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Budd-Chiari Syndrome

It results from the obstruction to hepatic venous outflow. The obstruction can be at 3 sites:
  1. Hepatic vein obstruction – (Commonest site)
    The causes are:
    1. Pregnancy, post partum state
    2. Oral contraceptives
    3. Malignancy – Hepatocellular carcinoma, renal cell carcinoma, colonic carcinoma.Q
    4. Hematological – PNH, polycythemia, sickle cell anemia.Q
    5. Sarcoidosis
    6. Vasculitis
    7. Underlying liver disease – cirrhosis, hepatitis
  2. Inferior vena cava obstruction – congenital. There is increased association of IUC obstruction with hepatocellular carcinoma.
  3. Obstruction of small branches of hepatic veins – veno occlusive disease.
    Due to fibrous obliteration of hepatic venules and small hepatic veins.
    The causes are:
    1. Pyrrolizidine alkaloids in bush tea (Bush Tea disease)Q
    2. AzathioprineQ
    3. Graft versus host reaction
    4. Radiation
    5. Inflammatory bowel disease
  1. Nutmeg liverQ
  2. Congestive hepatomegaly in early stage and cardiac cirrhosis in late stages.
Clinical features – The onset can be acute, subacute or chronic. The presentations can be:
  1. Gross ascites with abdominal painQ
  2. Acute abdominal pain with shock and fulminant hepatic failure. 
  3. HematemesisQ
Laboratory features:
U/S with Doppler flow is Ist test is to be done in BC syndrome
  1. Hepatic venogram – confirms the diagnosis.
  2. Inferior venacavography.
  1. Surgical correction is the only definitive treatment.
  2. Shunt surgery is useful if done early before irreversible hepatic damage and before thrombus extends to involve IVC.

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