Rapid Communication| Volume 43, ISSUE 8, e1-e3, August 2008

Download started.


Tracheal agenesis and esophageal atresia with proximal and distal bronchoesophageal fistulas


      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.

      Key words

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Journal of Pediatric Surgery
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Pratap A.
        • Saha G.S.
        • Bhattarai B.K.
        • et al.
        Tracheal agenesis type B: further evidence to a lethal congenital tracheal malformation.
        J Pediatr Surg. 2007; 42: 1284-1287
        • Spitz L.
        Oesophageal atresia.
        Orphanet J Rare Dis. 2007; 2 ([Review]): 24
        • Payne W.A.
        Congenital absence of trachea.
        Brooklyn Med J. 1900; 14: 568
        • Das B.B.
        • Nagaraj A.
        • Rao A.H.
        • et al.
        Tracheal agenesis: report of three cases and review of the literature.
        Am J Perinatol. 2002; 19: 395-400
        • Faro R.S.
        • Goodwin C.D.
        • Organ Jr., C.H.
        • et al.
        Tracheal agenesis..
        Ann Thorac Surg. 1979; 28: 295-299
        • Kluth D.
        • Steding G.
        • Seidl W.
        The embryology of foregut malformations.
        J Pediatr Surg. 1987; 22: 389-393
        • Evans J.A.
        • Reggin J.
        • Greenberg C.
        Tracheal agenesis and associated malformations: a comparison with tracheoesophageal fistula and the VACTERL association.
        Am J Med Genet. 1985; 21: 21-38
        • Evans J.A.
        • Greenberg C.R.
        • Erdile L.
        Tracheal agenesis revisited: analysis of associated anomalies.
        Am J Med Genet. 1999; 82 ([Review]): 415-422
        • Buchino J.J.
        • Meagher Jr., D.P.
        • Cox J.A.
        Tracheal agenesis: a clinical approach.
        J Pediatr Surg. 1982; 17: 132-137
        • Effmann E.L.
        • Spackman T.J.
        • Berdon W.E.
        • et al.
        Tracheal agenesis.
        Am J Roentgenol Radium Ther Nucl Med. 1975; 125: 767-781
        • Statz T.
        • Lynch J.
        • Ortmann M.
        • et al.
        Tracheal agenesis: a case report.
        Eur J Pediatr. 1989; 149: 203-204
        • Hiyama E.
        • Yokoyama T.
        • Ichikawa T.
        • et al.
        Surgical management of tracheal agenesis.
        J Thorac Cardiovasc Surg. 1994; 108 ([discussion 1052]): 830-833
        • Jacobs J.P.
        • Quintessenza J.A.
        • Andrews T.
        • et al.
        Tracheal allograft reconstruction: the total North American and worldwide pediatric experiences.
        Ann Thorac Surg. 1999; 68: 1043-1051
        • Neville W.E.
        • Bolanowski P.J.
        • Soltanzadeh H.
        Prosthetic reconstruction of the trachea and carina.
        J Thorac Cardiovasc Surg. 1976; 72: 525-538