Esophageal atresia/tracheoesophageal fistula

Abstract

Esophageal atresia (EA) and tracheoesophageal fistula (TEF) are congenital abnormalities that occur in approximately 1 in 4,000 births. These abnormalities are related to defective orientation of the tracheoesophageal septum as the trachea forms from the caudal foregut during the 6th week of gestation. There are five basic variants. EA with normal tracheal development is designated as type A. Unlike the other types, it is due to a defect in recanalization of the esophagus in the 8th week of gestation, and it occurs in 80 % of cases. Type B is EA with proximal TEF, occurring in 1 % of cases. The most common variant (85 %) is type C, consisting of EA with distal TEF. The TEF is typically located near the level of the carina. EA with both distal and proximal TEF, type D, occurs in 2 % of cases. Finally, type E consists of TEF with no EA. The defect is usually located high on the trachea and occurs in 4 % of cases. It is also known as H-type TEF.