ImageAssist: A Novel Smartphone Application for Standardized Clinical Photography That Implements and Automates American Society of Plastic Surgery/Plastic Surgery Foundation Photographic Guidelines With Background Deletion.

STATEMENT OF THE PROBLEM: Standardized medical photography of the face is a vital part of patient documentation, clinical evaluation, and scholarly dissemination. Because digital photography is a mainstay in clinical care, there is a critical need for an easy-to-use mobile device application that could assist users in taking a standardized clinical photograph. ImageAssist was developed to answer this need. The mobile application is integrated into the electronic medical record (EMR); it implements and automates American Society of Plastic Surgery/Plastic Surgery Research Foundation photographic guidelines with background deletion. INITIAL PRODUCT DEVELOPMENT: A team consisting of a craniofacial plastic surgeon and the Health Information Technology product group developed and implemented the pilot application of ImageAssist. The application launches directly from patients' chart in the mobile version of the EMR, EPIC Haiku (Verona, Wisconsin). Standard views of the face (90-degree, oblique left and right, front and basal view) were built into digital templates and are user selected. Red digital frames overlay the patients' face on the screen and turn green once standardized alignment is achieved, prompting the user to capture. The background is then digitally subtracted to a standard blue, and the photograph is not stored on the user's phone. EARLY USER EXPERIENCE: ImageAssist initial beta user group was limited to 13 providers across dermatology, ENT, and plastic surgery. A mix of physicians, advanced practice providers, and nurses was included to pilot the application in the outpatient clinic setting using Image Assist on their smart phone. After using the app, an internal survey was used to gain feedback on the user experience. In the first 2 years of use, 31 users have taken more than 3400 photographs in more than 800 clinical encounters. Since initial release, automated background deletion also has been functional for any anatomic area. CONCLUSIONS: ImageAssist is a novel smartphone application that standardizes clinical photography and integrated into the EMR, which could save both time and expense for clinicians seeking to take consistent clinical images. Future steps include continued refinement of current image capture functionality and development of a stand-alone mobile device application.

Severity of Mandibular Dysmorphology in Treacher Collins Syndrome for Stratification of Perioperative Airway Risk.

BACKGROUND: Patients with Treacher Collins syndrome (TCS) and attendant airway dysmorphology may be predisposed to airway complications in the perioperative period. However, limited data correlates severity of mandibular hypoplasia and airway status. This study aims to improve risk stratification for perioperative airway insufficiency in TCS by using a previously proposed mandibular severity index. METHODS: Patient demographics, perioperative airway status, difficulty of intubation, and Cormack Lehane grade were collected and compared using a TCS mandibular hypoplasia severity grading scale in patients with TCS treated between 2000 and 2022. RESULTS: Twenty-six patients underwent 222 procedures with institutional mandibular severity gradings as follows: 23% Grade I, 31% Grade II, 39% Grade III, 8% Grade IV. Our severity index was associated with intubation difficulty ( P <0.001) and difficult airway status ( P <0.001), with 72% of difficult airways found in grade III and grade IV patients. Mandibular retrusion and ramal hypoplasia subscores were positively correlated with measures of airway severity ( P <0.001), whereas the gonial angle was negatively correlated ( P <0.001). Age was negatively correlated with difficult visualization for endotracheal intubation ( P =0.02) but had no association with difficult airway status ( P =0.2). CONCLUSIONS: This study found a positive correlation between severity of maxillomandibular dysmorphology and perioperative airway difficulty in TCS patients. Our findings suggest that severely affected patients require heightened vigilance throughout life, as difficult airways may not completely resolve with aging. Given the risk of morbidity and mortality associated with airway complications, proper identification and preparation for challenging airways is critical for TCS patients.

Machine Learning for Automatic Detection of Velopharyngeal Dysfunction: A Preliminary Report.

