Pathology of non-cirrhotic portal hypertension and incomplete septal cirrhosis

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.