Esophageal atresia (EA) is a rare condition requiring surgical repair to restore esophageal continuity. Long-gap esophageal atresia (LGEA) repairs are complicated by a lack of sufficient esophageal tissue at birth, precluding immediate primary repair. Multiple surgical techniques have been described involving delayed primary repair, traction techniques to lengthen native tissue, intestinal conduits, and placement of tissue-engineered grafts. The current literature is composed primarily of single-center retrospective reviews and does not establish a clear definition for LGEA, much less describe a single preferred procedure. To combat this lack of data, expert opinion consensus guidelines were established by the American Pediatric Surgical Association (APSA), as well as the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) and the International Network of Esophageal Atresia (INoEA). This chapter summarizes these recommendations for pre-operative evaluation, indications for various surgical techniques based on their relative advantages and limitations, and postoperative management.

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Management of Long-Gap Esophageal Atresia

  • Anne M. Sescleifer,
  • Shaun M. Kunisaki

摘要

Esophageal atresia (EA) is a rare condition requiring surgical repair to restore esophageal continuity. Long-gap esophageal atresia (LGEA) repairs are complicated by a lack of sufficient esophageal tissue at birth, precluding immediate primary repair. Multiple surgical techniques have been described involving delayed primary repair, traction techniques to lengthen native tissue, intestinal conduits, and placement of tissue-engineered grafts. The current literature is composed primarily of single-center retrospective reviews and does not establish a clear definition for LGEA, much less describe a single preferred procedure. To combat this lack of data, expert opinion consensus guidelines were established by the American Pediatric Surgical Association (APSA), as well as the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) and the International Network of Esophageal Atresia (INoEA). This chapter summarizes these recommendations for pre-operative evaluation, indications for various surgical techniques based on their relative advantages and limitations, and postoperative management.