Noncirrhotic portal fibrosis

Abstract

In India noncirrhotic portal fibrosis (portal hypertension without demonstrable intrahepatic or extrahepatic obstruction) accounted for seventy-five (25 per cent) of 300 cases of portal hypertension studied by us. The majority of patients presented with marked splenomegaly of long duration and recurrent hemorrhage from esophageal varices (usually well tolerated), with minimal features of hepatocellular failure. There was an increase of alpha₂ globulin, beta globulins and immunoglobulin G (IgG). Hemodynamically, two significant findings were a marked gradient between the splenic pressure and wedged hepatic vein pressure, and a normal hepatic blood flow; in some patients wedged hepatic vein pressure was high. Roentgenologically, there was dilatation and tortuosity of the portal and splenic veins, with massive varices and hepatofugal collaterals. The intrahepatic vascular pattern was not much distorted, in contrast to cirrhosis. At shunt surgery the gross appearance of the liver varied from near normal to irregular nodularity resembling cirrhosis. There was at times shrinkage of the left lobe and hypertrophy of the right lobe. A needle biopsy specimen of the liver showed nonspecific changes, I.e., small focal areas of necrosis and regeneration, focal infiltrates, Kupffer cell hyperplasia and portal scars of varying size. Wedge biopsy and autopsy specimens revealed focal occlusive changes in the medium-sized radicals of the portal vein, changes considered specific for this disease. Electron microscopy revealed widening and collagenization of the space of Disse, with laying down of large collagen bundles in and around the sinusoids.Shunt surgery was performed in twenty-six patients. The operative mortality was 10.7 per cent. Splenorenal shunt was better tolerated than portocaval shunt but was associated with postoperative bleeding in 18.75 per cent of the cases.