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Hirschsprung Disease (cont.)

What are the symptoms of Hirschsprung's disease?

The main symptoms of Hirschsprung disease are constipation or intestinal obstruction, usually appearing shortly after birth. Constipation in infants and children is common and usually comes and goes, but if your child has had ongoing constipation since birth, Hirschsprung disease may be the problem.

Symptoms in Newborns

Newborns with Hirschsprung disease almost always fail to have their first bowel movement within 48 hours after birth. Other symptoms include

Symptoms in Symptoms in Toddlers and Older Children

Symptoms of Hirschsprung disease in toddlers and older children include

  • not being able to pass stools without laxatives or enemas. A laxative is medicine that loosens stool and increases bowel movements. An enema is performed by flushing water, or sometimes a mild soap solution, into the anus using a special wash bottle
  • swelling of the abdomen
  • lots of gas
  • bloody diarrhea
  • slow growth or development
  • lack of energy because of a shortage of red blood cells, called anemia.

How is Hirschsprung's disease treated?

Hirschsprung disease is diagnosed based on symptoms and test results.

A doctor will perform a physical exam and ask questions about your child's bowel movements. Hirschsprung disease is much less likely if parents can identify a time when their child's bowel habits were normal.

If Hirschsprung disease is suspected, the doctor will do one or more tests.

X rays

An X-ray is a black-and-white picture of the inside of the body. To make the large intestine show up better, the doctor may fill it with barium liquid. Barium liquid is inserted into the large intestine through the anus.

If Hirschsprung disease is the problem, the last segment of the large intestine will look narrower than normal. Just before this narrow segment, the intestine will look bulged. The bulging is caused by blocked stool stretching the intestine.

Manometry

During manometry, the doctor inflates a small balloon inside the rectum. Normally, the rectal muscles will relax. If the muscles don't relax, Hirschsprung disease may be the problem. This test is most often done in older children and adults.

Biopsy

Biopsy is the most accurate test for Hirschsprung disease. The doctor removes a tiny piece of the large intestine and looks at it with a microscope. If nerve cells are missing, Hirschsprung disease is the problem.

Pull-through Procedure

Hirschsprung disease is treated with surgery called a pull-through procedure. A surgeon removes the segment of the large intestine lacking nerve cells and connects the healthy segment to the anus. The pull-through procedure is usually done soon after diagnosis.

Ostomy surgery

An ostomy allows stool to leave the body through an opening in the abdomen. Although most children with Hirschsprung disease do not need an ostomy, a child who has been very sick from Hirschsprung disease may need an ostomy to get better before the pull-through procedure.

For ostomy surgery, the surgeon first takes out the diseased segment of the large intestine. The end of the healthy intestine is moved to an opening in the abdomen where a stoma is created. A stoma is created by rolling the intestine's end back on itself, like a shirt cuff, and stitching it to the abdominal wall. An ostomy pouch is attached to the stoma and worn outside the body to collect stool. The pouch will need to be emptied several times each day.

If the surgeon removes the entire large intestine and connects the small intestine to the stoma, the surgery is called an ileostomy. If the surgeon leaves part of the large intestine and connects it to the stoma, the surgery is called a colostomy.

Later, during the pull-through procedure, the surgeon removes the stoma and closes the abdomen with stitches.


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Source: MedicineNet.com
http://www.medicinenet.com/hirschsprung_disease/article.htm

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