Position Paper of INoEA Working Group on Long-Gap Esophageal Atresia: For Better Care.

INoEA is the International Network of Esophageal Atresia and consists of a broad spectrum of pediatric specialties and patient societies. The working group on long-gap esophageal atresia (LGEA) set out to develop guidelines regarding the definition of LGEA, the best diagnostic and treatment strategies, and highlight the necessity of experience and communication in the management of these challenging patients. Review of the literature and expert discussion concluded that LGEA should be defined as any esophageal atresia (EA) that has no intra-abdominal air, realizing that this defines EA with no distal tracheoesophageal fistula (TEF). LGEA is considerably more complex than EA with distal TEFs and should be referred to a center of expertise. The first choice is to preserve the native esophagus and pursue primary repair, delayed primary anastomosis, or traction/growth techniques to achieve anastomosis. A cervical esophagostomy should be avoided if possible. Only if primary anastomosis is not possible, replacement techniques should be used. Jejunal interposition is proposed as the best option among the major EA centers. In light of the infrequent occurrence of LGEA and the technically demanding techniques involved to achieve esophageal continuity, it is strongly advised to develop regional or national centers of expertise for the management and follow-up of these very complex patients.