To determine whether or not a true primary repair, without myotomies and with the gastroesophageal junction below the diaphragm, can be accomplished across the esophageal atresia (EA) spectrum. Our hypothesis is that the esophageal anastomosis can withstand significant tension. The consequences, particularly for those patients with a very long gap atresia, were assessed.Summary of Background Data
Difficulties arise roughly in proportion to the size of the gap between esophageal segments. Reported surgical complications remain frequent, and particularly at the far end of the EA spectrum, not all children are left with a satisfactorily functioning esophagus or esophageal substitute.Methods
The outcomes of all infants who had a true primary repair of EA from 1976-1997 were determined. Surgically, the methods used to achieve a reliable true primary repair were expanded to accomplish this, even for a very long gap EA.Results
From 1976-97, 70 infants with or without associated tracheoesophageal fistula (TEF) had primary repairs performed with no surgery-related deaths and 11% later deaths. No interpositions were performed since 1983. There were no discernible anastomotic leaks and one late recurrent TEF related to the early use of balloon dilation. Ten infants had gaps of 5.0-6.8 cm and, among these, four had gaps of 5.5-6.8 cm that could not be pulled together initially. Traction sutures in the esophageal ends, however, produced sufficient lengthening within 6-10 days for a true primary repair. Very long gap repairs produced more reflux (10 of 10 required a fundoplication versus 24 of 70 overall) and more dilations to relieve strictures. Two infants underwent stricture resection with no recurrence. On follow-up, all patients over 2 years of age were eating well or satisfactorily, and none had a gastrostomy tube.Conclusions
(1) The esophageal anastomosis can withstand considerable tension and allows a reliable true primary repair for the full EA spectrum. (2) Growth is rapid and traction sutures will produce significant esophageal lengthening within days. (3) With increasing tension, gastroesophageal reflux (GER) and strictures are more common; however, both are treatable. Follow-up reveals the benefits of true primary repair over other solutions.