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Hirschsprung-associated Enterocolitis

  • Cynthia M. Johnson, MA
Publication Type:


Hirschsprung-associated Enterocolitis



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.


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HAEC may be caused by:

  • An intestinal blockage caused by HD
  • A 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 HAEC 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


Problems may be:

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


The doctor will ask about the child’s symptoms and past health. A physical exam will be done.

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

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


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. Saltwater 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.


There are no known methods to prevent HAEC.





  • Hirschsprung disease. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/hirschsprung-disease.
  • Hirschsprung’s disease. About Kids GI—International Foundation for Functional Gastrointestinal Disorders website. Available at: https://aboutkidsgi.org/lower-gi/hirschsprungs-disease.


  • Kari Kuenn, MD
Last Updated:

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.