Long-term results of treatment of Budd-Chiari syndrome with portal decompression
M. J. Orloff, M. S. Orloff and P. O. Daily
Department of Surgery, School of Medicine, University of California San Diego, La Jolla.
Thirty-three patients with Budd-Chiari syndrome were studied for 1 to 19
years following portal decompression. All had ascites, hepatomegaly,
abnormal liver function, angiographic demonstration of inferior vena cava
and/or hepatic vein occlusion, and biopsy specimens showing intense hepatic
congestion and necrosis. When thrombosis was confined to hepatic veins (20
patients), side-to-side portacaval shunt resulted in 95% operative
survival, 90% prolonged survival, permanent shunt patency, relief of
ascites, reversal of liver dysfunction, and reversal or improvement of
hepatic lesions. When thrombosis involved the inferior vena cava,
mesoatrial shunt (eight patients) was unsatisfactory because of a 63%
mortality rate from liver failure due to shunt thrombosis. In contrast, a
new procedure consisting of combined portacaval and caval-atrial shunts
(five patients) has been highly successful, with 100% survival, shunt
patency, relief of ascites, and reversal of pathologic abnormalities.