Failed Emergence After Pediatric Epilepsy Surgery: Is Propofol-Related Infusion Syndrome to Blame?

Propofol infusion syndrome was first reported in the literature by Bray in 1998. He described a series of fatal outcomes after a presenting constellation of symptoms observed in pediatric patients who had received prolonged propofol infusions. Profound metabolic acidosis and bradycardia are the disease’s hallmark features, which can further develop expeditiously to rhabdomyolysis, renal failure, and heart failure. It has been subsequently theorized that a triggering mechanism or a precipitating factor sets up the progressive physiologic spiral which can ensue. The name of the disease was expanded to Propofol Related Infusion Syndrome (PRIS), as propofol alone was no longer considered the culprit. The disease process is rare and can present with an insidious onset in some cases, causing much speculation of whether there is a proper grasp of the disease entity in its entirety as currently reported. The case discussed in this article depicts an adverse neurologic outcome following a craniotomy for temporal lobectomy in a child with lesional epilepsy. Since there was no obvious causative factor for these findings, PRIS became a diagnosis that was robustly discussed among the involved services.