Intestinal atresia and stenosis

  • Nutrition is absorbed by the intestines similar to the way that water is absorbed by a sponge. But some children are born with defects that prevent their intestines from “soaking up” all the nutrition their growing bodies need. This most often happens in the small intestine (small bowel), and very occasionally in the large intestine.

    If your child has intestinal atresia, it means that his intestines haven’t formed correctly. There may be areas of blockage that prevent nutrition from flowing through the digestive tract, or some sections may not be connected together.  

    With intestinal stenosis, the intestine isn’t completely blocked, but the inside space (lumen) has narrowed so much that it’s difficult for nutrition to move through. Both intestinal atresia and intestinal stenosis are also sometimes referred to as “intestinal obstructions,” and they:

    • are thought to be caused by an inadequate supply of blood to your baby’s intestines during fetal development
    • are usually discovered within a day or two of birth
    • affect boys and girls in equal numbers
    • appear to run in families, although a specific genetic cause hasn’t been discovered yet

    Intestinal obstructions can nearly always be removed with surgery, but the procedure carries some risk. During surgery, the surgeon meticulously removes as little of the intestine as absolutely necessary, but sometimes fixing the obstruction means your child is left with not enough of his small intestine to absorb all the nutrition his growing body needs. This condition is called short bowel syndrome (SBS).

    Fortunately, there are many treatments for intestinal obstructions, and it is no longer the life-threatening condition it once was.  

    The Boston Children’s Hospital approach

    At Children’s, we treat children with intestinal atresia and stenosis in our Center for Advanced Intestinal Rehabilitation (CAIR), one of the world's premier programs for treating children with of short bowel syndrome, a complex and often devastating disorder caused by the loss of part of the small bowel.

    Our program is staffed by a team of experts with lots of experience in caring for children with intestinal problems and their families. Our specialists include:

    • physicians trained in surgery, gastroenterology and nutrition
    • registered dieticians
    • nurse practitioners
    • nurses
    • pharmacist
    • social worker

    Our clinical care is informed by our research. Our researchers have made major contributions to the field: In 2002, Children's doctors performed the world's first serial transverse enteroplasty (STEP) procedure, a surgical technique developed by Tom Jaksic, MD and Heung Bae Kim, MD, which lengthens the bowels of children with short bowel syndrome. Call 617-355-5275 for an appointment.

     

    Intestinal stenosis / atresia: Reviewed by Christopher P. Duggan, MD, MPH
    © Children’s Hospital Boston, posted in 2011

  • Background

    The small intestine (small bowel) varies in length between 10 and 28 feet, and is divided into three main sections:

    1. The duodenum is a relatively short section of the small intestine, measuring about 10 to 12 inchesl. It links the stomach to the rest of the small intestine.

    2. The jejunum is the largest section, because its many folds give it increased surface area for absorbing nutrients.

    3. The ileum is the last and longset section of the small intestine, accounting for around 3/5 of the small intestine. It connects to the large intestine.

    4. The empty space in the intestine (through which digested food travels) is called the lumen.

    What are the types of intestinal atresia and stenosis?

    Intestinal obstructions are usually classified according to where in your child’s intestine the absence, blockage or narrowing is found. Obstruction in the duodenum is known as duodenal atresia/stenosis and obstructions in the jejunum or the ileum are both called jejunoileal atresia/stenosis.

    How common are intestinal obstructions?

    Intestinal obstructions occur in about one out of every 1,500 babies, and boys and girls are equally affected. 

    Can intestinal atresia and stenosis be detected before birth?

    Yes, intestinal obstructions are being more and more frequently identified during prenatal ultrasounds.

    What are the complications of intestinal atresia and stenosis? 

    While every child’s situation is unique, there sometimes relate to other conditions, and they seem to vary according to location:

    • Jejunal obstruction may accompany intestinal malrotation, hernia or abdominal wall defects that strangle the small intestine, interrupting its blood supply.
    • About half of all infants with duodenal obstruction are born prematurely, and more than 30 percent have Down’s syndrome. Between 50 and 75 percent have other anomalies, which often affect other parts of the digestive system, the heart or the kidneys.


    Causes

    What causes intestinal atresia and stenosis?

    Intestinal atresia and stenosis are thought to be caused by an inadequate supply of blood to your baby’s intestines during fetal development. They appear to run in families, although a specific genetic cause has yet to be discovered.


