Budd-Chiari Syndrome: Illustrated Review of Current Management

John D. Horton; Francisco L. San Miguel; Jorge A. Ortiz

Disclosures

Liver International. 2008;28(4):455-466. 

In This Article

Diagnostic Evaluation

Work-up of suspected BCS begins with imaging modalities such as Doppler ultrasound, computed tomography (CT) scan and magnetic resonance imaging (MRI). Radiographic analysis should attempt to determine the patency of the hepatic veins, IVC and portal vein because this information will determine therapeutic options. Other radiographic findings seen in BCS include inhomogeneous parenchymal enhancement, presence of intrahepatic collaterals, hypervascular nodules and caudate lobe hypertrophy (Fig. 1).[20] Doppler ultrasound, often the first study obtained, has a sensitivity and specificity as high as 85%.[21] CT scans allow for the evaluation of ascites, hepatic vein patency, vena cava patency and caudate lobe hypertrophy. MRI may differentiate chronic from acute disease and provide further delineation of vascular anatomy. On MRI, patients with chronic BCS demonstrate heterogeneous signal intensity throughout the liver, while those with acute BCS show divergent signal intensities and decreased contrast enhancement in the periphery relative to the central region of the liver.[22] Other investigations that may aid in management include infra- and suprahepatic caval pressures (Fig. 2), hepatic venography and liver biopsy.[16] Imaging studies also play an important role in postoperative evaluation and surveillance of surgical shunts (Fig. 3).

Pressure Gradient of 9 mm Hg Measured Across an Inferior Vena Cava (IVC) Stenosis (large arrow).

Axial Computed Tomography (CT) in a Patient With a Cavoatrial Shunt Showing (a) Compression and Occlusion (arrow) of the Graft Posterior to the Liver. (b) Axial Magnetic Resonance Imaging (MRI) Showing a Patent Portocaval Shunt (arrow).

Underlying hypercoagulable states should be investigated in all patients.[16] Patients found to have membranous obstruction should also undergo hypercoagulable work-up, as recent evidence suggests that thrombosis may be the inciting event for membranous web formation.[10,11,12] Of note, low levels of antithrombin III, protein C and protein S may be seen in patients with any disease resulting in hepatocellular dysfunction. Bone marrow biopsy should be considered to identify occult myeloproliferative diseases.[23] Some authors recommend testing for JAK2 mutations in all patients with BCS in order to uncover a possible latent myeloproliferative disorder.[1]

Liver biopsy may reveal a wide range of histological findings from sinusoidal congestion and inflammation to cirrhosis and parenchymal necrosis. Extravasation of red cells into the liver-cell plate and space of Disse is a characteristic feature of BCS. Sample error during biopsy may occur as parenchymal changes may be heterogeneous. No correlation has been observed between histological findings and patient outcomes.[16,19,24] Moreover, some authors argue that biopsy results should not play a dominant role in decision making because patients with fibrosis at initial presentation may do well with procedures other than transplantation.[25]

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