How is Hirschsprung disease (HD) treated?

The treatments for Hirschsprung disease (HD) are as follows:

Pull-through surgery: HD can be treated with a surgery called a pull-through operation. There are three common ways to do a pull-through, the Swenson, the Soave, and the Duhamel procedures. Although done a little differently, each of these procedures involve taking out the part of the intestine that does not work and connecting the remaining healthy part to the anus. After pull-through surgery, the child has a working intestine. Colostomy and ileostomy: Often, the pull-through can be done right after the diagnosis is confirmed. However, children who have been very sick may first need a surgery called an ostomy. This surgery helps the child get healthy before having the pull-through. Some doctors do an ostomy in every child before doing the pull-through.

In an ostomy, the doctor takes out the diseased part of the intestine. Then, the doctor cuts a small hole, called a stoma, in the baby's abdomen. The doctor connects the upper part of the intestine to the stoma. The stool leaves the body through the stoma, while the lower part of the intestine heals. The stool goes into a bag attached to the skin around the stoma. This bag needs to be emptied several times a day.

If the doctor removes the entire large intestine and connects the small intestine to the stoma, the surgery is called an ileostomy. If the doctor leaves part of the large intestine and connects it to the stoma, the surgery is called a colostomy.

Later, the doctor performs the pull-through by disconnecting the intestine from the stoma and attaching it just above the anus. As the stoma is not needed any more, the doctor either sews it up during surgery, or waits about six weeks to make sure that the pull-through has worked.

This answer is based on source information from the National Digestive Diseases Information Clearinghouse (NDDIC)

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