Hirschprung's Disease Flashcards
1
Q
what
A
congenital condition where nerve cells of myenteric plexus are absent in distal bowel and rectum
bowel looses motility
2
Q
key pathophysiology
A
absence of parasympathetic ganglion
3
Q
presentation
A
intestinal obstruction shortly after birth delay passing meconium abdo pain abdo distension vomit FTT
4
Q
Hirschsprung-Associated Enterocolitis
A
inflammation and obstruction of intestine
occurs 20% neonates with hirschprung’s
5
Q
Hirschsprung-Associated Enterocolitis - features
A
presents 2-4wks after birth
fever
abdo distention
diarrhoea w blood
6
Q
Hirschsprung-Associated Enterocolitis- Mx
A
antib
fluid resus
decompression
7
Q
Ix
A
abdo x-ray
rectal biopsy ***
8
Q
rectal biopsy finding
A
absence of ganglionic cells
9
Q
Mx
A
fluid resus
antib if HAEC
surgical removal of aganglionic bowel
