Conditions + Treatments

Esophageal Atresia in Children

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Contact the Esophageal Atresia Treatment Program

  • 1-617-355-3038

Esophageal atresia (EA) is a rare birth defect in which a baby is born without part of the esophagus (the tube that connects the mouth to the stomach). About 1 in 4,000 babies in the U.S. is born with esophageal atresia.

EA frequently occurs along with tracheoesophageal fistula (TEF), and as many as half of all babies with EA/TEF have another birth defect, as well.

The Esophageal Atresia Treatment Program at Boston Children's Hospital is one of the only programs in the country specifically designed to care for children with this condition.

What is Esophageal Atresia?

Esophageal atresia (EA) is a birth defect in which part of your child’s esophagus is missing. Instead of forming a tube between the mouth and the stomach, the esophagus grows in two separate segments that do not connect.

Esophageal atresia is a lethal disease unless treated. Without a working esophagus, your baby cannot swallow or feed normally. Once esophageal atresia is diagnosed, your child will probably be fed intravenously at the hospital until doctors perform surgery to repair the esophagus. 

Although EA can be life-threatening in its most severe forms and could cause long-term nutritional concerns, the majority of children fully recover if it’s detected early.

The best treatment is usually surgery to reconnect the two ends of the baby’s esophagus to each other. In some children, however, so much of the esophagus is missing that the ends can’t be easily connected. This is known as long-gap esophageal atresia.

Esophageal Atresia and Tracheoesophageal Fistula

EA and TEF commonly occur together. In children who have both EA and TEF, the X-ray will show gas in the baby’s stomach (because the esophagus has not formed properly, the only way gas can get into the stomach is through an abnormal channel between the esophagus and the trachea).

After the tests are complete, our team of experts will meet to review and discuss what they have learned about your child's condition. Then we will meet with you and your family to discuss the results and outline the best treatment options.

What Causes Esophageal Atresia?

The exact cause of esophageal atresia is still unknown, but it appears to have some genetic components. Up to half of all babies born with esophageal atresia have one or more other birth defects, such as other digestive tract problems, kidney and urinary tract problems, heart problems or muscular or skeletal problems.

If caught early and treated properly, most babies with esophageal atresia can eat normally within two to three months. Your child may need monitoring for strictures (places of narrowing) in their esophagus, but these are treatable, and in general the outlook is quite good. In fact, the Foker process most often results in an esophagus that looks just like one that developed normally.

Questions to Ask Your Doctor About Your Child’s Care

After your child is diagnosed with EA, you may feel overwhelmed with information.  Your neonatologist or pediatric surgeon may suggest that your child be treated at the hospital where you delivered.

However, given the rarity of this condition and the importance of a successful first treatment (repeated procedures can result in complications), it is important to know all of your options.  You want the best possible care for your child, which means working with surgeons who have significant experience treating this condition.

Here are some questions you may want to ask your doctor:

  • Is my child’s esophageal atresia associated with another condition?
    • VACTERL?
    • Laryngeal cleft?
    • TEF?
    • Tethered spinal cord?
    • Congenital heart disease?
    • Spinal or rib malformations?
    • Tracheomalacia?
    • Anorectal anomalies?
    • Kidney malformations?
    • Radial limb malformation?
  • How will you manage my child’s symptoms?
  • What’s the best treatment for my child right now?
  • What are the possible short and long-term complications of treatment?
  • How many cases of EA have been treated at your hospital in the last year? How many of those cases were long gap EA?
  • Do any of your surgeons have experience with the Foker process or the jejunum interposition technique?
  • What is the long-term outlook for my child?
    • How have your patients with this specific problem done over time?
    • May I speak with some parents of children who have been treated for this condition here?
  • What services are available to help my child and my family to cope?

Make an Appointment

To make an appointment or speak with a member of our team, please call 617-355-3038.

International Patients

For families residing outside of the United States, please call Boston Children's International Health Services at +01-617-355-5209.

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- Sandra L. Fenwick, President and CEO

Boston Children's Hospital 300 Longwood Avenue, Boston, MA 02115 617-355-6000 | 800-355-7944