    Symptoms

    What are the signs of intestinal atresia and stenosis?

    If your baby has an intestinal obstruction, it will usually be discovered within a day or two of his birth. You and/or your child’s doctors may notice him:

    • not tolerating feedings
    • vomiting
    • with an abdomen looks bloated (distended)


    FAQ

    Q: What is the treatment for intestinal obstruction?
    A:
    When possible, surgery can remove the obstruction and/or reconnect your baby’s intestines. If surgery is not an option, intestinal obstruction can sometimes be treated with medication or nutritional protocols. 

    Q: What’s the long-term outlook for my child?
    A:
    Your child’s prognosis greatly depends on several factors, including how much of the small bowel is left after surgery and whether he has an associated condition. Many babies do quite well after surgery, and never have a problem again.

    Other babies need to continue nutritional support (a special formula through an IV or tube) for weeks, months or years, or even need an intestinal transplant. You can rest assured that your baby’s health care team will closely monitor his progress after treatment, and keep you aware of his status and any additional treatment options that may help.

  • The first step in treating your child is forming an accurate and complete diagnosis. If doctors suspect an intestinal obstruction after your baby was born, they will run tests to see whether there is an obstruction, and if so, determine its location. These diagnostic tests may include:

    • An x-ray and/or ultrasound of your baby’s abdomen can create images of his intestines that may show an obstruction. Or the doctors may give your child barium, a chalky liquid that coats the inside of your child’s organs and lets certain blockages and narrowing appear on an x-ray.

    • To examine the upper parts of the small intestines, your child will swallow the barium. This is known as an upper GI (gastrointestinal) test, and is also sometimes called a barium swallow.

    • For the lower parts of the small intestine, your child will be given barium through an enema. This is called a lower GI test, or a barium enema.

    Doctors may also perform imaging studies of your child’s heart and kidneys to check for the anomalies that sometimes accompany intestinal obstructions.


  • We understand that it can be very upsetting to learn that your baby was born with an intestinal obstruction. We look at the diagnosis as the very first step in determining the best treatment plan for your child. Here's how we can help your child: 

    1. At the Center for Advanced Intestinal Rehabilitation at Boston Children's Hospital, we have the latest innovations and most up-to-date clinical information at our disposal to ensure the best possible outcome for your baby.

    2. Our physicians are focused on family-centered care: From your first visit, you'll work with a team of professionals who are committed to supporting all of your family's physical and psychosocial needs.

    Surgery

    Surgery is often the best option for treating intestinal obstructions. Our surgeons are specialized pediatric surgeons with lots of experience working with newborns.

    What happens during surgery?
    First, your baby's surgery team will make sure that he is stable enough for surgery, and continue to monitor his breathing, body temperature, blood pressure, fluids and other vital signs throughout the surgery.

    Next, we'll give your baby medicine to help him sleep. Depending on the location of the obstruction in your baby's intestine, he may have a bloated abdomen. If this is the case, the doctor will pass a thin tube down his mouth or nose down into his stomach to remove the contents and relieve the pressure.

    Then the surgeon will make an incision in your baby's abdomen, and remove the affected part of the intestine, taking care to leave as much of your baby's healthy intestine in place as possible. Then she will reconnect your baby's intestines.

    What happens after surgery?
    After surgery, your baby may receive nutrition (a special, easily digested formula) through an IV, and our team will monitor him carefully to assess how well his intestines are healing. When the time is right, we'll transition him to fewer IV feedings and more oral or tube feedings, with the goal of weaning him off of IV feedings completely. 

    How long will this take?
    Unfortunately, there's no way to know in advance. This depends on how much of your baby's small intestines his surgeons had to remove, and how well the remaining intestines are working.

    Some babies are able to leave the hospital in a week, other babies develop short bowel syndrome and might stay on IV feedings for years. Sometimes too, babies will progress from the IV feedings to tube feedings. 

    Treatments for small bowel syndrome include a groundbreaking surgical procedure developed by Children's doctors  and intestinal transplant

    Follow up

    Your child's healthcare team will monitor your child carefully – whether he's here at the hospital or coming in for regular follow-up visits - to determine how well his respond after treatment. And please remember that we're always here to answer any questions you might have.
     

