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[Results of portasystemic shunts in the treatment of the Budd-Chiari syndrome].

Seven patients (6 women and 1 man) with a Budd-Chiari syndrome were treated by a portal systemic shunt (6 mesocaval and 1 portoatrial shunts). Budd-Chiari syndrome occurred after treatment by estrogen-progestational drugs in 3 cases and following delivery in one. It was due to a myeloproliferative syndrome in 2 cases and to narrowing of the ostium of the hepatic veins in one. All patients had ascites before operation and 5 had an increased serum activity of transaminases. One patient was operated in emergency for progressive massive liver cytolysis. There was no operative mortality. In all patients ascites cleared after operation and transaminases returned to normal values. There was no chronic encephalopathy. Six patients are alive and well with a follow-up of 5 to 66 months. One patient died of acute myeloblastic leukemia 19 months after mesocaval shunt. A liver biopsy was obtained in 2 patients 18 months and 2 years after operation. Hemorrhagic necrosis and sinusoidal dilatation had disappeared and there was annular fibrosis. These results suggest that portal-systemic shunting is a good treatment of the Budd-Chiari syndrome. Since the natural history of this syndrome is still poorly known, a surgical shunt should be rapidly decided upon.