Liver cirrhosis and hepatocellular carcinoma in hepatic vena cava disease, a liver disease caused by obstruction of inferior vena cava

Abstract

Purpose Hepatic vena cava disease (HVD), a form of Budd-Chiari syndrome, is caused by the obstruction of hepatic portion of the inferior vena cava. It is a chronic disease characterized by the development of liver cirrhosis (LC) and hepatocellular carcinoma (HCC). As HVD occurred in areas with high incidence of hepatitis B virus (HBV) infection and some patients tested HBsAg positive, it was thought to be the cause of LC and HCC. To assess the pathogenesis of LC or HCC in HVD, a long-term follow-up study was done. Method Fifty-six patients with HVD diagnosed by ultrasound (US) and confirmed by cavography in 31 and liver biopsy in 34 were followed up for an average of 14.8 ± 9 years. The occurrence of LC was diagnosed by US and/or liver biopsy and that of HCC by US, elevated level of a-fetoprotein, and liver biopsy or fine-needle aspiration cytology, or computed tomographic scan. Other risk factors for LC/HCC such as alcohol use and HBV and hepatitis C virus (HCV) infections were assayed. Results Forty-four (78.5%) and 6 (10.7%) patients developed cirrhosis and HCC, respectively. LC/HCC occurred more frequently among those who had severe or frequent acute exacerbations (P = 0.017), but it was not related to alcohol use or HBV and HCV infections. Conclusion HVD is independent risk factors for LC and HCC. Severe and/or recurrent loss of hepatocytes caused by hepatic venous outflow obstruction and/or thrombotic obstruction of small radicals of hepatic and portal veins that occurred during acute exacerbations was considered important in the pathogenesis of LC and HCC in HVD.

Development of a canine model with diffuse hepatic vein obstruction (Budd-Chiari syndrome) via endovascular occlusion

“…The development of cirrhosis from hepatic fibrosis takes a relatively long time. For instance, it takes 2-15 years (average, 8.7 years) for congestive liver fibrosis of the hepatic vena cava disease (a form of BCS) to advance to cirrhosis (22). It was hypothesized that, with time, cirrhosis may also develop following hepatocyte necrosis in the canine BCS model of the present study.…”

Hepatic Venous Outflow Obstruction: Suggestion of a New Classification

“…Patients with HVCS often have associated endophlebitis of medium-sized HVs and PVs. The associated ischemic liver damage eventually results in development of veno-portal type of cirrhosis (9)(10)(11). Development of LC within 3-9 months of liver biopsy for acute diseases has been documented in patients with sinusoidal obstruction syndrome (SOS) (12, 13), Budd-Chiari syndrome (BCS) (14), and HVCS (11).…”

Subacute Hepatic Failure: Its Possible Pathogenesis

“…On resolution, the lesion transforms into mild-to-severe stenosis or complete obstruction. 13,34 Ascites in HVCS was associated with high incidence of bacterial peritonitis. 14 The clinical features of SAHF in AH thus may be explained by associated bacterial infection resulting in thrombophlebitis of the hepatic portion of the IVC.…”

“…It is associated with bridging type of ischemic loss of hepatocytes and ultimate development of venocentric and venoportal cirrhosis. 34,40 HVCS was complicated by high incidence of cirrhosis, which occurred more frequently in patients with prolonged severe disease or frequent acute exacerbations. 34 Thus, the histological features of BN, thin cord like arrangement of the surviving hepatocytes with dilated sinusoids and rapid development of cirrhosis is more likely to be due to vascular lesion rather than to immunological damage of the liver by viral infection.…”

“…34,40 HVCS was complicated by high incidence of cirrhosis, which occurred more frequently in patients with prolonged severe disease or frequent acute exacerbations. 34 Thus, the histological features of BN, thin cord like arrangement of the surviving hepatocytes with dilated sinusoids and rapid development of cirrhosis is more likely to be due to vascular lesion rather than to immunological damage of the liver by viral infection. Based on these observations it is postulated that the entity called SAHF in AH is most probably caused by superadded bacterial infection resulting in acute or subacute HVCS.…”