In all cases of Hirschsprung disease, surgery is the definitive treatment. The segment of intestine without the specialized nerves is removed. The segment of intestine that has been shown by biopsy to be normal is then pulled down to the anus.
The trend is to perform this surgery without a protective colostomy during the neonatal period. However, in many cases in which a child is ill from infections, has an obvious intestinal obstruction, has other serious conditions or has a significantly enlarged bowel, a colostomy is required.
The colostomy is placed in the part of the intestine that functions normally and the child may eat and grow. The operation to remove the abnormal segment of bowel is performed at a later date.
When there is an early diagnosis and when circumstances are favorable (such as otherwise good health and lack of infection), the definitive surgical procedure can be performed in a single stage. This can be done with a minimally invasive surgical technique, with laparoscopy, or sometimes entirely through the anus with no scars at all. With proper surgical technique of preserving the sphincters and anal canal, fecal continence should remain.