Correlation of clinical and pathologic characteristics in Mexican patients with porto-sinusoidal vascular disease

Abstract

Porto-sinusoidal vascular disease (PSVD) is an uncommon disorder characterized by hepatic microvascular alterations and noncirrhotic portal hypertension. Its recent redefinition includes patients with and without portal hypertension, even in the presence of concomitant liver disease. The present study aimed to correlate the clinical, radiologic, and histopathologic findings in Mexican patients with PSVD and identify key diagnostic challenges.

A retrospective study was conducted on ten patients diagnosed with PSVD according to updated criteria, in whom liver biopsy showed no cirrhosis. Clinical, laboratory, imaging, and histopathologic data were collected. Upper gastrointestinal bleeding was the most frequent initial presentation, followed by mild thrombocytopenia. Biopsies revealed obliterative portal venopathy, nodular regenerative hyperplasia, and incomplete septal fibrosis. Magnetic resonance imaging identified periportal hyperintensity, which aided in differentiating PSVD from cirrhosis.

The findings confirm PSVD heterogeneity, underscoring the importance of integrating different diagnostic tools for its timely detection.