Management of long-gap esophageal atresia

A uniquely challenging subset of infants diagnosed with esophageal atresia (EA) are those born with long-gap EA (LGEA). The common unifying feature in infants with LGEA is that the proximal and distal segments of the esophagus are too far apart to enable primary anastomosis via a single operation in the newborn period. Although any type of EA can technically result in a long gap, the Gross type A variant occurs in 8% of all EA cases and is most commonly associated with LGEA. In this review, we provide an evidence-based approach to the current challenges and management strategies employed in LGEA. There are fortunately a range of available surgical techniques for LGEA repair, including delayed primary repair, staged repair based on longitudinal traction strategies to lengthen both ends (e.g., Foker procedure, internal traction), and esophageal replacement using other portions of the gastrointestinal tract. The literature on the management of LGEA has long been dominated by single-center retrospective reviews, but the field has recently witnessed increased multi-center collaboration that has helped to increase our understanding on how to best manage this challenging patient population. Delayed primary repair is strongly preferred as the initial approach in management of LGEA in the United States as well as several European countries and is supported by the American Pediatric Surgery Association recommendations. Should esophageal replacement be required in cases where salvaging the native thoracic esophagus is not possible, gastric conduits are the preferred approach, based on the relative simplicity of the operation, low postoperative morbidity, and long-term durability. Long-term followup for monitoring of swallowing function, nutritional status, aspiration/respiratory illnesses, gastroesophageal reflux, and associated comorbidities is essential in the comprehensive care of these complex patients.