Altered Hemodynamics in the Budd-Chiari Syndrome Demonstrated by Selective Hepatic and Selective Splenic Angiography

Abstract

Symptomatic occlusion of the hepatic veins, which bears the eponym of the Budd-Chiari syndrome, is uncommon (6). Definitive diagnosis prior to laparotomy or autopsy is often difficult. This communication will describe previously unreported angiographic findings seen on sequential selective hepatic and selective splenic arterial injections of contrast medium. These findings, as observed in 2 patients with the Budd-Chiari syndrome in whom the diagnosis was proved by autopsy, form the basis for this report. In these cases the direction of flow of contrast agent in the portal venous system was abnormal, because of the altered hemodynamics secondary to the blockage of the normal blood flow through the hepatic veins. The hepatogram phase of the selective hepatic angiogram was also abnormal, probably due to contrast medium in the dilated, congested sinusoids. Angiographic Findings on Selective Hepatic Artery and Selective Splenic Artery Injection When there is complete obstruction of the hepatic venous outflow, a selective hepatic arterial injection of contrast medium produces a more intense and prolonged (more than twenty seconds) hepatogram than is seen in the normal or in any other pathological condition. There is a unique, peculiar, finely mottled appearance that is probably due to stasis within the sinusoids. During the hepatogram phase opacification of the portal vein results from reversal of flow (hepatofugal flow) within it (Figs. 2 and 9, B). Hepatomegaly, which is due to congestion, is easily seen on both the arterial and the hepatogram phases (Figs. 1 and 9,A). Selective splenic artery injection of contrast medium demonstrates stasis within the spleen and does not opacify the portal vein (Figs. 4 and 10, B). When obstruction of the hepatic veins is limited to a lobe or segment, the hemodynamics are altered in a more localized way. The degree of hepatic enlargement may not be as profound. After hepatic artery injection there is localized retrograde (hepatofugal) flow of contrast medium through the branches of the portal vein in the obstructed area of the liver. There is subsequent visualization of the portal veins in nonobstructed areas where flow is in the normal (hepatopetal) direction (Fig. 7). After selective splenic artery injection opacification of the portal vein from the spleen is normal except that portal pressure may be increased, and thus varices or other collateral pathways may fill (Fig. 8, B). Case Reports Case I (3): This 12-year-old girl entered the Massachusetts General Hospital with massive ascites and edema of the lower extremities of sudden onset. Liver function studies showed severe parenchvmal disease. Seven days after admission selective hepatic and selective splenic angiography were performed to rule out possible cancer of the liver (Figs. 1–4). Seven days after admission selective hepatic and selective splenic angiography were performed to rule out possible cancer of the liver (Figs. 1–4).