Primary repair of ultra-long-gap esophageal atresia: results without a lengthening procedure.

Ultra-long-gap esophageal atresia, defined as a gap length of 3.5 cm or greater, has proved difficult to repair. When primary repair has been attempted, even with bougienage, circular myotomy, or intraabdominal esophageal mobilization to lessen anastomotic tension, leaks, anastomotic disruptions, and recurrent tracheoesophageal fistulas are frequent. Consequently, interposition grafts are commonly used. For long-term function the intact native esophagus should be preferable to an interposition graft or the consequences of circular myotomy. Therefore, even when an ultra-long gap is present, we have carried out a primary repair using our single-layer technique without myotomies. Since 1979, 8 of 58 infants (14%) with esophageal atresia had gaps ranging from 3.5 to 6 cm. All had a primary repair with follow-up from 1 to 11 years. Despite severe anastomotic tension in all cases, there were no anastomotic leaks, disruptions, recurrent tracheoesophageal fistulas, or deaths. The tension, however, may have led to major gastroesophageal reflux in 5 of 8 patients (62.5%), all treated by a Nissen fundoplication, and a stricture in 4 of 8 infants (50%). Three strictures responded to dilation and one was resected. Now, all children are eating a normal diet for age. In conclusion, this technique has allowed primary repair of ultra-long-gap esophageal atresia. Although the severe tension may contribute to strictures needing dilation and gastroesophageal reflux requiring fundoplication, primary repair resulted in a clinically functional native esophagus.