Tracheoesophageal fistula

Alternative names
Esophageal atresia

Definition
Esophageal atresia is a disorder of the digestive system in which the esophagus does not develop properly. The disorder is a congenital defect, which means it occurs before birth. There are several types.

Causes, incidence, and risk factors

The esophagus is the tube that normally carries food from the mouth to the stomach. In most cases of esophageal atresia, the upper esophagus ends blindly and does not connect with the lower esophagus and stomach. In these cases, the top end of the lower esophagus is connected with the trachea (windpipe). This connection between the lower esophagus and the trachea is called a tracheoesophageal fistula (often abbreviated TEF).

Over 30% of people affected by this condition will also have other abnormalities, such as heart disorders or other gastrointestinal tract disorders. Other types of esophageal atresia involve narrowing of the esophagus and may also be associated with other birth defects.

This condition is frequently complicated by the infant’s breathing saliva and secretions into the lungs, causing pneumonia, choking, and possibly death. It is considered a surgical emergency.

The disorder is usually detected shortly after birth when feeding is attempted and the infant coughs, chokes, and turns blue (cyanosis). Immediate surgical repair of this disorder is required so that the lungs are not damaged and the baby can be fed.

Symptoms

     
  • Excessive salivation  
  • Drooling  
  • Poor feeding  
  • Coughing, gagging, and choking associated with attempted feeding  
  • Bluish coloration to the skin (cyanosis) associated with attempted feedings  
  • Excessive amniotic fluid during pregnancy (polyhydramnios)

Signs and tests

Prior to birth, the presence of excessive amniotic fluid will arouse suspicion of esophageal atresia or other obstruction of the gastrointestinal tract.

After birth, as soon as the diagnosis is suspected, an attempt to pass a small feeding tube (nasogastric tube) through the mouth or nose into the stomach should be made. The feeding tube will not be able to pass all the way to the stomach in a baby with esophageal atresia.

An X-ray of the esophagus shows an air-filled pouch and air in the stomach and intestine. If a feeding tube was passed it will be seen coiled up in the upper esophagus.

Treatment

Surgery to repair the esophagus is done quickly after the infant has been stabilized. Before the surgery, the infant is not fed by mouth, and care is taken to prevent the infant from breathing secretions into the lungs.

Expectations (prognosis)

An early diagnosis gives a better chance of a good outcome.

Complications

     
  • Prematurity may complicate the condition  
  • aspiration pneumonia  
  • Feeding difficulties  
  • Reflux (the repeated regurgitation of food) is common after surgery

Calling your health care provider

This disorder is usually diagnosed shortly after the birth of an infant.

Call your infant’s health care provider if the infant regurgitates repeatedly after feedings, or if the infant develops breathing difficulties.

Johns Hopkins patient information

Last revised: December 8, 2012
by Armen E. Martirosyan, M.D.

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