Hirschsprung disease Flashcards
1
Q
what is hirschsprung’s disease?
A
Hirschsprung’s disease is an absence of ganglion cells in the neural plexus of the intestinal wall
2
Q
how does it present?
A
The delayed passage of meconium together with distension of abdomen is the usual clinical presentation.
3
Q
what do investigations show?
A
- A plain abdominal x ray will demonstrate dilated loops of bowel with air/fluid levels
- barium enema
-
demonstrates a cone with dilated ganglionic proximal colon and the distal aganglionic bowel failing to distend.
4
Q
when does hirschsprung disease present?
A
24-48 hours after birth
5
Q
what is the difinitive diagnosis?
A
Full-thickness rectal biopsy for diagnosis
6
Q
mx
A
Treatment is with rectal washouts initially, after that an anorectal pull through procedure
