Hyperammonemia of unknown origin

Abstract

Idiopathic portal hypertension is a benign long-standing non-cirrhotic portal hypertension with no typical laboratory findings and absence of stigmata of chronic liver disease. The disease is diagnosed by the presence of evidence of portal hypertension with preserved liver function and absence of extrahepatic portal vein obstruction. We report the case of a 71-year-old woman who was admitted in hospital with encephalopathy and hyperammonemia. Liver biochemical tests excluded cirrhosis but revealed pancytopenia; preliminary abdomen ultrasound was normal. Ultrasound doppler and abdomen computed tomography of portal vein revealed patent portal vein with impaired portal intrahepatic perfusion and portal-systemic shunts.