What to do with an incidental finding of a fused sagittal suture: a modified Delphi study.

OBJECTIVE: As many as 5% of normocephalic children may have a prematurely fused sagittal suture, yet the clinical significance and best course of management of this finding remain unclear. Providers in the Synostosis Research Group were surveyed to create a multicenter consensus on an optimal treatment and monitoring algorithm for this condition. METHODS: A four-round modified Delphi method was utilized. The first two rounds consisted of anonymous surveys distributed to 10 neurosurgeons and 9 plastic surgeons with expertise in craniosynostosis across 9 institutions, and presented 3 patients (aged 3 years, 2 years, and 2 months) with incidentally discovered fused sagittal sutures, normal cephalic indices, and no parietal dysmorphology. Surgeons were queried about their preferred term for this entity and how best to manage these patients. Results were synthesized to create a treatment algorithm. The third and fourth feedback rounds consisted of open discussion of the algorithm until no further concerns arose. RESULTS: Most surgeons preferred the term "premature fusion of the sagittal suture" (93%). At the conclusion of the final round, all surgeons agreed to not operate on the 3- and 2-year-old patients unless symptoms of intracranial hypertension or papilledema were present. In contrast, 50% preferred to operate on the 2-month-old. However, all agreed to utilize shared decision-making, taking into account any concerns about future head shape and neurodevelopment. Panelists agreed that patients over 18 months of age without signs or symptoms suggesting elevated intracranial pressure (ICP) should not undergo surgical treatment. CONCLUSIONS: Through the Delphi method, a consensus regarding management of premature fusion of the sagittal suture was obtained from a panel of North American craniofacial surgeons. Without signs or symptoms of ICP elevation, surgery is not recommended in patients over 18 months of age. However, for children younger than 18 months, surgery should be discussed with caregivers using a shared decision-making process.

Pediatric Facial Fractures: Demographics, Injury Patterns, and Associated Injuries in 3334 Patients.

Pediatric craniofacial fractures are fundamentally distinct from their adult counterparts because of unique injury patterns and effects on future growth. Understanding patterns and injury context informs management and risk mitigation. Previous studies include only inpatients, operative patients, or are specialty-specific. In contrast, our study presents a comprehensive assessment of all pediatric facial fracture patients seen at a single institution. Patients under 18 years old who were evaluated for facial fractures at a level I pediatric trauma center between 2006 and 2021 were reviewed. Subanalysis was performed for groups defined by age. Variables studied included demographics, etiology, fracture pattern, associated injuries, management, and outcomes. Three thousand thirty-four patients were included. Mean age at presentation was 11.5 to 4.9 years. The majority were Caucasian (82.6%) and male (68.4%). Sports were the leading cause of injury in older patients (42.2% of patients over 12 y), compared with activities of daily living in patients under 6 years (45.5%). Thirty-two percent of patients were hospitalized, 6.0% required ICU care, and 48.4% required surgery. Frequency of ICU admission decreased with age (P<0.001), whereas operative intervention increased with age (P<0.001). Zygomaticomaxillary complex (P=0.002) and nasal fractures (P<0.001) were common in older patients, whereas younger patients experienced more skull (P<0.001) and orbital fractures (P<0.001). The most associated injuries were soft tissue (55.7%) and neurologic (23.6%). This large-scale study provides updated characterization of craniofacial fractures in the pediatric population, providing a necessary framework for future studies on outcomes assessments and preventative care.

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.

A Longitudinal Analysis of Pre- and Post-Operative Dysmorphology in Metopic Craniosynostosis.

OBJECTIVE: The purpose of this study is to objectively quantify the degree of overcorrection in our current practice and to evaluate longitudinal morphological changes using CranioRateTM, a novel machine learning skull morphology assessment tool.  . DESIGN: Retrospective cohort study across multiple time points. SETTING: Tertiary care children's hospital. PATIENTS: Patients with preoperative and postoperative CT scans who underwent fronto-orbital advancement (FOA) for metopic craniosynostosis. MAIN OUTCOME MEASURES: We evaluated preoperative, postoperative, and two-year follow-up skull morphology using CranioRateTM to generate a Metopic Severity Score (MSS), a measure of degree of metopic dysmorphology, and Cranial Morphology Deviation (CMD) score, a measure of deviation from normal skull morphology. RESULTS: Fifty-five patients were included, average age at surgery was 1.3 years. Sixteen patients underwent follow-up CT imaging at an average of 3.1 years. Preoperative MSS was 6.3 ± 2.5 (CMD 199.0 ± 39.1), immediate postoperative MSS was -2.0 ± 1.9 (CMD 208.0 ± 27.1), and longitudinal MSS was 1.3 ± 1.1 (CMD 179.8 ± 28.1). MSS approached normal at two-year follow-up (defined as MSS = 0). There was a significant relationship between preoperative MSS and follow-up MSS (R2 = 0.70). CONCLUSIONS: MSS quantifies overcorrection and normalization of head shape, as patients with negative values were less "metopic" than normal postoperatively and approached 0 at 2-year follow-up. CMD worsened postoperatively due to postoperative bony changes associated with surgical displacements following FOA. All patients had similar postoperative metopic dysmorphology, with no significant association with preoperative severity. More severe patients had worse longitudinal dysmorphology, reinforcing that regression to the metopic shape is a postoperative risk which increases with preoperative severity.

