Abstract
The operative management of patients born with long-gap esophageal atresia (LGEA) remains a major challenge for most pediatric surgeons, due to the rarity and complex nature of this malformation. In LGEA, the distance between the proximal and distal esophageal end is too wide, making a primary anastomosis often impossible. Still, every effort should be made to preserve the native esophagus as no other conduit can replace its function in transporting food from the oral cavity to the stomach satisfactorily. In 1981, Puri et al. observed that in newborns with LGEA spontaneous growth and hypertrophy of the two segments occur at a rate faster than overall somatic growth in the absence of any form of mechanical stretching, traction or bouginage. They further noted that maximal natural growth arises in the first 8-12 weeks of life, stimulated by the swallowing reflex and reflux of gastric contents into the lower esophageal pouch. Since then, creation of an initial gastrostomy and continuous suction of the upper esophageal pouch followed by delayed primary anastomosis at approximately 3 months of age has been widely accepted as the preferred treatment option in most LGEA cases, generally providing good functional results. The current article offers a comprehensive update on the various aspects and challenges of this technique including initial preoperative management and subsequent gap assessment, while also discussing potential postoperative complications and long-term outcome.