Tracheoesophageal Fistula and Esophageal Atresia

Epidemiology

Tracheoesophageal fistula (TEF) and esophageal atresia (EA) are part of a complex of congenital malformations characterized by abnormal communication between the esophagus and trachea. The incidence is 1 in 3000 to 4000 births. Thirty percent of patients are born prematurely. There is no reported gender predilection. Coexisting anomalies are present in up to 50% of patients with these congenital anomalies. The best known pattern of anomalies is referred to as VATER (v ertebral defects, imperforate a nus, t racheo e sophageal fistula, r adial and r enal dysplasia) and VACTERL (v ertebral, a nal, c ardiac, t racheal, e sophageal, r enal, and l imb deformities). Other coexisting anomalies include Down syndrome and atresias of the gastrointestinal tract, including duodenal atresia. Cardiovascular anomalies include patent ductus arteriosus, ventricular septal defect, and right-sided aortic arch. Patients with TEF have an increased incidence of tracheomalacia, with the latter being an important cause of postoperative respiratory complications. Rarely, TEF may be acquired by erosion from a granulomatous inflammatory process, tumor, or foreign body.

Embryology

This group of anomalies is believed to result from a disorder in the separation of the primitive foregut into the trachea and esophagus. The most likely cause is thought to be a failure of complete formation of the tracheoesophageal septum that separates the respiratory and digestive systems. The developmental error is thought to most likely occur between the fourth to fifth weeks of gestation.