Closure of a distal splenorenal shunt as a therapy for refractory hyperammonaemia in setting of neuropsychiatric symptoms

Abstract

Before the widespread use of the transjugular intrahepatic portosystemic shunt, portal hypertension was addressed by the creation of an operative distal splenorenal shunt (DSRS). DSRS was largely regarded as a safe and effective therapy, though it did have a risk of precipitating hyperammonaemia and hepatic encephalopathy.A woman suffered a gunshot wound to the abdomen. This trauma led to portal vein thrombosis, cavernous transformation of the vein and eventually bleeding oesophageal varices from portal hypertension. A DSRS was created to decrease portal hypertension. Years later, she had profound depression with psychomotor slowing in the setting of hyperammonaemia. To reduce hepatic encephalopathy as a contributor to her neuropsychiatric condition, her shunt was closed. The hyperammonaemia resolved and neuropsychiatric symptoms improved.This case highlights several interesting clinical features, including portal hypertension due to chronic portal vein occlusion, cavernous transformation, a surgical DSRS causing hyperammonaemia and hepatic encephalopathy manifesting as neuropsychiatric symptoms.