Our department’s general practice when advising on patients with organic acidemias who require surgery is to avoid the use of propofol. This practice was developed after a patient with methylmalonic acidemia (MMA) developed pancreatitis after a surgical procedure during which profofol was administered.
However a recent review of the anesthesia administration in a cohort of 28 patients with MMA or defects of cobalamin metabolism requiring 39 sedated procedures/surgeries showed that, in the setting of metabolic stability, propofol was administered safely. (Ktena et al, JIMD, 2015, Volume 38, Issue 5, pp 847-853).
This has caused me to reconsider my practice of absolutely recommending against propofol in MMA in the setting of a metabolically stable patient requiring an elective procedure.
Hilary Vernon, MD PhD