Single-Stage Posterior Vomerine Ostectomy, Premaxillary Setback, Bilateral Gingivoperiosteoplasties and Primary Bilateral Cheiloplasty in Patients with Protuberant Premaxilla.

Various patients with complete bilateral cleft lip and palate present with a protruded premaxilla. Several techniques have been described for correctional repair of the projection with a plethora of unsatisfactory outcomes. This poses a challenge not only for the cleft team providing care but also for the patients and their respective families. Multiple patients suffer from residual deformities after inadequate primary repair, which increase surgical, financial, and psychological burden. Premaxillary setback with posterior vomerine ostectomy and complete bilateral cleft lip repair can promote alignment of the premaxilla with the maxillary prominences. To effectively address this challenging deformity, we describe a single-stage surgical technique that includes vomerine ostectomy posterior to the vomero-premaxillary suture, bilateral gingivoperiosteoplasties with complete bilateral cleft lip repair, and primary cleft rhinoplasty. Careful surgical planning is essential for adequate matching between the length of the protruded premaxilla and the extent of ostectomy. The described technique offers several advantages for the management of complete bilateral cleft lip with a projected premaxilla. It can be applied anywhere around the world and is most beneficial in underprivileged areas where patients suffer from restricted access to healthcare, absence of presurgical orthodontics and lack of sufficient resources.

Cleft Summit 2022: The Impact of a Unified Voice.

OBJECTIVE: The inaugural Cleft Summit aimed to unite experts and foster interdisciplinary collaboration, seeking a collective understanding of velopharyngeal insufficiency (VPI) management. DESIGN: An interactive debate and conversation between a multidisciplinary cleft care team on VPI management. SETTING: A two-hour discussion within a four-day comprehensive cleft care workshop (CCCW). PARTICIPANTS: Thirty-two global leaders from various cleft disciplines. INTERVENTIONS: Cleft Summit that allows for meaningful interdisciplinary collaboration and knowledge exchange. MAIN OUTCOME MEASURES: Ability to reach consensus on a unified statement for VPI management. RESULTS: Participants agreed that a patient with significant VPI and a dynamic velum should first receive a surgery that lengthens the velum to optimize patient outcome. A global, multicenter prospective study should be done to test this hypothesis. CONCLUSION: The 1st Cleft Summit successfully distilled global expertise into actionable best-practice guidelines through iterative discussions, fostering interdisciplinary collaboration and paving the way for a transformative multi-center prospective study on VPI care.

A Modified and Refined Classification System for Secondary Cleft Nasolabial Deformities.

Secondary cleft lip (CL) deformities are commonly encountered in cleft management. Various attempts have been made to create a classification system that can comprehensively encapsulate residual CL deformities after primary repair. The aim of this study is to reinforce valid classification systems and to introduce a new classification subtype (Type V Revision). A longitudinal retrospective analysis was done in 35 outreach programs in 4 countries (Ecuador, Lebanon, Peru, and El Salvador) between 2015 and 2023. Two hundred sixty-nine patients, between the ages of 9 months and 45 years, with residual CL deformities after primary lip repair, were classified into one of the 5 classification types. Patients with syndromes were excluded from the study. Patients received one of 5 revision types depending on their initial preoperative evaluation and intraoperative analysis of anatomic involvement. The mean age at surgery was 12.86 years. Twenty-five patients received type I revision, 29 patients received type II revision, 81 patients received type III revision, 106 patients received type IV revision, and 28 patients received type V revision. As the surgical complexity advanced from type I to V, there was a gradual increase in the average surgical time. The Congruence between preoperative and postoperative revision types was substantial. Residual CL defects cause a significant burden on both the patient and cleft care provider. This classification system, with the newly introduced type V revision, enhances and eases the categorization and management of secondary CL deformities.

Ergonomics for Cleft Providers: A Brief Communication.

Musculoskeletal (MSK) injuries are the most common and debilitating work-related injuries among healthcare providers. These injuries often occur due to a lack of awareness and insufficient guidance during the early years of medical training. Recognizing the need to address this issue, the Comprehensive Cleft Care Workshop (CCCW) has taken steps to integrate an ergonomics session into its curriculum. The goal of this initiative is to enhance awareness on ergonomics, improve the integration of ergonomics into daily routine, and ultimately reduce the occurrence of MSK injuries among healthcare professionals.

