Tracheoesophageal Fistula

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.