establish the spectrum of severity, and a point-of-care tool was developed and validated. RESULTS: Over the study period (2019 to 2021), 254 patients with metopic craniosynostosis and 92 control patients who underwent CT scanning between the ages of 6 and 18 months were included. CT scans were processed using an unsupervised machine-learning based dysmorphology quantification tool, CranioRate. The average MSS was 0.0 ± 1.0 for normal controls and 4.9 ± 2.3 ( P < 0.001) for those with metopic synostosis. The average CMD was 85.2 ± 19.2 for normal controls and 189.9 ± 43.4 ( P < 0.001) for those with metopic synostosis. A point-of-care user interface (craniorate.org) has processed 46 CT images from 10 institutions. CONCLUSIONS: The resulting quantification of severity using MSS and CMD has shown an improved capacity, relative to conventional measures, to automatically classify normal controls versus patients with metopic synostosis. The authors have mathematically described, in an objective and quantifiable manner, the distribution of phenotypes in metopic craniosynostosis.

True Incidence of Marginal Mandibular Nerve Palsy Following Neonatal Mandibular Distraction Osteogenesis.

INTRODUCTION: In children with PRS, MDO is routinely performed to alleviate airway obstruction; however, it involves risk of injury to the MMN. We hypothesize that MMN palsy incidence following MDO, reported at 1-15%, is underestimated. This study investigates the true incidence of MMN palsy after MDO to better guide follow-up care and improve treatment of this complication. METHODS: A retrospective review of PRS patients who underwent MDO at a single, tertiary pediatric hospital between September 2007 and March 2021 was conducted. Patients who underwent MDO under one year of age and had postoperative clinical evaluations detailing MMN function were included. Logistic regression analysis was performed to investigate predictors of MMN injury. RESULTS: Of 93 patients who underwent MDO, 59.1% met inclusion criteria. 56.4% were female, 43.6% were syndromic, and average age at MDO was 1.52 ± 2.04 months. The average length of mandibular distraction was 17.3 ± 4.36mm, average duration of intubation was 6.57 ± 2.37 days, and average time until hardware removal was 111.1 ± 23.6 days. Sixteen patients (29.1%) presented with permanent MMN dysfunction, comprised of 8 patients with bilateral weakness and 8 with unilateral weakness. An additional five patients (9.1%) presented with transient MMN weakness that resolved within a year. Average length of follow-up postoperatively was 6.02 years, and no significant predictors of nerve injury were found. CONCLUSION: In this 14-year review of patients with PRS who underwent MDO, 38.2% demonstrated evidence of MMN palsy (29.1% permanent, 9.1% transient), which is much greater than previously described.

Quantifying the Severity of Metopic Craniosynostosis Using Unsupervised Machine Learning.

BACKGROUND: Quantifying the severity of head shape deformity and establishing a threshold for operative intervention remains challenging in patients with metopic craniosynostosis (MCS). This study combines three-dimensional skull shape analysis with an unsupervised machine-learning algorithm to generate a quantitative shape severity score (cranial morphology deviation) and provide an operative threshold score. METHODS: Head computed tomography scans from subjects with MCS and normal controls (5 to 15 months of age) were used for objective three-dimensional shape analysis using ShapeWorks software and in a survey for craniofacial surgeons to rate head-shape deformity and report whether they would offer surgical correction based on head shape alone. An unsupervised machine-learning algorithm was developed to quantify the degree of shape abnormality of MCS skulls compared to controls. RESULTS: One hundred twenty-four computed tomography scans were used to develop the model; 50 (24% MCS, 76% controls) were rated by 36 craniofacial surgeons, with an average of 20.8 ratings per skull. The interrater reliability was high (intraclass correlation coefficient, 0.988). The algorithm performed accurately and correlates closely with the surgeons assigned severity ratings (Spearman correlation coefficient, r = 0.817). The median cranial morphology deviation for affected skulls was 155.0 (interquartile range, 136.4 to 194.6; maximum, 231.3). Skulls with ratings of 150.2 or higher were very likely to be offered surgery by the experts in this study. CONCLUSIONS: This study describes a novel metric to quantify the head shape deformity associated with MCS and contextualizes the results using clinical assessments of head shapes by craniofacial experts. This metric may be useful in supporting clinical decision making around operative intervention and in describing outcomes and comparing patient population across centers.

