Budd-Chiari Syndrome

Background/Epidemiology

  • Rare (1-2/1000000) condition caused by thrombosis of the large hepatic veins (and occasionally IVC)
  • The underlying cause cannot be found in up to 50%

Pathophysiology

  • Venous congestion causes hepatomegaly, which can cause stretch of the liver capsule and right upper quadrant pain (can be severe)
  • Increased sinusoidal pressure can cause hepatocellular necrosis and can eventually cause fibrosis/cirrhosis (although this is late feature and may not present)

Causes

  • Haematological disorders 
    • Polycythaemia vera; myeloproliferative disorders
    • Thrombophillic conditions e.g. Protein C/S deficiency, antithrombin III deficiency or factor V leiden
    • Antiphospholipid syndrome
  • Reduced blood flow
    • Vena cava abnormalities e.g. webs; right heart failure, constrictive pericarditis, right atrial myxoma
  • Pregnancy
  • Drugs
    • OCP, HRT
  • Chronic infections e.g. TB, aspergillosis
  • Chronic inflammatory conditions e.g. IBD, sarcoid, SLE, Sjogren’s, Behcet’s
  • Malignancy
    • Hepatocellular carcinoma; renal cell carcinoma; Wilm’s tumour; adrenal carcinoma
  • Trauma/surgery
  • Alpha 1-antitrypsin deficiency

Presentation

  • Acute occlusion causes acute onset right upper quadrant pain, often marked ascites and occasionally acute liver failure
  • Insidious occlusion causes ascites and progressive abdominal discomfort
  • Hepatomegaly (often tender, particularly at the caudate lobe)
  • Peripheral oedema may occur if IVC obstruction is present
  • Patients may also present with features of acute renal injury

Investigations

  • LFTs- can be variable but may show a hepatocellular picture
  • Ascitic fluid can show a high protein concentration (exudative) although this may fall later in the process
  • Imaging
    • Doppler USS
      • May show destruction of the hepatic veins and reversed flow or thrombosis in the portal vein
    • CT/MRI
      • Enlarged caudate lobe (redirection of blood flow)
      • Occlusion of the hepatic veins/IVC
  • Liver biopsy (rarely required)
    • Centrilobular congestions +/- fibrosis

Management

  • Manage any underlying cause as best as possible
  • Obstruction can be managed with either streptokinase or heparin and then warfarin
  • The prognosis is poor, with 3 year survival estimated at 10%
    • Intractable ascites and liver failure most common cause of death
  • Surgical shunting (TIPSS) may improve outcome (3 year survival 50%)
  • Rarely, transplantation is an option

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