Long gap esophageal atresia with or without tracheoesophageal fistula: several management options but a continuous challenge

Background: The management of long gap esophageal atresia/tracheo-esophageal fistula (EA/TEF) continue to challenge pediatric surgeons and although there were several advances in its management, none of the available options is ideal. Patient and Methods: This is a retrospective chart review of 42 patients with long gap esophageal atresia treated over a 20 years period. The review included age at diagnosis, sex, type of esophageal atresia, associated anomalies and most importantly the methods of repair and their outcomes. Results: Out of 42 patients with long gap EA with or without TEF, 23 patients were treated by primary repair of their native esophagus, 15 had esophageal substitution and 4 died before any definitive treatment was performed. Conclusions: The management of long gap esophageal atresia is challenging and every attempt should be made to preserve the native esophagus. During The second half of the study period we adopted a more conservative approach with the aim of avoiding esophageal replacement using primary repair under severe tension or delayed primary repair after a period of observation. Although this approach is associated with a high incidence of esophageal stricture, we found it to be a better alternative to esophageal replacement in patients with long gap esophageal atresia.