Management of long gap esophageal atresia: A systematic review and evidence-based guidelines from the APSA Outcomes and Evidence Based Practice Committee

Background: Treatment of the neonate with long gap esophageal atresia (LGEA) is one of the most challenging scenarios facing pediatric surgeons today. Contributing to this challenge is the variability in case definition, multiple approaches to management, and heterogeneity of the reported outcomes. This necessitates a clear summary of existing evidence and delineation of treatment controversies. Methods: The American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee drafted four consensus-based questions regarding LGEA. These questions concerned the definition and determination of LGEA, the optimal method of surgical management, expected long-term outcomes, and novel therapeutic techniques. A comprehensive search strategy was crafted and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were utilized to identify, review and report salient articles. Results: More than 3000 publications were reviewed, with 178 influencing final recommendations. In total, 18 recommendations are provided, primarily based on level 4–5 evidence. These recommendations provide detailed descriptions of the definition of LGEA, treatment techniques, outcomes and future directions of research. Conclusions: Evidence supporting best practices for LGEA is currently low quality. This review provides best recommendations based on a critical evaluation of the available literature. Based on the lack of strong evidence, prospective and comparative research is clearly needed. Type of study: Treatment study, prognosis study and study of diagnostic test. Level of evidence: Level II–V.