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GS DISEASE
Kim Eligio, RN
Hirschsprungs Disease
Motor disorder of the colon, which is caused by the
absence of enteric neurons (ganglia) in the
myenteric and submucosal layer of the colon. The
resulting aganglionic segment of the colon fails to
relax, causing a functional obstruction.
Also called as Anganglionic Megacolon
1 per 5000 live births
4x Male > Female
Children with HD usually diagnosed at first 2 years
of age
Etiology
During fetal development, neuroblasts that
originated from the neuronal crest cells failed
to migrate to the colon between 8th to 12th
weeks
Accelerated cellular destruction in the
intestine can also be a cause
PATHOPHYSIOLOGY
OF HIRSCHSPRUNGS
Peristalsis takes place in normal colon
Rectal Manometry
o shows failure to demonstrate reflex
relaxation of the internal anal sphincter in
response to inflation of a rectal balloon.
Diagnosis