The Risk of Medication-Related Osteonecrosis of the Jaw in Children: Guidance for Antiresorptive Use in Pediatric Patients.

BACKGROUND: Antiresorptive targeted cancer therapies, such as denosumab and bisphosphonates, are used in adults, but their application in pediatric cancer is more recent. Side effects such as osteonecrosis of the jaw (ONJ) observed in adults have curtailed use of these medications in the pediatric population. PURPOSE: This study assesses the frequency of ONJ, other side effects, and the indications for use of denosumab versus bisphosphonates in pediatric subjects. STUDY DESIGN, SETTING, SAMPLE: A retrospective cohort study of pediatric subjects who underwent bisphosphonate or denosumab therapy at our institution from 2007-2023 was conducted. Subjects aged ≥ 18 years at therapy initiation were excluded. INDEPENDENT VARIABLE: The independent variable was antiresorptive therapy divided into 2 groups, treatment with intravenous bisphosphonates or denosumab. MAIN OUTCOME VARIABLE(S): Primary outcomes were development of bisphosphonate-related and denosumab-related ONJ. Secondary outcomes included additional side effects. COVARIATES: ONJ risk factors, subject demographics, indications for use, timing, duration, and cumulative dose of antiresorptive therapy were abstracted. ANALYSES: Univariate and bivariate statistics were computed to describe the sample and measure associations between antiresorptive therapy and outcomes. P values < .05 conferred statistical significance. RESULTS: The sample was composed of 178 subjects with a mean age of 11.7 ± 6.1 years. There were 14 (7.9%) and 164 (92.1%) subjects treated with denosumab and bisphosphonate therapies, respectively. There were 0 cases of ONJ across all subjects. The most common indication for treatment was adjuvant targeted therapy for aggressive tumors and malignancy (39.3%) followed by osteoporosis (14.6%). Subjects treated with denosumab had higher frequencies of hypercalcemia and severe bone pain than subjects treated with bisphosphonates, 28.6% versus 1.2% (P < .001) and 14.3% versus 0.00% (P < .001), respectively. CONCLUSION AND RELEVANCE: While invasive dental procedures are ideally performed before antiresorptive treatment, our data suggest that bisphosphonates may be used safely in the pediatric population with low concern for ONJ. Our data also demonstrated bisphosphonates may have a more tolerable side effect profile than denosumab. If the perceived benefits are similar, we recommend using bisphosphonates as first-line therapy in children while reserving denosumab for refractory cases. Future studies will help determine long-term side effects and differences in efficacies of these medications.

Comparing Outcomes of Traditional Lip Repair Versus Early Cleft Lip Repair on a National Scale.

BACKGROUND: Cleft lip (CL) is one of the most common congenital anomalies and has traditionally been repaired surgically when the patient is between 3 and 6 months of age. However, recent single-institutional studies have demonstrated the efficacy and safety of early CL repairs (ECLRs) during the neonatal period. This study seeks to evaluate the outcomes of ECLR (repair <1 month) versus traditional lip repair (TLR) by comparing outcomes on a national scale. METHODS: The American College of Surgeons National Surgical Quality Improvement Program Pediatric Date File was used to query patients who underwent CL repairs between 2012 and 2022. The main outcome measures were anesthesia times and perioperative complications. The main predictive variable was operative group (ECLR vs TLR). Patients were considered to be in the ECLR cohort if they were younger than 30 days after birth at the time of cleft repair. Student t test and χ2 analyses were used to evaluate categorical and continuous differences, respectively. Multiple logistic regression was performed to model the association of ECLR versus TLR with death within 30 days, overall complication rates, dehiscence rates, readmission within 30 days, and reoperation rates while controlling for various covariates. RESULTS: Multiple linear regression determined that the ECLR cohort had significantly shorter operative times when controlling for operative complications, sex, cardiac risk factors, and American Society of Anesthesiologists class (coefficient = -34.4; confidence interval, -47.8 to -20.9; P < 0.001). Similarly, multiple linear regression demonstrated ECLR patients to have significantly shorter time of exposure to anesthesia (coefficient = -35.0; 95% confidence interval, -50.3 to -19.7; P < 0.001). Multiple logistic regression demonstrated that ECLR was not significantly associated with an increased likelihood of any postoperative complication when controlling for sex, cardiac risk factors, and American Society of Anesthesiologists class (P = 0.26). CONCLUSIONS: The findings of this study provide nationwide evidence that ECLR does not lead to an increased risk of adverse outcomes or complications. In addition, ECLR patients have shorter surgeries and shorter exposure to anesthesia compared with TLR. The results provide further evidence that ECLR can be done safely where earlier intervention may result in better feeding/weight gain and subsequently improve cleft care. However, longer-term studies are warranted to further elucidate the effects of this protocol.

