DELAYED PRIMARY REPAIR OF ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL FISTULA - IS IT WORTH THE WAIT

Objective: To characterize a successful approach to the management of infants with long-gap esophageal atresia (EA) with tracheoesophageal f istula (TEF), significant prematurity with respiratory distress syndro me (RDS), or both, so as to preserve the native esophagus. Design: A r eview of the medical records and office charts of a cohort of patients with EA and TEF. Setting: A tertiary care children's hospital affilia ted with a major university. Patients: 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 inclu ded. Intervention: Of the 118 infants, 88 received primary repair of E A 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 p rematurity (n = 6), (2) long-gap EA (gap length >4 cm or the upper pou ch above the thoracic inlet (n = 10), or (3) associated cardiac defect s (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 an astomosis of native esophagus. Comparison of incidence of gastro esoph ageal reflux; anastomotic complications, or survival between groups un dergoing primary or delayed repair. Results: Primary EA was accomplish ed in 88 patients. Delayed EA was successfully accomplished in 18 of t he 19 surviving patients within 5 months, thereby preserving the nativ e esophagus in all surviving infants. There was no difference in anast omotic complications, gastroesophageal reflux, or survival when the de layed group was compared with those who had a primary repair. Conclusi ons: 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 a nd TEF.