Global Prevalence and Burden of Orofacial Clefts: A Systematic Analysis for the Global Burden of Disease Study 2019.

Orofacial clefts, in particular cleft lip and cleft palate, are among the most common congenital anomalies. Despite guidelines recommending early surgical correction, a global backlog of untreated patients persists. This has made orofacial clefts an attractive target for global cleft care initiatives. The most recent global burden of orofacial clefts was estimated to be 529,758.92 disability-adjusted life years (95% uncertainty interval: 362,492.88-798,419.69 disability-adjusted life years), whereas the global prevalence of orofacial clefts was estimated to be 4.6 million (95% uncertainty interval: 3.8-5.7 million). An inverse relationship exists between the Sociodemographic Index and the burden of orofacial clefts. Sub-Saharan Africa, Middle East/North Africa, and South Asia are the regions carrying the most significant burden of orofacial clefts. This manuscript provides updated estimates of the global burden and prevalence of orofacial clefts, acting as a guide to direct future investments, resources, and initiatives from individuals and organizations engaged in global cleft care delivery with the goal of building sustainable cleft care capacity where it is needed the most.

Single-Stage Posterior Vomerine Ostectomy and Primary Cheiloplasty in Patients with Bilateral Cleft Lip & Palate and Protuberant Premaxilla.

OBJECTIVE: A protruded premaxilla has always been challenging to care for by cleft care professionals. This study aims to fortify the use of a single-stage premaxillary setback, with posterior vomerine ostectomy and primary cheiloplasty to achieve proper care for patients with bilateral cleft lip and palate (BCLP) and protruded premaxilla. DESIGN: Longitudinal retrospective analysis. SETTING: Twenty-three outreach programs to four countries (Ecuador, Lebanon, Peru, and El-Salvador) between 2016-2022. PATIENTS/PARTICIPANTS: Sixty-five patients between the ages of 3 months and 6 years and 5 months, with BCLP and severely protruded premaxilla underwent premaxillary setback via posterior vomerine ostectomy and primary cheiloplasty. Patients with diagnosed syndromes and inaccessible vomer bone due to fused palates were excluded from the study. INTERVENTIONS: Premaxillary setback with posterior vomerine ostectomy, bilateral gingivoperiosteoplasties (GPP), and primary cheiloplasty. MAIN OUTCOME MEASURE(S): Postoperative complications and aesthetic outcomes. RESULTS: The mean age at surgery was 13.17 ± 14.1 months, with an average follow-up time of 26 ± 17 months. Patients underwent their procedures in Ecuador (72%), Peru (9%), Lebanon (8%) and El-Salvador (1%). The majority of patients were aged 1 year or less (66.7%) and were males (58.5%). All patients were operated on successfully and had good aesthetic outcomes. Only one patient developed partial necrosis. CONCLUSION: Patients with BCLP and severe premaxillary protrusion have always carried immense social, psychological, and financial burdens, especially in outreach settings. Our described single-stage technique has proven to be safe and effective with good aesthetic results. Further follow-up after primary repair should be done to document and ensure proper facial growth and normal nasolabial maturation.

Global Smile Foundation's Cleft Surgical Outreach Program: Clinical and Economic Impact During the Past 14 Years.

Clefts of the lip and/or palate can result in significant morbidity as well as economic and psychosocial distress for patients and families. Global Smile Foundation is a non-profit organization committed to providing comprehensive cleft care to patients with cleft of the lip/palate around the world. Primary cleft lip and primary cleft palate repairs performed by the Global Smile Foundation in the last decade were reviewed. Averted disability-adjusted life years were estimated and assessed for their economic value. A total of 15,310 disability-adjusted life years were averted. The financial gain was estimated between $78,323,624 and $152,906,604, with an average financial benefit of $48,021 to $93,750 per patient.

Application of Safety Protocols for International Cleft Outreach Programs During the COVID-19 Pandemic: Global Smile Foundation Precaution Guidelines and Implications.