Custom Endoprosthesis-Assisted Pediatric Microsurgical Jaw Reconstruction.

Surgical treatment of pediatric maxillary and mandibular tumors can cause significant postresection disfigurement, mastication, and speech dysfunction. The need to restore form and function without compromising growth at the recipient and donor sites poses a particular reconstructive dilemma. This study evaluates outcomes of the custom endoprosthesis (CE) compared with noncustom reconstruction (NCR) and introduces an algorithm using CE to optimize available soft tissue reconstructive options. An Institutional Review Board-approved retrospective review of all patients undergoing maxillary or mandibular reconstruction between 2016 and 2022 was completed. The independent variable of interest was CE utilization. Primary outcomes of interest included hardware failure/removal or exposure, major complications, and revision surgeries. Covariates of interest included patient demographics, medical comorbidities, tumor size, and pathologic diagnosis. Statistical analyses including independent t test, χ2 analyses, and univariate/multivariate logistic regression were performed using RStudio version 4.2.1. Fifty-one patients (37 mandible and 14 maxilla) underwent CE or NCR. Of patients, 37% (n = 19) received CE. Of patients who underwent mandibular reconstruction, there were significantly lower rates of hardware exposure (14.3% versus 47.8%, P = 0.018), failure (7.1% versus 43.5%, P = 0.048), major complications (28.6% versus 78.2%, P = 0.008), and revisions (11.1% versus 50.0%, P = 0.002) in the CE cohort compared with the NCR cohort. The rates of hardware failure, exposure, major complications, and revisions did not significantly differ in maxillary reconstructions, however, CE successfully reconstructed significantly larger defects (179.5 versus 74.6 cm3, P = 0.020) than NCRs. Deviating from NCR, the authors propose an algorithm considering anatomical location, extent of resection, and patient age for soft tissue selection. This algorithm yielded improved mandibular reconstructive outcomes and no increase in complications rate in maxillary reconstruction despite larger resection defects. Furthermore, the authors' initial findings demonstrate that CE is a safe option for pediatric maxillary and mandibular reconstruction that may, in addition, facilitate improved form and function.

A National Analysis of Craniosynostosis Demographic and Surgical Trends Over a 10-Year Period.

The pathogenesis of craniosynostosis, characterized by the premature fusion of calvarial sutures, is multifaceted and often the result of an amalgamation of contributing factors. The current study seeks examine the possible contributors to craniosynostosis development and its surgical trends over time. A multicenter/national retrospective cohort study was conducted of patients who underwent surgical repair of craniosynostosis (n=11,279) between 2012 and 2021 identified in the American College of Surgeons National Surgical Quality Improvement Program Pediatric Data File. Main outcome measures included risk factors and trends relating to surgical repair of craniosynostosis. Nationwide reports of craniosynostosis in the NSQIP-P database have increased between 2012 and 2021 by 195%. The prevalence of craniosynostosis per overall cases has remained between 1.0% and 1.3%. There were predominantly more White male patients in the craniosynostosis cohort (P<0.001). Craniosynostosis patients had significantly greater birth weights, gestational ages, and were less likely to be premature (P<0.05). Linear regression demonstrated that operative time, anesthesia time, and length of stay significantly decreased over the study period (P<0.001). This national data analysis highlights trends in craniosynostosis repair indicating potential improvements in safety and patient outcomes over time. While these findings offer insights for health care professionals, caution is warranted in extrapolating beyond the data's scope. Future research should focus on diverse patient populations, compare outcomes across institutions, and employ prospective study designs to enhance the evidence base for craniosynostosis management. These efforts will help refine diagnostic and treatment strategies, potentially leading to better outcomes for patients.

A Review of Socioeconomic Disparities in Submucous Cleft Diagnosis and Outcomes.

