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The term tracheoesophageal fistula (TEF) describes a communication between the gastrointestinal tract and the airway. This defect can present at birth as a congenital anomaly or later in life as an acquired pathology secondary to trauma, malignancy, or inflammation. Management of TEF requires expedient diagnosis with thoughtful planning and implementation of tailored single or multistage therapy. As in all diseases of the esophagus and trachea, patient outcome depends on a clear understanding of the pathophysiology and anatomy of the disease, expert treatment, and sound surgical technique. This chapter focuses on the management of both congenital and acquired TEF.


Congenital TEF is most frequently associated with esophageal atresia (EA). Thomas Durston first described EA in 1670. In 1696, Thomas Gibson described TEF with EA. However, it was not until 1939 that Thomas Lanman and Logan Leven reported a successful staged repair. This was soon followed by the first report of a primary repair by Cameron Haight in 1941.1

Congenital TEF occurs in about 1 of every 3000 to 4000 live births.2 The prevalence increases with advancing maternal age and maternal diabetes, and there is a genetic predisposition in children born to affected parents.3 TEF and EA are believed to develop during the fourth week of gestation after the lung bud has begun to separate from the foregut. This is usually followed by separation of the trachea from the esophagus, which begins at the level of the carina and moves cephalad toward the larynx. It is postulated that abnormal endoderm–mesoderm interactions that occur early in development cause incorrect signaling and inappropriate separation and development of the tracheobronchial tree and esophagus.4

Diagnosis and Preoperative Assessment

TEF and EA were first classified by Gross with a lettering system and Ladd with a numbering system. It is now more common to describe them in terms of the anatomic abnormalities of the esophagus and trachea. The most common variant is EA with distal TEF (85%) (Fig. 58-1A), followed by EA alone without TEF (7%) (Fig. 58-1B) and TEF alone without EA (N- or H-type) (4%) (Fig. 58-1C). Other very rare variants include EA with proximal TEF (Fig. 58-1D) and EA with proximal and distal TEF (Fig. 58-1E).1,2

With advances in surgical management of TEF and EA, neonates who undergo successful repair can be expected to live near-normal lives. However, both TEF and EA are associated with other congenital abnormalities in 30% to 50% of patients, and these anomalies can have a significant impact on long-term outcomes. Associated defects include cardiac, gastrointestinal, neurologic, skeletal, and genitourinary. The most common cardiac defects are atrial and ventricular septal defects, patent ductus arteriosus, tetralogy of Fallot, and aortic arch abnormalities.5 The most common gastrointestinal abnormality is imperforate anus, which occurs ...

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