Budd-Chiari Syndrome

Question 1

what is it?

Answer 1

hepatic venous outflow obstruction which causes increased hepatic sinusoidal pressure and portal HT

Question 2

aetiology?

Answer 2

commonest:

• hypercoagulable states
• myeloproliferative disorders

Question 3

Symptoms?

Answer 3

classic triad of:

• abdo pain
• ascites
• hepatomegaly

varices & splenomegaly in chronic form

Question 4

Ix?

Answer 4

• Bloods
  
  • LFTs
    
    • raised AST/ALT, ALP, billirubin
    • low albumin
  • thrombophilia screening
  • coagulation profile
    
    • raised PT in fulminant BCS
• Genetic testing for JAK2
• Colour and pulsed doppler US

Question 5

Mx?

Answer 5

• asymptomatic: anticoagulation
• symptomatic: thrombolysis
• ascites mx: low salt diet and diuretics

Question 6

prognosis?

Answer 6

untreated–> mortality of 80%