Submucous cleft palate (SMCP) is a common congenital anomaly characterized by a diastasis of the levator veli palitini muscle. The subtlety of SMCP on physical examination can contribute to diagnostic delays. This study aims to analyze the factors contributing to delays in care and subsequent postoperative outcomes in patients with SMCP. All patients with surgical indications for SMCP who underwent palatoplasty at an urban academic children's hospital were included. Patient socioeconomic characteristics, medical history, and postoperative outcomes were collected. Patients were compared based on insurance type and government assistance utilization. Statistical analyses including independent t-test, Wilcoxon ranked sum test, χ2 analyses, Fisher's exact test, and stepwise logistic regression were performed. Among the 105 patients with SMCP, 69.5% (n=73) had public insurance and 30.5% (n=32) private. Patients with public insurance were diagnosed later (5.5±4.6 versus 2.6±2.4 years old; p<0.001) and underwent palatoplasty later (7.3±4.1 versus 4.4±3.4 years old; p<0.001) than those with private insurance. Patients receiving government assistance experienced higher rates of post-surgical persistent velopharyngeal insufficiency (74.5% versus 44.8%; p=0.006). The authors' results suggest a disparity in the recognition and treatment of surgical SMCP. Hence, financially vulnerable populations may experience an increased risk of inferior speech outcomes and subsequent therapies and procedures.

Dental Protraction Versus Surgery for Cleft Lip and Palate: A Budget Impact Analysis.

Class III malocclusion for individuals with cleft lip and palate has historically been managed with surgery. Orthodontic protraction is a noninvasive alternative that may be associated with lower costs. This analysis investigated the budget impact of protraction versus surgery from an institutional perspective. Using a decision tree, analysis was conducted using costs derived from Medicaid reimbursement codes and using actual institutional reimbursement. Probabilities of success, failure, and complications were based on a clinical trial comparing the 2 treatment modalities. One-way and probabilistic sensitivity analyses tested the robustness of results to model parameters. Based on Medicaid fee schedules and failure rates requiring additional surgery, the total cost of protraction was $79,506 versus $172,807 for surgery, resulting in $93,302 cost-savings per patient. The cost and probability of surgery success, as well as the cost of surgery failure and repeat surgery, had the largest impact on these cost-savings. Probabilistic sensitivity analysis showed cost-savings of nearly $92,000 or higher in >50% of simulations. This study showed that protraction is associated with lower costs than surgery and may present a cost-effective alternative to surgery in eligible, appropriate patients.

A Nationwide Analysis of the Impact of Cardiopulmonary Anomalies on Cleft Palate Surgical Outcomes.

OBJECTIVE: To increase awareness and improve perioperative care of patients with cleft palate (CP) and coexisting cardiopulmonary anomalies. DESIGN: Retrospective cohort. SETTING: Multi-center. PATIENTS/PARTICIPANTS: Patients who underwent surgical repair of CP between 2012-2020 identified in the American College of Surgeons National Surgical Quality Improvement Program Pediatric Data File. Chi-squared analysis and Student's t-test were implemented to make associations between congenital heart disease (CHD) and congenital pulmonary disease (CPD) and postoperative complications. Multiple logistic regression was performed to identify associations between CP and CHD/CPD while controlling for age, gender, and ASA class. C2 values were used to assess the logistic regressions, with a significance level of 0.05 indicating statistical significance. MAIN OUTCOMES MEASURES: Length of stay (LOS), perioperative complications (readmission, reoperation, reintubation, wound dehiscence, cerebrovascular accidents, and mortality). RESULTS: 9 96 181 patients were identified in the database, 17 786 of whom were determined to have CP, of whom 16.0% had congenital heart defects (CHD) and 13.2% had congenital pulmonary defects (CPD). Patients with CHD and CPD were at a significantly greater risk of increased LOS and all but one operative complication rate (wound dehiscence) relative to patients with CP without a history of CHD and CPD. CONCLUSION: This study suggests that congenital cardiopulmonary disease is associated with increased adverse outcomes in the setting of CP repair. Thus, heightened clinical suspicion for coexisting congenital anomalies in the presence of CP should prompt referring providers to perform a comprehensive and multidisciplinary evaluation to ensure cardiopulmonary optimization prior to surgical intervention.

Inpatient versus Outpatient Alveolar Bone Grafting: A Nationwide Cost Analysis.

OBJECTIVE: To compare postoperative outcomes and costs between inpatient and outpatient ABG in the United States. DESIGN: Retrospective cohort. SETTING: Multi-institutional/national. PATIENTS AND PARTICIPANTS: Patients who underwent ABG (n = 6649) were identified in the National Surgical Quality Improvement Program Pediatric database from 2012-2021. Inpatient and outpatient cohorts were matched using coarsened exact matching. MAIN OUTCOMES MEASURE(S): Thirty-day readmission, reoperation, and complications. A modified Markov model was developed to estimate the cost difference between cohorts. One-way and probabilistic sensitivity analyses were performed. RESULTS: After matching, 3718 patients were included, of which 1859 patients were in each hospital-setting cohort. The inpatient cohort had significantly higher rates of reoperations (0.6% vs. 0.2%; p = 0.032) and surgical site infections (0.8% vs. 0.2%; p = 0.018). The total cost of outpatient ABG was estimated to be $10,824 vs. $20,955 for inpatient ABG, resulting in $10,131 cost savings per patient. Probabilistic sensitivity analysis revealed that all 10,000 simulations resulted in consistent cost savings for the outpatient cohort that ranged from $8000 to $24,000. CONCLUSIONS: Outpatient ABG has become increasingly more popular over the past ten years, with a majority of cases being performed in the ambulatory setting. If deemed safe for the individual patient, outpatient ABG may confer a lower risk of nosocomial complications and offer significant cost savings to the healthcare economy.