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.

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.

Perceptual Barriers to Becoming a Plastic Surgeon among Underrepresented Medical Students.

Background: The field of plastic surgery has experienced difficulty increasing diversity among trainees, despite significant efforts. Barriers to recruitment of underrepresented in medicine (URM) students are poorly understood. This study assesses URM students' exposure to plastic surgery, access to mentors and research opportunities, and the importance of diversity in the field. Methods: A survey was designed and distributed to members of the Student National Medical Association over 3 months. Survey data were collected using Qualtrics and descriptive statistics, and logistical regressions were performed using SAS. Results: Of the 136 respondents, 75.0% identified as Black (n = 102/136), and 57.4% (n = 66/115) reported a plastic surgery program at their home institution. Of the total respondents, 97.7% (n = 127/130) were concerned about racial representation in plastic surgery, and 44.9% (n = 53/114) would be more likely to apply if there were better URM representation. Most respondents disagreed that there was local (73.4%, n = 58/79) or national (79.2%, n = 57/72) interest in URM recruitment. Students whose plastic surgery programs had outreach initiatives were more likely to have attending (OR 11.7, P < 0.05) or resident mentors (OR 3.0 P < 0.05) and access to research opportunities (OR 4.3, P < 0.05). Conclusions: URM students feel there is an evident lack of interest in recruiting URM applicants in plastic surgery. Programs with outreach initiatives are more likely to provide URM students access to mentorship and research opportunities, allowing students to make informed decisions about pursuing plastic surgery.

Fracture patterns, associated injuries, management, and treatment outcomes of 530 pediatric mandibular fractures.

BACKGROUND: Mandibular fractures account for up to 48.8% of pediatric facial fractures; however, there are a wide range of available treatment modalities, and few studies describe trends in adverse outcomes of these injuries. This study describes fracture etiology, pattern, management, and treatment outcomes in pediatric mandibular fracture patients. METHODS: A retrospective review was performed of patients under 18 years of age who were evaluated for mandibular fractures at a pediatric level I trauma center between 2006 and 2021. Variables studied included demographics, etiology, medical history, associated facial fractures, other associated injuries, treatments, and outcomes. RESULTS: A total of 530 pediatric patients with 829 mandibular fractures were included in the analysis. Most isolated mandibular fractures were treated with physical therapy (PT) and rest (n=253, 47.7%). Patients with combination fractures, specifically those involving the parasymphysis and angle, were 2.63 times more likely to undergo surgical management compared to patients with a single facial fracture (p<0.0001). Older age (p<0.001), gender (p=0.042), mechanism (p=0.008) and cause of injury (p=0.002), as well as specific fractures (e.g., isolated angle (p=0.001)) were more associated with adverse outcomes. The odds of adverse outcomes were higher for patients treated with CREF or ORIF compared to conservative management (OR=1.8; 95% Cl 1.0-3.2 and OR=2.1; 95% Cl 1.2-3.5, respectively). CONCLUSION: Fracture type, mechanism of injury, and treatment modality in pediatric mandibular fractures are associated with distinct rates and types of adverse outcomes. Large scale studies characterizing these injuries are critical for guiding physicians in the management of these patients.

Social Determinants of Health in Early Otologic and Audiologic Evaluation in an Interdisciplinary Cleft-Craniofacial Clinic.

OBJECTIVE: Investigate associations between socioeconomic indicators of healthcare access with family compliance with cleft-related otologic and audiologic care within an interdisciplinary model. DESIGN: Retrospective case series. SUBJECTS AND SETTING: Children born 2005-2015 who presented to the Cleft-Craniofacial Clinic (CCC) at a quaternary care children's hospital. INTERVENTIONS: Associations between main outcome measures and Area Deprivation Index (ADI), median household income for zip code, distance from hospital, and insurance status were evaluated. MAIN OUTCOME MEASURES: Cleft types, ages at presentation to outpatient clinic (cleft, otolaryngology, and audiology), and ages at procedures (first tympanostomy tube insertion (TTI), lip repair, and palatoplasty) were measured. RESULTS: Most patients were male (147/230, 64%) with cleft lip and palate (157/230, 68%). Median age at first cleft, otolaryngology, and audiology visits were 7 days, 86 days, and 5.9 months, respectively. Private insurance predicted lower no-show rates (p = .04). Age at first CCC visit was younger for patients with private insurance (p = .04) and older for those who lived further from the hospital (p = .002). Age at lip repair was positively correlated with national ADI (p = .03). However, no socioeconomic status (SES) proxy or proximity to hospital was associated with delays in first otolaryngology or audiology examination or TTI. CONCLUSION: Once children become established within an interdisciplinary CCC, SES appears to bear little influence on cleft-related otologic and audiologic care. Future efforts should aim to elucidate which aspects of the interdisciplinary model maximize multisystem cleft care coordination and increase access for higher risk populations.

