Tracheoesophageal Fistula (TEF)

TEF is an abnormal opening in one or more places between the esophagus (tube going from the mouth to the stomach) and the trachea (windpipe that goes from the throat to the lungs). These are normally two separate tubes. Saliva or gastric juices can pass from the esophagus, through the opening, and into the lungs. This can cause breathing and lung problems.

In premature infants who have underdeveloped lungs and a TEF, air may go from the trachea into the esophagus and stomach, causing severe belly swelling, possible stomach rupture, and respiratory distress.

Esophageal Atresia (EA)

TEF is often seen with another birth defect known as esophageal atresia. In this condition, the esophagus does not fully form and there is not a connection from the mouth to the stomach.

What Causes Tracheoesophageal Fistula and Esophageal Atresia?

As a baby grows during pregnancy, different organ systems develop and mature. Although next to each other, the trachea and the esophagus develop as two separate tubes. When the wall between them does not form properly, TEF and / or EA can occur. About one in 3,000 to 5,000 babies in the United States is born with one or both of these problems.

Risk Factors and Associated Disorders

These two conditions are not thought to be inherited, and cases in which multiple family members are affected are rare.

Babies with other birth defects often have a TEF and / or EA. Birth defects that often occur with these two conditions are:

• Trisomy 13, 18, or 21
• Other digestive tract problems, such as diaphragmatic hernia, duodenal atresia or imperforate anus
• Heart problems, such as ventricular septal defect, tetralogy of Fallot, or patent ductus arteriosus
• Kidney and urinary tract problems, such as horseshoe or polycystic kidney, absent kidney, or hypospadias
• Muscular or skeletal problems
• VACTERL association, which involves vertebral, anal, cardiac, TE fistula, renal, and limb abnormalities

Signs and Symptoms of Tracheoesophageal Fistula and Esophageal Atresia

Symptoms are usually seen soon after birth. They may include:

• Frothy white bubbles in the mouth
• Coughing or choking when feeding
• Vomiting
• Blue color of the skin, especially during feeding
• Trouble breathing
• Very round, full stomach (TEF)
• Unusually flat stomach (isolated EA)

How are Tracheoesophageal Fistula and Esophageal Atresia Diagnosed?

A complete medical history is taken and a physical examination is done. This includes trying to place a tube into the stomach. If this cannot be easily done, there is a high chance of a blocked esophagus, which may a sign of esophageal atresia. Chest and abdominal X-rays are done to see whether one or both conditions are present.

What are the Best Treatment Options for Tracheoesophageal Fistula and Esophageal Atresia?

If a child (infant) has a TEF or EA, surgery will be needed, and this can be done quickly after the infant has been stabilized. The timing and type of surgery performed depends upon the following:

• Type of problem
• Overall health of the baby and medical history
• Opinion of the surgeon and other doctors involved in the baby's care
• Expectations for the course of the condition

When a TEF is repaired, the connection between the esophagus and the trachea is closed. In most cases, the two ends of the esophagus can be brought together in a single operation. Occasionally, when the distance between the two segments is too far to bring the two ends together, the decision is made to wait to connect the esophagus and give time for the two ends to possibly grow closer together. Sometimes more complex reconstructions, using portions of the intestines or stomach, can be done to create a new esophageal tube. This may require more than one operation.

Possible Complications

• Recurrent fistula (another operation is required in 1% of cases)
• Narrowing of the esophagus caused by scarring that occurs after surgical repair of the esophagus (10% to 15% of cases)
• Gastroesophageal reflux (approximately 50% of cases)
• Aspiration (uncommon)

Long-Term Outlook

Some children born with EA have long-term problems. Swallowing food or liquids may be difficult due to:

• Absent or abnormal contraction of esophageal muscles
• Scarring in the esophagus can partially block the passage of foods

In some children, a narrowed esophagus can be successfully widened with a special endoscopic dilatation procedure performed under general anesthesia. This procedure may need to be done multiple times in order to widen the esophagus. In other children, additional surgery may be needed to open up the esophagus so that food can pass to the stomach properly.

Approximately half of the children who have had surgical repair for EA will have problems with gastroesophageal reflux disease (GERD). GERD causes acid to move up into the esophagus from the stomach. As it moves, it causes a burning or painful feeling known as heartburn. GERD is usually treated with medications. However, an anti-reflux operation is sometimes needed.