The management of long gap esophageal atresia

Present management of esophageal atresia has enabled the survival rate to approach 95%. Controversy remains concerning the many options for the surgical management of long gap esophageal atresia without tracheoesophageal fistula and represents the difficulty of this pathology. In the last couple of years, we have had a nonexplained outbreak of cases of long gap esophageal atresia without tracheoesophageal fistula. This article reports our experience in the management of these children.

It is a retrospective study of all cases of long gap esophageal atresia without tracheoesophageal fistula managed in our institution since 1992, focusing on the antenatal period, delivery with weight and term, the associated malformations, the initial management, and the definitive surgery. Mann-Whitney U test was used for statistical analysis.

Ten cases (8.7%) of long gap esophageal atresia according to Ladd's classification, 6 during the past 2 years, were taken in charge at Robert Debré Hospital between 1992 and 2002. There were 4 girls and 6 boys. Ten had a prenatal diagnosis of esophageal atresia. The average birth weight was 2496 g (range, 1400-3400 g) with an average term of 36.6-week gestation (range, 31.5-39.6). Delayed reconstruction was done in all children between 41 and 147 days of life (average of 102 days). Six had a direct anastomosis and 4 had a colonic esophagoplasty (3 with an esogastric disconnection during the same procedure). The average follow-up was 60 months (range, 27-133). There was 1 death owing to adenovirus infection at 5 years of age. Four children required a Nissen fundoplication for severe gastroesophageal reflux. At least, 2 children presented an anastomotic stricture which required pneumatic dilatations.

Treatment options for long gap esophageal atresia generally require several stages over several months. We propose, for their management, a direct anastomosis at 4 months of age whenever it is possible. If not, we use a colonic esophagoplasty with an esogastric disconnection to control the gastroesophageal reflux which is responsible for strictures and respiratory impairment and does not obstruct the aperistaltic tube.