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
These symptoms may be caused by other conditions. If your child has any of these symptoms, tell the doctor right away.

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