Partial-Exchange Blood Transfusion: An Effective Method for Preventing Mortality in a Child With Propofol Infusion Syndrome

Abstract

Here we describe a case of propofol-related infusion syndrome (PRIS) in a child with malignant refractory status epilepticus treated with partial-exchange blood transfusion (PEBT), an innovative method of resuscitation that has the potential to reduce the mortality rate associated with this syndrome. Our patient is a 4-year-old boy with malignant status epilepticus associated with bacterial meningitis. Propofol was used because of persistent seizure activity refractory to adequate doses of phenytoin, phenobarbital, levetiracetam, and midazolam infusion at 0.7 mg/kg per hour. Propofol was escalated from 0.6 mg/kg per hour to an electroencephalogram-burst-suppressing dose of 15.6 mg/kg per hour. Signs of PRIS were noticed after 48 hours on propofol. The severe bradycardia responded only to infusions of calcium gluconate. PEBT corrected all the cardiac abnormalities and returned enough hemodynamic stability to permit continuous veno-venous hemodialysis for renal failure and removal of toxins. PEBT is a safe and innovative option for correcting the metabolic abnormalities that result in cardiac dysfunction, which is typically the most serious and usually terminal event in PRIS. When done with small aliquots, it avoids the severe hemodynamic instability that is usually a hindrance with hemodialysis, continuous veno-venous hemodialysis, and extracorporeal membrane oxygenation, which are other methods of supporting these children during the crisis that are mentioned in the literature.

Neuroimaging of Propofol Infusion Syndrome: A Case Report and Review of Literature

“…It must be noted that our patient was not genetically tested for inborn errors of metabolism that may have predisposed him to developing PRIS. While there are no specific protocols for management of PRIS, treatment typically includes immediate discontinuation of propofol, correction of pH and electrolyte imbalances, supportive care including extracorporeal membrane oxygenation when necessary, and hemodialysis in select cases where electrolyte or pH imbalances cannot be corrected via other means [ 11 , 12 ]. At this time, given the risks associated with prolonged propofol use in young patients, propofol is not currently recommended for long term sedation in pediatric patients [ 1 ].…”

Propofol Infusion Syndrome in Refractory Status Epilepticus: A Case Report and Topical Review

“…From a total of 16 case reports, there were 3 cases of PRIS who developed cardiac arrest out of a total of 14 patients who developed one or more criteria of PRIS from Iyer et al's retrospective study [ 3 ], 3 cases from Kumar et al [ 4 ], and 2 cases from Hanna and Ramundo [ 5 ] and the rest are single case-based reports [ 6 – 18 ].…”

Prolonged propofol infusion for mechanically ventilated children

“…The description of propofol infusion syndrome has been followed by subsequent reports . Few studies have compared propofol with other sedative agents .…”

“…Propofol may reduce mortality compared with midazolam or lorazepam . Children are less often sedated for long periods with propofol as it may pre‐dispose to severe side‐effects referred to as ‘propofol infusion syndrome’ . Other studies suggest that low‐dose propofol is safe in children .…”