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What is tracheoesophageal fistula?

Normally, the esophagus (the tube that connects the throat to the stomach) and the trachea (the tube connecting the throat to the windpipe and lungs) are separate. A tracheoesophageal fistula (TEF) is an abnormal connection between these two tubes. As a result, swallowed liquids or food can be aspirated (inhaled) into your child's lungs. Feeding into the stomach directly can also lead to reflux and aspiration of stomach acid and food. TEF usually occurs with a related condition called esophageal atresia (EA).

What causes tracheoesophageal fistula?

As a fetus is growing and developing in the mother's uterus before birth, the trachea and the esophagus begin developing as one single tube. At about four to eight weeks after conception, a wall forms between the fetus' esophagus and trachea to separate them into two distinct tubes. If this wall does not form properly, a range of anomalies, including tracheoesophageal fistula (TEF), can occur.

After repair of a primary TEF, there is a chance the TEF will return (recurrent TEF). Sometimes the cause is an infection during surgery that damages the trachea. Once a TEF recurs, the increased esophageal pressure forces fluids into the airway during swallowing. Eventually the TEF becomes lined with mucosa and becomes permanent.

What are the symptoms of tracheoesophageal fistula?

Babies who have TEF by itself (without esophageal atresia, or EA) often don't show any symptoms at birth. Symptoms that eventually emerge include:

  • Coughing while feeding
  • Frequent lung infections

If your child has both TEF and EA, the symptoms are usually obvious right after birth. The most common signs of combined EA/TEF are:

  • Breathing problems
  • Coughing or choking trying to swallow

Babies with EA/TEF may have other problems that are linked to the conditions.

How we care for tracheoesophageal fistula

Many surgeons try but fail to repair TEF with minimally invasive techniques, increasing the risk that it will reoccur. The scarring caused by these procedures makes further repair more difficult. Recurring TEFs can be larger and potentially damage other structures, such as the voice box. At Boston Children's Esophageal and Airway Treatment Center, our doctors usually repair TEF in one operation in the first day or two of your baby's life, using techniques that don't result in recurrence.

Tracheoesophageal Fistula | Diagnosis & Treatments

How is tracheoesophageal fistula diagnosed?

Like other disorders of the trachea, tracheoesophageal fistula (TEF) is rarely diagnosed before birth. Once your baby is born and symptoms have been observed, your doctor may recommend:

  • imaging studies, such as x-rays
  • endoscopy or bronchoscopy, which are techniques for looking at the inside of your child's airways using a thin tube fitted with a small light and camera

Symptoms of coughing with eating or drinking, or recurrent episodes of respiratory infection may suggest recurrent TEF. TEF may come and go, making it difficult to diagnose. The procedures listed above will typically be the first step in getting a correct diagnosis.

How is tracheoesophageal fistula treated?

Surgeons at Boston Children's Hospital have developed highly successful techniques for treating TEF and recurrent TEF that do not result in further recurrence. If your baby has TEF without esophageal atresia (EA), we can usually repair it in the first day or two of life with one short operation. This procedure has also been successful for those children suffering from recurrent TEF. The procedure is customized to your child to ensure the best possible result. During this surgery, your child's doctor will:

  • Make a small incision in your child's neck or back, depending on the location of the TEF.
  • Divide the fistula, closing the connection between the esophagus and the trachea.
  • Remove the pouch from the back of the trachea where the TEF originated. This is done with bronchoscopy guidance, so that the surgeons can see the repair from the inside of the airway.
  • Repair the hole in the esophagus, which is often necessary to correct an area of narrowing in the esophagus that may be present.
  • Correct tracheaomalacia, if present, with a posterior tracheopexy. This is done with bronchoscopy to ensure a good result.
  • Rotate the esophagus away from the trachea so the TEF does not come back.

If your child has TEF with EA, our doctors will follow the same approach as above and then connect the two ends of the esophagus together, if they are close enough together to do so. If this is not possible, our surgeons will discuss the best options to grow the esophagus long enough for the repair. If your child has TEF and long-gap EA, meaning that the two disconnected ends of the esophagus are far apart, they will require a special treatment known as the Foker process. This highly successful procedure is only offered routinely at Boston Children's.

Children who undergo these surgical procedures usually do very well. To date, we have not seen a single recurrence of TEF after these procedures. Because children with EA/TEF are at higher risk for experiencing acid reflux, Barrett's esophagus (a precancerous condition) and other concerns, they should undergo routine follow-up endoscopy for life.

Tracheoesophageal Fistula | Programs & Services