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Hirschsprung-associated Enterocolitis

(HAEC)

How to Say It: HIRSH-sprung ah–SO-shee-ay-ted ENT-ero-co-LYE-tis

Definition

Hirschsprung-associated enterocolitis (HAEC) is an inflammation or infection of the bowel in babies who have Hirschsprung disease (HD). HD is when an area of the colon (large intestine) cannot push stool through as it should. This can lead to a partial or full blockage.

HAEC can happen suddenly. Care is needed right away.

Enterocolitis
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Causes

HAEC may be caused by:

  • An intestinal blockage caused by HD
  • Bacterial or viral infection—bacteria may grow more quickly in the intestines of babies with HD
  • Other changes in the intestines caused by HD

Risk Factors

Things that may raise the risk of this problem are:

  • Undiagnosed HD—HD is usually diagnosed in infants, but it may not be diagnosed until a child is older.
  • Pull-through surgery to treat HD—An unhealthy area of the colon is removed. The healthy colon is then joined to the rectum.
  • Down syndrome
  • Having long sections of the colon that are affected by HD

Symptoms

Problems may be:

  • Bloating
  • Severe diarrhea
  • Vomiting
  • Fever
  • Lack of energy
  • Poor feeding
  • Rectal bleeding

Diagnosis

You will be asked about your child’s symptoms and health history. A physical exam will be done.

Your child's bodily fluids may be tested. This can be done with blood tests.

Images may be needed of the intestines and other nearby structures. This can be done with abdominal x-rays.

Treatment

The goal of treatment is to ease inflammation and infection. Options are:

  • Antibiotics to treat infection
  • Rectal irrigation—A catheter will be pushed into the colon. Salt water will be pushed through it to allow gas and stool to come out of the rectum.

Some children may need surgery. This is not common.

Prevention

There are no known methods to prevent this health problem.

RESOURCES:

Healthy Children—American Academy of Pediatrics
https://www.healthychildren.org

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

CANADIAN RESOURCES:

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

Caring for Kids—Canadian Paediatric Society
https://www.caringforkids.cps.ca

REFERENCES:

Hirschsprung disease. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/hirschsprung-disease. Accessed August 30, 2021.

Hirschsprung’s disease. About Kids GI—International Foundation for Functional Gastrointestinal Disorders website. Available at: https://aboutkidsgi.org/lower-gi/hirschsprungs-disease. Accessed August 30, 2021.

Last reviewed July 2021 by EBSCO Medical Review Board Kari Kuenn, MD  Last Updated: 8/30/2021