Hirschsprung's disease is caused by the absence of nerve cells in the wall of the bowel. This means that the muscles of a portion (usually) of the bowel do not contract, the bowel remains collapsed and stools cannot pass. Bowel contents build up behind the obstruction. Most often it begins in the last foot or two of bowel, called the sigmoid colon and rectum. The disorder rarely involves the entire bowel. Symptoms include constipation, abdominal swelling, and vomiting. Symptoms in older children include passing small watery stools, diarrhea, and a lack of appetite.
Hirschsprung's disease is a genetic disorder named for Harold Hirschsprung, the Dutch physician who first described the disease in 1886 after caring for two boys who were unable to have spontaneous bowel movements. It is a congenital disease, which means a person is born with it. The disease may also be hereditary, which means a parent can pass it to a child.
Hirschsprung's disease affects mainly infants and children. although some people do not develop symptoms until childhood or even adulthood.