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Esophageal Atresia and Tracheoesophageal Fistula

By William J. Cochran, MD, Associate, Department of Pediatrics GI and Nutrition;Clinical Professor, Department of Pediatrics, Geisinger Clinic, Danville, PA;Temple University School of Medicine

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In esophageal atresia, the esophagus narrows or comes to a blind end. Most newborns with esophageal atresia also have an abnormal connection between the esophagus and the windpipe (trachea) called tracheoesophageal fistula.

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).

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).

Symptoms

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

Diagnosis

  • Before birth, prenatal ultrasonography

  • After birth, passage of a tube down the esophagus

  • X-rays

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.

Treatment

  • Surgery

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.

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