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  • Coughing, cyanosis, or vomiting with onset of feeds.

  • Inability to pass an orogastric 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.

  • Association with VACTERL.

General Considerations

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.


Esophageal atresia and TEF are classified based on the presence of atresia and the relation of the fistula location to the atresia. The most commonly used classification system, developed by Dr. Robert Gross, uses the letters A to E to distinguish the different types (Figure 37–1). The incidence of each type is found in Table 37–1.

Figure 37–1

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

Table 37–1Incidence of esophageal atresia and tracheoesophageal fistula (TEF).

A. Type A

Isolated, or “pure,” esophageal atresia comprises 7.3% of cases. The lower pouch is usually only 1 to 2 cm above the diaphragm, whereas the upper pouch ends near the thoracic inlet, creating a long gap between the 2 ends that can complicate repair. This anomaly does not allow amniotic fluid to pass to the remainder of the developing gut, which explains the finding of polyhydramnios on prenatal ultrasound. Because there is no fistula to the trachea, these babies will have a gasless abdomen on x-ray.

B. Type B

Esophageal atresia with proximal TEF is the least commonly encountered anomaly, comprising < 1% of cases. The fistula angles downward from the trachea to the esophagus. The space between the 2 esophageal ends is pronounced.

C. Type C

Esophageal atresia with a distal TEF is the most common anomaly, comprising 85.4% of 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 ...

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