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Congenital anomalies of the intrathoracic airways and tracheoesophageal fistula

Christopher M Oermann, MD
Section Editor
Gregory Redding, MD
Deputy Editor
Alison G Hoppin, MD


Congenital anomalies of the respiratory tract include malformations located anywhere from the nose to the alveoli. Anomalies of the intrathoracic airways, including the trachea, bronchi, and lower airways, are reviewed here. Disorders of the upper respiratory tract are discussed separately. (See "Congenital anomalies of the nose" and "Congenital anomalies of the jaw, mouth, oral cavity, and pharynx".)


Tracheal atresia, also known as agenesis or aplasia of the trachea, is a rare, usually lethal disorder [1]. The defect consists of partial or complete absence of the trachea below the larynx. The lower respiratory tract often is connected to the gastrointestinal tract via a distal tracheo- or bronchoesophageal fistula. Lack of a fistula between the airway and esophagus is incompatible with life [2,3].

Tracheal atresia is classified based upon the presence and extent of the tracheal remnant [4-8]. Several classification systems have been proposed; the most widely applied recognizes three distinct lesions [9]:

Type I – a short segment of trachea connects to the anterior esophagus

Type II – the airway and esophagus are fused at the level of the carina, with no tracheal remnant

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Literature review current through: Nov 2017. | This topic last updated: Nov 22, 2017.
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