Long-gap oesophageal atresia.

The various options for the management of long-gap oesophageal atresia are discussed. Of 89 infants treated, 27 had isolated atresia, 6 distal atresia with proximal fistula, and 56 atresia with distal fistula. The preferred approach for the former two groups was oesophageal replacement via gastric transposition. Recently, delayed primary anastomosis has been attempted after 6-12 weeks. For wide-gap atresia with distal fistula, primary anastomosis under marked or extreme tension with elective paralysis and mechanical ventilation for 5 days postoperatively achieved highly successful results in 39 infants. There were no major anastomotic disruptions and only 7 minor leaks. Strictures developed in 72% of cases and gastro-oesophageal reflux in 54%, 66% of whom required antireflux surgery. There was only 1 death in the 43 patients undergoing oesophageal replacement (none after gastric transposition, n = 34). A highly satisfactory outcome was achieved in 85%-90% of infants undergoing a replacement procedure.