Hirschsprung's disease with skip area (segmental aganglionosis)

Hirschsprung's disease is characterized by a single aganglionic segment of colon extending distally to the anal margin. Well documented reports of segmental aganglionosis have been rare. We report a case of segmental aganglionosis in which there were two distinct aganglionic segments resected. The entire transverse colon between the two aganglionic segments was normally ganglionated, preserved, and utilized and functions in a normal fashion.