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Tuesday, January 22, 2008

Budd Chiari Syndrome

Budd - Chiari Syndrome (BCS)

BCS is a rare disorder wherein there is wide spread clotting of the veins draining the liver. The main focus of the pathology is hepatic venous outflow obstruction resulting from either hepatic vein or Caval thrombosis or caval web or narrowing above the liver (Thrombotic states are responsible for >70% of BCS in the US and Europe while membranous Caval webs are common in Asia). The other causes include liver cancer, polycythemia, myeloproliferative disorders and oral contraceptive usage. The cause of this condition is multifactorial. There is a strong association with Hughe's syndrome or Anti phospholid syndrome (APLS), sticky blood caused by increased pro-coagulants in the blood. It is caused by a single point mutation in factor V gene, resulting in resistance against activated protein C.

Presentation: Can present acutely with pain in the abdomen and sudden onset liver failure with jaundice and coma or can present insidiously with a chronic course. The marked failure of the chronic phase is severe abdominal distension from large fluid accumulation in the belly, fatigue and poor nutritional status. When due to occlusion of the Vena Cava there is accompanying swelling of the feet and kidney failure.

Diagnosis: Angiography by transjugular approach and measurement of heptic wedge pressure are the gold standard for establishing the diagnosis. CT scan, MRI and Doppler examination can give equally reliable diagnosis. Level of serum albumin helps in determining the severity of liver disease. The workup should investigate the cause of BCS with complete analysis of procoagulant state (anti thrombin-III deficiency, Protein-C, Protein-S, Lupus anticaogulant, igG anticardiolipin antibody, polycythemia vera, Essential thrombocytocis etc). Peripheral smear and bone marrow biopsy may be required to rule out myeloproliferative disorders. Liver biopsy shows intense centrilobular congestion as well as pressure necrosis of the hepatocytes and in chronic cases fibrosis. Though liver biopsy is not required for diagnosis it helps in planning the appropriate therapy.

Treatment: Non surgical therapies with anticoagulant therapy are seldom successful. In the absence of supracaval obstruction, poto-caval or meso-caval shunts are useful. In presence of high grade caval obstruction meso-atrial shunt may be indicated. Early in the disease process it may be ideal to consider TIPSS (Trans jugular Intrahepatic Porto Systemic Shunt). Orthopotic liver transplant is indicated when the synthetic function of the liver is poor (ie; Albumin <> 3 mg/d1). Patients with fibrosis on the biopsy with no decompensation may be benefit with shunt surgery. Though progressive fibrosis and cirrhosis will dominate the clinical course of some post-shunt patients. The other factors which influence the decision to transplant include nutritional status and presence of malignancy or antiphospholipid syndrome.

Current opinions and Prognosis: Acute BCS with fulminant hepatic failure will need OLT. Chronic BCS with fibrosis and synthetic dysfunction should have the benefit of OLT(50-80% 3 year survival). Chronic BCS without synthetic dysfunction but fibrosis on liver biopsy may either have shunt surgery or OLT depending on the center's expertise. When early without fibrosis most patients will benefit from TIPSS and if they progress to have cirrhosis may later go in for OLT. Some of the procoagulant states caused by factor V gene mutation may be cured by OLT.

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