Analysis of morbidity and mortality in 227 cases of esophageal atresia and/or tracheoesophageal fistula over two decades

Abstract

Objective: This report analyzes the morbidity and mortality in 227 infants (127 boys and 100 girls) with variants of esophageal atresia and/or tracheoesophageal fistula who were treated from 1971 to 1993.

Design: Data were collected retrospectively from hospital and office records. Mean follow-up was 76 months, ranging from 1 month to 22 years.

Setting: Patients were treated at a tertiary care children's hospital.

Results: The mean birth weight was 2557 g (range, 1100 to 4460 g), and the mean gestational age was 38 weeks (range, 28 to 42 weeks). Classification included 29 cases of type A esophageal atresia (13%); two cases of type B (1%), 178 cases of type C (78%), five cases of type D (2%), and 13 cases of type E (6%). Associated anomalies occurred in 146 infants (64%), including cardiac defects in 86 (38%), skeletal defects in 44 (19%), neurological defects in 34 (15%), renal defects in 35 (15%), anorectal defects in 18 (8%), and other abnormalities in 30 (13%). A single-layer anastomosis was performed in 81%, and a two-layer repair, in 17%. Esophagomyotomy was necessary in 9% of the patients. Anastomotic complications included leakage (16%), symptomatic stricture (35%), and recurrent tracheoesophageal fistula (3%). Gastroesophageal reflux was present in 127 cases (58%), with 56 (44%) requiring an antireflux procedure. Tracheomalacia occurred in 32 cases (15%), and 13 required operative treatment. Postoperative esophageal dysmotility was documented in 56 children (30%). The overall survival rate was 95%. The cause of death in 12 patients included severe cardiac anomalies (n = 3), fatal sleep apnea (n = 1), renal failure (n = 1), trisomy 18 (n = 2), accidental decannulation of tracheostomy (n = 1), pulmonary failure (n = 1), and unknown causes (n = 3).

Conclusions: Early diagnosis, improved surgical technique, neonatal anesthesia, sophisticated ventilatory support, advanced intensive care management, early treatment of associated anomalies, responsiveness of anastomotic strictures to dilatation, and aggressive treatment of gastroesophageal reflux have influenced survival positively. Improved survival rates were noted irrespective of the traditional Waterston criteria, which now seem outdated. With few exceptions, most infants with esophageal atresia and/or tracheoesophageal fistula should survive in the current era.