Remimazolam-based anesthesia with intraoperative motor evoked potential monitoring in a patient with Leigh syndrome undergoing scoliosis surgery: a case report.

BACKGROUND: We present a case of scoliosis surgery performed under general anesthesia with remimazolam in a 16-year-old patient with Leigh syndrome (LS), a subtype of mitochondrial encephalomyopathy. Anesthetic management in such patients is challenging because of the risks of malignant hyperthermia with inhalational agents and propofol infusion syndrome, and because many of these patients present with impaired consciousness and respiratory compromise, anesthetic management becomes extremely difficult. To date, very few reports have described remimazolam use in LS, and none have described cases requiring intraoperative motor-evoked potential (MEP) monitoring. CASE PRESENTATION: A patient diagnosed with LS at 7 months of age underwent corrective scoliosis surgery. Anesthesia was induced and maintained using remimazolam supplemented with opioids and muscle relaxants. Some intraoperative MEP signals were attenuated but remained monitorable. The patient was extubated with flumazenil and admitted to the intensive care unit, where a transient decrease in oxygenation was observed. However, the patient recovered without any complications and was discharged uneventfully. CONCLUSION: Remimazolam may be a feasible anesthetic option for patients with LS undergoing surgery requiring MEP monitoring. However, its use should be carefully determined based on factors such as the patient’s age, level of consciousness, respiratory function, and history of epilepsy.