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Congenital atresia of the esophagus and tracheoesophageal fistula occur in 1 of every 2500 to 3000 live births. Other congenital disorders of the esophagus, such as esophageal webs, strictures, duplications, and extrinsic vascular rings are far less common. Acquired esophageal lesions include strictures due to caustic injury, gastroesophageal reflux or eosinophilic esophagitis, and, rarely, diverticula.

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The embryologic mechanisms responsible for both normal and abnormal trachea and esophageal development are not fully elucidated. At day 26 or 27 of gestation, a ventral diverticulum is formed from the caudal end of the primitive pharyngeal foregut. This laryngotracheal diverticulum undergoes elongation and differentiation to eventually form the larynx, trachea, bronchi, and lungs. In order to separate the dorsal foregut (future esophagus) from the ventral laryngotracheal diverticulum, longitudinal tracheoesophageal folds fuse to form a septum that completely separates these structures (eFig. 392.1). It is believed that failure of these folds to completely form, or improper timing of their formation, leads to the anomalies of esophageal atresia and tracheoesophageal fistula (TEF).

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eFigure 392.1.
Graphic Jump Location

Schematic diagram demonstrating the development of the laryngotracheal diverticulum from the primitive pharyngeal foregut. Cross-sectional views show the tracheoesophageal folds creating a septum dividing the trachea and esophagus.

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The genetic factors that lead to malformation of the trachea and esophagus have not yet been elucidated. There is a small increased risk of TEF of about 2% if there is an affected sibling. There are associated anomalies, with cardiac malformations being the most common, in about half of the cases of esophageal atresia (syndromatic esophageal atresia). In the remainder (nonsyndromic cases), esophageal atresia and tracheoesophageal fistula occur in isolation. The specific genes and signaling pathways that lead to esophageal malformations are yet to be determined.1

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William Durston first described isolated esophageal atresia (EA) in 1670. He later described 40 cases of EA and tracheoesophageal fistula (TEF) and astutely noted that the fistulas typically arose just proximal to the carinal bifurcation. In 1697, Thomas Gibson was first to describe EA with TEF. Gibson was summoned to care for an infant with aspiration associated with all feeding attempts. The infant died, and on autopsy Gibson noted the EA and TEF. It was not until the 1900s that operative interventions were attempted. The first survivors were reported by William Ladd and N. Logan Leven in 1939. Both used a staged approach that consisted of fistula ligation, cervical esophagostomy, and gastrostomy tube placement. Later esophageal continuity was established via an antethoracic esophagus.2,3 Cameron Haight performed the first successful extrapleural primary repair of an EA and TEF in 1941.4

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William Durston first described isolated esophageal atresia (EA) in 1670. He later described 40 cases of EA and tracheoesophageal fistula (TEF) and astutely noted that the fistulas typically arose just proximal to the carinal bifurcation. In 1697, Thomas Gibson was first ...

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