Table 1.
Clinical Signs of Portal Hypertension (Any One of the Following †) • Splenomegaly or hypersplenism • Esophageal varices • Ascites (non-malignant) • Minimally increased hepatic venous pressure gradient • Portovenous collaterals |
Exclusion of Cirrhosis on Liver Biopsy |
Exclusion of chronic liver disease causing cirrhosis or non-cirrhotic portal hypertension ‡ • Chronic viral hepatitis B or C • Non-alcoholic or alcoholic steatohepatitis • Autoimmune hepatitis • Hereditary hemochromatosis • Wilson’s disease • Primary biliary cholangitis |
Exclusion of Conditions Causing Non-Cirrhotic Portal Hypertension • Congenital liver fibrosis • Sarcoidosis • Schistosomiasis |
Patent Portal and Hepatic Veins (Doppler Ultrasound or CT Scanning) |
All criteria must be fulfilled to diagnose portal sinusoidal vascular disease † Splenomegaly must be accompanied by additional signs of portal hypertension to fulfil this criterion. ‡ Chronic liver disease must be excluded, because severe fibrosis might be understaged on liver biopsy Addapted from De Gottardi et al. [5].