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Hirschprung's Disease

Signs and Symptoms
Diagnosis
Treatment

Signs and Symptoms

Hirschsprung's Disease is a birth defect that affects about one out of 5,000 newborns. Babies with this birth defect are born without intestinal nerve cells called ganglion cells. These nerve cells allow the intestine to relax so stool can pass through the intestine and out of the body. Without these special nerve cells, the intestine can't relax and it becomes very narrow.

In most babies with Hirschsprung's Disease, only a small part of the large intestine (near the rectum) is missing ganglion cells. In some babies, all of the large intestine is missing ganglion cells. Very rarely, babies can be born without ganglion cells in both the large and small intestine.

When the intestine is narrowed, stool can't easily pass. As a result, babies with Hirschsprung's Disease can't have bowel movements on their own and have severe constipation.

Most babies have a bowel movement in the first one to two days after birth. Hirschsprung's Disease is usually suspected when a baby doesn't have a bowel movement for several days following birth. Babies with Hirschsprung's Disease often have large, swollen abdomens and may vomit green bile after feeding.

Reviewed by health care specialists at UCSF Children's Hospital.
Last updated April 19, 2007