Primary repair without routine gastrostomy is the treatment of choice for neonates with esophageal atresia and tracheoesophageal fistula.

Gastrostomy and staged repair are techniques frequently recommended for the management of esophageal atresia with distal tracheoesophageal fistula (EA-TEF), especially for those infants at high risk. We describe 42 consecutive patients with EA-TEF treated during the past 8 years. Staged repair and preliminary gastrostomy were not routinely employed. Fifteen infants were considered to be at high risk (Waterston class C). Surgical treatment via an extrapleural approach consisted of fistula division and primary single-layer end-to-end esophageal anastomosis. Four patients required proximal esophageal circular myotomy. Four patients early in the series received a gastrostomy at or before definitive repair for various life-threatening indications. One patient had fistula division only and died before esophageal anastomosis was possible. Two neonates died before repair and another died after repair. The deaths in this series of patients were unrelated to EA-TEF. One patient developed a clinically significant anastomotic leak. Four patients required multiple dilatations for anastomotic stricture. Fundoplication was necessary in 3 patients with symptomatic gastroesophageal reflux. Our data demonstrate that excellent overall survival (90%) with low morbidity (15%) can be achieved using primary repair without preliminary gastrostomy in most neonates with EA-TEF. We believe that mortality in high-risk patients with EA-TEF is due to associated life-threatening anomalies.