Hereditary hemorrhagic telangiectasia (HHT) is a rare disorder characterized by telangiectasias and arteriovenous malformations (AVMs), which can involve multiple organ systems. Although hepatic involvement is common, the development of portosystemic encephalopathy is extremely rare. We herein report a 72-year-old woman with HHT-induced portosystemic encephalopathy secondary to hepatic arteriovenous malformations. She presented with disturbance of consciousness, and her serum ammonia level was elevated at 270 mg/dL. Color Doppler ultrasonography and contrast-enhanced computed tomography showed hepatic AVMs and shunts, which were useful for making the definite diagnosis. Portosystemic encephalopathy should be considered as a differential diagnosis in HHT patients presenting with disturbance of consciousness.
Keywords: arteriovenous malformation; hereditary hemorrhagic telangiectasia; portosystemic encephalopathy.