Background and aims: Esophageal atresia (EA) with or without tracheo-esophageal fistula (TEF) is a rare condition that can be nowadays succesfully treated. The current interest therefore is focused on the management of the difficult cases, on thoracoscopic approach, and on some aspects of the long-term results.
Methods: The current strategies for the difficult or impossible anastomoses in pure and long-gap EA, the introduction of thoracoscopic repair and the causes, mechanisms and management of post-operative gastro-esophageal reflux (GER) are reviewed.
Results: Methods of esophageal elongation and multi-staged repair of pure and long-gap EA allow anastomosis but with functional results that are often poor. Esophageal replacement with colon or stomach achieves at least similar results and often requires less procedures. Thoracoscopic repair is a promising adjunct, but the difficulties for setting it as a gold-standard are pointed out. GER is a part of the disease and its surgical treatment, that is often required, is burdened by high failure rates.
Conclusions: EA with or without TEF can be successfully treated in most cases, but a number of unsolved issues remain and the current approach to difficult cases will certainly evolve in the future.