To characterize a successful approach to the management of infants with long-gap esophageal atresia (EA) with tracheoesophageal fistula (TEF), significant prematurity with respiratory distress syndrome (RDS), or both, so as to preserve the native esophagus.
A review of the medical records and office charts of a cohort of patients with EA and TEF.
A tertiary care children's hospital affiliated with a major university.
A total of 118 children with EA and TEF admitted from February 1986 through December 1996. All of the patients diagnosed as having EA and TEF during this period were included.
Of the 118 infants, 88 received primary repair of EA and TEF within 48 hours of birth. An additional 23 children had the TEF divided and a gastrostomy placed secondary to (1) severe RDS and prematurity (n = 6), (2) long-gap EA (gap length >4 cm or the upper pouch above the thoracic inlet (n = 10), or (3) associated cardiac defects (n = 7). Delayed primary EA repair was done when the RDS resolved or the gap length was 2 cm or less.
Main Outcome Measures
Successful anastomosis of native esophagus. Comparison of incidence of gastroesophageal reflux, anastomotic complications, or survival between groups undergoing primary or delayed repair.
Primary EA was accomplished in 88 patients. Delayed EA was successfully accomplished in 18 of the 19 surviving patients within 5 months, thereby preserving the native esophagus in all surviving infants. There was no difference in anastomotic complications, gastroesophageal reflux, or survival when the delayed group was compared with those who had a primary repair.
Using delayed EA repair, all children with EA and TEF, regardless of gap length, can have their esophagus preserved. The primary cause of mortality was the association of a severe cardiac anomaly with EA and TEF.