Key Points: Hirschsprung's Disease

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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 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.