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Esophageal Atresia and Tracheoesophageal Fistula

By

William J. Cochran

, MD, Geisinger Clinic

Last full review/revision Apr 2021| Content last modified Apr 2021
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Esophageal atresia is a birth defect in which the esophagus narrows or comes to an end. Most newborns with esophageal atresia also have an abnormal connection between the esophagus and the windpipe (trachea) called tracheoesophageal fistula.

The esophagus is the long tubelike organ that connects the mouth to the stomach. In esophageal atresia, the esophagus is narrow or develops as two separate sections that do not connect (atresia). Because of the defect, food and fluids are delayed or prevented from going from the esophagus to the stomach.

The windpipe (trachea) is the main airway to the lungs. Normally, the esophagus and the trachea are separate. However, in tracheoesophageal fistula, an abnormal connection (fistula) forms between the esophagus and the windpipe. Because of the defect, food and fluids go into the windpipe and lungs.

Esophageal atresia and tracheoesophageal fistula often occur together.

Doctors do not know what causes esophageal atresia and tracheoesophageal fistula, but many children with these birth defects have other abnormalities, such as defects of the spine, heart, kidneys, genitals, ears, and limbs and retardation of mental development, physical development, or both.

Atresia and Fistula: Defects in the Esophagus

In esophageal atresia, the esophagus narrows or comes to a blind end. It does not connect with the stomach as it normally should. A tracheoesophageal fistula is an abnormal connection between the esophagus and the trachea (which leads to the lungs).

Atresia and Fistula: Defects in the Esophagus

Symptoms

A newborn with esophageal atresia coughs, chokes, and drools after attempting to swallow while feeding.

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).

Diagnosis

  • Before birth, prenatal ultrasonography

  • After birth, passage of a tube down the esophagus and x-rays

Doctors may suspect esophageal atresia and tracheoesophageal fistula before birth based on results of prenatal ultrasonography or after birth based on symptoms.

If doctors suspect these defects 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.

Treatment

  • Surgery

Before surgery to repair the defects 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 after birth to establish a normal connection between the esophagus and stomach and to close the fistula.

Some complications may develop after surgery. Leakage and scar tissue at the site where the esophagus was reconnected are the most common problems. Many children have feeding difficulties. About half of children who have feeding difficulties develop gastroesophageal reflux (the backward movement of food and acid from the stomach into the esophagus), so doctors may give drugs that suppress stomach acid to newborns who have esophageal atresia. If the drugs do not control the reflux, a procedure called fundoplication is done. In fundoplication, the surgeon wraps the top of the stomach around the lower end of the esophagus to make that junction tighter and decrease reflux.

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