Total Colonic Hirschsprung Disease

Hirschsprung disease is a condition that results when certain nerve cells that are normally present in the wall of the intestine fail to develop properly during fetal development. This abnormality is called aganglionosis. In Hirschsprung’s disease, the affected colon and rectum is missing the nerves (ganglion cells) that allow it to relax, thus, the colon and rectum are in a constant squeezing state, prohibiting gas and stool from passing.

Neonates with Hirschsprung disease will always require surgery and the extent of the removal will depend on the level of change from ganglion cells to no ganglion cells in the intestine. The greater the amount of intestinal removal, the more likely the child will require parenteral nutritional support.

Diagnosis is with use of abdominal x-ray, barium enema contrast studies, antroduodenal manometry motility studies, and rectal biopsy. Treatment is surgical removal of the defective bowel.

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