BACKGROUND: Since COVID-19 was declared a worldwide pandemic by the World Health Organization (WHO) in March of 2020, foundation-based cleft outreach programs to Low- and Middle-Income Countries (LMICs) were halted considering global public health challenges, scarcity of capacity and resources, and travel restrictions. This led to an increase in the backlog of untreated patients with cleft lip and/or palate, with new challenges to providing comprehensive care in those regions. Resumption of international outreach programs requires an updated course of action to incorporate necessary safety measures in the face of the ongoing pandemic. In this manuscript, the authors outline safety protocols, guidelines, and recommendations implemented in Global Smile Foundation's (GSF) most recent outreach trip to Beirut, Lebanon. METHODS: COVID-19 safety protocols for outreach cleft care and an Action Response Plan were developed by the GSF team based on the published literature and recommendations from leading international organizations. RESULTS: GSF conducted a 1-week surgical outreach program in Beirut, Lebanon, performing 13 primary cleft lip repairs, 7 cleft palate repairs, and 1 alveolar bone grafting procedure. Safety protocols were implemented at all stages of the outreach program, including patient preselection and education, hospital admission and screening, intraoperative care, and postoperative monitoring and follow-up. CONCLUSIONS: Organizing outreach programs in the setting of infectious diseases outbreaks should prioritize the safety and welfare of patients and team members within the program's local community. The COVID-19 protocols and guidelines described may represent a reproducible framework for planning future similar outreach initiatives in high-risk conditions.

Enhanced Recovery After Cleft Lip Repair: Protocol Development and Implementation in Outreach Settings.

INTRODUCTION: Clefts of the lip are of the most common congenital craniofacial anomalies. The development and implementation of an enhanced recovery after surgery (ERAS) protocol among patients undergoing cleft lip repair may decrease postoperative complications, accelerate recovery, and result in earlier postoperative discharge. METHODS: A modified ERAS program was developed and applied through Global Smile Foundation outreach craniofacial programs. The main components of this protocol include: (1) preoperative patient education, (2) nutrition screening, (3) smoking cessation when applicable, (4) use of topical anesthetic adjuncts, (5) facial nerve blocks, (6) postoperative analgesia, (7) preferential use of short-acting narcotics, (8) antibiotic administration, (9) use of elbow restraints, (10) early postoperative oral feeding and hydration, and (11) discharge planning. RESULTS: Between April 2019 and March 2020, GSF operated on 126 patients with cleft lip from different age groups and 58.8% of them were less than 1 year of age. Three patients (2.4%) had delayed wound healing and one (0.8%) had postoperative bleeding. There were no cases of mortality, length of hospital stay did not exceed 1 postoperative day, and patients were able to tolerate fluids intake at discharge. CONCLUSION: The implementation of an ERAS protocol among patients undergoing cleft lip repair has shown to be highly effective in minimizing postoperative discomfort while reducing opioids use, decreasing the length of stay in hospital, and leading to early oral feeding resumption. The ERAS principles described carry increased relevance in the context of the ongoing COVID-19 pandemic and opioid crisis and can be safely applied in resource-constrained settings.

Quality Assurance Standards for Outreach Cleft Lip and Cleft Palate Repair Programs in Low-Resource Settings.

BACKGROUND: Comprehensive cleft care is a multidisciplinary team endeavor. While untreated craniofacial conditions have multiple undue repercussions, cleft care in outreach settings can be fraught with significant perioperative morbidity risks. AIM: Propose updated quality assurance standards addressing logistic and operational considerations essential for the delivery of safe and effective cleft lip and /or palate (CL/P) care in low and middle-income countries (LMICs) settings. METHODS: Based on American Cleft Palate-Craniofacial Association (ACPA) quality standards, published literature, published protocols by Global Smile Foundation (GSF), and the senior author's three-decade experience, updated standards for outreach cleft care were synthesized. RESULTS: Ten axes for safe, effective, and sustainable cleft lip and palate care delivery in underserved settings were generated: 1) site assessment, 2) establishment of community partnerships, 3) team composition and credentialing, 4) team training and mission preparation, 5) implementation of quality assurance guidelines, operative safety checklists, and emergency response protocols, 6) immediate and long-term postoperative care, 7) medical record keeping, 8) outcomes evaluation, 9) education, and 10) capacity building and sustainability. Subsequent analysis further characterized essential components of each of those ten axes to delineate experience derived and evidence-based recommendations. DISCUSSION: Quality assurance guidelines are essential for the safe delivery of comprehensive cleft care to patients with CL/P in any setting. Properly designed surgical outreach programs relying on honest community partnerships can be effectively used as vehicles for local capacity building and the establishment of sustainable cleft care ecosystems.

