RADIOLOGICAL STUDY OF IDIOPATHIC BUDD-CHIARI SYNDROME COMPLICATED BY HEPATOCELLULAR-CARCINOMA - A REPORT OF 4 CASES

During an ll-yr period (1979-1989), we have experienced five patients with idiopathic Budd-Chiari syndrome (BCS), four (80%) of whom had ass ociated hepatocellular carcinoma (HCC). In contrast, the incidence of BCS complicated by HCC was 0.7% of a total of 556 patients who underwe nt surgery for HCC or were autopsied. Hepatitis B virus-related antige n or antibody was positive in one patient each. Four of our five patie nts were asymptomatic and were initially diagnosed by ultrasonography (n = 3) or computed tomography (n = 1). The hepatic parenchyma histopa thological findings were cirrhosis and fibrosis in one each. Infection of hepatitis B virus rather than BCS was speculated as a causative fa ctor for HCC in two patients. Membranous obstruction with spotty calci fication, intrahepatic bizarre communicating vessels, and the dilated anterior longitudinal veins in spinal canal were recognized in three p atients. Three patients had two HCCs which were similar in size and ar ose from the right and left hepatic lobe, separately, suggesting multi centricity of HCC. Both percutaneous transluminal angioplasty with Gru ntzig balloon catheters for the obstruction of the inferior vena cava and hepatic arterial embolization for HCC(s) were performed in three p atients. These patients survived 29.3 months on average after the diag nosis of BCS complicated by HCC(s). The opened IVC was confirmed to be patent on an average of 26.3 months after the first angioplasty.