Abstract
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.
Highlights
Propofol-related infusion syndrome (PRIS) is a rare and potentially fatal syndrome characterized by severe metabolic acidosis, rhabdomyolysis, renal failure, and heart failure [1]
While the exact mechanism behind propofol infusion syndrome is not fully elucidated, it is a widely accepted theory that the syndrome bears a striking resemblance to the symptomatology of patients with mitochondrial disease experiencing significant metabolic stressors [3]
Our patient demonstrated a concerning postoperative presentation with abnormal brain imaging in correlation with a failure to emerge from anesthesia after consecutive propofol-based anesthetics
Summary
Propofol-related infusion syndrome (PRIS) is a rare and potentially fatal syndrome characterized by severe metabolic acidosis, rhabdomyolysis, renal failure, and heart failure [1]. Bray and colleagues initially reported PRIS as a result of increased morbidity and mortality in pediatric intensive care patients receiving long term (>48 hours) and high dose (>4mg/kg/hr or 67 mcg/kg/min) propofol infusions [2]. The increase in seizure frequency had resulted in worsening academic performance (A to C student), blunting of effect, inability to continue with soccer, and impaired peer relationships He was scheduled for right frontal lobe resection for focal, lesional epilepsy. On the morning of surgery, he received his scheduled valproic acid and levetiracetam but experienced breakthrough seizure activity immediately prior to surgery He was transferred from the Pediatric Intensive Care Unit to the operative suite urgently. The patient exhibited no neurologic sequelae at subsequent outpatient follow-up visits with his neurologist with a significant improvement from his baseline symptoms and was free to resume all activities
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