BACKGROUND: Even after palatoplasty, the incidence of velopharyngeal dysfunction (VPD) can reach 30%; however, these estimates arise from high-income countries (HICs) where speech-language pathologists (SLP) are part of standardized cleft teams. The VPD burden in low- and middle-income countries (LMICs) is unknown. This study aims to develop a machine-learning model that can detect the presence of VPD using audio samples alone. METHODS: Case and control audio samples were obtained from institutional and publicly available sources. A machine-learning model was built using Python software. RESULTS: The initial 110 audio samples used to test and train the model were retested after format conversion and file deidentification. Each sample was tested 5 times yielding a precision of 100%. Sensitivity was 92.73% (95% CI: 82.41%-97.98%) and specificity was 98.18% (95% CI: 90.28%-99.95%). One hundred thirteen prospective samples, which had not yet interacted with the model, were then tested. Precision was again 100% with a sensitivity of 88.89% (95% CI: 78.44%-95.41%) and a specificity of 66% (95% CI: 51.23%-78.79%). DISCUSSION: VPD affects nearly 100% of patients with unrepaired overt soft palatal clefts and up to 30% of patients who have undergone palatoplasty. VPD can render patients unintelligible, thereby accruing significant psychosocial morbidity. The true burden of VPD in LMICs is unknown, and likely exceeds estimates from HICs. The ability to access a phone-based screening machine-learning model could expand access to diagnostic, and potentially therapeutic modalities for an innumerable amount of patients worldwide who suffer from VPD.

Sociodemographic Factors Predict Long-Term Attrition from Multidisciplinary Cleft Clinic.

OBJECTIVE: Identify and describe factors associated with retention and attrition of patients during longitudinal follow-up at multidisciplinary cleft clinic. DESIGN: Retrospective cohort study. SETTING: Single, tertiary care center. PATIENTS, PARTICIPANTS: Patients born between 1995 and 2007 with a diagnosis of cleft palate with or without cleft lip attending multidisciplinary cleft clinic. INTERVENTIONS: None tested, observational study. MAIN OUTCOME MEASURE(S): Age at last clinical appointment with a multidisciplinary cleft team provider. Attrition was defined as absence of an outpatient appointment following 15 years of age. RESULTS: Six hundred seventy-eight patients were included. The average age at last appointment across the entire cohort was 13.1 years (IQR 6.6-17.2). Patients who were Black (HR 1.60, 95% CI 1.10-2.32, p = 0.014) and other races (HR 1.90, 95% CI 1.22-2.98, p = 0.004) were more likely to be lost to follow-up compared to white patients. Publicly insured patients were more likely to experience attrition than those who were privately insured (HR 1.30, 95% CI 1.03-1.65, p = 0.030). Estimated income was not significantly associated with length of follow-up (p = 0.259). Those whose residence was in the fourth quartile of driving distance from our center experienced loss to follow-up significantly more than those who lived the closest (HR 2.04, 95% CI 1.50-2.78, p < 0.001). CONCLUSIONS: There is a high degree of follow-up attrition among patients with cleft lip and palate. Race, insurance status, and driving distance to our center were associated with attrition in a large, retrospective cohort of patients who have reached the age of cleft clinic graduation.

Incomes to Outcomes: A Global Assessment of Disparities in Cleft and Craniofacial Treatment.

OBJECTIVE: Recent investigations focused on health equity have enumerated widespread disparities in cleft and craniofacial care. This review introduces a structured framework to aggregate findings and direct future research. DESIGN: Systematic review was performed to identify studies assessing health disparities based on race/ethnicity, payor type, income, geography, and education in cleft and craniofacial surgery in high-income countries (HICs) and low/middle-income countries (LMICs). Case reports and systematic reviews were excluded. Meta-analysis was conducted using fixed-effect models for disparities described in three or more studies. SETTING: N/A. PATIENTS: Patients with cleft lip/palate, craniosynostosis, craniofacial syndromes, and craniofacial trauma. INTERVENTIONS: N/A. RESULTS: One hundred forty-seven articles were included (80% cleft, 20% craniofacial; 48% HIC-based). Studies in HICs predominantly described disparities (77%,) and in LMICs focused on reducing disparities (42%). Level II-IV evidence replicated delays in cleft repair, alveolar bone grafting, and cranial vault remodeling for non-White and publicly insured patients in HICs (Grades A-B). Grade B-D evidence from LMICs suggested efficacy of community-based speech therapy and remote patient navigation programs. Meta-analysis demonstrated that Black patients underwent craniosynostosis surgery 2.8 months later than White patients (P < .001) and were less likely to undergo minimally-invasive surgery (OR 0.36, P = .002). CONCLUSIONS: Delays in cleft and craniofacial surgical treatment are consistently identified with high-level evidence among non-White and publicly-insured families in HICs. Multiple tactics to facilitate patient access and adapt multi-disciplinary case in austere settings are reported from LMICs. Future efforts including those sharing tactics among HICs and LMICs hold promise to help mitigate barriers to care.

