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 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.

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.

Safety and Sustainability: Optimizing Outcomes and Changing Paradigms in Global Health Endeavors.

Background: The need to address inequities in global surgical care has garnered increased attention since 2015, after the Lancet Commission on Global Surgery underscored the importance of ensuring safe, accessible, affordable, and timely surgical and anesthetic care. The vast unmet global plastic surgery needs make plastic surgery care essential in reducing the global burden of disease. In the past, many nonprofit organizations undertook humanitarian activities within low- and middle-income countries that were primarily service-provision oriented. The Lancet Commission on Global Surgery report prompted a shift in focus from direct patient care models to sustainable global surgical models. The realization that 33% of deaths worldwide were due to unmet surgical needs led to a global shift of strategy toward the development of local systems, surgical capacity, and a focus on patient safety and quality of care within international global surgery partnerships. Methods: In this report, the authors explore some of the primary components of sustainable international global surgical partnerships discussed in a recent panel at the American Society of Plastic Surgeons Plastic Surgery The Meeting 2022, titled "Safety and Sustainability Overseas: Optimizing Outcomes and Changing Paradigms in Global Health Endeavors." A literature review elaborating the topics discussed was performed. Results: This report focuses on cultural competence and humility, international collaboration, and the use of technology and innovation, all of which are needed to promote sustainability and patient safety, within global surgery efforts. Conclusions: The adoption of these components into international surgical collaborations will lead to greatly enhancing the development and sustainability of mutually beneficial relationships.

Systematic Review of Postoperative Velopharyngeal Insufficiency: Incidence and Association With Palatoplasty Timing and Technique.

Cleft palate is among the most common congenital disorders worldwide and is correctable through surgical intervention. Sub-optimal surgical results may cause velopharyngeal insufficiency (VPI). When symptomatic, VPI can cause hypernasal or unintelligible speech. The postoperative risk of VPI varies significantly in the literature but may be attributed to differences in study size, cleft type, surgical technique, and operative age. To identify the potential impact of these factors, a systematic review was conducted to examine the risk of VPI after primary palatoplasty, accounting for operative age and surgical technique. A search of PubMed, Embase, and Web of Science was completed for original studies that examined speech outcomes after primary palatoplasty. The search identified 4740 original articles and included 35 studies that reported mean age at palatoplasty and VPI-related outcomes. The studies included 10,795 patients with a weighted mean operative age of 15.7 months (range: 3.1-182.9 mo), and 20% (n=2186) had signs of postoperative VPI. Because of the heterogeneity in reporting of surgical technique across studies, small sample sizes, and a lack of statistical power, an analysis of the VPI risk per procedure type and timing was not possible. A lack of data and variable consensus limits our understanding of optimal timing and techniques to reduce VPI occurrence. This paper presents a call-to-action to generate: (1) high-quality research from thoughtfully designed studies; (2) greater global representation; and (3) global consensus informed by high-quality data, to make recommendations on optimal technique and timing for primary palatoplasty to reduce VPI.

The Personal Impact of Involvement in International Global Health Outreach: A National Survey of Former Operation Smile Student Volunteers.

BACKGROUND: Humanitarian surgical organizations such as Operation Smile provide global health opportunities for students and medical trainees. Prior studies have shown a positive benefit for medical trainees. This study aimed to determine if the international global health experiences of young student volunteers impact their career choices as adults. METHODS: A survey was sent to adults who were involved with Operation Smile as students. The survey elicited information about their mission trip experience, education, career, and current volunteer and leadership activities. Data were summarized with descriptive statistics and qualitative analysis. RESULTS: 114 prior volunteers responded. The majority participated in leadership conferences (n = 110), mission trips (n = 109), and student clubs (n = 101) while in high school. Many graduated from college (n = 113, 99%) and completed post-graduate degrees (n = 47, 41%). The most highly represented occupational industry was healthcare (n = 30, 26%), including physicians and medical trainees (n = 9), dentists (n = 5), and other healthcare providers (n = 5). Three-fourths reported that their volunteer experience impacted their career choice, and half reported that their experience allowed them to connect with career mentors. Their experience was associated with the development of leadership skills, including public speaking, self-confidence, and empathy, and increased awareness of cleft conditions, health disparities, and other cultures. Ninety-six percent continued to volunteer. Narrative responses revealed that the volunteer experiences impacted their inter- and intrapersonal development into adulthood. CONCLUSIONS: Participation in a global health organization as a student may encourage a long-term commitment to leadership and volunteerism and foster interest in a healthcare career. These opportunities also encourage development of cultural competency and interpersonal skills. LEVEL OF EVIDENCE: III, Cross-Sectional Study.

