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Essentials of Diagnosis

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  • Coughing, cyanosis, or vomiting with onset of feeds.
  • Association with VACTREL.
  • Inability to pass the feeding tube.
  • Orogastric tube curled in upper chest or neck on chest -x-ray.
  • Intestinal gas indicates esophageal atresia with tracheoesophageal fistula; no gas represents isolated esophageal atresia.

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General Considerations

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Esophageal atresia and tracheoesophageal fistula (TEF) have a prevalence of 1 in 3000 live births. The male-to-female ratio is equal. Infants with these conditions are often premature, and polyhydramnios is commonly diagnosed prenatally.

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Classification

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Esophageal atresia and TEF are classified based on the presence of atresia and the relation of the fistula location to the atresia. Older classification methods have been replaced with anatomical descriptions (Figure 35–1). The incidence of these two conditions is found in Table 35–1.

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Figure 35–1.
Graphic Jump Location

Types of esophageal atresia and tracheoesophageal fistula. (A) Type 1, esophageal atresia with distal tracheoesophageal fistula; (B) Type 2, esophageal atresia without tracheoesophageal fistula; (C) Type 3, tracheoesophageal fistula without esophageal atresia; (D) Type 4, esophageal atresia with proximal and distal tracheoesophageal fistula; and (E) Type 5, esophageal atresia with proximal tracheoesophageal fistula.

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Table Graphic Jump Location
Table 35–1. Incidence of Esophageal Atresia and Tracheoesophageal Fistula (TEF).
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Types of esophageal atresia and tracheoesophageal fistula: (A) Type 1, esophageal atresia with distal tracheoesophageal fistula; (B) Type 2, esophageal atresia without tracheoesophageal fistula; (C) Type 3, TEF without esophageal atresia; (D) Type 4, esophageal atresia with proximal and distal tracheoesophageal fistula; and (E) Type 5, esophageal atresia with proximal tracheoesophageal fistula.

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Type 1

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Esophageal atresia with a distal TEF is the most common anomaly, comprising 85.4% cases. The lower esophageal segment begins as a fistula that arises from the distal trachea near the carina. The proximal esophageal pouch is found as a blind-ending segment near the thoracic inlet. The blood supply to the superior esophageal segment is via the thyrocervical trunk, whereas branches of the gastric arteries supply the distal esophageal segment.

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Type 2

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Isolated esophageal atresia comprises 7.3% of cases. The lower pouch is usually only 1–2 cm above the diaphragm, whereas the upper pouch ends near the thoracic inlet, creating a long gap between the two ends that can complicate repair. This anomaly does not allow amniotic fluid to pass to the remainder of the developing gut, explaining the finding of polyhydraminos prenatally. However, esophageal atresia with a relatively narrow distal TEF can produce similar findings.

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Type 3

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