General anesthesia with remimazolam in a patient with mitochondrial encephalomyopathy: a case report

JA Clin Rep. 2021 Jun 23;7(1):51. doi: 10.1186/s40981-021-00454-8.

Abstract

Background: Systemic anesthetic management of patients with mitochondrial disease requires careful preoperative preparation to administer adequate anesthesia and address potential disease-related complications. The appropriate general anesthetic agents to use in these patients remain controversial.

Case presentation: A 54-year-old woman (height, 145 cm; weight, 43 kg) diagnosed with mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes underwent elective cochlear implantation. Infusions of intravenous remimazolam and remifentanil guided by patient state index monitoring were used for anesthesia induction and maintenance. Neither lactic acidosis nor prolonged muscle relaxation occurred in the perioperative period. At the end of surgery, flumazenil was administered to antagonize sedation, which rapidly resulted in consciousness.

Conclusions: Remimazolam administration and reversal with flumazenil were successfully used for general anesthesia in a patient with mitochondrial disease.

Keywords: Flumazenil; Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes; Mitochondrial myopathy; Remimazolam.