A Single Institution 19 Year Comparison of Furlow and Straight Line Palatoplasty Techniques in Bilateral Cleft Lip and Palate.

OBJECTIVE: This study aims to compare patients' speech correcting surgery and fistula rates between the Furlow and Straight Line (SLR) palatoplasty techniques when combined with greater palatine flaps for complete bilateral cleft lip and palate (BCLP) repair. DESIGN: This was a single-center IRB approved retrospective cohort study. SETTING: This study took place at an urban tertiary academic center. PATIENTS, PARTICIPANTS: All patients with BCLP anomalies that underwent repair between January 2003 and August 2022 were included. Patients with index operations at an outside institution or incomplete medical charting were excluded. INTERVENTIONS: A total of 1552 patients underwent palatoplasty during the study period. Of these, 192 (12.4%) met inclusion criteria with a diagnosis of BCLP. MAIN OUTCOME MEASURES: Primary outcomes of this study included rate of fistula and incidence of speech correcting surgery. Secondary outcomes included rate of surgical fistula repair. RESULTS: One hundred patients underwent SLR (52.1%) and 92 Furlow repair (47.9%). There was no significant difference in fistula rates between the SLR and Furlow repair cohorts (20.7% vs. 15.0%; p = 0.403). However, SLR was associated with lower rates of speech correcting surgery when compared to the Furlow repair (12.5% vs. 29.6%; p = 0.011). CONCLUSIONS: This study compares the effect of Furlow and SLR on speech outcomes and fistula rates in patients with BCLP. Our findings suggest that SLR resulted in an almost three times lower rate of velopharyngeal dysfunction requiring surgical intervention in patients with BCLP, while fistula rates remained similar.

The Forgotten Flap: The Pedicled Trapezius Flap's Utility in Pediatric Head and Neck Reconstruction-A Systematic Review.

BACKGROUND: When free tissue transfer is precluded or undesired, the pedicled trapezius flap is a viable alternative for adults requiring complex head and neck (H&N) defect reconstruction. However, the application of this flap in pediatric reconstruction is underexplored. This systematic review aimed to describe the use of the pedicled trapezius flap and investigate its efficacy in pediatric H&N reconstruction. METHODS: A systematic review was performed using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Articles describing the trapezius flap for H&N reconstruction in pediatric patients were included. Patient demographics, surgical indications, wound characteristics, flap characteristics, complications, and functional outcomes were abstracted. RESULTS: A systematic review identified 22 articles for inclusion. Studies mainly consisted of case reports (n = 11) and case series (n = 8). In total, 67 pedicled trapezius flaps were successfully performed for H&N reconstruction in 63 patients. The most common surgical indications included burn scar contractures (n = 46, 73.0%) and chronic wounds secondary to H&N masses (n = 9, 14.3%). Defects were most commonly located in the neck (n = 28, 41.8%). The mean flap area and arc of rotation were 326.4 ± 241.7 cm2 and 157.6 ± 33.2 degrees, respectively. Most flaps were myocutaneous (n = 48, 71.6%) and based on the dorsal scapular artery (n = 32, 47.8%). Complications occurred in 10 (14.9%) flaps. The flap's survival rate was 100% (n = 67). No instances of functional donor site morbidity were reported. The mean follow-up was 2.2 ± 1.8 years. CONCLUSION: This systematic review demonstrated the reliability of the pedicled trapezius flap in pediatric H&N reconstruction, with a low complication rate, no reports of functional donor site morbidity, and a 100% flap survival rate. The flap's substantial surface area, bulk, and arc of rotation contribute to its efficacy in covering soft tissue defects ranging from the proximal neck to the vertex of the scalp. The pedicled trapezius flap is a viable option for pediatric H&N reconstruction.

Does Early Referral Lead to Early Repair? Quality Improvement in Cleft Care.

