Porto-Sinusoidal Vascular Disease: A Concise Updated Summary of Epidemiology, Pathophysiology, Imaging, Clinical Features, and Treatments

Abstract

Porto-sinusoidal vascular disease (PSVD) is a rare disorder usually characterized by signs of portal hypertension in the absence of an identifiable etiology, such as cirrhosis, and is largely underrecognized because of insufficient disease awareness among physicians. The prevalence of PSVD varies geographically, and it is more commonly reported in developing countries than in developed countries. Imaging findings suggestive of PSVD include the absence of liver surface nodularity, hypertrophy of segments IV and I, and normal or mildly elevated liver stiffness values in patients with signs of portal hypertension. Liver biopsy is mandatory for the diagnosis of PSVD, and specific histologic signs include obliterative portal venopathy, nodular regenerative hyperplasia, and incomplete septal fibrosis. The treatment of PSVD focuses on managing portal hypertension-related complications and adopts the same strategy as that for patients with cirrhosis. The long-term prognosis of PSVD, which is generally better than that of cirrhosis, is associated with age, specific signs of portal hypertension, including ascites, and underlying conditions.