Hirschsprung’s disease

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Digestive system

Large intestine

Hirschsprung's disease is a blockage of the large intestine due to improper muscle movement in the bowel. It is a congenital condition, which means it is present from birth.

Congenital megacolon

Muscle contractions in the gut help digested materials move through the intestine. This is called peristalsis. Nerves in between the muscle layers trigger the contractions.

In Hirschsprung's disease, the nerves are missing from a short or long part of the bowel. Areas without such nerves can not push material through. This causes a blockage. Intestinal contents build up behind the blockage, causing the bowel and abdomen to become swollen. If the condition is severe, the newborn may fail to pass meconium or stool, and the newborn may vomit.

Milder cases may not be diagnosed until a later age. In older children, the disease may be cause chronic constipation, abdominal swelling, and decreased growth.

Hirschsprung's disease causes about 25% of all newborn intestinal obstructions. It occurs 5 times more frequently in males than in females. Hirschsprung's disease is sometimes associated with other inherited or congenital conditions such as Down syndrome.

Review Date: 12/18/2006
Reviewed By: Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor of Pediatrics, NYU School of Medicine, New York, NY. Review provided by VeriMed Healthcare Network.