Abstract
A 65-year-old woman was admitted to our hospital for further examination of recurrent consciousness disturbance with an elevation of blood ammonia level which had first occurred 8 years ago. She had noticed telangiectasias and subcutaneous vascular dilatation of the skin at the right upper chest from an early age. Her laboratory data showed pancytopenia, hypoalbuminemia and retention of plasma ICG. Celiac arteriography suggested a shunt between hepatic artery branches and hepatic vein branches. Superior mesenteric arteriography suggested an extrahepatic shunt between artery and portal vein and also an intrahepatic shunt between portal vein branches and hepatic vein branches. Gastrofiberscopy showed a cherry-red like capillary dilatation on the posterior wall of the anglulus. On laparoscopy, a marked scattering vascular dilatation was observed on the atrophied right lobe surface. Microscopic findings of the biopsy specimen taken from the left lobe showed no fibrotic nor cirrhotic changes. Because of these systemic vascular abnormalities involving the skin, the stomach and the liver, diagnosis of Rendu-Osler-Weber's disease was made, although the heredity was not proved. The cause of encephalopathy was thought to be a portal-systemic shunting due to hepatic vascular anomalies.
