Budd-Chiari syndrome

• Budd-Chiari syndrome is a vascular liver disorder due to obstruction of hepatic venous outflow
• BCS describes a classic triad of hepatomegaly, abdominal pain and ascites due to hepatic venous obstruction
• Can occur due to a variety of underlying disorders that each result in obstruction anywhere from the small hepatic venules in the liver to the entrance of the IVC at the right atrium
• • Primary BCS: obstruction due to a predominantly venous process (e.g. thrombosis, phlebitis)
  • Secondary BCS: obstruction due to external compression or invasion of the hepatic veins/IVC (e.g. tumour)
• Definition = Describes a condition characterised by occlusion or partial occlusion of any, or all, the three major hepatic veins. This may occur with or without occlusion of the IVC. Commonly due to thrombosis with well characterised underlying conditions including myeloproliferative disorders (50% of cases)
• The majority of conditions leading to Budd-Chiari are associated with a hypercoagulable state that increases the risk of venous thromboembolism (VTE). Most importantly, myeloproliferative disorders are implicated in up to 50% of cases.
• Causes of BCS:

  • Myeloproliferative disorders
  • Malignancy
  • Infection and benign liver lesions
  • Oral contraceptives and pregnancy
  • Other: hypercoagulable states, connective tissue diseases
• Myeloproliferative disorders:

  • Polycythaemia ruba vera - overproduction of erythrocytes
  • Essential thrombocytosis - overproduction of platelets
  • Chronic myeloid leukaemia - overproduction of leucocytes
• BCS may lead to acute (fulminant) liver failure or rapid progression of fibrosis with development of cirrhosis. This spectrum of severity is dependent on the speed of occlusion to the hepatic venous flow.
• Symptoms:

  • Abdominal pain
  • Abdominal swelling (ascites)
  • Nausea & vomiting
  • Itching
  • Leg cramps
  • GI bleeding: more common if acute presentation with/without liver failure
• Signs

  • Hepatomegaly
  • Ascites
  • Distended abdominal veins (predominant if IVC obstruction)
  • Peripheral oedema (predominant if IVC obstruction)
  • Jaundice (usually absent and at worst mild)
• Features of acute liver failure

  • Hepatic encephalopathy: confusion, asterixis (flapping tremor)
  • Bruising (coagulopathy)
  • Jaundice
  • Ascites
  • GI bleeding (e.g. variceal)
• The diagnosis of BCS is made by demonstrating hepatic venous outflow obstruction on imaging.

  • First line = doppler ultrasound
  • CT/MRI
  • Venography
• Basic investigations

  • Full blood count
  • Urea & electrolytes
  • Liver function tests
  • Bone profile
  • C-reactive protein
  • Coagulation
• Further investigations:

  • Non-invasive liver screen
  • Imaging
  • Thrombophilia screen
  • Haematological screen
  • Liver biopsy
• Stepwise algorithm to treat:

  • Treat the underlying obstruction - anticoagulation, interventional radiology, TIPSS and liver transplant
  • Treat the consequences of portal hypertension
  • Treat the underlying condition
• Patients with Budd-Chiari syndrome (hepatic vein thrombosis) should receive systemic anticoagulation to reduce clot extension and new thrombotic episodes. The initial anticoagulant of choice is usually low molecular weight heparin (LMWH)
• Patients may be considered for interventional radiological procedures that include angioplasty (unblocking a vein using balloon dilatation) with/without stenting and/or catheter-directed thrombolysis.
• Patients who fail to respond to medical therapy with anticoagulation and first-line interventional procedures may be considered for TIPSS. TIPSS is another interventional radiology procedure, which is a definitive treatment.

  TIPSS involves creation of an artificial channel between the portal inflow and hepatic vein outflow. This channel is known as a shunt and is kept open by placement of a PTFE-covered stent.
• Acute forms of BCS with acute liver failure is usually fatal without liver transplantation.