Dominique Valla - Vascular Disorders of the Liver: VALDIGs Guide to Management and Causes

This Springer imprint is published by the registered company Springer Nature Switzerland AG

The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland

Vascular liver disorders (VLD) encompass a wide spectrum of clinico-pathological entities resulting from damage to the hepatic vascular system, that includes hepatic arteries, portal and hepatic veins, sinusoids, and lymphatics. Among them, the vascular structures most often damaged at liver biopsy examination include portal and hepatic veins, as well as sinusoids.

According to the type of vascular structure involved, specific morphological criteria are recognized, and histological analysis may thus be contributive in the management of patients []. In addition to identifying elementary morphological features suggestive of vascular disorders, the analysis of liver biopsy will also assess the extent of changes and their chronicity through the evaluation of fibrosis and architectural distortion. Nevertheless, as for other liver diseases, interpretation of liver histology (i.e. liver biopsy) should be integrated into a multidisciplinary approach including pathologists, clinicians and radiologists.

Recent advances have been made in the description of liver pathological changes associated with portal hypertension in the absence of portal vein thrombosis and cirrhosis, the entity previously recognized as idiopathic non-cirrhotic portal hypertension, for which the denomination porto-sinusoidal vascular disease has been recently proposed [].

This chapter describes the main morphological features observed in VLD, as well as the different patterns associated with each VLD. Indications of liver biopsy will be also recapitulated.

The histological assessment of liver biopsy for the study of vascular disorders is based on the analysis of serial sections to ensure adequate examination, taking into account potential sampling variability. In addition to standard hematoxylin and eosin staining, which can elucidate most histological features, red Sirius or trichrome for connective tissue staining, and Perls for iron identification are performed. Reticulin staining provides a more accurate evaluation of hepatic architecture than the former, which is most helpful to highlight regenerative processes in a context of vascular disorders.

The interpretation of liver biopsy is based, as in other liver diseases, on a systematic analysis of the different morphological structures of the liver, with a specific attention to centrilobular and portal veins, and sinusoids. As for other chronic liver diseases, morphological lesions in VLD may not be evenly distributed and may vary in their severity, thus challenging the reliability of biopsy. Accordingly, while no specific study has been carried out in the specific context of VLD, a 25-mm biopsy is considered an optimal size for accurate pathological evaluation [].