BACKGROUND: Cleft lip and palate is the most common congenital defect of the head and neck, occurring in 1 of 700 live births. Diagnosis often occurs in utero by conventional or 3-dimensional ultrasound. Early cleft lip repair (ECLR) (<3 months of life) for unilateral cleft lip (UCL), regardless of cleft width, has been the mainstay of lip reconstruction at Children's Hospital Los Angeles since 2015. Historically, traditional lip repair (TLR) was performed at 3 to 6 months of life ± preoperative nasoalveolar molding (NAM). Previous publications highlight the benefits of ECLR, such as enhanced aesthetic outcomes, decreased revision rate, better weight gain, increased alveolar cleft approximation, cost savings of NAM, and improved parent satisfaction. Occasionally, parents are referred for prenatal consultations to discuss ECLR. This study evaluates timing of cleft diagnosis, preoperative surgical consultation, and referral patterns to validate whether prenatal diagnosis and prenatal consultation lead to ECLR. METHODS: Retrospective review evaluated patients who underwent ECLR versus TLR ± NAM from 2009 to 2020. Timing of repair, cleft diagnosis, and surgical consultation, as well as referral patterns, were abstracted. Inclusion criteria dictated: age < 3 months for ECLR or 3 to 6 months for TLR, no major comorbidities, and diagnosis of UCL without palatal involvement. Patients with bilateral cleft lip or craniofacial syndromes were excluded. RESULTS: Of 107 patients, 51 (47.7%) underwent ECLR whereas 56 underwent TLR (52.3%). Average age at surgery was 31.8 days of life for the ECLR cohort and 112 days of life for the TLR cohort. Furthermore, 70.1% of patients were diagnosed prenatally, yet only 5.6% of families had prenatal consults for lip repair, 100% of which underwent ECLR. Most patients were referred by pediatricians (72.9%). Significance was identified between incidence of prenatal consults and ECLR (P = 0.008). In addition, prenatal diagnosis was significantly correlated with incidence of ECLR (P = 0.027). CONCLUSIONS: Our data demonstrate significance between prenatal diagnosis of UCL and prenatal surgical consultation with incidence of ECLR. Accordingly, we advocate for education to referring providers about ECLR and the potential for prenatal surgical consultation in the hopes that families may enjoy the myriad benefits of ECLR.

Rethinking the Rule of 10s: Early Cleft Lip Repair Improves Weight Gain.

OBJECTIVE: This study compares patients undergoing early cleft lip repair (ECLR) (<3-months) and traditional lip repair (TLR) (3-6 months) with/without nasoalveolar molding (NAM) to evaluate the effects of surgical timing on weight gain in hopes of guiding future treatment paradigms. DESIGN: Retrospective review. SETTING: Children's Hospital of Los Angeles, California. PATIENT, PARTICIPANTS: A retrospective chart review evaluated patients who underwent ECLR or TLR ± NAM from November 2009 through January 2020. INTERVENTIONS: No intervention was performed. MAIN OUTCOME MEASURE(S): Patient demographics, birth and medical history, perioperative variables, and complications were collected. Infant weights and age-based percentiles were recorded at birth, surgery, 8-weeks, 6-months, 12-months, and 24-months postoperatively. The main outcomes were weight change and weight percentile amongst ECLR and TLR ± NAM groups. RESULTS: 107 patients met inclusion criteria: ECLR, n = 51 (47.6%); TLR + NAM, n = 35 (32.7%); and TLR-NAM, n = 21 (19.6%). ECLR patients had significantly greater changes in weight from surgery to 8-weeks and from surgery to 24-months postoperatively compared with both TLR ± NAM (P < .05). Age-matched weights in the ECLR group were significantly greater than TLR ± NAM at multiple time points postoperatively (P < .05). CONCLUSIONS: ECLR significantly increased patient weights 24-months postoperatively when compared to TLR ± NAM. Specifically compared to TLR-NAM, ECLR weights were significantly greater at all time points past 6-months postoperatively. The results of this study demonstrate that ECLR can mitigate feeding difficulties and malnutrition traditionally seen in patients with cleft lip.

Tympanostomy Tubes: Are They Necessary? A Systematic Review on Implementation in Cleft Care.