Psychosocial Status and Self-Perception in Patients with Cleft Lip and/or Palate.

OBJECTIVE: To describe how the psychosocial status of patients with cleft lip and/or palate (CL/P) relates to patient-reported outcomes (PROs). DESIGN: Cross-sectional retrospective chart review. SETTING: Tertiary care pediatric hospital. PATIENTS/PARTICIPANTS: Patients aged 8 to 29 years attending cleft team evaluations during a 1-year period. MAIN OUTCOME MEASURES: CLEFT-Q. RESULTS: Patients (N = 158) with isolated or syndromic CL/P and mean age 13.4 ± 3.0 years were included. Fifteen (9%) patients had siblings who also had CL/P. Of 104 patients who met with the team psychologist, psychosocial concerns were identified in 49 (47%) patients, including 25 (24%) with Attention-Deficit/Hyperactivity Disorder or behavior concerns, 28 (27%) with anxiety, and 14 (13%) with depression or mood concerns. Younger age and having siblings with cleft were associated with better PROs, while psychosocial concerns were associated with worse PROs on Speech, Psychosocial, and Face Appearance scales. CONCLUSIONS: Patient perception of cleft outcomes is linked to psychosocial factors.

Prior fronto-orbital advancement associated with complications from transcranial midface surgery in patients with syndromic craniosynostosis.

PURPOSE: Our center adopted posterior vault distraction osteogenesis (PVDO) as a first-line intervention for cranial expansion in syndromic craniosynostosis in 2008, and we have a growing cohort of patients undergoing transcranial midface advancement who have not had prior fronto-orbital advancement (FOA). The purpose of this study was to evaluate whether a history of FOA influences the risk profile of transcranial midface advancement in patients with syndromic craniosynostosis. METHODS: Patients undergoing transcranial fronto-facial advancement from 2000 to 2022 were retrospectively divided into cohorts based on preceding history of fronto-orbital advancement (FOA- and FOA+). Perioperative outcomes including operative time, length of stay, intraoperative dural injury, and complications (Clavien-Dindo score) were compared between groups with appropriate statistics. RESULTS: Thirty-eight patients were included (15 in FOA- group and 23 in FOA+ group). The overall complication rate was 47% (10% minor, 37% major). Compared to the FOA- group, the FOA+ group had a higher incidence of dural tears (65% v 20%, p = 0.006) and major complications (48% v 13%, p = 0.028). These findings were recapitulated in multivariate logistic regression controlling for other predictors. CONCLUSIONS: Prior FOA is associated with increased rates of major complications and dural tears in patients with syndromic craniosynostosis undergoing fronto-facial surgery. Options for cranial vault expansion that avoid the frontal region, such as PVDO, may favorably alter the risk profile of fronto-facial advancement.

Oxycephaly-systematic review, case presentation, and diagnostic clarification.

PURPOSE: Oxycephaly is a specific phenotype of multi-suture craniosynostosis that is often misrepresented. This study aims to review the relevant literature, clarify the diagnostic criteria, and present an alternate approach to its management. METHODS: Published literature regarding oxycephaly was reviewed from 1997, when the largest series was published, until 2022. All cases at a single institution were then retrospectively reviewed. RESULTS: Over the last 25 years, four studies met the inclusion criteria, none of which specifically defined oxycephaly. One case, in one study, was potentially consistent with the phenotype. An institutional review yielded two patients who met the original diagnostic criteria set forth by Renier and Marchac. Both patients had unexplained speech delays, mild retinal nerve fiber layer thickening, and diffuse inner table scalloping, along with the characteristic oxycephalic phenotype. One patient also had a direct intracranial pressure (ICP) measurement of 25 mmHg, and the other had a Chiari I malformation. Both were treated with posterior vault distraction osteogenesis (PVDO) to alleviate the cephalo-cranial disproportion while simultaneously allowing for turricephaly correction. CONCLUSIONS: Oxycephaly presents with late onset multi-suture fusion. Patients have patent sutures at birth. Midface hypoplasia and known syndromic associations are absent. Patients demonstrate supraorbital recession, an obtuse fronto-nasal angle, and turricephaly without substantial brachycephaly. Over 60% of patients have symptomatic ICP elevation, the presentation of which ranges from headaches to rapidly progressive blindness. This rare form of craniosynostosis is particularly virulent and likely often missed due to diagnostic ambiguity and its relatively mild phenotype.

