Surgical treatment of Budd-Chiari syndrome induced by Behcet's disease

Although Budd-Chiari syndrome in Japanese is usually chronic, and of unknown etiology and idiopathic, Behcet's disease is rare as an underlying disorder of Budd-Chiari syndrome in Japanese. To clarify the Behcet-induced Budd-Chiari syndrome, the clinical course and pathologic findings of patients with Behcet-induced Budd-Chiari syndrome were compared with those of patients with idiopathic Budd-Chiari syndrome.

We treated 45 patients (15 women and 30 men) with our devised surgical procedure. With normothermic partial bypass, the occluded vena cava and hepatic veins were reopened. The age of the patients ranged from 24 to 76 years (mean, 48.9 13.0 years). In two patients, Budd-Chiari syndrome was induced by Behcet's disease (Behcet group). The other 43 patients (control group) had no distinct underlying disorder. The Behcet group was compared to the control group with regards to (1) onset of symptoms and duration of illness prior to medical treatment, (2) preoperative laboratory data including liver function, (3) intraoperative findings, (4) microscopic findings of liver tissue, and (5) postoperative course.

(1) In the Behcet group, duration of illness from diagnosis to surgical treatment was markedly shorter (P=0.027, 8.5 months vs. 10.1 10.6 years). (2) The preoperative laboratory data of liver function were similar in both groups with moderately impaired hepatic function. (3) The Behcet group had no patent hepatic vein (P=0.025 vs. 1.22 0.57). (4) Microscopic examination of the liver tissue showed liver cirrhosis or liver fibrosis in the control group, and centrilobular marked congestion only in the Behcet group. (5) During hospitalization, one patient of the control group died due to preoperative severe hepatic failure. One patient with Behcet's disease underwent reoperation due to reocclusion by Behcet-induced vasculitis, and the other died of peritonitis by intestinal Behcet's disease.

In Budd-Chiari syndrome in Japanese, the Behcet-induced Budd-Chiari syndrome had an acute clinical course, and its postoperative prognosis depends on the prognosis of the Behcet's disease.