Hirschsprung’s Disease

Hirschsprung’s Disease (HD) is a disease of the large intestine (colon) that causes severe constipation or intestinal obstruction. Bowel movements occur less often than normal and stool is difficult to pass. Some children with HD can’t pass stool at all which causes a complete blockage. If HD is not treated, stool can fill up in the large intestine and cause serious problems like infection, bursting of the colon, and even death.

Hirschsprung’s disease develops before the child is born. Normal nerve cells grow in the baby’s intestine while the mother is carrying the child. These nerve cells grow down from the top of the intestine to the anus and are supposed to contract the rectal muscles in order to have a bowel movement. With HD, the nerve cells stop growing before they reach the end. No one knows why these cells stop growing.

Hirschsprung’s Disease occurs in about 1 in every 5000 children. In some cases, HD is hereditary, which means mothers and fathers could pass it to their children. Children are born with HD and are usually diagnosed when they are infants. Less severe cases are sometimes diagnosed when a child is older.

Symptoms

Symptoms experienced by newborn babies include:

  • Not passing stool on the first or second day of life
  • Green or brown vomit
  • Swollen belly
  • Bloody Diarrhea

Symptoms experience by older children and adolescents may include:

  • Constipation
  • Swollen abdomen
  • Gas
  • Lack of weight gain
  • Fatigue
  • Bloody diarrhea

Diagnosis/Testing

If the doctor suspects the child may have Hirschsprung’s disease, there are several tests that can be done:

  • Barium enema – involves an x-ray after the doctor flushes barium into the rectum to evaluate the anatomy of the colon.
  • Rectal Suction Biopsy – involves taking a small pinch of tissue from the rectum to be examined under the microscope.
  • Manometry – a balloon is inserted into the rectum to measure the pressure of the muscles when the person is relaxed’

Treatment

The treatment for Hirschsprung’s disease is surgery to remove the portion of the colon that has no nerve cells called apull-through. The rest of the colon is joined to the rectum so waste can leave the body normally. In very sick children, surgery may be done in two steps. The bad section of the intestine will be cut out and the doctor will attach the good intestine to a small hole (stoma) that has been made in the child’s abdomen. Removing the entire colon is called anIleostomy. Removing part of the colon is called a colostomy. Stool leaves through a bag attached to the stoma. This allows the lower part of the colon to heal. After allowing time for the child to recover from the first surgery, the surgeon will do a second surgery where the healthy section of the intestine is attached to the rectum and the stoma in the abdomen is closed.

It will be important for the child to consume a diet that is high in fiber in order to prevent constipation. Consider meeting with one of our dieticians for a nutritional consult. Call (865) 546-3998 to schedule an appointment.