Budd-Chiari Syndrome Computed Tomographic Imaging: Elucidating the Devil in the Details

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Objective: Hepatic venous outflow obstruction, due to hepatic venous thrombosis is hallmark of Budd-Chiari Syndrome(BCS), a relatively uncommon disorder. The scope of the study, was to explore imaging details and causes, and define invasive and non-invasive interventions for achieving successful portosystemic shunting (PSS).

Methods: Fifty-three BCS cases, diagnosed on sonographic and clinical evaluation during past twenty months were studied. The cases underwent dynamic contrast enhanced computed tomography, variations in imaging topology and associated risk factors were studies.

Results: Of 53 cases [32 males,21 females; mean age:34.4±13.5 years; Child-Pugh Score (CPS)-A 12, CPS-B 36 and CPS-C 5; median MELD-Na:10], five cases were Hepatitis-B virus(HBV) positive, twelve HCV positive and six were co-infected. Imaging features included mottled cirrhotic / nutmeg hepatic parenchyma(median size:19.8cm; range:16-24.6cm), thrombosed hepatic veins with/without portal vein and/or IVC thrombosis, left lobe atrophy, caudate hypertrophy, splenomegaly, portosystemic varices and ascites. Three cases had subacute onset, two had acute while rest were chronic BCS. 13 cases had hepatocellular carcinoma with Alpha-Fetoprotein(AFP) range:1125-20,000 ng/ml, rest had AFP<6ng/ml. Thrombosis of main portal vein and its tributaries was seen in nine and cavernous transformation in seven cases. The etiology was protein C, S and anti-thrombin deficiency(45%), JAK2 mutation(6%), lupus antibody with increased homocysteine levels (9%) and cryptogenic in 40%. 12 cases were offered liver transplantation; 20 were planned to undergo TIPS; rest were optimized medically.