Hirschprung's Disease Flashcards
1
Q
what is this?
A
congenital condition where there is absence of parasympathetic ganglion cells in distal bowel / rectum
2
Q
aetiology?
A
familial inheritance
3
Q
presentation?
A
- failure to pass meconium >48h
- chronic constipation
- abdo pain & distension
- acute bowel obstruction
4
Q
Ix?
A
- Rectal biopsy
- used to confirm diagnosis - absence of ganglionic cells
- AXR: if bowel obstruction
- distended bowel loops
5
Q
Mx?
A
excision or bypass of anganglionic segment of bowel
6
Q
complication?
A
enterocolitis
7
Q
presentation of hirschprung associated enterocolitis?
A
- fever
- abdo distension
- diarrhoea
- features of shock
medical emergency –> can lead to toxic megacolon
8
Q
Rx of HAEC?
A
- IV fluids
- IV Abx
- decompression of obstructed bowel
