Hirchsprung’s disease

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 

Answer

a

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