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 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.

Failure Rates Based on Alveolar Cleft Volume: An Analysis of the Critical-Sized Defect for Alveolar Bone Grafting.

BACKGROUND: Alveolar bone grafting (ABG) using iliac crest bone graft (ICBG) is best practice for children with complete cleft lip and palate. With the advent of recombinant human bone morphogenetic protein (rhBMP-2) and demineralized bone matrix (DBM), excellent results can still be achieved while avoiding donor-site morbidity. This study aims to determine the critical-sized defects by analyzing graft failure rates for ICBG and rhBMP-2/DBM to guide surgeons performing ABG. METHODS: A retrospective review was conducted evaluating patients who underwent ABG from 2016-2022. Patients with preoperative and postoperative cone beam computed tomography (CBCT) imaging were included. Volumetric defect sizes were calculated using preoperative imaging. Graft success criteria were based on both clinical and radiographic outcomes. Logistic regressions analyzed graft failure rates to identify an optimal cutoff, which defined the critical-sized defect. RESULTS: Ninety-three patients were included. Bone graft cohorts included ICBG (n=30) and rhBMP-2/DBM (n=63). The critical-sized defects were calculated to be 810 mm 3 and 885 mm 3 for ICBG and rhBMP-2/DBM, respectively. There were significantly higher graft failure rates beyond the critical size compared to below for both ICBG (71.4% vs. 0.0%; p<0.001) and rhBMP-2/DBM (65.0% vs. 14.0%; p<0.001). CONCLUSION: This study identified critical-sized defects based on alveolar cleft volume for ICBG or rhBMP-2/DBM with higher graft failure rates beyond the predicted thresholds. Distinct ranges in cleft volume were identified where patients might benefit from each select graft option.

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.

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.

A Calvarial Defect Model to Investigate the Osteogenic Potential of Umbilical Cord Stem Cells in Bone Regeneration.

BACKGROUND: The standard graft material for alveolar cleft repair (ACR) is autogenous iliac crest. A promising alternative potential graft adjunct-newborn human umbilical cord mesenchymal stem cells (h-UCMSCs)-has yet to be explored in vivo. Their capacity for self-renewal, multipotent differentiation, and proliferation allows h-UCMSCs to be harnessed for regenerative medicine. This study sought to evaluate the efficacy of using tissue-derived h-UCMSCs and their osteogenic capabilities to improve ACR in a murine model. METHODS: Foxn1 mice were separated into three groups with the following calvarial defects: no treatment (empty defect; n = 6), poly(D,L-lactide-co-glycolide) (PLGA) scaffold ( n = 6), or h-UCMSCs with PLGA ( n = 4). Bilateral 2-mm-diameter parietal bone critical-sized defects were created using a dental drill. Microcomputed tomography (microCT) imaging was performed 1, 2, 3, and 4 weeks postoperatively. The mice were euthanized 4 weeks postoperatively for RNAScope, immunohistochemical, and histological analysis. RESULTS: No mice experienced complications during the follow-up period. MicroCT imaging and histological analysis demonstrated that the no-treatment and PLGA-only defects remained patent without significant defect size differences across groups. In contrast, the h-UCMSCs with PLGA group had significantly greater bone fill on microCT and histological analysis. CONCLUSIONS: This study demonstrates a successful calvarial defect model for the investigation of h-UCMSC-mediated osteogenesis and bone repair. Evidence reveals that PLGA alone has neither short-term effects on bone formation nor any unwanted side effects, making it an attractive scaffold. Further investigation using h-UCMSCs with PLGA in larger animals is warranted to advance future translation to patients requiring ACR. CLINICAL RELEVANCE STATEMENT: The authors' results demonstrate a successful murine calvarial defect model for the investigation of h-UCMSC-mediated osteogenesis and bone repair, and they provide preliminary evidence for the safe and efficacious use of this graft adjunct in alveolar cleft repair.

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.

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.

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.

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.

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.

Fungating Malignancies: Management of a Distinct Wound Entity.

GENERAL PURPOSE: To provide information on the surgical management of fungating malignancies as a distinct wound entity. TARGET AUDIENCE: This continuing education activity is intended for physicians, physician assistants, nurse practitioners, and nurses with an interest in skin and wound care. LEARNING OBJECTIVES/OUTCOMES: After participating in this educational activity, the participant will:1. Identify characteristics of patients in a study examining the treatment of fungating malignancies.2. Select common symptoms experienced by patients with fungating malignancies.3. Explain issues related to the surgical treatment of fungating malignancies.4. Identify a reason why patients with fungating breast masses may avoid medical care.

To address the literature gap on malignant fungating wound treatment by reporting two institutions' experiences with this disease process and proposing practices to improve care. A multi-institutional retrospective review was conducted of 44 patients with 45 malignant fungating wounds over an 11-year period. Patient characteristics, treatment history, and outcomes were analyzed. Of the 44 patients who met the inclusion criteria, 31 (70.5%) were women and 13 (29.5%) were men. The average age at presentation was 63.0 (SD, 16.1) years. The most common malignancy was breast cancer, accounting for more than half of cases (54.5%). The average surface area of the tumors at presentation was 110.3 (SD, 215.0; range, 2.2­1,140) cm 2 , whereas the average surface area at time of discharge/death was 104.6 (SD, 310.7; range, 0­1,800) cm 2 . Neither surface area at presentation ( P = .504) nor surface area at time of final follow-up ( P = .472) were significantly associated with death during the study time frame. In the era of advancing technologies and medical innovation, the benefits of palliative surgery, which helps mitigate an open wound, should not be overlooked. Improving end-of-life care is beneficial to the patient and families alike. As surgeons, we strive for a tangible cure, but providing palliative resection to enable death with dignity might be the most humane service of all.

