Hirschsprung's disease is a disorder of the gut caused by the congenital absence of ganglion cells in the submucosal and myentric plexus of the intestine, also known as megacolon or congenital aganglionic megacolon. It is caused by the congenital absence of autonomic parasympathetic ganglion cells. Surgical management involves removing the aganglionic bowel followed by anastomosis of the remaining portion, done in two stages - a temporary colostomy followed later by a pull through procedure to excise the aganglionic segment and connect the bowel.

1. HIRSCHSPRUNG’S
DISEASE/CONGENITAL
MEGACOLON
PREPARED BY
DR.SHALI B.S
PROFESSOR
MAMATA COLLEGE OF NURSING.KHAMMAM

5. HIRSCHSPRUNG’S DISEASE/CONGENITAL
MEGACOLON
• It is a disorder of the gut caused due to congenital absence of
gangalion cells in the submucosal and myentric plexus of
intestine.This disease is also known as megacolon or congenital
Aganglionic megacolon.

17. PATHOPHYSIOLOGY
• It is caused by congenital absence of autonomic parasympathetic
ganglion cells in the submucosal and myenteric plexus of the intestine

40. MEDICAL MANAGEMENT
• Administration of isotonic enema
• Administration of stool softeners
• Low residue diet

41. Surgical management
• The aim of the surgery is to remove the aganglionic bowel followed by
anastomosis of the remaining portion.
• The surgery involves two steps
• In first stage
• A temporary colostomy is done above the transition zone of
ganglionic and aganglionic bowel in the sigmoid or transverse
colon.This enables the normal distal bowel to return to its orginal
tone and size.
• Second stage involves definitive surgery ,which is done when the child
weight and condition is appropriate.

43. • Pull through procedure; Excision of aganglionic segment enabling an
anastomosis to be done by corrective surgeries