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.
There are three main types of TEF. In 85 to 90 percent 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 by a small passageway. This is the most difficult type of tracheoesophageal fistula to diagnose, because both eating and breathing are possible. TEFs often occur in babies with additional birth defects.
Demographics
TEFs occur in about one of every 3,000 live births. They are slightly more common in boys than in girls. Some studies suggest that the occurrence of TEFs increases with the age of the mother.
Causes and 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.
When to call the doctor
TEFs are normally diagnosed within hours of birth, because feeding and breathing problems are immediately apparent. Some H type defects are not detected until later in life.
KEY TERMS
Endoscopy—Visual examination of an organ or body cavity using an endoscope, a thin, tubular instrument containing a camera and light source. Many endoscopes also allow the retrieval of a small sample (biopsy) of the area being examined, in order to more closely view the tissue under a microscope.
Esophageal atresia—Blockage or closure of the esophagus, the tube leading from the mouth to the stomach.
Gastrostomy tube—A tube that is inserted through a small incision in the abdominal wall and that extends through the stomach wall into the stomach for the purpose of introducing parenteral feedings. Also called a gastric tube, gastrointestinal tube, or stomach tube.
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 locate 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 is usually done at a hospital that has special facilities for treating seriously ill newborns. However, surgery cannot always be performed immediately because of prematurity, the presence of other birth defects, or complications from aspiration pneumonia.
While awaiting surgery, the infant's condition is stabilized. Preoperative care concentrates on avoiding aspiration pneumonia and includes the following:
elevating the head to avoid reflux and aspiration of the stomach contents
using a suction catheter to continuously remove mucus and saliva that could be inhaled
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
The survival rate of infants with tracheoesophageal fistulas improved dramatically toward the end of the twentieth century. In uncomplicated cases, the survival rate is close to 100 percent. However, often babies with TEFs have other birth defects that limit their recovery.
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, in which the acidic contents of the stomach back up into the bottom of the esophagus and cause ulcers and scarring. Long-term follow-up, however, finds that 80 to 90 percent of children who have repaired TEFs as infants eat normally by the time they are in elementary school. As more individuals with corrected TEFs reach adulthood, there is some evidence that suggests they are more susceptible to esophageal cancers. However, as of 2004, there was not enough data to confirm these findings.
Prevention
Tracheoesophageal fistulas are defects in development of the fetus that cannot be prevented.
Parental concerns
Parents often worry about the effect a TEF may have on their child's later ability to eat and participate in normal activities such as sports. In the absence of other birth defects, almost all children have no restrictions on their eating and activities by the time they start school.
BOOKS
Moore, Keith L., et al. Before We Are Born: Essentials of Embryology and Birth Defects. Kent, UK: Elsevier—Health Sciences Division, 2002.
PERIODICALS
"Esophageal Atresia with or without Tracheoesophageal Fistula." eMedicine Medical Library. Available online at <www.emedicine.com/ped/topic2950.htm> (accessed August 18, 2004).
"Gastrointestinal Defects." The Merck Manual of Diagnosis and Therapy, 17th ed. Edited by Robert Berkow. Rahway, NJ: Merck Research Laboratories, 1999–2004. Available online at <www.merck.com/mrkshared/mmanual/home.jsp> (accessed October 18, 2004).
Kronemer, Keith, and Alison Snyder. "Esophageal Atresia/Tracheoesophageal Fistula." eMedicine Medical Library. Available online at <www.emedicine.com/radio/topic704.htm> (accessed October 18, 2004).
Minkes, Robert K., and Alison Snyder. "Congenital Anomalies of the Esophagus." eMedicine Medical Library. Available online at <www.emedicine.com/derm/topic396.htm> (accessed October 18, 2004).