Utilization of carbonated calcium phosphate cement for contouring cranioplasty in patients with syndromic craniosynostosis.

PURPOSE: Carbonated calcium phosphate (CCP) cement is an alloplastic material which has been increasingly utilized for cranioplasty reconstruction; however, there is a paucity of data investigating its use in patients with syndromic craniosynostosis. The purpose of this study was to characterize our institutional experience with CCP cement for secondary contouring cranioplasty in these patients to establish safety and aesthetic efficacy. METHODS: Patients with syndromic craniosynostosis undergoing cranioplasty with CCP cement from 2009 to 2022 were retrospectively reviewed for prior medical and surgical history, cranioplasty size, cement usage, and postoperative complications. Aesthetic ratings of the forehead region were quantified using the Whitaker scoring system at three timepoints: preoperative (T1), < 6 months postoperative (T2), and > 1 year postoperative (T3). RESULTS: Twenty-one patients were included. Age at surgery was 16.2 ± 2.8 years, forehead cranioplasty area was 135 ± 112 cm2, and mass of cement was 17.2 ± 7.8 g. Patients were followed for 3.0 ± 3.1 years. Whitaker scores decreased from 1.9 ± 0.4 at T1 to 1.4 ± 0.5 at T2 (p = 0.005). Whitaker scores at T2 and T3 were not significantly different (p = 0.720). Two infectious complications (9.5%) were noted, one at 4.5 months postoperatively and the other at 23 months, both requiring operative removal of CCP cement. CONCLUSION: Our results suggest that aesthetic forehead ratings improve after CCP contouring cranioplasty and that the improvement is sustained in medium-term follow-up. Complications were uncommon, suggesting that CCP is relatively safe though longer-term follow-up is needed before reaching definitive conclusions.

National Trends in Orthognathic Surgery: A Multi-Institutional Analysis of 6640 Patients.

BACKGROUND: LeFort I osteotomy (LF1) and bilateral sagittal split osteotomy (BSSO) have unique operative challenges and inherent anatomic considerations that predispose to certain adverse outcomes, yet their respective complication profiles continue to be debated given conflicting results from single-center investigations. The purpose of this study is to perform a multi-institutional analysis of complications, socioeconomic trends, and financial charges associated with orthognathic surgery. METHODS: A retrospective cohort study was conducted of orthognathic procedures performed in the United States from 2010 through 2020 using the Pediatric Health Information System. Patients younger than 12 years of age were excluded. Medical complications, surgical complications, and admission costs were compared across procedures. Socioeconomic determinants and trends across regions of the country were also analyzed. RESULTS: During the study interval, 6640 patients underwent orthognathic surgery, including LF1 (59.2%, n=3928), BSSO (14.4%, n=959), and double-jaw surgery (26.4%, n = 1753). Patients undergoing LF1 were more likely to experience overall complications ( P <0.001), infections ( P <0.001), and blood transfusions ( P <0.001) than those undergoing BSSO. High-volume hospitals were more likely to perform double-jaw procedures than other hospitals ( P <0.001), yet high-volume hospitals were less likely to have surgical complications ( P =0.014). Patient admission charges related to orthognathic operations at high-volume hospitals were less than other hospitals ( P <0.001). Household income was higher for orthognathic procedures performed at high-volume hospitals ( P <0.001). White patients were 1.5 times more likely to choose a farther, higher volume hospital for orthognathic surgery than the one locally available ( P =0.041). CONCLUSIONS: LeFort I osteotomy procedures had higher infection and transfusion rates than BSSO procedures. High-volume hospitals were more likely to perform double-jaw procedures, yet high-volume hospitals had fewer complications and decreased admission charges. Future study will be needed to further elucidate case-mix index details and socioeconomic determinants of health contributing to these disparities.

