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The term porto-sinusoidal vascular disorder (PSVD) was recently proposed to replace that of idiopathic non-cirrhotic portal hypertension (INCPH) to describe patients with typical histological lesions in absence of cirrhosis, irrespective of the presence/absence of portal hypertension (PH), and new diagnostic criteria were defined. The study aimed to compare the applicability between the diagnostic criteria of PSVD and those of INCPH. 53 patients affected by PSVD were enrolled. Biochemical, clinical, ultrasound and histological data, the presence and type of associated diseases were recorded in a database. According to the new criteria, histological data and signs of PH were divided into specific and non-specific. Percutaneous and transjugular biopsies were compared to establish the usability of the two methods for diagnostic purposes. In 85% of the patients the diagnosis of PSVD was obtained by applying the first criterion (25 had specific histological signs with specific signs of PH); one patient presented with specific histological signs but no PH. In 8 patients the diagnosis was obtained by applying the second criterion. 19% of patients had portal vein thrombosis. Finally, the prevalence of the various histological lesions was similar between the patients submitted to percutaneous and transjugular liver biopsy. The study confirms that the diagnostic criteria of PSVD lead to the inclusion of a greater number of patients than INCPH. [ABSTRACT FROM AUTHOR]

It is well established that portal hypertension can occur in the absence of cirrhosis, as reported in patients with immune disorders, infections and thrombophilia. However, similar histological abnormalities primarily affecting the hepatic sinusoidal and (peri)portal vasculature have also been observed in patients without portal hypertension. Thus, the term porto-sinusoidal vascular disorder (PSVD) has recently been introduced to describe a group of vascular diseases of the liver featuring lesions encompassing the portal venules and sinusoids, irrespective of the presence/absence of portal hypertension. Liver biopsy is fundamental for PSVD diagnosis. Specific histology findings include nodular regenerative hyperplasia, obliterative portal venopathy/portal vein stenosis and incomplete septal fibrosis/cirrhosis. Since other conditions including alcohol-related and non-alcoholic fatty liver disease, or viral hepatitis, or the presence of portal vein thrombosis may occur in patients with PSVD, their relative contribution to liver damage should be carefully assessed. In addition to histology and clinical diagnostic criteria, imaging and non-invasive tests such as liver and spleen stiffness measurements could aid in the diagnostic workup. The introduction of PSVD as a novel clinical entity will facilitate collaborative studies and investigations into the underlying molecular pathomechanisms encompassed by this term. [ABSTRACT FROM AUTHOR]

Abstract: This review will provide an overview of the pathological features of non-cirrhotic portal hypertension (NCPH) and incomplete septal cirrhosis (ISC), focussing on practical diagnostic approaches and problems. Although many liver diseases may be associated with portal hypertension in the absence of advanced fibrosis, the term NCPH is most widely used to describe a spectrum of changes resulting from occlusion of small portal vein branches, also referred to as “hepato-portal sclerosis” or “obliterative portal venopathy”. Other histological manifestations of NCPH include parenchymal atrophy, nodular regenerative hyperplasia, formation of shunt vessels and foci of sinusoidal dilatation. In more severe cases, foci of parenchymal collapse may be associated with formation of delicate non-linking fibrous septa, corresponding to incomplete septal cirrhosis. Although ISC is generally considered to be part of the morphological spectrum of NCPH, it can also be regarded as part of the spectrum of true cirrhosis – either reflecting cirrhosis that is incompletely developed or partially regressed. [Copyright &y& Elsevier]