Hepatic encephalopathy caused by congenital extrahepatic portosystemic venous shunt

Congenital portosystemic venous shunt is a relatively rare disease. Recently, a 60‐year‐old woman was admitted to our hospital for hepatic encephalopathy caused by congenital extrahepatic portocaval shunt. She had been in good health until the onset of this event, with no liver damage and no experience of abdominal surgery or history of abdominal trauma. In May 1993, hepatic encephalopathy manifested suddenly, with the chief complaint of orthostatic disturbance. Although conservative treatment was administered during the subsequent 5 years, on admission, liver damage and slight splenomegaly were shown, for which complete resection of the shunt vessel and splenectomy were performed. Postoperatively, the patient's symptoms have been alleviated. Hepatic encephalopathy caused by congenital portosystemic venous shunt requires long‐term conservative treatment, and the patient's quality of life is reduced. For this reason, surgical intervention or embolization with interventional radiology should be considered, and the maintenance of hepatic blood flow should also be considered.