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 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.

Sagittal Growth Restriction of the Midface Following Isolated Cleft Lip Repair: A Systematic Review and Meta-Analysis.

Midface hypoplasia (MFH) is a long-term sequela of cleft lip and palate repair, and is poorly understood. No study has examined the aggregate data on sagittal growth restriction of the midface following repair of the lip, but not palate, in these patients.A systematic review of 3780 articles was performed. Twenty-four studies met inclusion criteria and 11 reported cephalometric measurements amenable to meta-analysis. Patients with Veau class I-III palatal clefts were included so long as they had undergone only lip repair. Groups were compared against both noncleft and unrepaired controls.Cephalometrics were reported for 326 patients (31.3% female). Noncleft controls had an average SNA angle of 81.25° ± 3.12°. The only patients demonstrating hypoplastic SNA angles were those with unilateral CLP with isolated lip repair (77.4° ± 4.22°). Patients with repaired CL had SNA angles similar to noncleft controls (81.4° ± 4.02°). Patients with unrepaired CLP and CL tended toward more protruding maxillae, with SNA angles of 83.3° ± 4.04° and 87.9° ± 3.11°, respectively. Notably, when comparing SNA angles between groups, patients with CLP with isolated lip repair had significantly more hypoplastic angles compared to those with repaired CL (P < .0001). Patients with CLP with isolated lip repair were also more hypoplastic than noncleft controls (P < .0001). In contrast, there was no significant difference between the SNA of patients with repaired CL and controls (P = .648).We found that cleft lip repair only appeared to contribute to MFH in the setting of concurrent cleft palate pathology, suggesting that scarring from lip repair itself is unlikely to be the predominant driver of MFH development. However, studies generally suffered from inadequate reporting of timing, technique, follow-up time, and cleft severity.

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.

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.

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.

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.

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.

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.

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.

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.

The International Family Study of Nonsyndromic Orofacial Clefts: Design and Methods.

BACKGROUND: The majority of research to understand the risk factors of nonsyndromic orofacial clefts (NSOFCs) has been conducted in high-income populations. Although patients with NSOFCs in low- and middle-income countries (LMICs) are at the highest risk of not receiving care, global health infrastructure allows innovative partnerships to explore the etiologic mechanisms of cleft and targets for prevention unique to these populations. METHODS: The International Family Study (IFS) is an ongoing case-control study with supplemental parental trio data designed to examine genetic, environmental, lifestyle, and sociodemographic risk factors for NSOFCs in 8 LMICs (through August 2020). Interview and biological samples are collected for each family. The interview includes demographics, family history of cleft, diet and water sources, maternal pregnancy history, and other lifestyle and environmental factors. RESULTS: Seven of 8 countries are currently summarized (2012-2017) for a total of 2955 case and 2774 control families with 11 946 unique biological samples from Vietnam, Philippines, Honduras, Madagascar, Morocco, Democratic Republic of the Congo, and Nicaragua. The phenotype distribution was 1641 (55.5%) cases with cleft lip and palate, 782 (26.5%) with cleft lip (CL), and 432 (14.6%) with cleft palate (CP). DISCUSSION: The International Family Study is the largest case set of NSOFCs with an associated biobank in LMICs currently assembled. The biobank, family, and case-control study now include samples from 8 LMICs where local health care infrastructure cannot address the surgical burden of cleft or investigate causal mechanisms. The International Family Study can be a source of information and may collaborate with local public health institutions regarding education and interventions to potentially prevent NSOFCs.

Genomic analyses in African populations identify novel risk loci for cleft palate.

Orofacial clefts are common developmental disorders that pose significant clinical, economical and psychological problems. We conducted genome-wide association analyses for cleft palate only (CPO) and cleft lip with or without palate (CL/P) with ~17 million markers in sub-Saharan Africans. After replication and combined analyses, we identified novel loci for CPO at or near genome-wide significance on chromosomes 2 (near CTNNA2) and 19 (near SULT2A1). In situ hybridization of Sult2a1 in mice showed expression of SULT2A1 in mesenchymal cells in palate, palatal rugae and palatal epithelium in the fused palate. The previously reported 8q24 was the most significant locus for CL/P in our study, and we replicated several previously reported loci including PAX7 and VAX1.

The Local Mission: Improving Access to Surgical Care in Middle-Income Countries.

