Hirschsprung's Disease

Treatment Overview

Children with Hirschsprung's disease require surgery to remove the area of the large intestine that has no nerve cells. Sometimes a baby will need enemas to remove stool from the intestine until surgery can be performed. You may be instructed how to give the enemas.

In most cases, surgery is done within the first months after birth. During surgery, the affected portion of the intestine is removed. Two surgeries may be needed to remove the affected area of the large intestine:

• In the first surgery, the intestine is brought to the surface of the abdomen (colostomy) above the diseased area. The affected part of the large intestine is removed. Stool passes out of the body through the colostomy into a disposable pouch . This allows the remaining normal intestine time to recover.
• After a few weeks or months, the colostomy is closed in a second surgery, and healthy intestine is reattached. Stool will again pass from the body through the anus.

Most babies are in the hospital 2 to 3 days to 1 week for surgery for Hirschsprung's disease.

Some healthy babies need just one surgery. This avoids the need for a colostomy and second operation. In many situations, surgery can be done using a lighted instrument called a laparoscope.

Complications from surgery include a leak where the intestine is rejoined (anastomotic leaks) and scar tissue formation (strictures).

After surgery

After corrective surgery for Hirschsprung's disease, no further intestinal blockages are expected. But long-term outcomes after surgery are variable. Children treated for Hirschsprung's disease may leak stool (fecal incontinence) for years after successful surgery. Recurrent or chronic abdominal pain or constipation may also occur. Some of these problems may persist into adulthood.

Some children may get a serious condition called Hirschsprung's-associated enterocolitis (HAEC). The small and large intestines may become inflamed. And a hole (perforation) may form in the large intestine, causing stool to leak inside the abdomen. HAEC requires emergency surgery.

The cause of symptoms that won't go away is often unclear. A colonic manometry measures muscle and nerve function in the large intestine and can often help doctors determine the specific problem so it can be treated appropriately. During this test, a flexible, plastic tube (catheter) is put into your child's rectum and into the large intestine, where sensors detect movement after fluid is flushed through that area. This test is only available at a limited number of facilities. If bothersome symptoms are a continual problem, ask your doctor for a referral or for more information.

Depending on the type of problem with the large intestine or anal sphincter, treatment may include medicine, behavior modification, biofeedback, cognitive behavioral therapy, or more surgery.

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Author:	Debby Golonka, MPH	Last Updated: September 22, 2008
Medical Review:	Michael J. Sexton, MD - Pediatrics Brad W. Warner, MD - Pediatric Surgery
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