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  • Title: Tracheal agenesis and esophageal atresia with proximal and distal bronchoesophageal fistulas.
    Author: Demircan M, Aksoy T, Ceran C, Kafkasli A.
    Journal: J Pediatr Surg; 2008 Aug; 43(8):e1-3. PubMed ID: 18675618.
    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.
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