Children's Hospital Colorado

Esophageal Atresia and Tracheoesophageal Fistula

What is esophageal atresia and tracheoesophageal fistula?

Esophageal atresia (EA) is a rare birth defect in which a baby’s esophagus (the food tube) is not fully formed and does not connect to the stomach like it should. Instead, each end of the esophagus is closed.

Sometimes esophageal atresia occurs together with a condition called tracheoesophageal fistula (TEF). A TEF is an abnormal connection between the esophagus and trachea. The trachea is the tube that connects the throat to the lungs. Normally, the esophagus and trachea are not connected. If TEF occurs with EA, we refer to the condition as EA/TEF. There are different types of EA/TEF that are based on the shape of the esophagus, trachea and the connecting TEF.

What causes esophageal atresia and tracheoesophageal fistula?

During early development, the esophagus and airway initially form as a single tube that divides into two tubes in the first two months of pregnancy. If the two tubes do not separate correctly, the baby will have EA/TEF.

EA/TEF may be part of a group of defects referred to as VACTERL association – vertebral, anorectal, cardiac, tracheal, esophageal, renal and limb anomalies.

Who gets esophageal atresia and tracheoesophageal fistula?

Esophageal atresia (EA) and tracheoesophageal fistula (TEF) occur in 1 in 3,000 to 5,000 live births. Isolated tracheoesophageal fistula is much less common, occurring in only 4% to 5% of all children with TEF/EA.

Half of the patients with esophageal atresia also have other birth defects associated with a syndrome. A syndrome is a group of symptoms that occur together. These defects include cardiac (heart) malformations, which occur in 25% of cases. Some syndromes are inherited. Some cases of EA/TEF occur without any other defects and appear to be isolated.

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