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Hirschsprung’s-associated Enterocolitis

  • Definition

    Hirschsprung’s-associated enterocolitis (HAEC) is a complication of Hirschsprung’s disease . This is a rare condition that occurs in babies. It occurs when there are no nerve cells in the bowel. These nerve cells normally help control the bowel muscles that allow stool to move through the colon. The absence of these cells results in a bowel obstruction. This prevents normal bowel movements.
    Enterocolitis is an inflammation or infection of the bowel. HAEC can happen suddenly and requires immediate care by a doctor. In most cases, hospital care is needed.
  • Causes

    HAEC occurs when the bowel becomes inflamed or infected. This may be caused by:
    • An intestinal blockage caused by Hirschsprung’s disease.
    • Bacterial or viral infection—Because of Hirschsprung’s disease, bacteria may grow more quickly in the intestines.
    • Other changes in the intestines caused by Hirschsprung’s disease.
  • Risk Factors

    Risk factors for HAEC include:
    • Undiagnosed Hirschsprung’s disease—It is usually diagnosed in infancy. But it may not be diagnosed until your child is older.
    • Pull-through surgery—This is surgery to treat Hirschsprung’s disease. The unhealthy area of the colon is removed. Then, the healthy colon is joined to the rectum.
    • Down syndrome —HAEC occurs in nearly half of those with Down syndrome who have Hirschsprung’s disease.
    • Long section of colon affected by Hirschsprung's disease—The risk of HAEC is greater when long sections of the colon are affected.
  • Symptoms

    Symptoms may include:
    • Bloated abdomen
    • Severe diarrhea
    • Vomiting
    • Fever
    • Lethargy
    • Poor feeding
    • Rectal bleeding
    These symptoms may be caused by other conditions. If your child has any of these symptoms, tell the doctor right away.
  • Diagnosis

    You will be asked about your child’s symptoms and medical history. A physical exam will be done.
    Your child's bodily fluids may be tested. This can be done with blood tests.
    Abdominal x-rays may be used to evaluate the intestines or other nearby structures.
    A child who has had pull-through surgery to treat an intestinal blockage will be closely monitored for symptoms of HAEC. While most cases of HAEC occur within 2 years after pull-through surgery, it can occur up to 10 years following surgery.
    If the doctor suspects HAEC, barium enemas will be avoided. Barium enemas increases the risk of bowel perforation.
  • Treatment

    Talk with the doctor about the best treatment plan for your child. Treatment options include:
    • For serious cases, rectal irrigation and IV antibiotics are used. For rectal irrigation, a catheter will be gently pushed into the colon. Salt water will be pushed in through the catheter. It will slowly drain out. This allows gas and stool to come out of the rectum. Rarely, surgery is required to treat HAEC.
    • For mild cases, the doctor may use oral antibiotics and rectal irrigation.
  • Prevention

    To help reduce your child’s chance of HAEC, rectal irrigation may be done after pull-through surgery to try to prevent HAEC.

    Healthy Children—American Academy of Pediatrics

    International Foundation for Functional Gastrointestinal Disorders


    Canadian Association of Gastroenterology

    Caring for Kids—Canadian Paediatric Society


    Hirschsprung’s Disease. International Foundation for Functional Gastrointestinal Disorders About Kids GI website. Available at: Updated October 28, 2014. Updated October 30, 2014.

    Kessmann J. Hirschsprung’s disease: diagnosis and management. Am Fam Physician. 2006;74(8):1319-1322.

    Revision Information

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