Damaging variants in FOXI3 cause microtia and craniofacial microsomia.

PURPOSE: Craniofacial microsomia (CFM) represents a spectrum of craniofacial malformations, ranging from isolated microtia with or without aural atresia to underdevelopment of the mandible, maxilla, orbit, facial soft tissue, and/or facial nerve. The genetic causes of CFM remain largely unknown. METHODS: We performed genome sequencing and linkage analysis in patients and families with microtia and CFM of unknown genetic etiology. The functional consequences of damaging missense variants were evaluated through expression of wild-type and mutant proteins in vitro. RESULTS: We studied a 5-generation kindred with microtia, identifying a missense variant in FOXI3 (p.Arg236Trp) as the cause of disease (logarithm of the odds = 3.33). We subsequently identified 6 individuals from 3 additional kindreds with microtia-CFM spectrum phenotypes harboring damaging variants in FOXI3, a regulator of ectodermal and neural crest development. Missense variants in the nuclear localization sequence were identified in cases with isolated microtia with aural atresia and found to affect subcellular localization of FOXI3. Loss of function variants were found in patients with microtia and mandibular hypoplasia (CFM), suggesting dosage sensitivity of FOXI3. CONCLUSION: Damaging variants in FOXI3 are the second most frequent genetic cause of CFM, causing 1% of all cases, including 13% of familial cases in our cohort.

Premaxillary Setback With Posterior Vomerine Ostectomy and Complete Bilateral Cleft Lip Repair: Report of a Case With Challenging Anatomy.

Congenital clefts of the lip and/or palate are among the most common craniofacial malformations. Patients with bilateral cleft of the lip often present with projected premaxilla. Premaxillary setback with a vomerine ostectomy posterior to the vomero-premaxillary suture, bilateral cleft lip repair, bilateral gingivoperiosteoplasties, and primary cleft lip rhinoplasty are achieved in a single-stage surgery that provides a valuable alternative to patients, especially in the outreach settings. In this article, we present a case report of a patient born with a bilateral cleft of the lip and a protruded premaxilla. He had collapsed secondary palatine shelves requiring intraoperative manual expansion to ensure access to the vomer bone.

Harnessing the Power of Artificial Intelligence to Teach Cleft Lip Surgery.

Background: Artificial intelligence (AI) leverages today's exceptional computational powers and algorithmic abilities to learn from large data sets and solve complex problems. The aim of this study was to construct an AI model that can intelligently and reliably recognize the anatomy of cleft lip and nasal deformity and automate placement of nasolabial markings that can guide surgical design. Methods: We adopted the high-resolution net architecture, a recent family of convolutional neural networks-based deep learning architecture specialized in computer-vision tasks to train an AI model, which can detect and place the 21 cleft anthropometric points on cleft lip photographs and videos. The model was tested by calculating the Euclidean distance between hand-marked anthropometric points placed by an expert cleft surgeon to ones generated by our cleft AI model. A normalized mean error (NME) was calculated for each point. Results: All NME values were between 0.029 and 0.055. The largest NME was for cleft-side cphi. The smallest NME value was for cleft-side alare. These errors were well within standard AI benchmarks. Conclusions: We successfully developed an AI algorithm that can identify the 21 surgically important anatomic landmarks of the unilateral cleft lip. This model can be used alone or integrated with surface projection to guide various cleft lip/nose repairs. Having demonstrated the feasibility of creating such a model on the complex three-dimensional surface of the lip and nose, it is easy to envision expanding the use of AI models to understand all of human surface anatomy-the full territory and playground of plastic surgeons.

