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Esophageal Atresia

  • Esophageal atresia (EA) is a rare birth defect in which a baby is born without part of her esophagus (the tube that connects the mouth to the stomach). Boston Children's Hospital Esophageal Atresia Treatment Program is one of the only programs in the country specifically designed to care for children with this condition.

    Here’s what you need to know about esophageal atresia:

    • EA frequently occurs along with tracheoesophageal fistula (TEF).

    • Up to half of all babies with EA/TEF have another birth defect, as well.

    • Before treatment, babies are often unable to swallow, can’t feed normally and may have trouble breathing.

    • Although EA can be life-threatening in its most severe forms and 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.

    How Children’s approaches esophageal atresia

    Traditional surgical procedures for esophageal atresia allow the baby to swallow, but require long-term medical care and monitoring. Children’s Esophageal Atresia Treatment Program is the world’s only center offering a lifelong solution for children with long-gap atresia—the Foker process.

    John Foker, MD, a pediatric surgeon from the University of Minnesota, developed a technique to stimulate the upper and lower ends of the esophagus to make them grow, allowing them to then be joined together.Children's is the only pediatric hospital that offers the Foker process to treat EA and TEF. Foker has worked exclusively with Children’s Russell Jennings, MD to ensure that his successes are continued here in Boston. Learn more about the Foker process or watch our videos.

    In addition to esophageal atresia, our specialists also care for children with:

    • tracheomalacia–weakening of the esophageal walls
    • tracheoesophageal fistula–an abnormal connection between the trachea (windpipe) and esophagus
    • esophageal injury and trauma
    • lye strictures–chemical burn and scarring of the esophagus, caused by ingesting sodium hydroxide or potassium hydroxide
    • hiatal hernias–a condition in which the upper part of the stomach bulges through an opening in the diaphragm

     

    Esophageal Atresia: Reviewed by Russell W. Jennings, MD
    © Children’s Hospital Boston; posted in 2011

  • What is esophageal atresia?

    Esophageal atresia (EA) is a birth defect in which part of your baby’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.

    Children with esophageal atresia almost always have another birth defect called tracheoesophageal fistula, or TEF (a fistula is an abnormal connection). The esophagus and trachea should be two separate, unconnected tubes, but in TEF, they're connected.

    There are four types of esophageal atresia:

    • Type A  
      • The upper and lower segments of the esophagus end in pouches, like dead-end streets that don’t connect.
      • TEF is not present.
    • Type B
      • The lower segment ends in a blind pouch.
      • TEF is present on the upper segment.
      • This type is very rare.
    • Type C
      • The upper segment ends in a blind pouch.
      • TEF is present on the lower segment.
      • This is the most common type.
    • Type D
      • TEF is present on both upper and lower segments.
      • This is the rarest form of EA/TEF.

    How do babies with esophageal atresia eat?

    Without a working esophagus, your baby may be unable to swallow or feed normally. Once esophageal atresia is diagnosed, she will probably be fed intravenously at the hospital until doctors perform surgery to repair her esophagus.

    Is esophageal atresia common?

    About one in 4,000 babies in the United States is born with esophageal atresia, making it the 25th most common birth defect.

    Is esophageal atresia dangerous?

    Without a working esophagus, it’s impossible to receive enough nutrition by mouth. Babies with esophageal atresia are also more prone to infections like pneumonia and conditions such as acid reflux. Luckily, esophageal atresia is most often correctable.

    Causes

    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:

    Esophageal atresia and tracheoesophageal fistula are also often found in babies born with VACTERL syndrome. This is a non-random collection of abnormalities that may also involve the spine, heart, lower digestive tract, kidneys and limbs. Not all babies born with VACTERL syndrome have abnormalities in all of these areas.

    Long-gap esophageal atresia may also result from surgery to try to fix a milder case of esophageal atresia, or to repair a tracheoesophageal fistula.

    Signs and symptoms

    What are the signs and symptoms of esophageal atresia?