OBJECTIVE: To understand the indication for and the effects of early ventilation tube insertion (VTI) on hearing and speech for patients with cleft lip and/or palate (CLP). DESIGN: We conducted a Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA)-guided systematic review of relevant literature. SETTING: Setting varied by geographical location and level of clinical care across studies. PATIENTS, PARTICIPANTS: Patients with CLP who underwent VTI were included. INTERVENTIONS: No interventions were performed. MAIN OUTCOME MEASURE(S): Primary outcome measures were hearing and speech following VTI. Secondary outcome measures were tube-related and middle ear complications. Early VTI occurred before or at time of palatoplasty while late VTI occurred after palatoplasty. RESULTS: Twenty-three articles met inclusion criteria. Articles varied among study design, outcome measures, sample size, follow-up, and quality. Few studies demonstrated support for early VTI. Many studies reported no difference in hearing or speech between early and late VTI. Others reported worse outcomes, greater likelihood of complications, or needing repeat VTI following early tympanostomy placement. Several studies had significant limitations, including confounding variables, small sample size, or not reporting on our primary outcome. CONCLUSIONS: No consistency was found regarding which patients would benefit most from early VTI. Given the aforementioned variability and sub-optimal methodologies, additional studies are warranted to provide stronger evidence regarding VTI timing in cleft care.

Lessons Learned from a Single Institution's Eight Years of Experience with Early Cleft Lip Repair.

Background and Objectives: The traditional approach in managing wide cleft lip deformities involves presurgical nasoalveolar molding (NAM) therapy followed by surgical cleft lip repair between three and six months of age. This institution has implemented an early cleft lip repair (ECLR) protocol where infants undergo primary cleft lip repair between two and five weeks of age without NAM. This study aims to present this institution's ECLR repair protocol over the past eight years from 188 consecutive patients with unilateral or bilateral CL/P deformity. Materials and Methods: Retrospective review was conducted at Children's Hospital Los Angeles evaluating patients who underwent ECLR before three months of age and were classified as American Society of Anesthesiologists (ASA) class I or II from 2015-2022. Anthropometric analysis was performed, and pre- and postoperative photographs were evaluated to assess nasal and lip symmetry. Results: The average age at cleft lip repair after correcting for gestational age was 1.0 ± 0.5 months. Mean operative and anesthetic times were 120.3 ± 33.0 min and 189.4 ± 35.4, respectively. Only 2.1% (4/188) of patients had postoperative complications. Lip revision rates were 11.4% (20/175) and 15.4% (2/13) for unilateral and bilateral repairs, respectively, most of which were minor in severity (16/22, 72.7%). Postoperative anthropometric measurements demonstrated significant improvements in nasal and lip symmetry (p < 0.001). Conclusions: This analysis demonstrates the safety and efficacy of ECLR in correcting all unilateral cleft lip and nasal deformities of patients who were ASA classes I or II. At this institution, ECLR has minimized the need for NAM, which is now reserved for patients with bilateral cleft lip, late presentation, or comorbidities that preclude them from early repair. ECLR serves as a valuable option for patients with a wide range of cleft severity while reducing the burden of care.

Increased Distraction Magnitude Leads to Greater Reduction in the Apnea-Hypopnea Index in Infants With Robin Sequence.

PURPOSE: Upper airway obstruction seen in Robin Sequence (RS) is commonly treated with mandibular distraction osteogenesis (MDO). The purpose of this study is to evaluate the impact of distraction distance on sleep study outcomes in patients with obstructive sleep apnea (OSA) secondary to RS. METHODS: A retrospective cohort study was conducted for patients with isolated RS who underwent MDO at Children's Hospital Los Angeles between January 2006-September 2021. The predictor variable was distraction distance (maximal distraction using a 30 mm device vs sub-maximal distraction), and the primary outcome variable was OSA scores. Relationships between covariates, including demographic characteristics, preoperative sleep variables, and postoperative OSA outcomes using polysomnography, were also analyzed. Descriptive statistics and tests of statistical significance were performed using the Statistical Package for Social Sciences (SPSS) (version 28.0), including Student's t-test, proportions testing, multiple linear regression, and correlation analysis. RESULTS: Seventy-one patients met inclusion criteria (39.4% female, 60.6% male). Average age at MDO was 3.0 ± 10.2 months. Fifty-six patients were distracted maximally with a 30 mm distractor, while the remaining 15 patients experienced shorter distraction due to distractor limitations (25 mm distractor), persistent infection or family request. Looking at absolute values of postoperative sleep study variables, there were no significant differences between patients who were maximally and sub-maximally distracted across apnea-hypopnea index (AHI), highest carbon dioxide, lowest oxygen saturation, and oxygen requirement. However, both cohorts demonstrated significant improvements in lowest oxygen saturation, AHI, highest carbon dioxide level, and highest oxygen requirement compared to their pre-distraction levels. Compared with patients distracted <30 mm, maximal distraction had a significantly greater improvement in AHI when controlling for preoperative sleep study variables (P = .047). CONCLUSION: Patients with isolated RS who have more severe OSA experienced greater improvements in AHI, oxygen requirement, and oxygen saturation after MDO. Two-thirds of patients no longer had oxygen requirements after MDO. Our results suggest that MDO is helpful in treating patients with RS regardless of distraction distance. However, our study provides evidence that increasing the distraction distance may further improve AHI, which is particularly beneficial to patients with a significant preoperative AHI.

