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Purposeof Review: To review the clinical and histopathological features of obliterative portal venopathy (OPV). Recent Findings: OPV is one cause of non-cirrhotic portal hypertension and is part of the general group of vascular liver diseases termed porto-sinusoidal vascular disease (PSVD), along with nodular regenerative hyperplasia and incomplete septal fibrosis/cirrhosis. It is increasingly being found on liver biopsies in patients having abnormal liver tests and is reliably diagnosable only by liver biopsy. Radiologically, patients may appear to have a cirrhotic liver but typically their liver tests are normal or mildly abnormal, and the liver function is normal. Summary: Most cases of OPV are idiopathic but it can be seen in collagen vascular diseases, in the setting of portal vein thrombosis, in hematologic disorders, and immunodeficiency syndromes, and as a result of certain medications. Liver biopsy may show subtle histological findings so there must be a keener awareness amongst both clinicians and pathologists of this entity, as well as a discourse alerting the pathologist to the presence of portal hypertension. [ABSTRACT FROM AUTHOR]

Purpose of Review: Porto-sinusoidal vascular disease (PSVD) refers to a group of vascular diseases of the liver with lesions encompassing the portal venules and sinusoids, irrespective of portal pressure measurements and the presence or absence of cirrhosis. This article aims to provide an update on the pathologic, clinical, diagnostic, and therapeutic aspects of this underrecognized disease. Recent Findings: The diagnosis of PSVD requires a liver biopsy. Specific histology findings include nodular regenerative hyperplasia, obliterative portal venopathy or portal vein stenosis, and incomplete septal fibrosis/cirrhosis. The natural history of PSVD has not been elucidated, and screening guidelines for sequalae such as portal hypertension or portal vein thrombosis and the outcome of liver transplantation in these patients are unclear. Summary: Patients who have signs of portal hypertension despite unremarkable work-up for portal vein thrombosis and cirrhosis should be actively evaluated for PSVD. A heightened awareness of PSVD as a novel clinical entity should facilitate further collaborative studies and investigations. [ABSTRACT FROM AUTHOR]