Use of remimazolam as an adjunct to general anesthesia for an adolescent with MELAS syndrome.

Abstract

MELAS syndrome is an acronym so named because of the characteristic clinical and pathophysiologic features including mitochondrial encephalo-myopathy, lactic aci- dosis, and stroke-like episodes. As the disorder impacts mitochondrial function and oxidative phosphorylation, clinical symptoms characteristically involve tissues and organ systems with high energy requirements including the skeletal muscle, the heart, and the central nervous sys- tem. Classically considered in the group of disorders known as mitochondrial myopathies, specific periopera- tive concerns must be considered in such patients given the effect of various anesthetic agents including propofol or the volatile anesthetic agents on patients with myopa- thic conditions or disorders of mitochondrial function. We report an 1-year-old with MELAS syndrome who re- quired anesthetic care during eye muscle surgery. Due to potential concerns with volatile anesthetic agents and propofol, we used the novel benzodiazepine, remimazo- lam, with dexmedetomidine and remifentanil to provide maintenance anesthesia. Previous reports of anesthetic care in patients with MELAS syndrome are reviewed and the use of the novel benzodiazepine, remimazolam, is dis- cussed.

Keywords

• mitochondrial myopathy
• oxidative phosphorylation
• melas syndrome