    The first signs of esophageal atresia are usually clearly seen very soon after birth. The most common are:

    • frothy white bubbles in your baby’s mouth
    • coughing or choking when feeding
    • vomiting
    • blue color of the skin, especially when your baby is feeding
    • difficulty breathing
    • very round, full abdomen


    FAQ

    Q: What is the treatment for esophageal atresia?
    A:
    Treatments for EA vary depending on how severe it is. Usually, the best treatment is surgery to reconnect the two ends of your baby’s esophagus to each other when she is between 2 and 6 months old.

    In some children, however, the ends are so far apart they cannot be easily connected. This is known as long-gap esophageal atresia. The Foker process was developed to address this condition.

    Q: What is the Foker process?
    A: 
    Developed by John Foker, MD, a pediatric surgeon from the University of Minnesota, the Foker process is a technique to stimulate the upper and lower ends of the esophagus to grow so they could be joined together. It’s only available here at Children's. Learn more.

    Q: How long does the Foker process take?
    A: It depends on how far your child’s esophagus needs to grow, but surgeons are usually able to attach the two ends together in anywhere from two to five weeks.

    Q: What are the other treatments for esophageal atresia?
    A: 
    Other treatments for esophageal atresia involve different types of surgery. Your child’s care team work together to determine the best treatment for her EA. Learn more.

    Q: What’s the long-term outlook for children with esophageal atresia?
    A: 
    If caught early and treated properly, most babies with esophageal atresia can eat normally within two to three months. They may need to be monitored 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’s indistinguishable from one that has developed normally.

  • The first step in treating your child is forming an accurate and complete diagnosis.

    Esophageal atresia is detected shortly after birth, usually right after the baby first tries to feed.

    If a baby has esophageal atresia, she might sputter, cough and choke, because the liquid may be filling up the back of her throat and blocking her airway.

    If she has a tracheoesophageal fistula, an abnormal connection between the esophagus and the trachea that is commonly seen with EA, food may enter her lungs when she swallows, causing her to cough it up.

    If your baby is unable to cough out the food, doctors put a tube down her mouth or nose into her esophagus to suction it out. If her esophagus is blocked, the tube will only go a short way in. Then, the doctors usually order an x-ray and call in a surgeon.

    At Children’s, after we complete all necessary tests, our experts 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.

    Can esophageal atresia be diagnosed prenatally?

    Sometimes long-gap esophageal atresia may be indicated on a pre-natal ultrasound. A fetus may seem to have a very small or barely visible stomach. But this doesn’t necessarily mean that the baby has esophageal atresia.

  • You may have heard that esophageal atresia can be challenging to treat. Unfortunately, that's true. But the dedicated, compassionate staff at Children's is incredibly well-qualified to care for your child. We're known for our science-driven approach—we're home to the most extensive research enterprise located in a pediatric hospital in the world, and we partner with a number of top biotech and health care organizations—but we never forget that your child is a child, and not just a patient.

    The best treatment for esophageal atresia is usually surgery to connect the ends of the esophagus together. But in long-gap esophageal atresia, the ends are often too far apart to be easily connected.

    Traditionally, babies with this type of long-type atresia are treated in ways that require life-long care and monitoring, such as pulling up the stomach to connect directly to the end of the esophagus (gastric pull-ups), or creating more “esophagus” from a piece of the child's large intestine (colonic transposition). We are proud to offer an alternative to this through a one-time solution known as the Foker Process.

    The Foker Process

    Here's what happens during the Foker Process:

    1. The surgeon places around four or five sutures on the upper and lower ends of your child's esophagus. These are tiny stitches that connect the two ends.

    2. The tension on the sutures is increased bit by bit, causing each end of the esophagus to grow about one to two millimeters each day—just like a muscle grows when you exercise it. The surgeon applies the proper amount of tension to the ends of the esophagus to create just enough growth for it to be attached.

    Depending on how much your baby's esophagus needs to grow, this may take between two and five weeks. During this time, your baby is on respiratory and nutritional supportive devices and closely monitored by her health care team.

    3. Then, during a second operation, the sutures are removed, and the ends of the esophagus are sewn together.

    Other treatments

    Some babies with milder cases of esophageal atresia may not need the Foker process. Our doctors are experts in choosing the best treatment for each child. For children who don't have long-gap esophageal atresia, treatment options include:

    • Colonic interposition: Surgeons move a section of colon from its place in the gut to the space left by the esophageal gap, in effect creating a replacement esophagus. 
    • Jejunum interposition: Rather than a piece of colon, surgeons use a section of the jejunum (the middle part of the small intestine) as a replacement esophagus. 

