| Popis: |
A case of Cruveilhier-Baumgarten (C-B) disease is reported, apparently the first in which postsinusoidal portal hypertension has been demonstrated. This portal hypertension occurred in the absence of liver disease or abnormalities in the hepatic or in portal veins. Three liver biopsy specimens showed normal hepatic morphology and there was only questionable increase in portal connective tissue at autopsy. The hepatic, splenic, portal and mesenteric veins and hepatic arteries were demonstrated by hepatic venography, splenoportography, operative portography and selective abdominal arteriography, respectively, and were found to be patent. This patient, with no significant morphologic or functional abnormality of the liver, had portal hypertension, portal systemic anastomoses, and complications usually associated with cirrhosis of the liver, i.e., excessive salt and water retention and encephalopathy. The presence of postsinusoidal portal hypertension in C-B disease in the absence of distortion of the hepatic architecture is compatible with the hypothesis that the portal hypertension is caused by increased blood flow in the portal system. The developing portal hypertension results in progressive enlargement of a congenitally patent umbilical vein. The drainage of portal blood into the umbilical vein not only reduces portal hypertension but also deprives the liver of portal venous blood as in an "Eck fistula." |