Association of Patient Factors and Follow-Up in an International Volunteer Cleft Lip and Palate Repair Clinic in El Salvador.

BACKGROUND: In countries which lack robust health care systems, congenital conditions such as cleft lip and/or palate deformities are often untreated in certain individuals. Many volunteer organizations have stepped in to fill this gap but certain factors, such as continuity of care, are yet to be studied for these clinics. METHODS: This is a retrospective cohort study of 167 pediatric patients with cleft lip and/or palate residing in El Salvador treated by a nongovernmental organizations between 2011 and 2020. This data was used in univariate and multivariable models to associate particular patient factors to their likelihood of following up to their annual clinic visits. RESULTS: Each 1-year increase in duration of follow-up was associated with a 27% decrease in the odds of attending a visit. In addition, 33.7% of cleft lip and 49.7% of cleft palate/cleft lip and palate patients returned at least once. Males had 36% higher odds of attending a return visit compared with females but this difference was not statistically significant. Time spent travelling to the clinic had no effect on follow-up rates. CONCLUSION: Nongovernmental organizations utilizing a diagonal care model should consider using more strategies to maximize continuity of care by increasing communication with patients and emphasizing the need of following up during clinic visits. Continued and increased collaboration with the local team is also of great importance.

An ancient founder mutation located between ROBO1 and ROBO2 is responsible for increased microtia risk in Amerindigenous populations.

Microtia is a congenital malformation that encompasses mild hypoplasia to complete loss of the external ear, or pinna. Although the contribution of genetic variation and environmental factors to microtia remains elusive, Amerindigenous populations have the highest reported incidence. Here, using both transmission disequilibrium tests and association studies in microtia trios (parents and affected child) and microtia cohorts enrolled in Latin America, we map an ∼10-kb microtia locus (odds ratio = 4.7; P = 6.78e-18) to the intergenic region between Roundabout 1 (ROBO1) and Roundabout 2 (ROBO2) (chr3: 78546526 to 78555137). While alleles at the microtia locus significantly increase the risk of microtia, their penetrance is low (<1%). We demonstrate that the microtia locus contains a polymorphic complex repeat element that is expanded in affected individuals. The locus is located near a chromatin loop region that regulates ROBO1 and ROBO2 expression in induced pluripotent stem cell­derived neural crest cells. Furthermore, we use single nuclear RNA sequencing to demonstrate ROBO1 and ROBO2 expression in both fibroblasts and chondrocytes of the mature human pinna. Because the microtia allele is enriched in Amerindigenous populations and is shared by some East Asian subjects with craniofacial malformations, we propose that both populations share a mutation that arose in a common ancestor prior to the ancient migration of Eurasian populations into the Americas and that the high incidence of microtia among Amerindigenous populations reflects the population bottleneck that occurred during the migration out of Eurasia.

Simulation Use in Outreach Setting: A Novel Approach to Building Sustainability.

SUMMARY STATEMENT: Simulation is a well-studied teaching tool for multidisciplinary teamwork, crisis resource management, and communication skills. These attributes are essential for successful international medical missions, which include healthcare providers with different familiarities with the outreach environment and each team member's role. However, immersive simulation remains underused in similar settings. Our team designed a simulation-based curriculum that focuses on multidisciplinary teamwork and crisis resource management skills. In this commentary, we describe its implementation during high-risk cleft care outreach missions conducted by the Global Smile Foundation. We discuss the importance of a simple, feasible, and flexible platform to successfully overcome the limitations of time and resources inherent to outreach mission work while addressing the clinical and geographic needs specific to each site. We highlight challenges, including unpredictability of the outreach environment, a language barrier, and the short duration of missions. Finally, we offer a roadmap for groups involved in similar global health efforts.

Emergency Response Protocols for Overseas Outreach Settings: Global Smile Foundation Strategy.

