title: Tracheal agenesis and esophageal atresia with proximal and distal bronchoesophageal fistulas.
abstract: 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 the 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.
authors.affiliation: Department of Pediatric Surgery, Inonu University, Medical School, 44315 Malatya, Turkey.
authors.forename: Mehmet
authors.initials: M
authors.lastname: Demircan
authors.forename: Tugrul
authors.initials: T
authors.lastname: Aksoy
authors.forename: Canan
authors.initials: C
authors.lastname: Ceran
authors.forename: Ayse
authors.initials: A
authors.lastname: Kafkasli
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000601:surgery*; D001983:Bronchial Fistula / Q000175:diagnosis / Q000601:surgery*; D004933:Esophageal Atresia / Q000175:diagnosis / Q000601:surgery*; D004937:Esophageal Fistula / Q000175:diagnosis / Q000601:surgery*; D017809:Fatal Outcome; D006323:Heart Arrest / Q000628:therapy; D006801:Humans; D007231:Infant, Newborn; D007431:Intraoperative Complications / Q000175:diagnosis / Q000628:therapy; D008297:Male; D018570:Risk Assessment; D014132:Trachea / Q000002:abnormalities*; D014133:Tracheal Diseases / Q000151:congenital / Q000601:surgery