What is Hirschsprungâ€™s disease?
Hirschsprungâ€™s disease is an illness that children are born with that prevents them from having a normal bowel movement.
Squeezing of the muscles in the gut helps move digested food through the intestines. The squeezing of the muscles is triggered by nerves. Hirschsprung’s disease is a problem that happens when some of the nerves are missing. Without the nerves, the muscles cannot move digested food through the intestine, so the intestine gets blocked. This can make it hard for your child to pass bowel movements (constipation).
Hirschsprungâ€™s disease may affect only the area near your childâ€™s rectum, or it may affect a larger section of the large intestine.
What is the cause?
Before birth, a childâ€™s nerve cells normally grow along the intestines toward the anus. When a child has Hirschsprungâ€™s disease, the nerve cells stop growing too soon. Why the nerve cells stop growing is not known.
Sometimes Hirschsprungâ€™s is inherited, which means that itâ€™s passed from parents to children through their genes. Genes are inside each cell of your body. They contain the information that tells your body how to develop and work. This disease affects boys more often than girls.
What are the symptoms?
The symptoms depend on how much of the intestine is involved. Symptoms may include:
- Not being able to have a bowel movement within a few days after birth
- A lot of gas
- Swollen belly
- Diarrhea, which may be forceful
- Poor growth and trouble gaining weight because this disease can keep nutrients from being absorbed by the body
If your child has a mild case of Hirschsprungâ€™s, symptoms may not start until your child is older. In addition to some of the symptoms listed above, older children may have constipation that gets worse over time.
How is it diagnosed?
Your childâ€™s healthcare provider will ask about your childâ€™s symptoms and medical history and examine your child. Tests may include:
- Rectal exam, which your childâ€™s provider does by gently putting a lubricated and gloved finger in your childâ€™s rectum to check the intestines
- X-ray of the belly to show the general size and shape of the intestines and to check for constipation
- Barium enema, which is an X-ray taken of the belly after barium is inserted through the rectum to show the walls of the intestine. Barium is a liquid that helps the intestines show up well on the X-ray.
- Tests to check the strength of the anal and rectal muscles
- A rectal biopsy, which is the removal of a small sample of tissue from the rectum for testing
How is it treated?
Hirschsprungâ€™s disease is treated with surgery to remove the part of the intestine that has no nerve cells. The healthy part of the intestine is then connected to your childâ€™s anus. (The anus is the opening of the rectum where bowel movements leave the body.)
If your child is very sick at the time of diagnosis, your child may need a procedure called an ileostomy or colostomy for a few months to a year. These procedures are done to connect the healthy part of your childâ€™s intestine to a new opening in the belly called a stoma. Your childâ€™s bowel movements will empty through the opening and collect in a disposable bag outside the body. After your child heals, a second procedure may be done to connect the healthy intestine to the anus. The stoma will be closed during this procedure.
How can I take care of my child?
Your childâ€™s surgeon and nurses will teach you how to care for your child. After surgery, you will need to care for your childâ€™s incision area. If your child has stoma, you will also learn how to care for it. To help prevent infection and other problems, follow your healthcare providerâ€™s directions carefully.
Follow the full course of treatment prescribed by your childâ€™s provider. Ask your provider:
- How and when you will hear your childâ€™s test results
- How long it will take for your child to recover
- If there are activities your child should avoid and when your child can return to normal activities
- How to take care of your child at home
- What symptoms or problems you should watch for and what to do if your child has them
Make sure you know when your child should come back for a checkup. Keep all appointments for provider visits or tests.
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This content is reviewed periodically and is subject to change as new health information becomes available. The information is intended to inform and educate and is not a replacement for medical evaluation, advice, diagnosis or treatment by a healthcare professional.
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