Non-cirrhotic hyperammonaemia: are we missing the diagnosis?

Area of Science:

• Gastroenterology and Hepatology
• Neurology

Background:

• Hepatic encephalopathy is a common complication of chronic liver disease, typically linked to hyperammonaemia.
• Non-cirrhotic hyperammonaemia is an underrecognized cause of encephalopathy, necessitating its inclusion in diagnostic workups.

Observation:

• A middle-aged woman underwent a major abdominal surgery including total pancreatectomy-duodenectomy and splenectomy.
• Postoperatively, the patient developed acute encephalopathy on day 10.

Findings:

• The patient's encephalopathy was attributed to non-cirrhotic hyperammonaemia.
• This highlights a rare but critical postoperative complication in patients without pre-existing liver cirrhosis.

Implications:

• Prompt identification and management of non-cirrhotic hyperammonaemia are vital to avert severe neurological sequelae such as seizures and cerebral oedema.
• This case underscores the importance of considering hyperammonaemia in the differential diagnosis of postoperative encephalopathy, even in the absence of cirrhosis.