Hirschsprung's disease is a congenital disease which refers to the blockage of the large intestine due to improper muscle movement in the bowel. Hirschsprung's disease occurs in about one in 5,000 of live births. It is usually diagnosed in children, and affects boys more often than girls.
Symptoms that may be present in newborns and infants include:
There is no exact cause of Hirschsprung’s disease. It is believed that this disease occurs when nerve cells that surround the colon don’t form completely.
Hirschsprung's disease is treated with surgery to remove the portion of the colon that has no ganglia cells.
In children who are severely affected, 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.