Children who have
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
- 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
Complications from surgery
include a leak where the intestine is rejoined (anastomotic leaks) and scar
tissue formation (strictures).
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. Emergency surgery may be needed.
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
Depending on the type of problem with the large
intestine or anal sphincter, treatment may include medicine, behavior
cognitive-behavioral therapy, or more surgery.