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.

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.

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.

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.

Cleft Palate Repair: A History of Techniques and Variations.

Orofacial clefting is a common reconstructive surgical condition that often involves the palate. Cleft palate repair has evolved over three centuries from merely achieving anatomical closure to prioritizing speech development and avoiding midface hypoplasia. Despite centuries of advancements, there is still substantial controversy and variable consensus on technique, timing, and sequence of cleft palate repair procedures. Furthermore, evaluating the success of various techniques is hindered by a lack of universal outcome metrics and difficulty maintaining long-term follow-up. This article presents the current controversies of cleft palate repair and details how the history of cleft palate repair has influenced current techniques commonly used worldwide. Our review highlights the need for a global consortium on cleft care to gather expert opinions on current practices and outcomes and to standardize technique classifications. An understanding of global protocols is crucial in an attempt to standardize technique and timing to achieve anatomical closure with optimal velopharyngeal competence, while also minimizing the occurrence of maxillary hypoplasia and palatal fistulae.

High prevalence of deleterious mutations in concomitant nonsyndromic cleft and outflow tract heart defects.

Our previous work demonstrating enrichment of outflow tract (OFT) congenital heart disease (CHD) in children with cleft lip and/or palate (CL/P) suggests derangements in common underlying developmental pathways. The current pilot study examines the underlying genetics of concomitant nonsyndromic CL/P and OFT CHD phenotype. Of 575 patients who underwent CL/P surgery at Children's Hospital Los Angeles, seven with OFT CHD, negative chromosomal microarray analysis, and no recognizable syndromic association were recruited with their parents (as available). Whole genome sequencing of blood samples paired with whole-blood-based RNA sequencing for probands was performed. A pathogenic or potentially pathogenic variant was identified in 6/7 (85.7%) probands. A total of seven candidate genes were mutated (CHD7, SMARCA4, MED12, APOB, RNF213, SETX, and JAG1). Gene ontology analysis of variants predicted involvement in binding (100%), regulation of transcription (42.9%), and helicase activity (42.9%). Four patients (57.1%) expressed gene variants (CHD7, SMARCA4, MED12, and RNF213) previously involved in the Wnt signaling pathway. Our pilot analysis of a small cohort of patients with combined CL/P and OFT CHD phenotype suggests a potentially significant prevalence of deleterious mutations. In our cohort, an overrepresentation of mutations in molecules associated with Wnt-signaling was found. These variants may represent an expanded phenotypic heterogeneity within known monogenic disease genes or provide novel evidence of shared developmental pathways. The mechanistic implications of these mutations and subsequent developmental derangements resulting in the CL/P and OFT CHD phenotype require further analysis in a larger cohort of patients.

The influence of an all-female healthcare environment on mentorship and empowerment of female healthcare professionals.

Low- and middle-income countries (LMICs) have the greatest need for additional healthcare providers, and women outside the workforce help address the need. Women in healthcare need more mentorship and leadership training to advance their careers due to systemic barriers. This study evaluates how women working together on a medical team influences mentorship, leadership and empowerment. A single all-female volunteer team participating in a cleft surgery mission in Oujda, Morocco were surveyed before and after the mission. Statistical analysis with student's t-test or chi-squared were performed. 95 female volunteers from 23 countries participated on this team and 85% completed surveys. Volunteers from high-income countries (32%) and LMICs (68%) had similar mission roles (p = 0.58). Experience as a mission volunteer (p = 0.47), team leader (p = 0.28), and educator (p = 0.18) were equivalent between cohorts. 73% of women had previously received mentorship but 98% wanted more. 75% had previously mentored others, but 97% wanted to be mentors. 73% of volunteers who had no prior mentorship found their first mentor during the mission. All participants found a long-term peer relationship and felt motivated to mentor women at home. 95% were inspired to pursue leadership positions, advance professionally, and continue working with other women. This population of female healthcare professionals overwhelmingly desired more mentorship than is felt to be available. An all-female healthcare environment appears to provide opportunities for mentorship and create lasting motivation to teach, lead, and advance professionally. Findings raise the potential that increasing visibility of female professionals may effectively empower women in healthcare.

