Hirschsprung disease

Question 1

What is the pathophysiological process of hirschsprung disease?

Answer 1

Aganglionic segment of the bowel due to developmental failure of the parasympathetic meissner (SM) and myenteric plexus of the GIT (derived from vagal neural crest cells)
Rare - characterised by childhood constipation due to uncoordinated peristalsis resulting in a functional obstruction.

Question 2

What are some risk factors for hirschprung disease?

Answer 2

3 times more common in males
Down syndrome

Question 3

What are the common presentations of Hirschprung disease?

Answer 3

Neonatal - failure or delay to pass meconium
Older children - constipation, abdominal distention.

Question 4

What investigations should be done for suspected Hirschprung disease?

Answer 4

Abdominal x-ray
Rectal biospy - gold standard for diagnosis

Question 5

What is the acute management of Hirchsprung disease?

Answer 5

Rectal washout/bowel irrigation

Question 6

What is the long term/definitive management of Hirschsprung disease?

Answer 6

Surgery to affected segment of the colon.