Abnormal enteric nervous system and motor activity in the ganglionic proximal bowel of Hirschsprung's disease.

Hirschsprung's disease (HSCR) is a congenital defect in which the enteric nervous system (ENS) does not develop in the distal bowel, requiring surgical removal of the portions of bowel without ENS ganglia ('aganglionic') and reattachment of the 'normal' proximal bowel with ENS ganglia. Unfortunately, many HSCR patients have persistent dysmotility (e.g.