Budd Chiari Syndrome

Question 1

What?

Answer 1

Hepatic vein thrombosis

Question 2

Risk factors/causes

Answer 2

Usually seen in context of underlying haematological disease or other pro-coagulable state
Polycythaemia rubra vera
Myeloproliferative disorders
Thrombophilia - activated protein C resistence, antithrombin III deficiency, protein C & S deficiencies
Pregnancy/COCP

Question 3

Features

Answer 3

Generally requires more than one hepatic vein to be affected to give symptoms
Sudden onset, severe abdominal pain
Ascites
Tender hepatomegaly
Also AKI
Signs of liver congestion

Question 4

Investigations

Answer 4

LFTs - mild elevation
PT - may be prolonged (confusing if assoc with hypercoagulability state)
Ascitic fluid - high protein content (except if very acute); risks (eg bacterial peritonitis) should be considered before undertaken
MRI (better than CT) - prominent caudate lobe
Doppler US
?? Liver biopsy - centrilobular congestion

Question 5

Management

Answer 5

Treat underlying condition!! (often anticoagulation)
Ascites - diuretics (spironolactone first-line, then furosemide); salt and fluid restric
Local thrombolysis with radiological support
Surgical decompression of liver in cases of persistent congestion (eg via transjugular intrahepatic portosystemic shunt (TIPS))
Liver transplant if decompensated liver cirrhosis

Question 6

Complications/prognosis

Answer 6

Hepatic failure ± encephalopathy, portal HTN, varices, bacterial peritonitis, hepatorenal syndrome

Without treatment, often fatal; with treatment, 5-year survival is 90%
Good prognosis: young, absence/small amount of ascites, low serum creatinine
Poor prognosis: portal hypertension and portal vein thrombosis