Intrahepatic portal obstruction in Wilson's disease as demonstrated by hepatic venous catheterization and splenoportal venography

Abstract

RAPHY. W. Jape Taylor, F. C. Jackson and Wallace N. Jensen. Depts. of Medicine and Surgery, Univ. of Pittsburgh Medical School, and V. A. Hospital, Pittsburgh, Pa. Moderate to marked splenomegaly in four patients with hepatolenticular degeneration (Wilson’s disease) prompted investigation of the portal circulation in these patients. Hepatic venous catheterization, with blood flow and wedged venous pressure measurements, was performed in all four patients and percutaneous splenic pulp pressures with portal venograms were obtained in three. In three patients with primarily neurologic manifestations, the wedged hepatic venous pressure was normal (mean 8.0 mm. Hg) whereas in two of these three patients the splenic pulp pressures were elevated (mean 24.5 mm. Hg). The hepatic blood flow and bromsulphalein clearance were normal in these patients. In a fourth patient with long-standing, advanced liver disease the splenic pulp pressure and wedged hepatic venous pressure were elevated to the same degree. The hepatic blood flow and bromsulphalein clearance were low. The three patients who had portal venograms demonstrated patent, abnormally tortuous and dilated portal veins with collateral venous channels. A normal wedged hepatic venous pressure in the presence of an elevated splenic pulp pressure and a patent portal vein suggests that an intrahepatic presinusoidal block exists early in the course of Wilson’s disease. Comparable data are not available in mild Laennec’s cirrhosis, but in the more advanced stages of this disease? the splenic pulp pressure and wedged hepatic venous pressure are both elevated; indicating sinusoidal or postsinusoidal obstruction. With progression of the hepatic disorder in hepatolenticular degeneration, obstruction to intrahepatic circulation becomes more widespread, and presumably involves the sinusoidal or postsinusoidal bed, with resultant circulatory dynamics which resemble those of advanced Laennec’s cirrhosis.