Congenital Anomalies of the Esophagus and Trachea

  • Congenital anomalies of the esophagus and trachea are birth defects that occur before your baby is born. There are several kinds:

    • Esophageal atresia- With esophageal atresia, your child's esophagus does not form properly, resulting in two segments that don't connect to each other. Since the esophagus is in two segments, liquid that a baby swallows cannot pass normally through the esophagus and reach the stomach. Also, ingested liquids may spill over into the bronchial tubes and cause pneumonia or airway obstruction.
    • Tracheoesophageal fistula (TEF)- Often occurs with esophageal atresia. It's an abnormal connection in one or more places between your child's esophagus and the trachea. Normally, the esophagus and the trachea are two separate tubes that are not connected, but if a TEF is present, stomach content may regurgitate into the lungs. It is also possible, though rare, that a TEF can be present from the upper end of the esophagus to the trachea in which event, swallowed liquids would pass directly into the lungs.
    • Isolated tracheoesophageal fistula- (Very rare) Where there is an abnormal communication between an otherwise intact esophagus and trachea either as a narrow opening or there can also be a rare long communication between esophagus and trachea called a tracheoesophageal cleft.
    • Trachea atresia- (Very rare) an absence of part or all of your child's trachea.
    • Tracheal stenosis- (Very rare) is a constriction of the trachea.
    • Tracheal occlusion- (Very rare) is a blockage in the trachea.

    Pioneering treatment

    Boston Children's Hospital surgeons can create a nearly normal, functioning esophagus for your child by elongating and then joining together the incomplete segments. It's called the Foker Technique and Children's is the only pediatric hospital in the world that can do it.

    Contact Us

    Boston Children's Hospital
    300 Longwood Avenue
    Fegan 3
    Boston MA 02115
    617-355-3529


  • What causes congenital anomalies of the esophagus and trachea?

    As a fetus is growing and developing in its mother's uterus before birth, different organ systems are developing and maturing. 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 these anomalies can occur.

    How often do these problems occur?

    About one in 3,000 to 5,000 babies in the United States is born with tracheoesophageal fistula or esophageal atresia. Other anomalies of the esophagus and trachea are even more rare.

    These two problems are not thought to be inherited. Up to one-half of all babies with TEF or esophageal atresia have another birth defect, such as:

    What are the symptoms of congenital anomalies of the esophagus and trachea?

    The symptoms of TEF or esophageal atresia can usually be seen very soon after birth. Each child may experience symptoms differently, but here's a list of the most common ones:

    Symptoms of TEF or esophageal atresia may resemble other conditions or medical problems. Please consult your child's physician for a diagnosis.

  • How are congenital anomalies of the esophagus and trachea diagnosed?

    If esophageal atresia occurs alone, it can be diagnosed prenatally on prenatal ultrasound because it causes polyhydramnios (overproduction of amniotic fluid) to occur.

    Esophageal atresia with tracheoesophageal fistula, and other anomalies of the trachea are almost never diagnosed before birth. However, short segment atresias of the trachea or the larynx (voice box) can be diagnosed prenatally.

    Once your baby is born, imaging studies are usually done, along with a physical examination and medical history, to evaluate whether your baby has abnormalities of the esophagus and/or trachea. X-rays are taken to look at your baby's chest and abdomen.

  • If your baby has a congenital anomaly of the esophagus or trachea, she will need surgery to fix the problem.

    Esophageal atresia

    Repair of esophageal atresia depends on how close the two sections of esophagus are to each other. It may involve the placement of a feeding tube in your child's stomach and may require one or more operations to repair over several months.

    Esophageal atresia associated with a tracheoesophageal fistula

    This condition can usually be repaired in the first day or two of your baby's life in one operation that involves surgically closing the connections between the esophagus and the trachea and connecting the two ends of the esophagus together. Children who undergo this procedure usually do very well.

    Tracheal stenosis
    This condition can be repaired by removing the narrowed or constricted part of your baby's trachea and then connecting the unaffected parts of the trachea.

    Tracheal blockages
    A blockage in the trachea, or short segment atresias of the trachea or larynx may require specialized surgical treatment during delivery called an EXIT (ex utero intrapartum treatment) procedure.

    For this procedure, surgery is done as the baby is delivered via Cesarean section and still attached to the mother's placenta. This method gives surgeons time to perform multiple procedures to secure or open up the baby's airway while the blood flow and exchange of gases that normally occurs in the womb between the fetus and the placenta is preserved.

    Your baby's surgeon and other physicians will decide when it is best to do the operations, based on your baby's condition and the type of problem.

    Could my child have problems in the future?

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

    • problems with the normal movement of foods and liquids down the esophagus (peristalsis)
    • scarring that can occur in the esophagus after surgery as the wounds heal, which can partially block the passage of foods

    Sometimes, a narrowed esophagus can be widened or dilated with a special procedure done while the child is under general anesthesia. In other cases, another operation may be necessary to open up the esophagus so food can pass to the stomach properly.

    GERD

    About one-half of children who had esophageal atresia repaired will have problems with gastrointestinal reflux disease.

    Reflux causes acid to move up into the esophagus from the stomach. When acid moves from the stomach into the esophagus, it causes a burning or painful feeling known as heartburn.

    Reflux can usually be treated with medications prescribed by a physician. In some cases, when there is poor response to medical treatment, the operative treatment of reflux may be necessary.

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