Budd-chiari syndrome

Any pathophysiological process resulting in hepatic venous outflow tract obstruction.

• Primary Budd-Chiari - predominantly venous process (thrombosis or phlebitis)
• Secondary Budd-Chiari - compression or invasion of the hepatic veins and/or the IVC by a lesion from outside the lumen

Risk factors

• Typically 20-40yo
• Female 3:1
• Prothrombotic risk factors:
  • Myeloproliferative disorders - test via JAK2
  • COCP
  • Factor V leiden
  • Prothrombin G20210A mutation
  • APS
  • Protein C, protein S or antithrombin deficiency
  • Paroxysmal nocturnal haemoglobinuria
  • Behcet's disease
  • Hyperhomocysteinaemia
  • MTHFR mutation (read this in a textbook, but this is actually no longer thought to be clinically relevant)
  • UC
  • Hypereosinophilic syndrome
  • Granulomatous venulitis
  • Recent pregnancy

Aetiology (underlying disorder found in >80%)

• Myeloproliferative disorder
• Malignancy
  • Direct compression
  • Hypercoagulable state
• Infections and benign lesions of the liver
• COCP and pregnancy
• Other hypercoagulable state

Presentation

• Classically:
  • Hepatomegaly
  • RUQ pain
  • Ascites
  • Followed by jaundice
• Also:
  • Leg oedema
  • Portal HTN-related GIT bleeding
  • Hepatic encephalopathy
• Asymptomatic 20%
• Can progress to acute liver failure

Diagnosis

• SAAG > 1.1 with total ascitic protein >3g/dL: suggestive of hepatic congestion - need a TTE to exclude CCF
  • In chronic BCS, total ascitic protein might be lower than 2.5g/dL
• Bloods
  • LFTs non-specific, but can be very high
  • Coags often abnormal
• Imaging
  • Doppler USS
  • CT
    • Arterial, venous, washout phases
    • Caudate hypertrophy - drains directly into IVC so is spared and tries to compensate
    • Ascites
    • Splenomegaly
    • Concomitant extrahepatic PV thrombosis is present in 15%
  • MRI
  • Hepatic venography is gold standard
    • Occlusion of hepatic veins
    • Spiderweb morphology of small intrahepatic venules

Treatment

• Principles
  • Prevent clot propagation
  • Restore vascular patency
  • Decompress hepatic congestion
  • Treat complications of portal hypertension
  • Most patients will require some form of intervention
• Medical
  • Anticoagulation
    • Commence immediately - heparin
    • Most patients with a predisposition will require indefinite anticoagulation of some form
  • Workup for cause
  • Treatment for portal hypertension - sodium restriction and diuretics
  • Monitor renal function carefully
• Interventional radiology
  • TPA directly into portal vein
    • Only in acute BCS - otherwise low chance of working
  • Balloon angioplasty in focal occlusions
    • Be very careful in deciding to put in a stent - may interfere with eventual need for liver transplant
  • TIPS is essentially standard of care for medical non-responders
    • 84% 5 year survival, 78% 5 year transplant free survival
• Surgery
  • Portosystemic shunting
  • Liver transplant - decompensated liver disease or recurrent stent or shunt failure