Premaxillary Setback in Bilateral Cleft Lip and Palate Repair.

OBJECTIVE: The aim of this study is to evaluate surgical outcomes and maxillofacial growth in patients undergoing primary lip repair with or without premaxillary setback. DESIGN: Retrospective review. SETTING: Children's Hospital of Los Angeles, California. PATIENTS AND PARTICIPANTS: Patients with bilateral cleft lip ± palate (BCLP) who underwent lip repair with or without premaxillary setback from January 1975 to September 2021. INTERVENTIONS: No intervention was performed. MAIN OUTCOME MEASURE(S): Patient demographics, comorbidities, and syndromic status were obtained. Indications for premaxillary setback, incidence of midface hypoplasia, orthodontic and/or orthognathic treatments, follow-up, complications, and revisions were recorded and analyzed. Comparisons among long-term outcomes, particularly the development of midface hypoplasia were made between groups. RESULTS: Thirty-one patients who underwent BCLP repair with premaxillary setback (BCLP + PS) and 31 matched control patients who underwent BCLP repair without premaxillary setback (BCLP - PS) were included. Among the 2 groups, multiple logistic regression demonstrated that when controlling for comorbidities, syndromic status, timing of lip repair, and timing of palate repair, premaxillary setback was neither significantly associated with the development of midface hypoplasia (P = .076) nor the timing of midface hypoplasia development (P = .940) in those that ultimately acquired this facial dysmorphology. CONCLUSIONS: While a high incidence of midface hypoplasia was seen in both BCLP ± PS and BCLP - PS, our findings demonstrate no difference in midface hypoplasia irrespective of premaxillary setback in the setting of BCLP. Future prospective studies investigating the downstream ramifications of our suggested selection criteria for premaxillary setback are warranted.

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.

A Unique Case of Aggressive Central Giant Cell Granuloma in a 10-Year-Old Boy With 16p13.11 Microdeletion Syndrome.

Central giant cell granuloma (CGCG) is a rare disease characterized by sporadic, benign, intraosseous mandibular lesions of unknown etiology. Histologically, these lesions are indistinguishable from brown tumors of hyperparathyroidism and cherubism, and occasionally have been associated with different syndromes raising a question for genetic etiology. The CGCG has varied presentation ranging from nonaggressive and indolent to aggressive, destructive, and recurrent, often posing diagnostic and therapeutic challenges. Herein, we present the first case of a 10-year-old boy with CGCG and 16p13.11 microdeletion syndrome, highlight the diagnostic challenges inherent to this heterogeneous disorder, and discuss the genetics and treatment approaches of these complex lesions.

Early Cleft Lip Repair: Demonstrating Efficacy in the First 100 Patients.

BACKGROUND: Cleft lip and/or palate affects approximately one in 700 live births. Optimal timing for repair of cleft lip has yet to be objectively validated. Earlier repair takes advantage of a high degree of plasticity within the nasal cartilage and maxilla. The authors present patients enrolled in an early cleft lip repair protocol facilitating effective repair of the cleft lip and nostril. METHODS: American Society of Anesthesiologists class I to II patients with unilateral cleft lip and/or palate undergoing repair before 3 months of age were enrolled over 5 years. Perioperative data, surgical and anesthetic complications, preoperative and postoperative nostril breadth, nostril width, nasal angle, lip length, frontal nasal breadth, and commissure length measured as ratios between the cleft and noncleft sides were abstracted. Early cleft lip repair and nasoalveolar molding patients were matched for cleft lip severity using the cleft width ratios and compared. RESULTS: The surgical and anesthetic complication rate for 100 early cleft lip repair patients was 2 percent. Operative and anesthetic times were 123 ± 37 minutes and 177 ± 34 minutes, respectively. Hospital length of stay was 1 ± 0 day. Age at repair between early cleft lip repair and nasoalveolar molding was 33 ± 15 days and 118 ± 33 days, respectively. After early cleft lip repair, preoperative to postoperative distance from symmetry for all anthropomorphic measurements improved ( p < 0.001). Comparing severity-matched early cleft lip repair to nasoalveolar molding patients, similar improvements were observed, suggesting equivalent results ( p > 0.05). CONCLUSION: Early cleft lip repair provides an efficacious method for correcting the cleft lip and nasal deformity that simulates nasoalveolar molding. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Syndromic Synostosis: Frontofacial Surgery.

Frontofacial surgery, encompassing the monobloc with or without facial bipartition and the box osteotomy, can treat the frontal bone and midface simultaneously, providing comprehensive improvement in facial balance. Complex pediatric patients with genetic syndromes and craniosynostosis are most optimized by an interdisciplinary team of surgeons, pediatricians, geneticists, speech pathologists, audiologists, dietitians, pediatric dentists, orthodontists, and psychosocial support staff to manage the myriad of challenges and complications throughout early childhood and beyond. Despite early treatment of the anterior and posterior cranial vault, these patients frequently have resultant frontal and/or midface hypoplasia and orbital abnormalities that are best managed with simultaneous surgical treatment.

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.

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.