Wilsons Disease, Portal Hypertension and Intrahepatic Vascular Obstruction

Abstract

APPLICATION of the technics of hepatic-vein catheterization and percutaneous splenic puncture, with appropriate blood-flow and pressure measurements and radiologic visualization of the portal bed, has permitted the clear definition of portal hypertension as a factor in the causation of splenomegaly in Wilson's disease. Despite the universal pathologic involvement of the liver with an associated but variable degree of impairment of hepatic function, of splenomegaly in most cases and the occasional rupture of an esophageal varix (as in the patient described in Wilson's¹ original report), with disastrous results, portal hypertension has not been previously quantified or properly emphasized as a major . . .