    Learn more about other treatments for esophageal atresia.


    How will you follow up with my child?

    Each child is different, but often they're seen every year or two throughout childhood to make sure that there are no problems or complications.

    What's the long-term outlook for babies with esophageal atresia?

    If caught early and treated properly, most babies with esophageal atresia can eat normally within two to three months. They may need to be monitored 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's indistinguishable from one that has developed normally.

  • At Boston Children's Hospital, we’re known for our science-driven approach. In fact, we’re home to the world's most extensive pediatric hospital research enterprise; and we partner with elite health care and biotech organizations around the globe. But as specialists in innovative, family-centered care, our physicians never forget that your child is precious, and not just a patient.

    Until recently, esophageal atresia (EA) was a condition with no truly satisfactory treatment options. Previous treatments involved stressful stretching of the esophagus, drastic repositioning of internal organs or using transplanted tissue to build a replacement esophagus.

    For a child with long-gap esophageal atresia, the revolutionary Foker process encourages natural growth and strengthening of a child’s existing esophagus with the end result being a normal, functioning esophagus.

    The idea

    John Foker, MD, a pediatric surgeon from the University of Minnesota, knew that a fetus’ normal esophagus develops due to the tension placed on it by growing bones. To encourage this same tension-induced elongation for children with EA following a baby’s birth, he surgically attached traction sutures to the tiny esophageal ends and increased the tension on these sutures, bit by bit. To date, all of the patients treated with this method are able to eat and swallow like other children.

    How it works

    • at least two operations are required
    • first operation attaches traction sutures to both ends of the esophagus
    • tension increases on the sutures over the course of several days or weeks (depending on length of the gap)
    • traction sutures stimulate upper and lower ends of the esophagus to grow
    • another operation removes sutures and joins esophageal ends

    Bringing the Foker process to Boston Children's Hospital

    After inventing this process, Foker met with skepticism from his peers. Fortunately, Children’s surgeon Russell Jennings, MD, emerged as one believer. In 2009, Jennings visited Foker in Minnesota to assist him in operations employing the Foker process.

    Jennings now put his education to use at Children’s. Together with Bradley Linden, MD, he’s established Children’s Esophageal Advanced Treatment Center, the world’s only center offering the Foker process.

  • Stretching the limits: For Elliot Cleckler, millimeters made all the difference in treating his esophageal atresia

    Five weeks before his due date, Elliot Cleckler was diagnosed with esophageal atresia and tracheoesophageal fistula (TEF), an abnormal connection between the esophagus and the trachea that can cause food traveling down the esophagus or acid refluxing up from the stomach to pass into the lungs. His parents, Jay and Heather, were upset but hopeful. “We left that meeting thinking, it could be so much worse; at least it’s just an esophagus,” says Jay.

    “Everyone told us not to look up LGEA on the Internet,” says Jay. “So, of course we did.” What Jay and Heather found were stories of children like Elliot, who endured failed surgery after failed surgery. Many of the “success” stories they found centered on colonic transposition, in which a piece of excess colon is stitched into place as a replacement esophagus, and gastric pull-up, where the stomach is forced up into the chest and connected directly to the top section of esophagus. But both procedures have significant drawbacks.

    Elliot’s surgeons instead advocated for attempting to stretch the two nubs of his esophagus over time to see if the ends could be made to meet, but the Clecklers had reservations. In the best cases, stretching still constricts the internal flow through the esophagus and thins the external walls, making them less resilient. In the worst cases, the esophagus, like a rubber band, will tear if it reaches its elastic limit.

    None of the options gave the Clecklers much hope. “We kept coming across statistics showing increased esophageal cancer rates and low growth percentiles,” says Heather. “All of a sudden we realized we had gone from reading scary stories to living one”…

    Read more about Elliot’s journey at http://apps.childrenshospital.org/x-dream/winter10/stretching_the_limits.html

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