BACKGROUND/AIMS: Clinical manifestations and histological features of the liver in Budd-Chiari syndrome (BCS), with or without idiopathic membranous obstruction of the inferior vena cava (MOVC), vary according to whether BCS is acute, subacute or chronic. We clarified the diagnostic features in 6 patients with MOVC and in 1 without MOVC. METHODOLOGY: Five patients with subacute or chronic type BCS with MOVC complaining of epigastric pain, hematemesis and encephalopathy, and signs of portal hypertension or collateral circulation were seen. There was 1 asymptomatic patient with MOVC. One patient with acute type BCS without MOVC revealed hepatic and multi-organ failure. Liver function tests in BCS with MOVC were similar to those in liver cirrhosis, and laboratory data in acute type without MOVC were quite the same as those seen in fulminant hepatitis. Non-invasive image analysis by US, CT and MRI showed thrombi and obstruction of the IVC, and extrahepatic vasculature or communication between hepatic veins and IVC. Vena cavography showed the length of obstruction in IVC and collateral circulation in the extrahepatic or intrahepatic veins. Liver biopsy demonstrated massive hemorrhagic necrosis in acute type without MOVC, and laparoscopy with liver biopsy in asymptomatic, subacute and chronic type with MOVC showed subcapsular hemorrhage, congestion, fibrosis, and cirrhotic features. CONCLUSIONS: The non-invasive image analysis was complementary to vena cavography, and liver biopsy with or without laparoscopy was essential not only for diagnosis of acute, subacute, and chronic BCS, but also for therapeutic decision-making.
BACKGROUND/AIMS: Clinical manifestations and histological features of the liver in Budd-Chiari syndrome (BCS), with or without idiopathic membranous obstruction of the inferior vena cava (MOVC), vary according to whether BCS is acute, subacute or chronic. We clarified the diagnostic features in 6 patients with MOVC and in 1 without MOVC. METHODOLOGY: Five patients with subacute or chronic type BCS with MOVC complaining of epigastric pain, hematemesis and encephalopathy, and signs of portal hypertension or collateral circulation were seen. There was 1 asymptomatic patient with MOVC. One patient with acute type BCS without MOVC revealed hepatic and multi-organ failure. Liver function tests in BCS with MOVC were similar to those in liver cirrhosis, and laboratory data in acute type without MOVC were quite the same as those seen in fulminant hepatitis. Non-invasive image analysis by US, CT and MRI showed thrombi and obstruction of the IVC, and extrahepatic vasculature or communication between hepatic veins and IVC. Vena cavography showed the length of obstruction in IVC and collateral circulation in the extrahepatic or intrahepatic veins. Liver biopsy demonstrated massive hemorrhagic necrosis in acute type without MOVC, and laparoscopy with liver biopsy in asymptomatic, subacute and chronic type with MOVC showed subcapsular hemorrhage, congestion, fibrosis, and cirrhotic features. CONCLUSIONS: The non-invasive image analysis was complementary to vena cavography, and liver biopsy with or without laparoscopy was essential not only for diagnosis of acute, subacute, and chronic BCS, but also for therapeutic decision-making.