Creatine kinase level was 162 IU/L (normal range, 41–153). Although enhanced insulin therapy had been introduced, her HbA1c level was high (12.5%) immediately before surgery. Estimated glomerular filtration rate was low (27 mL/min) because of diabetic nephropathy. Cardiac contractility was diffusely decreased and cardiac ejection fraction was 45%. Transthoracic echocardiography showed circumferential thickening of the left ventricle and asymmetric hypertrophy of the septum, which was thicker than the posterior wall. Preoperative electrocardiography showed no abnormalities. We report the successful use of intravenous remimazolam and its antagonist flumazenil for general anesthesia in a patient with mitochondrial myopathy.Ī 54-year-old woman (height, 145 cm weight, 43 kg) diagnosed with mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes elected to undergo cochlear implantation to address her progressive bilateral sensorineural deafness. Remimazolam is rapidly degraded by carboxylesterases in the liver and its metabolites have negligible pharmacological activity.
Recently, remimazolam, an ultrashort-acting benzodiazepine, was approved for clinical use in Japan and is now available. The appropriate agents to use remain controversial. However, clinical trials of anesthetic agents in adult mitochondrial myopathy patients have not been performed. Systemic anesthetic management of patients with mitochondrial disease requires careful preoperative preparation to administer adequate anesthesia and address potential disease-related complications. Examples include ataxia, hearing loss, convulsions, cognitive decline, cardiomyopathy, myocardial conduction disorders, limb weakness, ophthalmoplegia, and diabetes. Disease symptoms range from mild to severe and may involve multiple organs. Cellular energy production is therefore disordered and organs with high energy demand are most affected. Mitochondrial diseases are caused by abnormalities in mitochondrial or nuclear genes that result in abnormal mitochondrial morphology or function. Remimazolam administration and reversal with flumazenil were successfully used for general anesthesia in a patient with mitochondrial disease. At the end of surgery, flumazenil was administered to antagonize sedation, which rapidly resulted in consciousness. Neither lactic acidosis nor prolonged muscle relaxation occurred in the perioperative period. Infusions of intravenous remimazolam and remifentanil guided by patient state index monitoring were used for anesthesia induction and maintenance. Case presentationĪ 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. The appropriate general anesthetic agents to use in these patients remain controversial.
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