Q) False statement about Hirchsprung's disease is:
a) Male and Female have equal incidence
b) In approximately 8% of the patients entire colon is affected
c) After surgery constipation is the most common problem
d) Down syndrome can be seen in up to 3-5% patients
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Hirchsprung's disease is a developmental disorder characterized by absence of ganglion cells in both Auerbach's and Meissner's plexus and males are more frequently affected than females
Absence of these ganglion cells lead to a very tight anal sphincter with resultant constipation.
This aganglionosis which is similar to Achalasia cardia begins at anorectal junction and involves rectosigmoid in 80% and entire colon in 8% of cases.
Various surgical options like Duhamel, Swenson and Soave procedures can be done and in all constipation is a common problem
Down's syndrome is associated in 5%
Ref: Sabiston 20th edition page 1876