Hirschsprung's

Question 1

What is Hirschsprung’s disease?

Answer 1

A congenital condition where the parasympathetic ganglionic cells of the myenteric plexus are absent

Question 2

What is the job of the myenetric plexus?

Answer 2

It runs along the length of the gut wall and is responsible for initiating peristalsis

Question 3

What is the pathophysiology of Hirschsprung’s?

Answer 3

A loss of parasympathetic ganglion cells

Question 4

What conditions is Hirschsprung’s associated with?

Answer 4

Downs syndrome
Neurofibromatosis
Waardenburg syndrome
Multiple endocrine neoplasia

Question 5

What is the presentation of Hirschsprung’s disease?

Answer 5

Delay in passing meconium
Chronic constipation since birth
Abdominal pain and distention
Vomiting
Poor weight gain and failure to thrive

Question 6

What is the definitive investigation for diagnosis of Hirschsprung’s?

Answer 6

Rectal suction biopsy

Question 7

What is the initial management of Hirschprung’s?

Answer 7

Rectal washouts - bowel decompression
IV antibiotics

Question 8

What is the definitive management of Hirschprung’s?

Answer 8

Surgery to affected section of colon

Question 9

Passage of meconium after what amount of time is a red flag?

Answer 9

48 hours