Predictors of Complication Following Cleft Lip and Palate Surgery in a Low-Resource Setting: A Prospective Outcomes Study in Nicaragua.

BACKGROUND: Higher rates of postoperative complication following cleft lip or palate repair have been documented in low resource settings, but their causes remain unclear. This study sought to delineate patient, surgeon, and care environment factors in cleft complications in a low-income country. DESIGN: Prospective outcomes study. SETTING: Comprehensive Cleft Care Center. PATIENTS: Candidate patients presenting for cleft lip or palate repair or revision. INTERVENTIONS: Patient anthropometric, nutritional, environmental and peri- and post-operative care factors were collected. Post-operative evaluation occurred at standard 1-week and 2-month postoperative intervals. MAIN OUTCOME MEASURES: Complication was defined as fistula, dehiscence and/or infection. RESULTS: Among 408 patients enrolled, 380 (93%) underwent surgery, of which 208 (55%) underwent lip repair (124) or revision (84), and 178 (47%) underwent palate repair (96) or revision (82). 322 (85%) were evaluated 1 week and 166 (44%) 2 months postoperatively. 50(16%) complications were identified, including: 25(8%) fistulas, 24(7%) dehiscences, 17(5%) infections. Mid-upper arm circumference (MUAC) ≤12.5 cm was associated with dehiscence after primary lip repair (OR = 28, p = 0.02). Leukocytosis ≥11,500 on pre-operative evaluation was associated with dehiscence (OR = 2.51, p = 0.04) or palate revision fistula (OR = 64, p < 0.001). Surgeons who performed fewer previous-year palate repairs had higher likelihood of palate complications, (OR = 3.03, p = 0.01) although there was no difference in complication rate with years of surgeon experience or duration of surgery. CONCLUSIONS: Multiple patient, surgeon, and perioperative factors are associated with higher rates of complication in a low-resource setting, and are potentially modifiable to reduce complications following cleft surgery.

Assessing the Safety of a Novel Neonatal Anesthesia Protocol: A Review of 101 Patients With Early Cleft Lip Repair.

PURPOSE: Our study introduces a novel anesthetic protocol for neonates without using volatile agents with the goal to minimize potentially neurotoxic and pro-apoptotic agents. The authors evaluated the short-term safety and efficacy of our anesthetic protocol in patients undergoing early cleft lip repair (ECLR). METHODS: A retrospective review of ECLR patients who underwent repair before 2.5 months of age within the last 4.3 years was performed. This sample was comprised of 2 groups, those who received either a standard volatile gas-based regimen or a dexmedetomidine-based neonatal anesthetic protocol (DBNAP). Patient demographics, medication dosing, anesthetic time (induction to extubation), major and minor complications, and medication side effects were compared between the 2 cohorts. RESULTS: A total of 101 patients underwent ECLR. All patients were American Society of Anesthesiologists class 1 or 2. Mean age at surgery was 31 ±â€Š13 days and mean anesthetic time was 179 ±â€Š36 minutes. DBNAP was used in 65 patients while the standard anesthetic protocol was used in 36 patients. Patient weight was significantly lower in the DBNAP group (4.01 ±â€Š0.61 versus 4.38 ±â€Š0.72 kg, P  = 0.007). There were no significant differences between the 2 cohorts when comparing anesthetic time, emergence time, complication rate, or medication side effects. CONCLUSION: For patients undergoing ECLR, DBNAP is perioperatively equivalent to the anesthetic standard of care, demonstrating no major complications and acceptable rates of minor complications and medication side effects.