Key Points: Hirschsprung's Disease

Full article available for Download HERE

Hirschsprung’s Disease

•Congenital aganglionic megacolon.
•There is an absence of parasympathetic ganglion cells in the muscular wall of the rectum and distal colon.
•This results in a lack of peristalsis in the affected segment.
•The affected area is narrow and the portion above it is greatly distended.

Signs and Symptoms:
•Failure to pass meconium within 24 hours.
•Signs of obstruction—bile-stained emesis, abdominal distension.
•Gastroenteritis with diarrhea stools & ulceration of colon.
•Severe constipation.

Diagnosis:
•Diagnosis is made with barium enema.
•Surgical repair is with abdominal resection of the affected segment with a Swenson’s pull-through.
•Initially child may need a temporary colostomy to relieve obstruction and resection is done later.
•The child may also be anemic.
 
;