Severe Hyperammonemia Due to Fecal Bowel Obstruction With a Congenital Portosystemic Shunt Resulting in Refractory Status Epilepticus and Cerebral Edema

Hyperammonemic encephalopathy is a neurological emergency that can lead to seizures and cerebral edema. Although early interventions have been suggested, no clear criteria have been established. Herein, we report a case of severe non-hepatic hyperammonemia resulting in refractory status epilepticus within a day. A 79-year-old woman presented with acute altered mental status. Initial evaluation revealed septic shock and hyperammonemia due to fecal bowel obstruction with congenital portosystemic shunt. The patient was unresponsive to medical treatment and developed refractory status epilepticus. After surgical drainage with colostomy and a decrease in ammonia level, the patient developed cerebral edema and did not recover from the coma. Severe hyperammonemia warrants early intervention, especially in critically ill patients, with treatment of the cause and augmented removal of ammonia with renal replacement therapy.