Hirschsprung's Disease :: Nationwide Children's Hospital

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Hirschsprung's Disease

Hirschsprung's (HERSH-prungz) disease, or HD, is a serious condition that affects the large intestine. The intestines are also called the bowels. With HD, a part of the bowel (also called the colon or large intestine) did not develop the nerves needed for it to work properly.

Usually, the section of the bowel that has no nerve cells is in the lower part of the colon (Pictures 1, 2 and 3). This section gets very narrow, and the bowel above it becomes enlarged. This is because stool (bowel movements) cannot pass through the narrow area. In a person with HD, the healthy muscles of the intestine push the stool until it reaches the part without the nerve cells. At this point, the stool stops moving. New stool then begins to build up behind it. Stool can fill up the large intestine and cause serious problems like infection, bursting of the colon and even death.

Picture 1 - The digestive system inside the body.
Image of the digestive system
Picture 2 - Healthy large intestine: Never cells are found throughout the intestines.
Image of nerve cells
Picture 3 - HD large intestine: Nerve cells are missing from the last part of the intestine.
Image of missing nerve cells

Symptoms

The symptoms of HD depend on the person’s age:

Newborns and Infants

Newborns with HD don't have their first bowel movement when they should. After eating, babies may throw up green liquid called bile. Their bellies may swell and the pain from constipation or gas may make them very fussy. Sometimes, babies develop infections in their intestines. Many babies with HD grow and develop more slowly than they should.

Young Children, Teens and Adults

Older children and adults usually have frequent diarrhea and most have always had severe constipation. Children and adults with HD might also have anemia, a shortage of red blood cells, because blood is lost in the stool. The anemia may cause pale-looking skin and they may feel tired easily.

How HD Is Diagnosed

Several tests* may be done to see if a child has this disease. (See page 3 for a list of Helping Hands.)

Rectal exam - the doctor examines the rectum with a gloved finger.

Barium enema* - (BARE  ee um   EN eh muh)  A thick, white liquid (barium) is put into the rectum through a small tube and an X-ray is taken. The barium shows up on X-rays and allows the doctor to see the outline of the bowel Anal Manometry* (Ay-nul  mah NAH meh tree)  A small tube with a balloon on the end is inserted into the rectum. Air is inserted into the balloon to see if the nerves and muscles of the colon are working right. 

Rectal biopsy* - a very small piece of the lining of the bowel is removed through a small tube which is inserted into the rectum. This small piece of bowel is studied in the lab under a microscope to see if the nerve cells are present. 

How HD Is Treated

The symptoms of Hirschsprung's disease may at first be treated with mild enemas or stool softeners and by having your child eat special foods.

Sometimes Hirschsprung’s disease is treated with surgery. The part of the bowel without nerve cells is removed and a colostomy (koe-LOSS-toe-mee) is not needed. In other cases, a temporary colostomy may need to be done to let the stool pass out of the body. A small opening is made in the child's abdomen (belly), and part of the healthy bowel is brought to the outside. A special pouch or bag is used to cover the opening and collect the stool. If your child has a temporary colostomy, a nurse will teach you how to care for it until it is no longer needed.

The surgeon decides when to close the colostomy. The decision is based on the child's age, symptoms and how well the child's bowel is doing. When the colostomy is closed surgically, the healthy bowel is reconnected to the rectum so the stool will come out normally. The colostomy will be gone, but there will be a small scar where the colostomy used to be. 

After the colostomy is closed, your child will be able to eat most foods and will learn to adjust to bowel training if he or she is old enough.  Most children have normal bowel function after surgery.

Infection

Infections can be very dangerous for a child with HD. Infection of the large and small intestines is called enterocolitis (EN ter oh koe LIE  tis). It can happen before or after surgery to treat the condition.

Watch for these signs and call your child’s doctor immediately if you see any of these signs:

Helping Hands

For more information, please ask your nurse for these Helping Hands:

Barium Enema, HH-III-2.

Anal Manometry, HH-III-10.

Rectal Biopsy (Suction Procedure), HH-III-75.

Ostomy Care:  Ordering Supplies, HH-II-99, and one of the following:

Ostomy Care:  One-Piece Pouch with Attached Skin Barrier, HH-II-107;

Ostomy Care:  One-Piece Pouch without Attached Skin Barrier, HH-II-4;

or

Ostomy Care:  Two-Piece Pouch, HH-II-33

If you have any questions, be sure to ask your doctor or nurse.

Hirschsprung's Disease (PDF)

HH-I-139 6/91, Revised 9/06 Copyright 1991-2006, Nationwide Children’s Hospital

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