Establishing a Novel Treatment Algorithm for Pediatric Mandibular Tumor Reconstruction.

INTRODUCTION: Guidelines for pediatric mandibular reconstruction (PMR) are not well-established. One must consider the growing craniofacial skeleton, mixed dentition, long-term dental occlusion, need for secondary reconstruction, and speech development. The traditional guideline (bone defect > 5 cm) for use of vascularized bone grafts (VBG) is not applicable given the variation of pediatric mandibular size and growth. We seek to propose a novel algorithm for PMR. MATERIALS AND METHODS: An Institutional Review Board approved retrospective review of patients who underwent PMR for tumor resections between 2005 and 2019 evaluated patients' demographics, complications, resection index (RI) (resection length to mandibular length), and surgical outcomes. Outcomes based on RI were analyzed to establish guidelines for VBG utilization. RESULTS: Twenty-four patients underwent PMR at a mean age of 9.1 years (range: 1 - 18). The mandibular defect (mean± standard deviation) fornon-VBG (n  = 18) and VBG (n  = 6) was 6.6 ±â€Š3.0 cm and 12.8 ±â€Š4.3 cm, respectively. The VBG group had fewer return trips to the operating room (P  = 0.028) and fewer major complications (P = 0.028). When non-VBG with RI > 32% were compared to <32%, there was statistically less returns to the operating room for complications and a lower rate of early (< 30 days) major complications. DISCUSSION: Our algorithm proposes an RI cutoff of 32% for VBG use for PMR. Patients with a sizable soft tissue defect, previous chemotherapy and/or radiation, planned adjuvant chemotherapy and/or radiation therapy, or a history of failed non-VBG should undergo reconstruction using VBG.

Meeting in the middle: pediatric abdominal wall reconstruction for omphalocele.

BACKGROUND: Omphalocele is a congenital abdominal wall defect with an incidence of 1/4,200 births. Repair timing varies from the neonatal period to the first few years of life. Surgical technique has changed over the last two decades. We sought to establish improved surgical/ventilation protocols for patients with omphaloceles requiring abdominal reconstruction. METHODS: An IRB-approved retrospective review was performed on patients with omphalocele requiring abdominal wall reconstruction by Plastics and/or Pediatric Surgery at a pediatric tertiary-care referral center (January 2006-July 2021). Birth history, comorbidities, surgical details, ventilation data, complications/recurrence were extracted. RESULTS: Of 129 patients screened, seven required Plastic Surgery involvement. Defect size was 102.9 cm2 (range: 24-178.5); five patients required component separation; zero patients received mesh; zero complications/recurrences were recorded. Two patients required postoperative ventilation for 2.5 days, based on increased peak inspiratory pressures at surgery stop versus start time. CONCLUSION: Patients with large defects secondary to omphalocele benefit from collaboration between Pediatric and Plastic Surgery for component separation and primary fascial closure without mesh. Future research should follow patients who mature out of pediatric clinics to evaluate the incidence of hernias in adults with Plastic Surgery-repaired omphaloceles.

Orthognathic Surgery Rate in Cleft Care.

