Perioperative management and outcomes in long-gap esophageal atresia: A retrospective analysis from the Eastern Pediatric Surgery Network

Abstract

BackgroundLess than 10% of infants born with esophageal atresia (EA) have a long gap that precludes primary anastomosis at birth. The purpose of this study was to evaluate the diagnosis, management, and outcomes of infants with long-gap esophageal atresia (LGEA) within a regional consortium of children's hospitals. MethodsAfter IRB approval, a multicenter, retrospective cohort study was conducted of LGEA patients managed by 13 member institutions between 2009 and 2018. LGEA was defined as Type A or B esophageal atresia with the inability to perform a primary esophageal anastomosis at birth based on preoperative imaging or operating findings. Study outcomes, including operative repair, postoperative outcomes, and complications, were collected to detect significant associations between variables (p < 0.05). ResultsThere were 62 LGEA patients identified, including 50 (81%) with Type A and twelve (19%) with Type B esophageal atresia. Most (77%) were diagnosed prenatally with 98% undergoing a gastrostomy before attempted EA repair. The mean gap length at repair was 3.24 ± 1.59 cm (n = 21). Most (95%) were managed with delayed repair (median age at repair of 96 days (IQR: 67.5-131), and 22 (35%) underwent an esophageal traction-induced lengthening process. Two (3.2%) required esophageal replacement. There was no significant difference in complications between different treatment strategies. ConclusionsIn this collaborative descriptive study of 62 infants with LGEA, delayed primary repair with or without traction was the preferred approach, with outcomes that were comparable between strategies, and with high rates of esophageal preservation. We encountered variability in gap length measurement and reporting. This study highlights a critical need for a prospective, multi-institutional registry with uniform care pathways to help aid in the development of evidence-based guidelines for these challenging patients.