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

Pathophysiology

Obstruction is usually caused by a thrombus, but may result from extrinsic compression (tumour, abscess, cysts), membranous webs within the inferior vena cava (IVC),[3] or postoperative complications following liver transplantation.[6,7,8] Membranous web occlusion of the IVC is much more common in the Asian population than in Western countries.[1,9] Membranous web occlusion was once thought to be a congenital lesion, but more recent evidence suggests that membranous webs are the sequelae of thrombus formation.[10,11,12] Additionally, membranous webs have been associated with hepatocellular carcinoma.[11] Posthepatic obstruction leads to increased sinusoidal pressure, sinusoidal congestion, hepatomegaly, hepatic pain, portal hypertension and ascites. Without venous outflow, the elevated sinusoidal pressure leads to perisinusoidal necrosis of hepatocytes in Rappaport zone 3 and eventually to liver failure.[3] Ischaemic injury is compounded by oxidative injury caused by the release of free radicals from sinusoidal cells.[13] BCS results from the occlusion of three hepatic veins in about 2/3 of the cases, isolated occlusion of the IVC in 10% of cases and combined occlusion in almost 1/3 of the cases.[1] Caudate lobe hypertophy (Fig. 1), which may further compress the IVC, can occur in up to 50% of chronic presentations.[1]

Coronal Computed Tomography (CT) Showing Caudate Lobe Hypertrophy (white arrow) and Inferior Vena Cava (IVC) Compression (black arrow).

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