Spectrum of histopathological changes in patients with non-cirrhotic portal fibrosis.

Abstract

Non-cirrhotic portal fibrosis (NCPF) is a clinical disorder characterized by features of portal hypertension in the absence of significant fibrosis. It is one of the commonest causes of portal hypertension in India. This study aimed to analyze histomorphological spectrum of NCPF in detail. There were 67 specimens from 66 patients which included 43 (65.2%) male and 23 (34.8%) female patients with a mean age of 31years (range: 7-61years). The liver function tests showed only a mild derangement. The average length of biopsy was 1.4cm (median: 1.2cm, range: 0.8-3.4cm) and the mean number of portal tracts per biopsy was 11.1 (median: 10, range: 5-30). Most cases showed a combination of histological features; the mean number of histological features per biopsy was 7.4 (median: 7, range: 3-12). Obliterative portal venopathy was seen in 47.8% cases. Portal angiomatosis (61.2%), paraportal shunt vessels (61.2%), portal vein dilatation (74.6%), hypoplastic portal tracts (56.7%), megasinusoids (64.1%), and abnormally dilated central veins (64.1%) were other prevalent features. Portal/periportal fibrosis and perisinusoidal fibrosis were seen in 77.6% and 61.2% cases; none showed bridging fibrosis or cirrhosis. The median hepatic venous pressure gradient (HVPG) and liver stiffness (LS) values were 8mm of Hg (range: 5-20mm of Hg) and 9.2kPa (range: 4.4-26.3kPa). There was no correlation of HVPG or LS with either portal/periportal fibrosis or perisinusoidal fibrosis. Due to relatively non-specific and non-pathognomonic nature, a combination of different histological features in the absence of significant fibrosis and appropriate clinico-radiological background is needed for diagnosing NCPF.