Type B hepatic encephalopathy due to a congenital superior mesenteric-caval shunt: clinical scenario and therapeutic approach

Case Study


Type B Hepatic encephalopathy (HE) due to a congenital extra-hepatic porto-systemic shunt is an extremely rare condition. We report the case of a 57-year-old woman, with recurrent episodes of confusion and neuropsychiatric symptoms, who had an elevated serum ammonia level and a superior mesenteric-caval shunt documented on abdominal computed topography (CT) scan. There was no evidence of cirrhosis or portal hypertension. A diagnosis of non-cirrhotic, non-portal hypertension hepatic encephalopathy was made after excluding other causes of confusion and cognitive impairment. The patient was successfully treated by radiologically guided endovascular shunt closure and during 9 months follow up, her neuropsychiatric symptoms did not recur and repeated serum ammonia level results were normal.

Graphical abstract