BACKGROUND: Billions of people lack access to quality surgical care. Short-term missions are used to supplement the delivery of surgical care in regions with poor access to care. Traditionally known for using international teams, Operation Smile has transitioned to using a local mission model, where surgical service is delivered to areas of need by teams originating within that country. This study investigates the proportion and location of Operation Smile missions that use the local mission model. METHODS: A retrospective review was performed of the Operation Smile mission database for fiscal years 2014 to 2019. Missions were classified into local or international missions. Countries were also classified by their income levels as well as their specialist surgical workforce (SAO) density. As no individual patient or provider data was recorded, ethics board approval was not warranted. RESULTS: Between 2014 and 2019, Operation Smile held an average of 144.8 (range 135-154) surgical missions per year. Local missions accounted for 97 ± 5.6 (67%) of the missions. Of the 34 program countries, 26 (76%) used local missions. Of the countries that had only international missions, six (75%) were low-income countries and the average SAO density was 1.54 (range 0.19-5.88) providers per 100,000 people. Of the countries with local missions, 24 (92%) were middle-income, and the average SAO density was 30.9 (range 3.4-142.4). CONCLUSION: International investments may assist in the creation of local surgical teams. Once teams are established, local missions are a valuable way to provide specialized surgical care within a country's own borders.

Impact of an All-Female Surgical Team on Moroccan Patient Perspectives of Female Healthcare Providers.

INTRODUCTION: Increasing numbers of women in medicine could address Morocco's 5.5-fold deficit in surgical providers. Cultural perceptions towards women limit female advancement in healthcare. This study evaluates the impact of an all-female surgical team on Moroccan attitudes. OBJECTIVE: This study aimed to evaluate how attitudes towards female healthcare professionals changed for Moroccan patients after exposure to a unique, all-female medical environment. METHODS: Cleft patients were surveyed after a surgery mission with all-female volunteers in Oujda, Morocco. Analysis included quantitative, qualitative, and mixed-methods approaches. RESULTS: Of 121 respondents (94%), 85% and 77% had prior exposure to a female nurse or doctor, respectively. 94% of respondents strongly agreed to receiving high-quality care. 75% developed increased confidence in female providers. 68% and 69% of respondents, regardless of gender (p = 0.950), felt that having a female nurse or doctor did not impact care. Female patients were more likely than male patients to strongly encourage female relatives to pursue medical careers (p = 0.027). Respondents without prior exposure to female nurses were more likely to: pursue medical careers (p = 0.034), believe female relatives could pursue medical careers (p = 0.006), and encourage them to do so (p = 0.011). CONCLUSIONS: Increased visibility of women improved patient attitudes towards female providers, especially in patients without prior exposure. Initiatives that increase female representation in healthcare may have greater effects in cultures with more gender inequity.

The Use of Crowdsourcing Technology to Evaluate Preoperative Severity in Patients With Unilateral Cleft Lip in a Multiethnic Population.

ABSTRACT: Crowd sourcing has been used in multiple disciplines to quickly generate large amounts of diverse data. The objective of this study was to use crowdsourcing to grade preoperative severity of unilateral cleft lip phenotype in a multiethnic cohort with the hypothesis that crowdsourcing could efficiently achieve similar rankings compared to expert surgeons. Deidentified preoperative photos were collected for patients with primary, unilateral cleft lip with or without cleft palate (CL ±â€ŠP). A platform was developed with C-SATS for pairwise comparisons utilizing Elo rankings by crowdsource workers through Amazon Mechanical Turk. Images were independently ranked by 2 senior surgeons for comparison. Seventy-six patients with varying severity of unilateral (CL ±â€ŠP) phenotype were chosen from Operation Smile missions in Bolivia, Madagascar, Vietnam, and Morocco. Patients were an average of 1.2 years' old, ranging from 3 months to 3.3 years. Each image was compared with 10 others, for a total of 380 unique pairwise comparisons. A total of 4627 total raters participated with a median of 12 raters per pair. Data collection was completed in <20 hours. The crowdsourcing ranking and expert surgeon rankings were highly correlated with Pearson correlation coefficient of R = 0.77 (P = 0.0001). Crowdsourcing provides a rapid and convenient method of obtaining preoperative severity ratings, comparable to expert surgeon assessment, across multiple ethnicities. The method serves as a potential solution to the current lack of rating systems for preoperative severity and overcomes the difficulty of acquiring large-scale assessment from expert surgeons.