Budd-Chiari Syndrome Workup
- Author: Praveen K Roy, MD, AGAF; Chief Editor: BS Anand, MD more...
Evaluate patients with Budd-Chiari syndrome for underlying predisposing conditions, such as malignancy or hypercoagulable states, and institute appropriate therapy.
Examination of ascitic fluid provides useful clues to the diagnosis, including the following:
Patients usually have high protein concentrations (>2 g/dL); this may not be present in persons with the acute form of Budd-Chiari syndrome
The white blood cell (WBC) count is usually less than 500/µL
The serum ascites–albumin gradient is usually less than 1.1 (except in the acute forms of the disease)
Routine biochemical test results are usually nonspecific in Budd-Chiari syndrome, although mild elevations in serum aminotransferase and alkaline phosphatase levels are present in 25-50% of patients.
Thrombi can be visualized; color-flow Doppler ultrasonography, the preferred mode, has a sensitivity and specificity of 85-90%.[20, 21] (See the images below.)
Computed tomography (CT) scanning
Detailed imaging studies are required to determine the precise level and degree of obstruction. CT scanning can rarely provide such detail, unless a mechanical obstruction, such as a locally invading tumor, is suspected.
Magnetic resonance imaging (MRI)
MRI, which has a sensitivity and specificity of 90% or higher, is becoming increasingly useful in providing less-invasive venography, angiography, and cholangiography findings. MRI may assist in differentiating acute from chronic Budd-Chiari syndrome, because it is able to provide a larger image of the vasculature, as well as determine if edema of the parenchyma is present (acute form).
Catheterization and venography can clearly delineate the nature and severity of an obstruction. Occasionally, therapeutic interventions can be undertaken at the same time, including balloon angioplasty, localized thrombolysis, and placement of a stent or transjugular intrahepatic portacaval shunt (TIPS).[22, 24, 25, 26]
Liver Biopsy and Histology
Percutaneous liver biopsy can be of prognostic assistance, particularly if liver transplantation is being considered, to establish the degree of hepatocellular damage and the presence and degree of fibrosis.
Pathologic findings in liver biopsy are (1) high-grade venous congestion and centrilobular liver cell atrophy, and, possibly, (2) thrombi within the terminal hepatic venules. The extent of fibrosis can be determined based on biopsy findings. The most severe findings can include fulminant hepatic failure with massive centrilobular necrosis.
Studies have indicated, however, that early pathology related to Budd-Chiari syndrome does not have a significant impact on survival.
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