PURPOSE: To determine the true need for orthognathic surgery in patients with repaired cleft lip and/or palate (CL/P) at a high-volume craniofacial center. METHODS: An institutional retrospective review of patients with CL/P born between 1975 and 2008 was performed. Patients with adequate documentation reflecting cleft care who were ≥ 18 years at the time of last craniofacial/dentistry follow-up were included. Patients with non-paramedian clefts or a comorbid craniofacial syndrome were excluded. Primary outcome variable was the total proportion of patients with CL/P who either underwent or were referred for orthognathic surgery Le Fort I (LF1) to correct midface hypoplasia. Secondary outcome variables were associations between cleft phenotype, midface hypoplasia severity, and number of cleft related surgeries with the eventual LF1 referral/recipiency. RESULTS: One hundred seventy-seven patients with CL/P met inclusion criteria. A total of 90/177 (51%) patients underwent corrective LF1; however, 110/177 (62%) of patients were referred for surgery. Patients with secondary cleft palate involvement were referred for and underwent LF1 at significantly greater rates than those without secondary palate involvement (referred: 65% versus 13%, P = 0.001; underwent: 55% versus 0%, P < 0.001). Patients with bilateral cleft lip/palate were referred for and underwent LF1 at significantly higher rates than those with unilateral cleft lip/palate (referred: 71.0% versus 50.4%, P= 0.04; underwent: 84% versus 71%, P = 0.02). Number of secondary palate surgeries was positively correlated with increased LF1 referral (P = 0.02) but not LF1 recipiency (P = 0.15). CONCLUSIONS: The incidence of orthognathic surgery redundant in patients with repaired CL/P was 51% at our institution, marginally above the higher end of previously reported rates. However, this number is an underrepresentation of the true requirement for LF1 as 62% of patients were referred for surgical intervention of midface hypoplasia. This distinction should be considered when counseling families.

Pediatric Odontogenic and Maxillofacial Bone Pathology: A Global Analysis.

BACKGROUND: Although pathology in the maxillary and mandibular bones is rare in young patients, the differential diagnosis is broad. The World Health Organization (WHO) updated its classification of maxillofacial bone pathology in 2017. Using these updated guidelines, a systematic review of common maxillofacial bone lesions in the pediatric population was performed. METHODS: A PubMed search was conducted capturing English language articles from inception to July 2020. Thirty-one articles were identified that described the frequency of maxillofacial bone pathology. Data were extracted and organized using the WHO 2017 classification of odontogenic and maxillofacial bone tumors. Prevalence data were analyzed among diagnostic categories and geographical regions. The SAS version 9.4 was used to complete statistical analyses. RESULTS: The articles included patients from birth to a maximum age of 14 to 19 years. The most common odontogenic cysts included radicular cyst (42.7%) and dentigerous cyst (39.0%) followed by odontogenic keratocyst (15.0%). Among odontogenic bone tumors, odontoma (49.3%) was most common followed by ameloblastoma (29.1%). The most common nonodontogenic bone tumor was fibrous dysplasia (42.4%), and the most common malignant bone tumor was osteosarcoma (75.0%). Significant variations were found by geographic region, with dentigerous cyst more common than radicular cyst, and ameloblastoma more common than odontoma in African and Asian countries (P < 0.0001). CONCLUSIONS: This systematic review uses the WHO 2017 guidelines to classify common odontogenic and nonodontogenic maxillofacial bone lesions around the world. Pathogenesis, presentation, and available treatment options for the most common maxillofacial bone lesions are reviewed.

Midface Growth Potential in Unoperated Clefts: A Systematic Review and Meta-Analysis.

ABSTRACT: Competing hypotheses for the development of midface hypoplasia in patients with cleft lip and palate include both theories of an intrinsic restricted growth potential of the midface and extrinsic surgical disruption of maxillary growth centers and scar growth restriction secondary to palatoplasty. The following meta-analysis aims to better understand the intrinsic growth potential of the midface in a patient with cleft lip and palate unaffected by surgical correction. A systematic review of studies reporting cephalometric measurements in patients with unoperated and operated unilateral cleft lip and palate (UCLP), bilateral cleft lip and palate (BCLP), and isolated cleft palate (iCP) abstracted SNA and ANB angles, age at cephalometric analysis, syndromic diagnosis, and patient demographics. Age and Region-matched controls without cleft palate were used for comparison. SNA angle for unoperated UCLP (84.5 ±â€Š4.0°), BCLP (85.3 ±â€Š2.8°), and ICP (79.2 ±â€Š4.2°) were statistically different than controls (82.4 ±â€Š3.5°), (all P ≤ 0.001). SNA angles for operated UCLP (76.2 ±â€Š4.2°), BCLP (79.8 ±â€Š3.6°), and ICP (79.0 ±â€Š4.3°) groups were statistically smaller than controls (all P ≤ 0.001). SNA angle in unoperated ICP (n = 143) was equivalent to operated ICP patients (79.2 ±â€Š4.2° versus 79.0 ±â€Š4.3° P = 0.78). No unoperated group mean SNA met criteria for midface hypoplasia (SNA < 80). Unoperated UCLP/BLCP exhibit a more robust growth potential of the maxilla, whereas operated patients demonstrate stunted growth compared to normal phenotype. Unoperated ICP demonstrates restricted growth in both operated and unoperated patients. As such, patients with UCLP/BCLP differ from patients with ICP and the factors affecting midface growth may differ.Level of Evidence: IV.