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Tracheoesophageal Fistula Treatment

Tracheoesophageal fistula (TEF) is a birth defect in which the upper esophagus, a segment of the body's digestive organs, connects to the trachea instead of the lower esophagus and stomach. It is usually part of a larger birth defect called esophageal atresia, defined by disconnection of the upper and lower esophagus. Roughly one in 4,000 newborns in America has one or both of these conditions. TEF and esophageal atresia are life-threatening, and immediate surgery is the standard option for treatment.

  1. TEF and Esophageal Atresia

    • In very early fetal development, the esophagus and trachea form a single tube inside the throat and torso. Normally, one to two months after conception, a wall develops between the two, leading to further development of the systems for eating and breathing. In fetuses with TEF or esophageal atresia, this separation never fully occurs, leading to problems soon after birth. Connection of the esophagus and trachea allows a direct pathway to breathe saliva or other fluids into the lungs (aspiration), leading to potential for choking or the development of pneumonia. Disconnection of the upper and lower esophagus creates additional problems, and can make it impossible for a child to swallow or digest food.

    Symptoms

    • Although TEF and esophageal atresia are birth defects, they are not inherited disorders. Still, babies with certain defects of the heart, kidneys, urinary tract, muscles and skeleton seem to have a heightened risk of esophageal problems. Indications of problems include breathing difficulties, choking or coughing during feeding, vomiting, white froth in the mouth, a distended abdomen and blue skin, especially during feeding. If your baby is experiencing any of these symptoms, notify your doctor immediately. While difficulties of this sort can indicate other problems, the seriousness of TEF makes quick diagnosis a necessity.

    Diagnosis and Surgery

    • If your baby does have TEF or esophageal atresia, your doctor will likely place her directly into a neonatal intensive care unit. There, an IV will be used for feeding. In some cases, a ventilator is used to assist breathing, and suctioning digestive fluids away from the lungs may also be necessary. If your baby is premature or low-weight, your doctor may keep her in intensive care until weight gain or growth make surgery safer. In these cases, doctors typically insert a temporary tube to allow feeding directly into the stomach.

      In surgery, doctors will make an opening between the ribs to get at the abnormal connection (fistula). They will then separate the esophagus and trachea, then reconnect them back in their proper places. In some situations where esophageal atresia is also present, the upper and lower parts of the esophagus are too far apart to be easily reconnected. If that is the case, your doctor will use a temporary tube for feeding and perform a later surgery to connect the esophagus.

    Aftercare

    • After surgery, your baby may have certain complications, including food leakage from the repair site, return of the fistula, collapsed lung or abnormal narrowing of the esophagus or trachea. Acid reflux from the stomach to the esophagus is also possible. Your doctor will monitor these conditions, and should take corrective action if necessary.

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