Journal of the American College of Surgeons
-
Review Comparative Study Clinical Trial
Budd-Chiari syndrome caused by Behçet's disease: treatment by side-to-side portacaval shunt.
Behçet's disease is a chronic multisystem vasculitis of unknown etiology that involves skin, mucous membranes, eyes, blood vessels, joints, central nervous system, digestive system, and occasionally other organs. Budd-Chiari syndrome from occlusion of the major hepatic veins is a rare and serious complication of Behçet's disease. Although the mortality rate of Behçet's disease is only 3% to 4%, development of Budd-Chiari syndrome in patients with Behçet's disease has been associated with a mortality rate of 61%. This report presents the largest reported experience of Behçet's disease-related Budd-Chiari syndrome confined to the hepatic veins, and results of treatment by side-to-side portacaval shunt (SSPCS). These results are compared with those we have obtained in Budd-Chiari syndrome confined to the hepatic veins without Behçet's disease, and with results of treatment of Budd-Chiari syndrome in Behçet's disease reported in the literature. ⋯ SSPCS permanently reduced the mean portal vein-IVC pressure gradient (mm saline) from 205 to 7 in the 5 patients with Behçet's disease, and from 250 to 4 in the 27 without Behçet's disease. There was only one operative death, a patient without Behcet's disease. One patient with Behçet's disease died 2 years postoperatively from diffuse vasculitis, a complication of Behçet's disease, and the other 4 (80%) remain alive. All 26 operative survivors in the group without Behçet's disease (96%) are alive. Only one patient developed occlusion of the SSPCS, a man without Behçet's disease, and he required liver transplantation as a result of hepatic decompensation, PSE, and recurrent ascites. All other patients with or without Behçet's disease remained free of ascites, required no diuretics, were free of PSE, and had reversal of hepatic dysfunction. Serial liver biopsies showed normal architecture in 60% of patients with Behçet's disease and 46% of those without Behçet's disease. Return to fulltime work or housekeeping occurred in 80% of patients with Behçet's disease and 96% without Behçet's disease. Comparison of outcomes of our patients with 42 cases of Behçet's disease with Budd-Chiari syndrome reported in the literature, 79% of whom were treated medically, showed striking differences with an overall mortality rate of 61% in generally shortterm followup. (ABSTRACT TRUNCATED)