Definition

Hirschsprung-associated enterocolitis (HAEC) is a complication of Hirschsprung disease. This is a rare condition that occurs in babies. Nerve cells normally help control muscles that allow stool to move through the colon. The absence of these nerve cells results in bowel obstruction, preventing normal bowel movements.

HAEC can happen suddenly and requires immediate care by a doctor. In most cases, hospital care is needed.

Enterocolitis

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Causes

HAEC is an inflammation or infection of the bowel. This may be caused by:

  • An intestinal blockage caused by Hirschsprung disease.
  • Bacterial or viral infection—Because of Hirschsprung disease, bacteria may grow more quickly in the intestines.
  • Other changes in the intestines caused by Hirschsprung disease.

Risk Factors

Factors that may increase your child's chances of HAEC:

  • Undiagnosed Hirschsprung disease—Usually diagnosed in infancy, but may not be diagnosed until your child is older.
  • Pull-through surgery—A procedure to treat Hirschsprung disease. The unhealthy area of the colon is removed. The healthy colon is then joined to the rectum.
  • Down syndrome —HAEC occurs in nearly half of those with Down syndrome who have Hirschsprung disease.
  • Long section of colon affected by Hirschsprung disease—The risk of HAEC is greater when long sections of the colon are affected.

Symptoms

Symptoms may include:

  • Bloating
  • 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 chances of HAEC, rectal irrigation may be done after pull-through surgery to try to prevent HAEC.