بسم الله الرحمن الرحیم

Peresented by Hamed Hooshang malamiri 2012/09/28

Hirschsprung's disease is a condition that affects the large intestine (colon) and causes problems with passing stool. Hirschsprung's disease is present when a baby is born (congenital) and results from missing nerve cells in the muscles of a portion of the baby's colon.

 Children with Hirschsprung's disease are often constipated. In severe cases of Hirschsprung's disease, a newborn child experiences an obstructed colon and is unable to have a bowel movement. In mild cases, doctors may not detect Hirschsprung's disease until later in a child's life.

 Hirschsprung's disease is treated with surgery to remove the affected portion of the colon. After surgery, most children pass stool normally.

symptoms  Signs and symptoms may vary with the severity of the condition. Sometimes they appear right after a baby is born. Other times they may not be apparent until later in life.

 In newborns, signs may include:  Failure to pass stool within the first or second day of life  Vomiting, including vomiting a green or brown substance  Constipation or gas, which may make a newborn fussy  Diarrhea

 In older children, signs can include:  Swollen abdomen  Constipation  Gas  Lack of weight gain  Fatigue

causes  It's not clear what causes Hirschsprung's disease. Doctors know that Hirschsprung's disease occurs when nerve cells that surround the colon don't form completely.

 Normally, as a baby grows in the womb, bundles of nerve cells (ganglia) begin to form between the muscle layers along the length of the colon. This process begins at the top of the colon and ends at the bottom (rectum). In children with Hirschsprung's disease, this process does not finish, and the ganglia do not form normally along the entire length of the colon. Sometimes the cells are missing from only a few centimeters of the colon. Other times a longer portion may be affected.

RISK FACTORS  Factors that may increase the risk of Hirschsprung's disease include:  Having a sibling with Hirschsprung's disease. Because Hirschsprung's disease can be inherited, if you have one child with the disease, your future children also may be at risk.

 Being male. Hirschsprung's disease is more common in males.  This condition occurs more frequently in boys than in girls, with as many as 4 boys affected for every girl.

 Having other inherited conditions. Hirschsprung's disease is associated with certain inherited conditions, such as inherited heart problems and Down syndrome. It may also be associated with multiple endocrine neoplasia, type IIB — a syndrome that causes noncancerous tumors in the mucous membranes and adrenal glands (located above the kidneys) and cancer of the thyroid gland (located at the base of the neck).

TESTS AND DIAGNOSIS  To determine whether Hirschsprung's disease is present, your child's doctor may conduct one or all of the following tests:  Abdominal X-ray. If Hirschsprung's is present, as stool backs up in the colon, the X- ray may reveal decreased air in the colon or areas in which the colon has stretched wider than normal.

 Hirschsprung disease. Frontal abdominal radiograph showing marked dilatation of the bowel with no gas in the rectum.

 X-ray using a contrast dye. A barium enema uses a contrast dye to help your doctor evaluate the entire colon with an X-ray. Barium or another contrast dye is placed into the bowel in an enema form. The barium fills and coats the lining of the bowel, creating a clear silhouette of the colon and rectum. Air may also be added to provide better contrast on the X-ray.

 Measuring control of the muscles around the rectum. A manometry test is typically done on older children and adults. During the manometry test, the doctor inflates a balloon inside the rectum. The surrounding muscle should relax as a result. If it doesn't, Hirschsprung's disease may be the cause

 Removing a sample of colon tissue for testing. Biopsy is a procedure to remove a sample of tissue for laboratory testing. A biopsy sample can be collected using a suction device and is done on an outpatient basis, meaning it doesn't require a hospital sta

Treatments and drugs  Surgery Hirschsprung's disease is treated with surgery to remove the portion of the colon that has no ganglia cells. The remaining portions of the colon and rectum are then joined, so that waste can leave the body normally. Surgery for Hirschsprung's disease is often done in one step immediately after diagnosis.

 In children who are very ill, a doctor may choose to complete the process in two steps. First, the doctor will remove the abnormal portion of the colon without ganglia cells and perform an ostomy. This involves creating a small hole (stoma) in the child's abdomen and connecting the top, healthy portion of the colon to the stoma. Stool then leaves the body through the stoma into a bag that attaches to it, allowing the lower part of the colon to heal. A stoma bag must be emptied several times a day. Ostomy may include

 Ileostomy. In ileostomy, the doctor removes the entire colon and connects the small intestine to the stoma.  Colostomy. In colostomy, the doctor leaves part of the colon and attaches this to the stoma.  After allowing time for the child to recover from the first surgery, the doctor will perform another procedure to close up the stoma and connect the healthy portion of the intestine to the rectum.

 Results of surgery After surgery, most children pass stool normally. Some may experience diarrhea initially, but after some time stool will become more solid. Toilet training may take longer because some children have difficulty coordinating the muscles used to pass stool. This improves with time in most children. Constipation may continue in some children, although laxatives can help. Eating high-fiber foods also can help with diarrhea and constipation.

 A child is also at risk of developing enterocolitis in his or her colon or small intestine after surgery. Be aware of signs and symptoms of enterocolitis, and call the doctor immediately if any of these occur:  Bleeding from the rectum  Diarrhea  Fever  Swollen abdomen  Vomiting

Lifestyle and home remedies  Children may experience constipation after surgery to correct Hirschsprung's disease. You can help manage constipation by having your child:

 Eat high-fiber foods. If your child eats solid foods, include high-fiber foods as part of your child's diet. Encourage your child to eat fruits and vegetables. High-fiber foods as part of your child's diet may help control constipation. But a sudden increase in high- fiber foods can make constipation worse, so add high-fiber foods slowly. If your child isn't eating solid foods yet, ask your doctor about formulas that may help relieve constipation.

 Increase fluids. Encourage your child to drink more water. Children with Hirschsprung's disease may have an increased risk of dehydration, since the colon absorbs water from food in the last stages of digestion. If a portion of your child's colon was removed, your child may have trouble absorbing enough water. Drinking more water can help your child stay hydrated, which may help ease constipation