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.

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.

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.

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.

Review of Diet Protocols Following Orthognathic Surgery and Analysis of Postoperative Weight Loss.

INTRODUCTION: Orthognathic surgery is routinely practiced, yet little comparative data exists to evaluate post-orthognathic surgery diet protocols. OBJECTIVE: To evaluate which postoperative diet protocols are recommended and to quantify post-orthognathic surgery weight changes in our institutional cohort. METHODS: An internet search was carried out on Google for "orthognathic surgery diet" and the postoperative diet recommendations from centers worldwide were quantified. Additionally, a retrospective analysis of patients that underwent orthognathic surgery at our institution was performed, and their preoperative and postoperative weights were recorded. RESULTS: The internet search yielded 58 centers that met our inclusion criteria. Most centers were in the United States (n = 37, 63.8%) and were oral and maxillofacial surgeon (OMFS)-led centers (n = 39, 67.2%). Postoperative diets were categorized into 7 distinct protocols, ranging from most to least restrictive-the most popular was liquid diet for 2 to 4 weeks followed by soft diet for 2 to 6 weeks. There were no significant patterns observed across different geographical regions or specialties.In our institution, 135 patients were identified. Overall, there was an average maximum weight loss of 4.1 kg by week 4, followed by a gradual increase in weight. Linear regression analysis showed that patients with greater preoperative body mass index (BMI) lost more weight postoperatively than patients with lower BMI (R2 = 0.25, P < .001). CONCLUSION: There is a significant variability in recommended postoperative diets following orthognathic surgery. Following a moderately restrictive diet at our institution, patients returned to their preoperative weight after approximately 4 months.

Prevalence and Management of Laryngomalacia in Patients With Pierre Robin Sequence.

OBJECTIVE: To characterize the prevalence and presentation of laryngomalacia and efficacy of supraglottoplasty (SGP) in a cohort of patients with Pierre Robin Sequence (PRS). DESIGN: Retrospective cohort study. SETTING: Tertiary-care children's hospital. PATIENTS, PARTICIPANTS: Consecutive patients with PRS born between January 2010 and June 2018. MAIN OUTCOME MEASURES: Chart review included demographics, comorbid airway obstruction including laryngomalacia, timing of surgical interventions, clinical symptoms, sleep study data, and modified barium swallow study data.126 patients with PRS were included; 54% had an associated syndrome, 64% had an overt cleft palate, and 22% had a submucous cleft palate. 64/126 were noted to have laryngomalacia (51%). Patients with concurrent PRS and laryngomalacia were significantly more likely to have submucous cleft palate (P = .005) and present with aspiration with cough (P = .01) compared to patients with PRS without laryngomalacia. Patients with concurrent laryngomalacia and PRS showed a significant decrease in apnea-hypopnea index (AHI) and obstructive AHI (OAHI) after mandibular distraction, with a median AHI and OAHI improvement of 22.3 (P = .001) and 19.8 (P = .002), respectively. Patients who underwent only SGP did not show significant improvement in these parameters (P = .112 for AHI, P = .064 for OAHI).The prevalence of laryngomalacia in our PRS cohort was 51%. Patients with PRS and laryngomalacia are more likely to present with overt aspiration compared to patients with PRS without laryngomalacia. These data support that laryngomalacia does not appear to be a contraindication to pursuing MDO.

"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.

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.

Delayed Presentation of Unicoronal Craniosynostosis-Masked by Ipsilateral Posterior Deformational Plagiocephaly.

Despite a greater awareness of the differential diagnosis of head shape abnormalities among pediatricians, the effect of deformational forces on calvarial morphology can complicate the diagnosis of craniosynostosis. In this report, we describe 2 patients diagnosed with unicoronal craniosynostosis (UCS) in a delayed fashion due to the presence of concomitant posterior deformational plagiocephaly (PDP). In both cases, the severity of each patients' PDP obscured changes typically associated with UCS. This unique presentation underscores the importance of having a high index of suspicion for possible premature suture fusion despite the presence of concomitant PDP.