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 



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

Relation of malignancy with ulcerative colitis

Q) A patient has ulcerative colitis with pan colitis. He is on medications and screening endoscopy 10 years later show high grade dysplasia. What is the management now?

a) Nothing for now and repeat colonscopy after 1 year

b) Total proctocolectomy

c) Resect the segment with high grade dysplasia

d) Increase the steroid dose

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