Idiopathic Presinusoidal Portal Hypertension (Banti's Syndrome)

Abstract

A 23-year-old man with portal hypertension in the presence of a normal liver and a patent portal vein is described. The patient presented with hemorrhage from esophageal varices and hyper splenism. Liver function studies and liver biopsy were normal. Splenoportography revealed a patent splenic and portal vein. Splenic pulp pressure was elevated to 460 millimeters of saline. Hepatic vein wedge pressure was 140 millimeters saline. Laparotomy revealed a large portal vein which was patent with whose thickened walls contained amorphous material and sclerotic plaques. Portacaval anastomosis lowered the portal pressure to 170 millimeters of saline. The patient represents a case of presinusoidal portal hypertension.