    Coping and support

    No parent wants her child to be ill, and it's important to remember that you and your family aren't alone. Many families have been down this path, and there's a lot of support available here at Children's for you and your family. Here are some of the ways we can help:

    Patient education: From the office visit to pre-op to the recovery room, our nurses will be on hand to walk you through your child's treatment and help answer any questions you may have — How long will I be separated from my child during surgery? What will the operating room be like? They will also reach out to you by phone, continuing the care and support you received while at Children's.

    Parent to parent: Want to talk with someone whose child has been treated for intestinal atresia or stenosis? We can put you in touch with other families who have been through similar experiences and can share with you their experience at Children's.

    Faith-based support: If you are in need of spiritual support, we will help connect you with the Children's chaplaincy. Our program includes nearly a dozen clergy representing Episcopal, Jewish, Lutheran, Muslim, Roman Catholic, Unitarian and United Church of Christ traditions who will listen to you, pray with you and help you observe your own faith practices during your hospital experience.

    Social work:  Our clinical social workers have helped many other families in your situation. Your social worker can offer counseling and assistance with issues such as coping with your child's diagnosis, stresses relating to coping with illness and dealing with financial difficulties. 

    Visit our For Patients and Families page for all you need to know about:

    • getting to Children's
    • accommodations
    • navigating the hospital experience
    • resources that are available for your family
  • Boston Children's Hospital is home to the world’s most extensive research enterprise at a pediatric hospital. We also have many partnerships with research, biotech and health care organizations, and we work together to find innovative ways to improve kids’ health.

    A new surgery

    Problem: The small intestine is a very important part of the digestive tract – it’s where we absorb most of the nutrients from the food we eat. But sometimes children may not be able to absorb all the nutrients their growing bodies require. This could happen because their intestines don’t work as they should, or because they don’t have enough small intestine, often as the result of surgery to correct congenital defects including intestinal atresia and stenosis. 

    When the child can’t absorb enough nutrition from her short intestines to meet her nutritional requirements, she has short bowel syndrome and is at risk for serious malnutrition, with all of its complications. 

    If a child doesn’t have enough small intestine, the remaining part tries to fix the problem on its own. It puffs up like a balloon, creating more surface area to draw in nutrients, just as we might set out more buckets if we wanted to collect more rainwater. But this has an unfortunate side effect: the wider the intestine, the longer it takes for the body to move nutrients through it. (Imagine holding a tube with water flowing through it. Now imagine squeezing the tube – the water flows faster.) More time in the intestines means more time for the bacteria that would normally be swept promptly along to multiply, increasing your child’s chance of infection.

    Solution: Children’s physicians Heung Bae Kim, MD, surgical director of the Pediatric Transplant Center, and Tom Jaksic, MD, PhD, surgical director of CAIR, figured out a way to solve both problems at once. The procedure is called serial transverse enteroplasty (STEP).

    STEP solves both problems:

    1. It exposes more usable surface area, similar to the way that sharpening a pencil exposes more lead.

    2. It creates a narrower space, which keeps food moving through your child’s digestive tract at an appropriate pace, carrying bacteria out of her body along with the rest of the body’s waste.

    Watch a video of an actual STEP procedure.

    Results: The STEP procedure is now being used worldwide, allowing some children to wean completely from IV nutrition and avoid the need for intestinal transplantation.  In the spring of 2004, the STEP Data Registry was started to track outcomes of children who have had the STEP procedure, so that we can better understand its effectiveness compared to other bowel-lengthening procedures.

     Learn more about the STEP procedure.
     

    Smarter nutrition

    Problem: Some children with intestinal failure can only receive nutrients intravenously (through an IV). But prolonged use of IV feedings often damages the liver, which can lead to liver failure and the need for a liver transplant.

    Solution: Children’s researchers Mark Puder, MD, Jenna Garza, MD, and Kathy Gura, PharmD discovered that the then-standard formula included a fat mixture that made fat accumulate in the liver, which may contribute to liver disease. They discovered that switching from the old fat to Omegavan, a fat mixture made from fish oil, (which has been shown to prevent fat accumulation) resulted in a formula that’s far less damaging to the liver.  

    Result: This discovery has caused a worldwide shift in treatment. Puder and colleagues are now conducting a formal clinical trial to learn how it can be used even more effectively.

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