Non-cirrhotic hyperammonaemia: are we missing the diagnosis?

Hepatic encephalopathy secondary to hyperammonaemia is a known complication of chronic liver disease. In contrast, non-cirrhotic hyperammonaemia is a lesser-known entity that should be considered in a patient with acute encephalopathy as part of the diagnostic workup as prompt identification can help to avoid complications such as seizures and cerebral oedema. We present a case of a middle-aged woman who presented electively for a total pancreatectomy-duodenectomy with splenectomy, hepatico-jejunostomy, gastro-jejunostomy and developed encephalopathy on postoperative day 10 due to non-cirrhotic hyperammonaemia. © BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.