Associations Between Medicaid Expansion and Timely Repair of Cleft Lip and Palate.

The expansion of Medicaid under the Affordable Care Act (ACA) increased access to health care for many low-income children. However, the impact of this expansion on the timing of primary cleft lip and palate repair remains unclear. This study aimed to evaluate whether Medicaid expansion improved access to timely cleft lip and palate repair and whether it reduced preexisting ethnoracial disparities. Using a quasi-experimental design, the study analyzed data from 44 pediatric surgical centers across the United States. The results showed that Medicaid expansion was associated with a 9.0 percentage-point increase in delayed cleft lip repairs, resulting in an average delay of 16 days. Non-White patients were disproportionately affected by this delay, experiencing a 14.8 percentage-point increase compared with a 4.9 percentage-point increase for White patients. In contrast, Medicaid expansion had no significant effect on the timing of palate repair or on ethnoracial disparities in palate repair. The study underscores the importance of monitoring unintended consequences of large-scale health system changes, especially those affecting disadvantaged populations. Delayed cleft lip repair can lead to worse outcomes for patients, and the disproportionate impact on non-White patients is concerning. Further research is needed to identify the reasons for this delay and to mitigate its effects. Overall, the study highlights the need for ongoing vigilance to ensure that health care policies and interventions do not inadvertently worsen health disparities.

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.

Long-Term Patient-Reported Outcomes in Internationally Adopted Children with Cleft Lip and Palate.

OBJECTIVE: To compare patient-reported outcomes (PROs) in internationally adopted patients with cleft lip and palate to those in non-adopted peers. DESIGN: Cross-sectional study. SETTING: Multidisciplinary cleft team at tertiary care hospital. PATIENTS: Patients aged ≥ 8 with cleft lip and palate attending routine cleft team evaluations September 2021 - September 2022. MAIN OUTCOME MEASURE: CLEFT-Q PRO scores. RESULTS: Sixty-four internationally adopted patients and 113 non-adopted patients with a mean age of 13 years were included. Compared to non-adopted peers, adopted patients demonstrated worse satisfaction with face appearance (mean 59 vs. 66, p = .044), speech function (mean 69 vs. 78, p = .005), and speech distress (mean 80 vs. 84, p = .032). No significant differences were observed on the nose, nostrils, teeth, lips, lip scar, jaws, psychological function, or social function scales (p > .05). Objective clinical evaluation corroborated these findings, with adopted patients demonstrating worse Pittsburgh Weighted Speech scores (mean 3.0 vs 1.9, p = .027) and greater incidence of articulation errors (64% vs 46%, p = .021). No significant differences were observed in rates of mood, anxiety, or behavior concerns identified on psychosocial assessment (p = .764). Among adopted patients, undergoing palatoplasty prior to adoption was associated with worse satisfaction with speech, appearance, school, and social function (p < .05). CONCLUSIONS: Patient-reported outcomes among internationally adopted adolescents and young adults with cleft lip and palate show slightly lower satisfaction with facial appearance and speech but otherwise demonstrate similar results to non-adopted peers on most appearance and psychosocial measures. PRO data correlated well with objective speech assessment and did not portend worse psychosocial function.

Characterizing Interventions and Family Assistance of a Nurse Navigation Program in Orofacial Cleft Care.

OBJECTIVE: Characterize the interventions and assistance employed by a cleft nurse navigator (CNN) which have mediated improvement in care equity at our institution. DESIGN: Retrospective study. SETTING: Academic tertiary care center. PATIENTS, PARTICIPANTS: Patients presenting with cleft lip and/or cleft palate presenting between August 2020 and August 2021 with exclusions for syndromic diagnosis, Pierre-Robin sequence, late (> 6 months) presentation, and prior cleft surgery at outside institutions. INTERVENTIONS: Multidisciplinary cleft nurse navigator program. MAIN OUTCOME MEASURE(S): Family interactions with the CNN by phone, text, and email across the first year of life including feeding support, nasoalveolar molding (NAM) assistance, appointment scheduling, financial assistance, addressing perioperative concerns, and facilitating physician consults. Patient weight and surgical timing were also recorded. RESULTS: Sixty-nine patients were included with a total of 639 interactions between the CNN and families. Scheduling support (30%), addressing perioperative concerns (22%), and feeding support (20%) were the most common interactions. Feeding support and NAM assistance were heavily distributed in the first 3 months of life compared to after 3 months (P < .001). Median age at first contact was 1 week (range: 22 weeks gestation-14 weeks). There was no difference in the proportion of families receiving feeding support, NAM assistance, or scheduling assistance based on insurance status or race (P > .05 for all). CONCLUSIONS: Scheduling assistance, addressing perioperative concerns, and feeding support are the predominant methods by which the CNN interacts with and assists families of patients with cleft conditions. CNN service distribution is largely equitable between demographic groups.

