Non-cirrhotic portal fibrosis

Abstract

Portal hypertension occurs commonly in patients with cirrhosis and rarely in those without cirrhosis of liver. The two most important causes of non-cirrhotic portal hypertension are non-cirrhotic portal fibrosis (NCPF) and extrahepatic portal venous obstruction (EHPVO). Unlike EHPVO, there is no thrombosis of the extrahepatic portal vein in NCPF. In NCPF, there occurs sclerosis of medium and small branches of the portal vein. The hepatic venous pressure gradient (HVPG) is normal in NCPF, when compared with cirrhosis where it is elevated. NCPF is also known as non-cirrhotic intrahepatic portal hypertension (NCIPH), idiopathic portal hypertension, hepatoportal sclerosis, and benign intrahepatic portal hypertension. It is a disease of obscure etiology, predominantly affecting the middle-aged males and females who present with hematemesis and massive splenomegaly.