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  • Hirschsprung’s-associated Enterocolitis



    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 feces 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.


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


    The doctor will ask about your child’s symptoms and medical history. A physical exam will be done.
    The doctor may do the following:
    • Lab tests—eg, complete blood count, electrolytes, and blood culture to check for infection
    • X-ray of the abdomen—a test that uses radiation to take a picture of structures inside the body
    If your child has had pull-through surgery to treat an intestinal blockage, he will be closely monitored for symptoms of HAEC. While most cases of HAEC occur within two years after pull-through surgery, it can occur up to 10 years following surgery.
    If the doctor suspects HAEC, barium enemas will be avoided. This is because of an increased risk of bowel perforation.


    Talk with the doctor about the best treatment plan for your child. Treatment options include:
    • For serious cases, rectal irrigation and IV antibiotics (given using a needle placed in the arm) are used. For rectal irrigation, the doctor will gently push a catheter into the colon. Saline (salt water) will be pushed in through the catheter. It will slowly drain out. This allows gas and feces 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.


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


    American Academy of Pediatrics http://www.aap.org/

    International Foundation for Functional Gastrointestinal Disorders http://www.iffgd.org/


    Canadian Association of Gastroenterology http://www.cag-acg.org/

    Canadian Paediatric Society http://www.cps.ca/


    Badash M. Hirschsprung’s Disease. EBSCO Health Library website. Available at: http://www.ebscohost.com/healthLibrary/ . Updated September 2010. Accessed October 22, 2010.

    Enterocolitis Associated with Hirschsprung’s Disease. University of Michigan Department of Surgery: Pediatric Surgery website. Available at: http://surgery.med.umich.edu/pediatric/clinical/physician%5Fcontent/a-m/enterocolitis.shtml. Updated January 11, 2010. Accessed October 22, 2010.

    Hirschsprung’s Disease. About Kids GI website. Available at: http://www.aboutkidsgi.org/site/about-gi-health-in-kids/functional-gi-and-motility-disorders/hirschsprungs-disease . Updated October 2, 2009. Accessed October 22, 2010.

    Hirschsprung’s Disease. Mayo Clinic website. Available at: http://www.mayoclinic.com/health/hirschsprungs-disease/DS00825 . Updated November 2008. Accessed October 22, 2010.

    Kessmann J. Hirschsprung’s disease: diagnosis and management. Am Fam Physician . 2006 Oct 15;74(8):1319-1322.

    Lucey JR. Necrotizing enterocolitis. EBSCO Health Library website. Available at: http://www.ebscohost.com/healthLibrary/ . Updated September 2010. Accessed October 22, 2010.

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