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.

Management of an open nasofrontal encephalocele during the first day of life.

Nasofrontal encephaloceles are extremely rare craniofacial defects that present with herniation of cerebral tissue through the junction of the frontal and nasal bones. They often have a cutaneous covering which allows management to be delayed until early infancy, decreasing the risks of anesthesia and acute blood loss. Further bone development also facilitates cranial remodeling. Encephaloceles with a compromised cutaneous envelope pose an imminent threat to life due to the risk of meningitis, necessitating surgical repair in the first days of life. This report presents a patient born with an open nasofrontal encephalocele that underwent encephalocele excision, dural repair, and cranial remodeling on the first day of life. Anterior encephaloceles represent a spectrum of uncommon craniofacial dysraphias. Given the rarity of these diseases, it is important to understand their many presentations and the treatment options that exist for each. When the cutaneous covering of the encephalocele is compromised, surgical correction must be undertaken urgently to mitigate the mortality associated with neonatal meningitis. This represents the first report of an open nasofrontal encephalocele managed in the first day of life. A multidisciplinary surgical approach involving the neurosurgical and craniofacial surgical teams is paramount to the treatment and survival of such complicated patients.

Managing Bilateral Oro-Sino-Orbital Fistulae in the Setting of Bilateral Tessier IV Clefts.

ABSTRACT: The number IV orofacial cleft is extremely rare, and reports on long-term outcomes are limited. Here we present the management of a child born with bilateral Tessier number IV clefts and microphthalmia, who developed bilateral oro-sino-orbital fistulae. He presented 8 years after his index procedure with persistent ocular drainage and radiographic evidence of bilateral oro-sino-orbital fistulae. His globes maintained light perception, and in order to protect them from chemical reflux he underwent fistula obliteration with demineralized bone matrix and acellular dermal matrix. His orbits were reconstructed by Imre and conjunctival flaps. In the presence of marginally functioning globes, the importance of timely intervention is critical in order to preserve residual ocular function. Light perception vision assists in the maintenance of circadian rhythm and physiologic functions that are critical to the developing pediatric patient. This case also emphasizes the importance of a multi-disciplinary team in the correction of complex craniofacial deformities.

Resorbable Versus Titanium Hardware for Rigid Fixation of Pediatric Upper and Midfacial Fractures: Which Carries a Lower Risk Profile?

PURPOSE: Titanium associated risks have led to interest in resorbable hardware for open reduction and internal fixation (ORIF) of pediatric facial fractures. This study aims to systematically review and compare the outcomes of titanium/resorbable hardware used for ORIF of upper/midfacial fractures to determine which hardware carries a higher complication rate in the pediatric patient. METHODS: Studies published between 1990 and 2020 on the ORIF of pediatric upper/midfacial fractures were systematically reviewed. A retrospective institutional review was also conducted, and both arms were compiled for final analysis. The primary predictor value was the type of hardware used and the primary outcome was the presence of a complication. Fisher's exact test and 2-proportion 2-tailed z-test calculations were used to determine statistical significance, which was defined as a P value < .05. The low quality of published evidence precluded meta-analysis. RESULTS: Systematic review of 23 studies identified 659 patients, and 77 patients were identified in the institutional review. A total of 736 patients (299 resorbable, 437 titanium) were included in the final analysis. Total complication rate was 22.8%. The titanium group had a higher complication rate (27 vs 16.7%; P < .01), and more often underwent elective hardware removal (87.3 vs 0%, P < .01). In each hardware subgroup, the incidence of complications was analyzed by fracture site. In the titanium group, complication incidence was higher when treating maxillary fractures (32.8 vs 22.9%, P = .03). When comparing the 2 hardware groups by fracture site, maxillary fractures had a higher rate of complications when treated by titanium hardware compared with resorbable hardware (32.8 vs 18%, P < .01). CONCLUSIONS: Upper/midfacial pediatric fractures requiring ORIF, especially maxillary fractures, may be best treated with resorbable hardware. Additional hardware-specific outcomes data is encouraged.

