Treatment

Pull-through Surgery

Hirschsprung's Disease (HD) is treated with surgery called a pull-through operation. There are three common ways to do a pull-through, and they are called the Swenson, the Soave, and the Duhamel procedures. Each is done a little differently, but all involve taking out the part of the intestine that doesn't work and connecting the healthy part that's left to the anus. After pull-through surgery, the child has a working intestine.

Before surgery: The diseased section is the part of the intestine that doesn't work.	Step 1: The doctor removes the diseased section.	Step 2: The healthy section is attached to the rectum or anus.

Colostomy and Ileostomy

Often, the pull-through can be done right after the diagnosis. However, children who have been very sick may first need 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 in the baby's abdomen. The hole is called a stoma. The doctor connects the top part of the intestine to the stoma. Stool leaves the body through the stoma while the bottom part of the intestine heals. Stool goes into a bag attached to the skin around the stoma. You will need to empty this bag several times a day.

Step 1: The doctor takes out most of the diseased part of the intestine.	Step 2: The doctor attaches the healthy part of the intestine to the stoma (a hole in the abdomen).

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 that to the stoma, the surgery is called a colostomy.

Later, the doctor will do the pull-through. The doctor disconnects the intestine from the stoma and attaches it just above the anus. The stoma isn't needed any more, so the doctor either sews it up during surgery or waits about 6 weeks to make sure that the pull-through worked.

After surgery

Ostomy

Most babies are more comfortable after having an ostomy because they can pass gas more easily and aren't constipated anymore.

Older children will be more comfortable, too, but they may have some trouble getting used to an ostomy. They will need to learn how to take care of the stoma and how to change the bag that collects stool. They may be worried about being different from their friends. Most children can lead a normal life after surgery.

Adjusting After Pull-through

After a pull-through, 9 out of 10 children pass stool normally. Some children may have diarrhea for a while, and babies may develop a nasty diaper rash. Eventually the stool will become more solid and the child will need to go to the bathroom less often. Toilet training may be delayed, as the child learns how to use the bottom muscles only after pull-through surgery. Older children might stain their underwear for a while after the surgery. It is not their fault. They can't control this problem, but it improves with time.

Some children become constipated because 1 in 10 children with HD has difficulty moving stool through the part of the colon without nerve cells. A mild laxative may also be helpful. Ask your doctor for suggestions.

Diet and Nutrition

One job of the large intestine is to collect the water and salts the body needs. Since your child's intestine is shorter now, it absorbs less. Your child will need to drink more to make sure his body gets enough fluids.

An infant who has long-segment disease requiring an ileostomy may need special tube feedings. The shortened intestine does not allow the bloodstream enough time to absorb nutrients from food before it is pushed out of the body as stool. Tube feedings that deliver nutrients can make up for what is lost.

Eating high-fiber foods like cereal and bran muffins can help reduce constipation and diarrhea.

Infection

Infections can be very dangerous for a child with Hirschsprung's disease. Infection of the large and small intestines is called enterocolitis. It can happen before or after surgery to treat Hirschsprung's disease. Here are some of the signs to look for:

• fever
• swollen abdomen
• vomiting
• diarrhea
• bleeding from the rectum
• sluggishness

Call your doctor immediately if your child shows any of these signs. If the problem is enterocolitis, your child may be admitted to the hospital. In the hospital, an intravenous (I.V.) line may be needed to keep body fluids up and to deliver antibiotics to fight the infection. The large intestine will be rinsed regularly with a mild salt water solution until all remaining stool has been removed. The rinse may also contain antibiotics to kill bacteria.

When the child has recovered from the infection, the doctor may advise surgery. If the child has not had the pull-through surgery yet, the doctor may prepare for it by doing a colostomy or ileostomy before the child leaves the hospital. If the child has already had a pull-through operation, the doctor may correct the obstruction with surgery.

Enterocolitis can be life threatening, so watch for the signs and call your doctor immediately if they occur.

Long-segment HD

Sometimes HD affects most or all of the large intestine, plus some of the small intestine. Children with long-segment HD can be treated with pull-through surgery, but there is a risk of complications such as infection, diarrhea, and diaper rash afterward. Parents need to pay close attention to their child's health. Also, since some, most, or all of the intestine is removed, drinking a lot of fluid is important.

Reference:

National Digestive Diseases Information Clearinghouse, USA.