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Key Points

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  1. Most cases of esophageal atresia and tracheoesophageal fistula (EA/TEF) occur sporadically. In 50% to 80% of cases, there are associated anomalies, most frequently musculoskeletal malformations, followed by cardiovascular, genitourinary, gastrointestinal, and chromosomal anomalies.

  2. Unlike other congenital malformations, EA is infrequently diagnosed prenatally.

  3. Polyhydramnios is present in 95% of patients with isolated EA without TEF, and in 35% of cases with distal TEF.

  4. All infants with EA should have an echocardiogram before operative repair because of the high incidence of congenital cardiac anomalies.

  5. In neonates with EA/TEF, the diagnosis can be confirmed by tracheobronchoscopy and/or esophagoscopy.

  6. Surgical repair should be done soon after birth. It is not an emergency procedure, but neonates requiring mechanical ventilation should be operated on promptly to avoid gastric distension and possible perforation.

  7. Physiological stable neonates with EA and TEF can be managed with a single operation to divide the distal fistula and perform the esophageal anastomosis.

  8. In 10% of neonates with EA and TEF who are very premature or physiological unstable, ligation of the TEF is performed first and the esophageal anastomosis is done as a secondary stage repair when the baby is stable.

  9. Thoracoscopic repair offers a scarless repair with faster recovery and less postoperative pain and may also potentially protect from later problems such as winged scapula and thoracic asymmetry.

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Introduction

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Esophageal atresia and tracheoesophageal fistula (EA/TEF) constitute a relatively rare malformation, affecting approximately 1 in 2500 to 4500 live births with a slight preponderance of males in the ratio of 3:2. It was first described in 1670 by Durston in a set of conjoined twins. This was followed by a report of EA with TEF by Thomas Gibson in his landmark treatise, The Anatomy of Human Bodies Epitomized, published in 1684.

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In 1913, Richter proposed a plan of management that involved dividing the TEF, feeding the neonate via a gastrostomy, and performing a delayed esophageal anastomosis when difficulties had been overcome. In 1939, William Ladd and N. Logan Leven were independently the first to achieve long-term survival for children with EA/TEF, but only by a staged approach. The first successful primary repair of an EA was performed by Cameron Haight on March 15, 1941, at the University of Michigan. This was accomplished through a left thoracotomy, which is now used only if a right-sided aortic arch is found on preoperative echocardiography. Haight's success was soon followed by others and survival increased, reaching approximately 75% in late 1960 and almost 100% today in children with no severe associated anomalies.

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Embryology

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The pathogenesis of EA remains unknown, and because most cases of EA occur sporadically, it is highly unlikely that a simple, inheritable genetic mechanism is responsible. The parallel development of both teratogenic and genetic models of EA/TEF has demonstrated that the fundamental developmental aberration appears to be the disturbance of the process of separating the foregut into ...

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