Characterization of the Saddle Nose Deformity Following Endoscopic Endonasal Skull Base Surgery.

Objective The endoscopic endonasal approach (EEA) is commonly employed in skull base surgery for neoplasm resection. While nasal deformity following EEA is described, this study aimed to perform a detailed qualitative and quantitative assessment of the associated saddle nose deformity (SND) in particular. Setting/Participants This is a retrospective review of 20 adult patients with SND after EEA for resection of skull base tumors over a 5-year period at the University of Pittsburgh Medical Center. Main Outcome Measures Fifteen measurements related to SND were obtained on pre- and postoperative imaging. Statistical analyses were performed to evaluate differences between pre- and postoperative anatomies. Results The most common EEA was transsellar. Reconstruction techniques included nine free mucosal grafts alone, eight vascularized nasoseptal flaps (NSFs), one combined free mucosal graft/abdominal fat graft, and one combined NSF/fascia lata graft. Imaging analysis showed a trend toward loss of mean nasal height, nasal tip projection, and nasolabial angle postoperatively. Subgroup analysis showed that patients with NSF reconstruction had a significantly decreased nasal tip projection (1.2 mm, p = 0.039) and increased alar base width (1.2 mm, p = 0.046) postoperatively. Patients without functional pituitary microadenomas demonstrated significantly increased nasofrontal angle and decreased nasal tip projection on postoperative imaging, in contrast to those with functional adenomas who had no measurable significant changes. Conclusion Clinically evident SND does not always lead to significant radiographic changes. This analysis suggests that patients who undergo surgery for indications other than functional pituitary microadenomas or who receive NSF reconstruction develop more marked SND on standard imaging tests.

Late-Presenting Sagittal Craniosynostosis: An Update to a Standardized Treatment Protocol.

BACKGROUND: The authors previously published their protocol to treat patients who present with sagittal craniosynostosis after the age of 1 year. The purpose of this article is to present a follow-up and update of this cohort to evaluate outcomes of their treatment protocol. METHODS: Patients with isolated sagittal craniosynostosis who presented after the age of 1 year between July of 2013 and April of 2021 were included. RESULTS: A total of 108 patients met inclusion criteria. The average age at presentation was 5.2 ± 3.4 years, and 79 patients (73.1%) were male. The indications for imaging were head shape (54.6%), headache (14.8%), trauma (9.3%), seizure (4.6%), papilledema (2.8%), and other (13.9%). Of the 108 patients, 12 (11.1%) underwent surgery following their initial consultation: five for papilledema, four for elevated intracranial pressure, two for severely scaphocephalic head shapes, and one for abnormal funduscopic findings. Two of these patients underwent additional reconstructive surgery, one for the recurrence of papilledema and headache and the other for progressive scaphocephaly. The average length of time between operations was 4.9 years. Of the 96 patients who were managed conservatively, four (4.2%) underwent surgery at an average of 1.2 ± 0.5 years later (average age, 4.4 ± 1.5 years) for brain growth restriction ( n = 2), aesthetic concerns ( n = 1), and refractory headaches ( n = 1). The average follow-up of all patients with craniofacial surgery was 2.7 ± 2.3 years (median, 2.1 years; interquartile range, 3.7 years). CONCLUSIONS: Patients with late-presenting sagittal craniosynostosis require surgical correction less often than younger patients, likely because of milder phenotype. Few patients in the conservative treatment arm ultimately required surgery (4%). CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.