Tracheoesophageal fistula

Definition

Tracheoesophageal fistula (TEF) is commonly a birth defect, with the trachea connected to the esophagus. In most cases, the esophagus is discontinuous, causing immediate feeding difficulties. TEFs may develop in adult life, secondary to the invasion of cancer in the area. In addition, TEFs may be deliberately constructed with surgery to aid talking in a patient who has the larynx removed (a laryngectomy).

Description

The trachea, or windpipe, carries air to the lungs. The esophagus carries food to the stomach. Sometimes during development, these two tubes do not separate completely, but remain connected by a short passage. When this happens, air enters the gastrointestinal system, causing the bowels to distend, and mucus is breathed into the lungs causing aspiration pneumonia and breathing problems.

Most tracheoesophageal fistulas are diagnosed when a child is born. There are three types. In 85-90% of tracheoesophageal fistulas, the top part of the esophagus ends in a blind sac, and the lower part inserts into the trachea. In the second type, the upper part of the esophagus is connected directly to the trachea, while the lower part ends in a pouch. In a rare type of fistula called an H type, both the esophagus and trachea are complete, but they are connected. This is the most difficult type of tracheoesophageal fistula to diagnose, because both eating and breathing are possible.

Causes & symptoms

Tracheoesophageal fistulas arise as a developmental abnormality. At birth, the infant has difficulty swallowing. Eating produces severe coughing spells that interfere with breathing. Aspiration pneumonia can develop from fluid breathed into the lungs.

Small H type fistulas may go undiagnosed until later in life. Symptoms of an H type fistula include frequent pulmonary infections and bouts of abdominal bloating.

Diagnosis

Diagnosis that the esophagus is interrupted is confirmed by the inability to insert a nasogastric suction tube into the stomach. The exact type and location of the fistula can be determined using a radiopaque catheter, which allows pictures to be taken of the esophagus. X rays may show air in the bowels. Endoscopy often fails to located the fistula if it is small.

Treatment

Babies with all but H type fistulas are unlikely to survive without surgical separation and repair of the trachea and the esophagus. Surgery can not always be performed immediately because of prematurity, the presence of other birth defects, or complications from aspiration pneumonia. It is usually done at a hospital that has special facilities for treating seriously ill newborns.

While awaiting surgery, the infant's condition is stabilized. Preoperative care concentrates on avoiding aspiration pneumonia and includes:

• Elevating the head to avoid reflux and aspiration of the stomach contents
• Using a suction catheter to continuously removed mucus and saliva that could be inhaled
• When necessary, placement of a gastrostomy tube
• Withholding feeding by mouth.

When surgery is performed, the esophagus is reconnected to make it continuous and separate from the trachea. If the two ends of the esophagus are too far apart to be reattached, a piece of tissue from the large intestine is used to join the parts.

Prognosis

Infants who have tracheoesophageal fistula often have other birth defects that affect their recovery. Even when the esophagus is successfully separated and reattached, many infants have difficulty swallowing, because the contractility of the esophagus is impaired. Infants may also have problems with gastroesophageal reflux, where the acidic contents of the stomach back up into the bottom of the esophagus and cause ulcers and scarring.

Prevention

Tracheoesophageal fistulas are not preventable birth defects.

Key Terms

Endoscopy

A procedure where an instrument containing a camera and a light source is inserted into the gastrointestinal tract so that the physician can visually inspect the gastrointestinal system.

Gastrostomy tube

Stomach tube for feeding.

Laryngectomy

Surgical removal of the larynx to treat cancer.

Further Reading

For Your Information

Books

• "Gastrointestinal Defects." In The Merck Manual of Diagnosis and Therapy, edited by Robert Berkow. 16th ed. Rahway, NJ: Merck Research Laboratories, 1992.

Gale Encyclopedia of Medicine. Gale Research, 1999.