Budd-Chiari syndrome computed tomographic imaging: Elucidating the devil in the details

Hepatic venous outflow obstruction, due to hepatic venous thrombosis is the hallmark of Budd-Chiari Syndrome (BCS), a relatively uncommon disorder. The aim of this study was to explore imaging details and causes, and define invasive and non-invasive interventions for achieving successful portosystemic shunting (PSS). The variations in imaging topology and associated risk factors in BCS cases, diagnosed during the past 20 months, were studied. Of the 53 cases (32 males, 21 females; mean age: 34.4±13.5 years), 5 (9%) were Hepatitis-B virus (HBV) positive, 12 (23%) were HCV positive and 6 (11%) were co-infected, rest were negative. Imaging features included mottled cirrhotic hepatic parenchyma, thrombosed hepatic and portal-vein with or without IVC thrombosis, left lobe atrophy, caudate hypertrophy, splenomegaly, portosystemic varices and ascites. Three cases had subacute onset; two had acute while the rest were chronic BCS. The aetiology was: protein C, S and anti-thrombin deficiency in 24 (45%), JAK2 mutation in 3 (6%), lupus antibody with increased homocysteine levels in 5 (9%) and cryptogenic in 21 (40%) cases. Twelve cases were planned for liver transplantation, 20 were prepared to undergo TIPS, and the rest were optimised medically.