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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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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 to ...