Budd-Chiari syndrome caused by Behçet’s disease: treatment by side-to-side portacaval shunt

Abstract

Background: 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. Study Design: SSPCS was performed in 5 patients with Behçet’s disease who had developed acute Budd-Chiari syndrome, and 27 patients with Budd-Chiari syndrome from other causes. In all patients, Budd-Chiari syndrome was confined to the hepatic veins without involvement of the inferior vena cava (IVC). Patients were studied prospectively and were followed up at regular intervals for from 1.5 to 26 years (mean 10.6 years, 81% more than 5 years). Followup was 100%. Patients were mainly young adults; mean age was 24.6 years in the patients with Behçet’s disease and 30.0 years in those without Behçet’s disease. All patients had massive ascites, abdominal pain, hepatosplenomegaly, and abnormal liver function. Diagnosis was based on angiographic demonstration of occlusion of the major hepatic veins, and liver biopsy findings of intense hepatic congestion and necrosis. SSPCS was performed within 4 months of the onset of Budd-Chiari syndrome in all but 3 patients. Every year or two in followup, patients underwent liver biopsy and evaluation of SSPCS by Doppler duplex ultrasonography and angiography with pressure measurements. Outcomes criteria included mortality rate, SSPCS patency, maintenance of portal decompression, liver function, presence of ascites, presence of portal-systemic encephalopathy (PSE), need for diuretics, quality of life, and return to work. Our results were compared with those reported in the literature in 42 patients who had Budd-Chiari syndrome with Behçet’s disease. Results: 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 Behçet’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. Conclusion: Because the prognosis for long survival is quite good in Behçet’s disease, early diagnosis of Budd-Chiari syndrome is imperative, and prompt treatment by portal decompression surgery is indicated. SSPCS results in reversal of liver damage and correction of hemodynamic disturbances, prolonged survival, and a life of good quality when it is performed early in the course of Budd-Chiari syndrome.