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INTRODUCTION: A long gap between the esophageal pouches in esophageal atresia is a relatively rare finding; it adds a
significant challenge in the surgical correction and final outcome.
MATERIAL AND METHODS: We retrospectively reviewed all cases of long gap esophageal atresia managed at our institution
between 2000 and 2006, focusing on antenatal period, delivery weight, maurity, associated malformations, initial
management and definitive repair.
RESULTS: Sixteen patients with esophageal atresia were observed. Five of them (31%) presented a long gap. Delayed
reconstruction was achieved in 4 children at an average age of 63 days (range 40-95 days). Primary repair was possible
at birth in one case. All children had a direct anastomosis with one or more Livaditis myotomies in four cases.
DISCUSSION: Surgical management of long gap esophageal atresia remains controversial. Most authors believe that elongation
of the native esophagus provides a better functional outcome. In particular conditions, when this goal cannot be
achieved, esophageal substitution is the last resort.
Many techniques have been proposed to obtain esophageal elongation; although all the procedures give acceptable results,
none of them has been unanimously accepted by pediatric surgeons. The circular or spiral myotomy is still the most commonly
used technique to lengthen the esophagus in the repair of long gap atresias.
CONCLUSIONS: In our limited experience circular myotomy (single or double) has proven an effective and reliable technique
which allows direct repair of the esophagus even in the presence of initial gaps of 4 vertebral bodies.
