Health Library

Tracheoesophageal Fistula and Esophageal Atresia

What Happens in a 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 trouble breathing.

What Causes Tracheoesophageal Fistula and Esophageal Atresia?

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

Are There Risk Factors or Other Conditions Linked to TEF and EA?

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

Babies born with other birth defects often also have a TEF and/or EA. Conditions that often happen alongside TEF and EA include:

• Chromosomal conditions, such as Trisomy 13, 18, or 21
• Digestive tract abnormalities, including diaphragmatic hernia, duodenal atresia, or imperforate anus
• Heart defects, such as ventricular septal defect, tetralogy of Fallot, or patent ductus arteriosus
• Kidney and urinary tract abnormalities, including horseshoe or polycystic kidney, absent kidney, or hypospadias
• Musculoskeletal conditions, such as rib or spinal abnormalities
• VACTERL association, a group of conditions involving vertebral, anal, cardiac, TE fistula, kidney and limb abnormalities

What Are the Signs and Symptoms of TEF and EA?

Symptoms are usually seen shortly 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 TEF and EA Diagnosed?

The medical team asks about the child’s full medical history and does a physical exam.  As part of this exam, they try to gently place a tube into the baby’s stomach. If this cannot be easily done, there is a high chance of a blocked esophagus, which may be a sign of esophageal atresia. Chest and belly X-rays are done to see whether one or both conditions are present.

What Are the Treatment Options for TEF and EA?

If an infant is born with a TEF or EA, surgery will be needed. The timing and type of surgery done depends on the following:

• Type of trachoesophageal fistula with or without esophageal atresia
• Overall health of the baby and their medical history
• Opinion of the surgeon and other doctors involved in the baby's care

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. In some cases, the two esophageal segments are too far apart to safely join them, and doctors may choose to delay repair to allow time for the ends to grow closer together. Sometimes more complex reconstructions using parts of the intestines or stomach are needed if the two esophageal segments can be joined.

What Are the Possible Complications After Surgery?

Possible complications after surgery can include:

• Recurrent fistula (another operation is needed in 90-100% of cases)
• Narrowing of the esophagus caused by scarring that happens after surgical repair of the esophagus (30% to 50% of cases)
• Gastroesophageal reflux (approximately 50% of cases)
• Aspiration/inhaling food or liquids (uncommon)

What Is the Long-Term Outlook for Children with TEF or EA?

Children born with EA may have long-term issues that can affect feeding, digestion, breathing and overall health. Swallowing food or liquids may be hard for many reasons, including:

• The muscles of the esophagus do not work normally
• Scar tissue can form in the esophagus and partially block food from passing through

In some children, a narrow part of the esophagus can be stretched open using a special procedure called endoscopic dilatation procedure that is done under general anesthesia. This procedure may need to be done more than once to widen the esophagus. In other children, additional surgery may be needed to open the esophagus so that food can pass to the stomach properly.

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