Anaesthesia with dexmedetomidine and remifentanil in a child with mitochondrial myopathy

Abstract

Patients with mitochondrial disorders have complex physiological issues which create a challenging scenario with regard to the safe provision of anaesthetic care. Within the spectrum of mitochondrial disorders, patients can be susceptible to multiple adverse effects and drug reactions from medications used during general anaesthesia. Although recent evidence suggests that inhalational anaesthetic agents may be used in patients with mitochondrial disorders, there is still a preference among some anaesthesia providers to use total intravenous anaesthesia (TIVA). In most scenarios, when TIVA is chosen, propofol is a major component. However, as a result of using propofol, patients with mitochondrial disorders may be susceptible to an acute metabolic crisis. It has been postulated that propofol, especially when given in large dosages, or when infused for prolonged periods of time, can adversely affect the function of the abnormal mitochondria that are present in patients with mitochondrial disorders. We present our experience with the use of dexmedetomidine as the primary component of a general anaesthetic regimen in a 10-year-old girl with a mitochondrial disorder and dystonia, who required anaesthetic care during a urological procedure. Previous reports on the use of dexmedetomidine as part of TIVA in patients susceptible to malignant hyperthermia are reviewed, and its benefits in patients with mitochondrial disorders, discussed. Additional concerns regarding the perioperative care of patients with mitochondrial disorders are deliberated.

Keywords: dexmedetomidine, malignant hyperthermia, mitochondrial myopathy