KidsMD Health Topics

Intestinal Malrotation

  • When a fetus is about five weeks old, her intestine exits her abdomen into the amniotic fluid (where there’s more space), and continues to grow there. At around ten weeks, the intestine re-enters the abdomen, and makes two turns. Sometimes the intestines don’t make the turns as they should, resulting in the congenital (present at birth) condition known as intestinal malrotation.

    Intestinal malrotation itself isn’t much of a concern, but it puts your child at higher risk for two serious complications:

    • volvulus – when the intestine twists in on itself, potentially cutting off the blood supply

    • intestinal obstruction – when a stalk of fibrous tissue known as Ladd’s bands creates a blockage that prevents the intestine from functioning

    Intestinal Malrotaion
    Intestinal malrotation with volvolus
    click to enlarge

    It’s fairly common that a baby is born with intestinal malrotation; it affects about one in every 500 babies in the United States. Some babies may not have symptoms until they become children, teens or adults. Others may go through their entire life with no symptoms, never have a problem and never be diagnosed.

    Of babies who are diagnosed with intestinal malrotation:

    • 25 to 40 percent are diagnosed in the first week of life
    • 50 to 60 percent are diagnosed within the first month of life
    • 75 to 90 percent are diagnosed by age 1

    Although malrotation occurs equally among boys and girls, boys are more likely to become symptomatic by the first month of life.

     

    How Children’s Hospital Boston approaches intestinal malrotation

    At Children’s, we treat children with intestinal malrotation in our Center for Advanced Intestinal Rehabilitation (CAIR)one of the largest centers of its kind in the United States. Each year, our program cares for about 200 children from the United States and abroad.

    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
    • residents, fellows and students

    Call 617-355-5275 for an appointment.

    Boston Children's Hospital
    333 Longwood Avenue,
    Fegan 3
    Boston MA 02115

     617-355-5275

  • What causes intestinal malrotation?

    As a fetus grows in its mother's uterus, its organ systems are developing and maturing. The digestive tract starts as a straight tube from the stomach to the rectum; for a while, a part of the intestine protrudes from the abdomen into the umbilical card. But once the fetus reaches 10 weeks, the intestine separates from the umbilical cord, returns to the abdomen and makes two turns, so that it is no longer a straight tube.

    Malrotation occurs when the intestine does not make these turns as it should.

    What complications may result from malrotation?

    Intestinal malrotation causes the end of the small intestine, called the cecum, to develop abnormally, which leads to abnormal positioning.

    Normally, the cecum is located in the lower right side of the abdomen. With malrotation, the cecum and appendix (which is attached) remain in the upper right side of the abdomen. Bands of tissue form between the cecum and the intestinal wall and can create a blockage in the beginning of the small intestine.

    Intestinal Malrotation
    Intestinal malrotation with volvulus
    (click to enlarge)

    A volvulus is a problem that can occur after the baby is born as a result of malrotation: The intestine becomes twisted, causing an intestinal blockage or cutting off blood flow to the intestine.

    How often does malrotation and volvulus occur?

    Intestinal malrotation occurs in 1 out of every 500 live births in the United States. Of those children who have malrotation and develop symptoms, most symptoms will occur in the baby's first year of life.

    • 25 to 40 percent of babies are diagnosed in the first week of life.

    • 50 to 60 percent of babies are diagnosed by the first month of life.

    • 75 to 90 percent of babies are diagnosed by age 1.

    • Some people who have malrotation go through their entire life without having any symptoms and are never diagnosed. Others may not have symptoms until childhood, adolescence or adulthood.

    Which children are at risk for having malrotation?

    Malrotation occurs equally in boys and girls. However, more boys become symptomatic by the first month of life than girls. Up to 30 percent of children with intestinal malrotation also have another congenital malformation, including the following:

    • digestive system abnormalities
    • cardiac abnormalities
    • abnormalities of the spleen
    • abnormalities of the liver

    Why is intestinal malrotation a concern?

    A child with malrotation is likely to experience a twisting of the intestine, known as a volvulus. This will cause an obstruction, preventing food from being digested normally. The blood supply to the twisted part of the intestine can also be cut off, which can lead to the death of that segment of the intestine. Ladd's bands, formed between the cecum and the intestinal wall, can also create a blockage in the duodenum, preventing food from being digested. A child can become dehydrated quickly when intestinal blockage occurs.

    What are the symptoms of malrotation and volvulus?

    Yellow or green vomit is an early sign of malrotation. If your child has this kind of discolored vomit, she should be evaluated immediately by a doctor. If your child's intestine becomes twisted or obstructed, her symptoms may include:

    • vomiting bile
    • drawing up the legs
    • abdominal pain
    • abdominal swelling or distention
    • rapid heart rate
    • rapid breathing
    • bloody stools

    Will my child have life-long problems, even after treatment?

    The majority of children with malrotation who experienced a volvulus do not have long-term problems if the volvulus is repaired promptly and they have no intestinal damage. Children with intestinal injury who had the damaged part removed may have long-term problems.

    When a large portion of the intestine is removed, the digestive process can be affected. Nutrients and fluids are absorbed from food in the small intestine. Removing a large segment of the intestine can prevent a child from getting adequate nutrients and fluids. In this case, nutrition may need to be supplemented with long-term, high-calorie IV (intravenous) solutions given through special IV catheters.

  • In addition to a physical examination and medical history, diagnostic procedures for malrotation and volvulus may include various imaging studies (tests that show pictures of the inside of your child's body). These tests are performed to evaluate the position of the intestine, and whether it is twisted or blocked. They may include:

    • abdominal x-ray - a diagnostic test that may show intestinal obstructions
       
    • barium swallow / upper GI test - a procedure performed to examine the intestine for abnormalities. A fluid called barium (a metallic, chemical, chalky, liquid used to coat the inside of organs so that they will show up on an x-ray) is swallowed. An x-ray of the abdomen may show an abnormal location for the small intestine, obstructions (blockages) and other problems.
       
    • barium enema - a procedure performed to examine the intestine for abnormalities.
  • Malrotation of the intestines is usually not evident until a child's intestine becomes twisted or obstructed and symptoms are present; a volvulus is considered a life-threatening problem.

    • Children may be started on IV fluids to prevent dehydration and antibiotics to prevent infection. A nasogastric tube may be guided from the nose, through the throat and esophagus, to the child's stomach to prevent a gas buildup.
       
    • A volvulus is usually surgically repaired as soon as possible. The intestine is untwisted and checked for damage. Ideally, the circulation to the intestine will be restored after it is unwound, and it will turn pink. If the intestine is healthy, it is replaced in the abdomen. Since the appendix is located in a different area than usual, it would be difficult to diagnose appendicitis in the future; therefore, an appendectomy (surgical removal of the appendix) is also usually performed.
       
    • If a child's blood supply to the intestine is in question, the intestine may be untwisted and placed back into the abdomen. Another operation will be done in 24 to 48 hours to check the health of the intestine. If it appears the intestine has been damaged, the injured section may be removed.

    If the injured section of intestine is large, a significant amount of intestine may be removed. In this case, the parts of the intestine that remain after the damaged section is removed may not be able to be attached to each other surgically. Doctors may perform an ostomy, during which the two remaining healthy ends of intestine are moved through openings in the abdomen. Stool will pass through the opening (called a stoma) and then into a collection bag. The ostomy will be temporary and, depending on the amount of intestine that needed to be removed, it will be closed after your child has fully recovered.

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