Budd-Chiari syndrome caused by Behcet's disease: Treatment by side-to-sideportacaval shunt

Background: Behcet'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 an d serious complication of Behcet's disease. Although the mortality rate of Behcet's disease is only 3% to 4%, development of Budd-Chiari syndrome in p atients with Behcet's disease has been associated with a mortality rate of 61%. This report presents the largest reported experience of Behcet's disea se-related Budd-Chiari syndrome confined to the hepatic veins, and results of treatment by side-to-side portacaval shunt (SSPCS). These results are co mpared with those we have obtained in Budd-Chiari syndrome confined to the hepatic veins without Behcet's disease, and with results of treatment of Bu dd-Chiari syndrome in Behcet's disease reported in the literature. Study Design: SSPCS was performed in 5 patients with Behcet's disease who h ad developed acute Budd-Chiari syndrome, and 27 patients with Budd-Chiari s yndrome from other causes. In ail patients, Budd-Chiari syndrome was confin ed to the hepatic veins without involvement of the inferior vena cava (NC). Patients were studied prospectively and were followed up at regular interv als for from 1.5 to 26 years (mean 10.6 years, 81% more than 5 years). Foll owup was 100%. Patients were mainly young adults; mean age was 24.6 years i n the patients with Behcet's disease and 30.0 years in those without Behcet 's disease. All patients had massive ascites, abdominal pain, hepatosplenom egaly, and abnormal liver function. Diagnosis was based on angiographic dem onstration of occlusion of the major hepatic veins, and liver biopsy findin gs 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 y ear or two in followup, patients underwent liver biopsy and evaluation of S SPCS by Doppler duplex ultrasonography and angiography with pressure measur ements. Outcomes criteria included mortality rate, SSPCS patency, maintenan ce of portal decompression, liver function, presence of ascites, presence o f 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 Behcet's diseas e. Results: SSPCS permanently reduced the mean portal vein-IVC pressure gradie nt (mm saline) from 205 to 7 in the 5 patients with Behcet's disease, and f rom 250 to 4 in the 27 without Behcet's disease. There was only one operati ve death, a patient without Behcet's disease. One patient with Behcet's dis ease died 2 years postoperatively from diffuse vasculitis, a complication o f Behcet's disease, and the other 4 (80%) remain alive. All 26 operative su rvivors in the group without Behcet's disease (96%) are alive. Only one pat ient developed occlusion of the SSPCS, a man without Behcet's disease, and he required liver transplantation as a result of hepatic decompensation, PS E, and recurrent ascites. All other patients with or without Behcet's disea se remained free of ascites, required no diuretics, were free of PSE, and h ad reversal of hepatic dysfunction. Serial liver biopsies showed normal arc hitecture in 60% of patients with Behcet's disease and 46% of those without Behcet's disease. Return to fulltime work or housekeeping occurred in 80% of patients with Behcet's disease and 96% without Behcet's disease. Compari son of outcomes of our patients with 42 cases of Behcet's disease with Budd -Chiari syndrome reported in the literature, 79% of whom were treated medic ally, 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 Behcet 's disease, early diagnosis of Budd-Chiari syndrome is imperative, and prom pt treatment by portal decompression surgery is indicated. SSPCS results in reversal of liver damage and correction of hemodynamic disturbances, prolo nged survival, and a life of good quality when it is performed early in the course of Budd-Chiari syndrome. (J Am Coil Surg 1999;188: 396-407. (C) 199 9 by the American College of Surgeons).