Digestive System Home > Hirschsprung's Disease

Hirschsprung's disease is a medical condition in which specialized nerve cells fail to form in the large intestine during a baby's development. Severe constipation is often the result. The causes of this disease are unknown; it is not caused by anything the mother does while pregnant. Treatment is required for this condition, often with some type of surgery.

What Is Hirschsprung's Disease?

Hirschsprung's disease is a disease of the large intestine (also known as the colon). It usually occurs in children before a child is born.
 
Most often, the disease causes severe constipation. Some children with the disease can't have bowel movements at all.
 
If Hirschsprung's disease is not treated, stool can fill up the large intestine and create a blockage. This can cause serious problems, like infection, bursting of the colon, and even death.
 

Understanding the Intestines

Normally, muscles in the intestine push stool to the anus, where it leaves the body. Special nerve cells in the intestine, called ganglion cells, make the muscles push. A person with Hirschsprung's disease does not have these ganglion nerve cells in the last part of the large intestine.
 
In a person with Hirschsprung's disease, the healthy muscles of the intestine push the stool until it reaches the part without the nerve cells. At this point, the stool stops moving. New stool then begins to stack up behind it.
 
Sometimes, the ganglion cells are missing from the whole large intestine, and even parts of the small intestine before it. When the diseased section reaches to or includes the small intestine, it is called long-segment disease. When the diseased section includes only part of the large intestine, it is called short-segment disease.
 
Written by/reviewed by:
Last reviewed by: Arthur Schoenstadt, MD
Last updated/reviewed:
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