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Key Concepts

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  • Epidemiology

    • Esophageal atresia and tracheoesophageal fistula (EA–TEF) occurs in 1 in 3400. Esophageal duplication cysts represent 10 to 15 percent of foregut duplication cysts and 5 to 10 percent of mediastinal cysts. The true incidence of esophageal duplication cysts is unknown. Congenital esophageal stenosis and webs occur in 1 in 25,000 to 50,000 live births.

  • Classification

    • Anatomic variations of EA–TEF include isolated EA, EA with distal TEF, EA with proximal TEF, EA with distal and proximal TEF, or N-type TEF. EA with distal TEF is the most common. Esophageal duplication cysts can be cervical, thoracic, or abdominal. Esophageal stenosis and webs can be characterized on the basis of etiology into fibromuscular thickening, trachobronchial remnants, and membranous web.

  • Clinical features

    • EA–TEF is suggested by polyhydramnios, and infants present with drooling, rhonchi, aspiration with subsequent respiratory distress. Esophageal duplication cysts are often asymptomatic. Symptoms due to esophageal duplication cysts are secondary to mass effect and include dysphagia and respiratory insufficiency. Congenital esophageal stenosis and webs present in infancy with dysphagia and vomiting of solid food.

  • Diagnosis

    • EA–TEF is readily diagnosed with passage of a 10-F esophageal tube and gentle insufflation, and plain chest radiograph. The presence of air in the stomach confirms a distal TEF, and its absence suggests isolated EA. Esophageal duplication cysts are also diagnosed on plain film and confirmed with esophagography and CT. Esophageal stenosis and web are diagnosed with esophagography and endoscopy.

  • Treatment

    • Surgical repair of EA–TEF consists of a single-layer primary anastomosis of the proximal and distal esophageal segments and ligation of any TEF. Esophageal duplication cysts are resected. Esophageal stenosis and webs are initially managed conservatively with dilatation. Surgical treatment is reserved for stenosis refractory to dilatation and consists of resection of the stenotic segment and primary anastomosis.

  • Complications

    • Outcomes following repair of EA–TEF are good. Early complications include anastomotic leak and subsequent sepsis. Mid-to-late-term complications include stricture, recurrent fistula, and gastroesophageal reflux.

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Esophageal Atresia and Tracheoesophageal Fistula

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Epidemiology

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The incidence of esophageal atresia (EA), with or without tracheoesophageal fistula (TEF), is approximately 1 in 3400.1 There is a reported slight preponderance in males and children of diabetic or older mothers.2 Chromosomal anomalies have been reported in 6 to 10 percent of cases.3 Although EA–TEF can be an isolated finding, epidemiologic studies suggest that up to 60 percent will have other anomalies.2 Some will present with other anomalies that lead to a specific genetic diagnosis, whereas others will present with a nonrandom association of other defects. These other defects include coloboma, heart, atresia choanae, retarded development, genital hypoplasia, and ear abnormalities (CHARGE associations), as well as vertebral, anal, cardiac, tracheoesophageal fistula, renal, and limb defects (VACTERL).

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Embryology

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EA constitutes a spectrum of incomplete esophageal development, which arises from interruptions in endoderm–mesoderm interactions. Although the exact embryologic mechanism of EA–TEF is not well characterized, some of ...

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