3 - Paeds - Gastro - Biliary Atresia

Question 1

what is it? causes what?

Answer 1

progressive, destruction/absence of extrahepatic biliary tree and intrahepatic biliary ducts

chronic liver failure and death unless surgery done

Question 2

Clinical features - weight? jaundice/excretion colour? abdo signs?

Answer 2

usually normal birth bt but FTT

mild jaundice and pale stools (after meconium) and dark urine - often hepto and splenomegaly 2ndary to portal HTN

Question 3

Ix? (3)

Answer 3

LFTs of little value
fasting abdo USS may show contracted or absent gall bladder
TIBIDA radioisotope scan shows no excretion into bowel
Liver biopsy shows extrahepatic biliary obstruction

Question 4

How is diagnosis confirmed

Answer 4

laparotomy - by operative cholangiography - shows abnormal biliary tree

Question 5

Post op complications?

Answer 5

cholangitis, malabsorption of fats and fat-soluble vitamins

transplant if unsuccessful

Question 6

MGMT of biliary atresia?

Answer 6

surgical bypass of fibrotic ducts - hepatoportoenterostomy (use jejunum)

if performed before 60d - 80% achieve bile drainage