Makindo Medical Notes.com

Related Subjects: |Liver Function Tests |Ascites Assessment and Management |Antiphospholipid syndrome |Thrombophilia testing |Protein C Deficiency |Protein S Deficiency |Factor V Leiden Deficiency |Antithrombin III deficiency (AT3)

Always consider diagnosis in sudden onset of ascites and painful hepatomegaly or Massive ascites with relatively normal liver functions. The primary objective of acute care in Budd-Chiari Syndrome is to quickly restore normal blood flow in the liver, manage any immediate complications, and prevent further liver damage.

About

• A cause of acute liver failure which should always be considered
• Narrowing and obstruction (occlusion) of the veins of the liver (hepatic veins).
• Look for a prothrombotic cause and get a USS

Aetiology

• Thrombosis of one or more hepatic veins
• There is centrilobular congestion and necrosis (zone 3) of the liver lobule
• Extravasation of RBCs into the space of Disse and liver cell plate.
• Acute liver failure ensues
• Most cases tend to affect individuals between the ages of 20 to 40

Clinical

• Abdominal pain, ascites and hepatomegaly.
• Ascites is an exudate initially and the protein falls to be a transudate
• The syndrome may come on acutely, Sub acutely or chronically
• Chronic disease may result in cirrhosis.
• The caudate lobe may hypertrophy as it has separate venous drainage into the IVC

Causes

• Myeloproliferative disorders - polycythaemia vera, essential thrombocythemia
• Cancer - renal cell cancer, hepatocellular carcinoma
• Prothrombotic states - Factor V Leiden mutation, pregnancy, oral contraceptive, Protein C and S deficiency, Paroxysmal nocturnal haemoglobinuria

BCS should be suspected when there is:

• Sudden onset of ascites and painful hepatomegaly
• Massive ascites with relatively normal liver functions
• Sinusoidal dilation in liver biopsy without heart disease
• Fulminant hepatic failure along with hepatomegaly and ascites
• Unexplained chronic liver disease
• Liver disease with a thrombogenic disorder.

Investigations

• FBC and Blood film, U&E. Liver enzymes, Prothrombin time
• Ascites (exudate or transudate): invaluable clues to diagnosis and form of presentation. Elevated protein levels >2g/d and a white blood cell count (WBC) < 500/microliter are mostly present in patients with the chronic form. The serum ascites-albumin gradient is usually <1.1 g/dl in the chronic form compared to patients with an acute form of the syndrome.
• Doppler ultrasonography is the initial test of choice and usually helps confirm the diagnosis. If it is unavailable, equivocal, or cannot be performed, computed tomography (CT) or magnetic resonance imaging (MRI) may be helpful. If these tests fail to establish a diagnosis, but suspicion is still high, venography may be helpful.
• Technetium Tc 99m colloid scan of the liver and spleen. This often reveals diminished function in all portions of the liver except the caudate lobe, which is spared because it is drained by the inferior vena cava rather than the hepatic vein.
• CT scanning is helpful if a mechanical obstruction is suspected as an underlying cause.
• MRI can demonstrate the cessation of flow through the hepatic vein or by venography and injecting or using a dye to demonstrate thrombus in the vein. MRI is becoming the non-invasive modality of choice with a sensitivity and specificity of >90%
• Venography can accurately show the site and severity of obstruction, but the invasive nature limits the test's usefulness.
• Thrombophilia screen

Potential Complications

• Hepatic encephalopathy
• Variceal haemorrhage
• Hepatorenal syndrome
• Portal hypertension
• Bacterial peritonitis in the presence of ascites
• Hepatocellular carcinoma

Management

• Manage as for liver failure. Can deteriorate over weeks with end stage liver failure. Anticoagulate usually. In patients with an acute form and identifiable clot, thrombolytic therapy is advisable.
• Treat any underlying disorder and considered catheter delivered thrombolysis in selected cases or stenting, as well as placement of a transjugular intrahepatic portosystemic shunt in acute forms of BCS, which fail to respond.
• Surgical decompression can also be considered in acute forms if other therapies fail.
• Liver transplantation - in those with Protein C and S deficiency the thrombophilia is also treated as these are produced by the donor liver (assuming the donor does not have Protein C and S deficiency)