| Liver Function Tests
| Ascites Assessment and Management
|Protein C Deficiency
|Protein S Deficiency
|Factor V Leiden Deficiency
|Antithrombin III deficiency (AT3)
- 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
- 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
- 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
- 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
- FBC and Blood film, Renal function
- Liver enzymes, Prothrombin time
- Tap Ascites (exudate or transudate)
- Investigate hepatic vein thrombosis with a routine 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.
- MRI or ultrasound 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.
- Thrombophilia screen
- Manage as for liver failure
- Treat any underlying disorder and considered catheter delivered thrombolysis in selected cases
- Dilatation and stenting of the hepatic vein and portosystemic shunts e.g. Portocaval
- 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)