Tracheal agenesis and esophageal atresia with proximal and distal bronchoesophageal fistulas

DEMİRCAN M. , Aksoy T., CERAN ÖZCAN C. , Kafkasli A.

JOURNAL OF PEDIATRIC SURGERY, vol.43, no.8, 2008 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 43 Issue: 8
  • Publication Date: 2008
  • Doi Number: 10.1016/j.jpedsurg.2008.04.015


Tracheal agenesis (TA) is an extremely rare, typically fatal congenital tracheal malformation. Lack of prenatal symptoms and emergent presentation usually lead to a failure to arrive at the correct diagnosis and manage die airway properly before the onset of irreversible cerebral anoxia. Esophageal atresia (EA) encompasses a group of congenital anomalies comprising an interruption of the continuity of the esophagus with or without a persistent communication with the trachea, In 86% of cases, there is a distal tracheoesophageal fistula (TEF); in 7%, there is no fistulous connection, whereas in 4%, there is a TEF without atresia. We report the case of an infant born with TA and EA with proximal and distal bronchoesophageal fistulas. During 3 consecutive antenatal ultrasound examinations, there had been polyhydramniosis, difficulty visualizing the stomach, and dilatation of proximal esophagus, leading to a presumptive diagnosis of EA. The clinical presentation, embryology, classification, and surgical management are discussed. (C) 2008 Elsevier Inc. All rights reserved.