What is Hirschsprung’s Disease?

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Hirschsprung disease is a birth defect in which nerve cells are missing at the end of a child’s bowel. Normally, the bowel contains many nerve cells all along its length that control how the bowel works. When the bowel is missing nerve cells, it does not work well. This damage causes blockages in the bowel because the stool does not move through the bowel normally.

Most often, the areas missing the nerve cells are the rectum and the sigmoid colon. However, some children are missing the nerve cells for the entire colon or part of the small intestine.

• In short-segment Hirschsprung disease, nerve cells are missing from the last part of the large intestine.

• In long-segment Hirschsprung disease, nerve cells are missing from most or all of the large intestine and sometimes the last part of the small intestine.

• Rarely, nerve cells are missing in the entire large and small intestine.

In a child with Hirschsprung disease, stool moves through the bowel until it reaches the part lacking nerve cells. At that point, the stool moves slowly or stops.

Copied from: NIDDK. Please click the link for even more information about Hirschsprung’s Disease

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