Pediatric Orbital Entrapment: Radiographic Findings and Their Predictive Values.

INTRODUCTION: Although orbital fractures are less common in children than adults, the literature suggests that orbital entrapment occurs more frequently in the pediatric population. Orbital entrapment is an emergency, and early diagnosis and prompt management are critical. Specific radiographic manifestations such as herniation of extraocular muscles (EOMs) through orbital fractures have led to increased suspicion and overdiagnosis of orbital entrapment. This study evaluated the value of specific radiographic findings in predicting clinical findings after pediatric orbital floor fractures. METHODS: A retrospective review at a pediatric level 1 trauma center was performed. ICD-9 and ICD-10, codes identified patients with orbital fractures. Computed tomography (CT) reports were queried for the following terms: "herniation," "herniated," "entrapped," and "entrapment." Patients 16 years and older were excluded, as were all patients who were unable to undergo EOM evaluation on presentation. Electronic medical records were reviewed for fracture type, mechanism of injury, clinical and radiographic findings, surgical interventions, and demographic information. Medical diagnosis tests, including positive and negative predictive values, sensitivity, as well as specificity, were calculated to identify the relationship between radiographic findings and specific symptomology. RESULTS: One hundred fifty-three patients with 265 orbital fractures were included. "Entrapment" was mentioned in 34.6% of CT reports, whereas the incidence of clinical entrapment was 12.4% (19/153). Radiographic fat herniation, EOM herniation, and EOM irregularity had positive predictive values of 25%, 31%, and 10%, respectively, for clinical entrapment diagnosis at the initial examination. Overall, 32.7% (50/153) of patients with orbital fractures required surgery. CONCLUSIONS: Although the radiographic description of soft tissue herniation has become interchangeable with entrapment, these CT findings alone are poorly predictive. Orbital entrapment should be diagnosed preoperatively by physical examination, with radiographs as an adjunct only. Radiographic statements without clinical correlation may lead to the improper management of pediatric facial trauma patients.

Influence of Radiographic Soft Tissue Findings on Clinical Entrapment in Patients With Orbital Fractures.

INTRODUCTION: Radiographic assessment of facial fractures with computed tomography (CT) scanning has become standard of care. As imaging resolution has improved, findings such as herniation of extraocular muscles (EOM) have become a means of diagnosing conditions like orbital entrapment. However, the sensitivity and specificity of these findings has not been well-studied. We sought to evaluate the value of radiographic findings such as fat herniation, EOM contour irregularity, and EOM herniation in predicting orbital entrapment after orbital fracture. Secondary endpoints include diplopia, abnormal EOM motility, and the need for surgical fixation. METHODS: A single institution, retrospective review at a regional level 1 trauma center was conducted. Patients with orbital fractures were identified by International Classification of Disease (ICD) codes and CT reports were queried for the terms "herniation," "herniated," "entrapped," and "entrapment." Four hundred records were analyzed. RESULTS: Sixty-seven percent of radiology reports mentioned "entrapped" or "entrapment," while the incidence of clinical entrapment was 2.8%. The odds of entrapment, diplopia, and abnormal EOM motility were higher in those with EOM herniation; however, EOM herniation had a positive predictive value of 7.9% for clinical entrapment. Fat herniation alone and EOM contour irregularity had positive predictive values of 4.2% and 4.8%, respectively. CONCLUSIONS: While the odds of entrapment appear higher in patients with EOM herniation, this imaging finding is not predictive of clinical entrapment. Fat herniation and EOM contour irregularity did not have higher odds of entrapment, nor were they predictive. Diagnosis of orbital entrapment should be based primarily on physical exam, with CT as an adjunct only. The assumption that radiographic findings indicate orbital emergencies may result in unnecessary interfacility transfers, subspecialist consultations, and emergency operative procedures.