Hirschsprung Disease

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Hirschsprung Disease

During normal development before birth, the nerve cells that control intestinal function work their way from the upper part of the gastro-intestinal tract all the way to the anus. In some patients, this “nerve cell migration” process is incomplete and normal nerve cells are missing. The disease usually affects the lower part of the colon. This is the most common variety of the disease, called “Recto-sigmoid Hirschsprung disease”. More of the colon is affected in “Long-segment Hirschsprung disease” and the entire large intestine is affected in “Total-colon Hirschsprung disease”. Rarely, even more of the intestine is affected (Small-bowel Hirschsprung disease, Total intestinal Hirschsprung disease).

Although the intestines are not physically blocked or obstructed in Hirschsprung disease, they behave as if there is an intestinal blockage. Most patients are diagnosed as newborns. Sometimes the diagnosis is made later in childhood. Treatments are surgical and involve removing the abnormal intestine all the way through the pelvis to a level just inside the anus. The intestine with normal nerve cells is then attached there. Some patients require a temporary colostomy. Some patients require only one operation, while in others two or more operations may be needed.

The pediatric surgeons at Penn State Children’s Hospital have a large experience in treating patients with all varieties of Hirschsprung disease.

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