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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 TEF or 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 multistaged 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. Thomas Gibson first described TEF with EA in 1696. 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 3000–4000 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 the anatomic abnormalities of the esophagus and trachea. The most common variant is EA with distal TEF (85%), followed by EA alone without TEF (7%) and TEF alone without EA (N- or H-type) (4%). Other very rare variants include EA with proximal TEF and EA with proximal and distal TEF1,2 (Fig. 49-1).

Figure 49-1.
Graphic Jump Location

EA with distal TEF (85%) is the most common variant, followed by EA alone without TEF (7%) and TEF alone without EA (N- or H-type) (4%). Other very rare variants include EA with proximal TEF and EA with proximal and distal TEF.


With these advances in surgical management of TEF and EA, neonates who undergo successful repair can be expected to live normal lives. However, TEF/EA is associated with other congenital abnormalities in 30–50% of patients that 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 ...

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