A case of hereditary hemorrhagic telangiectasia with hepatic encephalopathy due to portal hepatic venous shunt.

Abstract

An 86-year-old male who was able to perform all activities of daily living (ADL) was diagnosed with hereditary hemorrhagic telangiectasia (HHT) at 70 years of age. Following his diagnosis, he had been receiving treatment at our hospital. After the sudden onset of a consciousness disorder, he was admitted to our hospital's emergency department with asterixis, a high serum ammonia level, and hepatic encephalopathy. After angiography, he was diagnosed with hepatic encephalopathy due to portal hepatic venous shunts. HHT is characterized by abnormal blood vessel construction and the formation of peripheral vasodilatation and shunt blood vessels. Although rare, portal hepatic venous shunts may sometimes cause hepatic encephalopathy. The extent of this shunt increases with age. As Japan is an increasingly aging society, the number of HHT patients with hepatic encephalopathy is likely to increase markedly in the future.