Non-governmental organizations (NGOs) aim to alleviate unmet global disease burden and promote collaboration between visiting and host countries. Well-executed emergency response protocols are foundational to providing safe and quality care in an unpredictable global setting. Global Smile Foundation (GSF) instituted a protocol in 2012 based on over three decades of cleft care experience. Here, we update this protocol and provide information on how to address special circumstances such as humanitarian crises and disease outbreaks. The GSF response protocol was developed in conjunction with surgeons, anesthesiologists, nurses, and administrators to ensure all team members are adequately prepared to respond to emergency situations in global outreach. This protocol provides information on pre-departure preparation, onsite setup, operative precautions, and post-departure debriefings. Emphasis is placed on a standardized, reproducible workflow that accounts for necessary site-specific adjustments. Strategies include emergency simulations, site-specific safety checklists, standardized operating room protocols, and well-defined individual responsibilities. The authors also provide anticipatory guidance in addressing unexpected circumstances, such as disease outbreak and natural disaster, during global outreach. In conclusion, a proactive and systematic approach to emergency response and prevention is vital to minimizing morbidity and mortality during surgical outreach initiatives. The GSF protocol represents a reproducible approach that other organizations can adopt and adapt to their unique needs.

Perceived Barriers to Comprehensive Cleft Care Delivery: Results From A Capacity-Building Educational Initiative and Implications.

INTRODUCTION: We analyzed the perceptions of participants and faculty members in simulation-based comprehensive cleft care workshops regarding comprehensive cleft care delivery in developing countries. METHODS: Data were collected from participants and faculty members in 2 simulation-based comprehensive cleft care workshops organized by Global Smile Foundation. We collected demographic data and surveyed what they believed was the most significant barrier to comprehensive cleft care delivery and the most important intervention to deliver comprehensive cleft care in developing countries. We also compared participant and faculty responses. RESULTS: The total number of participants and faculty members was 313 from 44 countries. The response rate was 57.8%. The majority reported that the most significant barrier facing the delivery of comprehensive cleft care in developing countries was financial (35.0%), followed by the absence of multidisciplinary cleft teams (30.8%). The majority reported that the most important intervention to deliver comprehensive cleft care was creating multidisciplinary cleft teams (32.2%), followed by providing cleft training (22.6%). We found no significant differences in what participants and faculty perceived as the greatest barrier to comprehensive cleft care delivery (P = 0.46), or most important intervention to deliver comprehensive cleft care in developing countries (P = 0.38). CONCLUSIONS: Our study provides an appraisal of barriers facing comprehensive cleft care delivery and interventions required to overcome these barriers in developing countries. Future studies will be critical to validate or refute our findings, as well as determine country-specific roadmaps for delivering comprehensive cleft care to those who need it the most.

Comprehensive Cleft Care Delivery in Developing Countries: Impact of Geographic and Demographic Factors.

INTRODUCTION: The authors analyzed the insights of participants and faculty members of Global Smile Foundation's Comprehensive Cleft Care Workshops concerning the barriers and interventions to multidisciplinary cleft care delivery, after stratification based on demographic and geographic factors. METHODS: During 2 simulation-based Comprehensive Cleft Care Workshops organized by Global Smile Foundation, participants and faculty members filled a survey. Surveys included demographic and geographic data and investigated the most relevant barrier to multidisciplinary cleft care and the most significant intervention to deliver comprehensive cleft care in outreach settings, as perceived by participants. RESULTS: The total response rate was 57.8%. Respondents reported that the greatest barrier to comprehensive cleft care was financial, and the most relevant intervention to deliver multidisciplinary cleft care was building multidisciplinary teams. Stratification by age, gender, and geographical area showed no statistical difference in reporting that the greatest barrier to cleft care was financial. However, lack of multidisciplinary teams was the most important barrier according to respondents with less than 5 years of experience (P = 0.03). Stratification by gender, years in practice, specialty, and geographical area showed no statistical difference, with building multidisciplinary teams reported as the most significant intervention. However, increased training was reported as the main intervention to cleft care for those aged less than 30 years old (P = 0.04). CONCLUSIONS: Our study delivers an assessment for barriers facing multidisciplinary cleft care delivery and interventions required to improve cleft care delivery. The authors are hoping that stratification by demographic and geographic factors will help them delineate community-specific road maps to refine cleft care delivery.