Hirschsprung's disease

Hirschsprung's disease occurs when some of your baby’s intestinal nerve cells don’t develop properly, leading to an obstruction of the intestine.

• As food is digested, muscles move food forward through the intestines in a movement called peristalsis.
• When we eat, nerve cells that are present in the wall of the intestines receive signals from the brain telling the intestinal muscles to move food forward.
• In children with Hirschsprung's disease, a lack of nerve cells in part of the intestine interrupts the signal from the brain and prevents peristalsis in that segment of the intestine.
• Because stool cannot move forward normally, the intestine can become partially or completely obstructed, and it begins to expand.

This same obstructed intestine is prone to the development of a serious infection, enterocolitis, which causes a child to have a fever, pain and diarrhea.

• The problems a child will experience with Hirschsprung's disease depend on how much of the intestine has normal nerve cells.
  • Seventy percent of babies with Hirschsprung's disease are missing nerve cells in only a portion of the last one to two feet of their large intestines.

Hirschsprung's disease causes 15 to 20 percent of intestinal obstructions that occur in newborns.

Less pain, faster recovery

Standard surgical treatment of Hirschsprung’s disease requires two surgeries: the first to place your child’s temporary colostomy, the second to pull the patient’s functioning colon through to the anorectum. However, due to advances is minimally invasive surgery, surgeons at Boston Children's Hospital frequently can perform a single-stage operation. Sometimes, a colostomy can be avoided all together and the minimally invasive technique means that your child will recover more quickly from the surgery.