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Hirschsprung's Disease

Signs and Symptoms
Diagnosis
Treatment

Signs and Symptoms

Hirschsprung's disease is a birth defect that causes a blockage or narrowing of the intestines. Babies with this condition lack intestinal nerve cells called ganglion cells, which allow the intestine to relax and contract so stool can pass through and out of the body. Without these nerve cells, the intestine narrows.

In most babies with Hirschsprung's disease, ganglion cells are missing from only a small part of the large intestine or colon, near the rectum. In some babies, ganglion cells are missing from throughout the large intestine. Rarely, babies are born without ganglion cells in both the large and small intestine.

When the intestine narrows, stool can't easily pass and babies are unable to have bowel movements on their own, resulting in severe constipation.

Most babies have a bowel movement in the first one to two days after birth. Hirschsprung's disease is usually suspected if a newborn doesn't have a bowel movement within the first two days after birth. Babies with Hirschsprung's disease may have swollen abdomens and may vomit green bile after feeding.

Reviewed by health care specialists at UCSF Children's Hospital.
Last updated January 15, 2008