Normally, the esophagus, a long tubelike organ, connects the mouth to the stomach. In esophageal atresia, food is delayed or prevented from going from the esophagus to the stomach.
Many children with esophageal atresia and tracheoesophageal fistula have other abnormalities, such as defects of the spine, heart, kidneys, genitals, ears, and limbs and retardation of mental development, physical development, or both.
A tracheoesophageal fistula is dangerous because it allows swallowed food and saliva to travel through the fistula to the lungs, leading to coughing, choking, difficulty breathing, and possibly aspiration pneumonia (due to inhaling food or saliva). Food or fluid in the lungs may impair oxygenation of blood, leading to a bluish discoloration of the skin (cyanosis).
(See also Overview of Digestive Tract Birth Defects.)
Doctors may suspect esophageal atresia before birth based on results of prenatal ultrasonography or after birth based on symptoms.
If doctors suspect this disorder after birth, they try to pass a tube down the infant's esophagus. If passage of the tube is blocked, they take x-rays to confirm the diagnosis and to locate the problem.
Before surgery to repair the defect can be done, preparations are done to prevent complications, such as aspiration pneumonia. First, feedings by mouth are stopped, and a tube is placed in the upper esophagus to continuously suction saliva before it can reach the lungs. Then the infant is fed by vein (intravenously).
Surgery needs to be done soon to establish a normal connection between the esophagus and stomach and to close the connection between the esophagus and the trachea.