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Hirschsprung's Disease - Topic Overview

What is Hirschsprung's disease?

Hirschsprung's disease is a birth defect that affects the nerve cells in the large intestine. These nerve cells control the muscles that normally push food and waste through the large intestine.

But in babies who have Hirschsprung's disease, the muscles in the wall of the large intestine don't relax, which prevents stool from passing. This can lead to trapped stool, infection, pain and swelling, and bowel problems.

Most of the time, the disease is found soon after birth in about 1 out of every 5,000 newborns. It is most common in male babies.1

In rare cases, the disease can be life-threatening.

What causes Hirschsprung's disease?

Doctors don't know what causes the disease, but it tends to run in families. It may also be linked to other medical problems, such as Down syndrome and congenital heart disease.

What are the symptoms?

Symptoms include a swollen belly and problems passing stool. Newborns with the disease may not pass their first stool until at least 48 hours after birth.

Other symptoms depend on a child’s age, but can include vomiting, having a poor appetite, refusing to eat, and not growing or gaining weight.

How is Hirschsprung's disease diagnosed?

Most children are diagnosed with Hirschsprung’s disease during their first year. A doctor may think that a child has the disease based on the child's symptoms and the results of a physical exam.

Other tests may be done to confirm the diagnosis, such as:

  • A tissue sample from the rectum (biopsy).
  • An abdominal X-ray.
  • An X-ray of the large intestine (barium enema).

If there is a delay in diagnosing Hirschsprung’s disease, the child could end up with serious, life-threatening complications.

How is it treated?

Children with Hirschsprung’s disease need surgery to remove the diseased part of the large intestine. Surgery is often done within the first days or month of life, soon after the disease is found. Children with Hirschsprung’s disease need one or two surgeries.

Long-term (chronic) problems with stomachaches and bowel problems are common after surgery. But most of the time, these problems are not severe. Depending on the problem, there are a number of treatment options. These include medicine, behavior modification, biofeedback, cognitive-behavioral therapy, and more surgery.

In a few cases, emergency surgery may be needed if a dangerous problem such as serious swelling of the small and large intestines (enterocolitis) occurs.

Frequently Asked Questions

Learning about Hirschsprung's disease:

Being diagnosed:

Getting treatment:

Living with Hirschsprung's disease:

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WebMD Medical Reference from Healthwise

Last Updated: September 09, 2010
This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.
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