Leigh脳症患者に対してレミマゾラムを用いて麻酔管理を行った1症例

Anesthetic Management of Tracheostomy in a Pediatric Patient with Leigh Syndrome：A Case Report

要旨

　Leigh脳症はミトコンドリア病の一病型であり，悪性高熱症のリスクと肝代謝低下が懸念されるほか，痙攣と呼吸中枢の障害が加わるため，麻酔薬の選択など周術期管理で注意すべき点が多い。今回われわれは，Leigh脳症の1歳6カ月の児に対して，レミマゾラムを用いた全静脈麻酔下で気管切開術の麻酔管理を経験したため報告する。

　The heredity mitochondrial disorder Leigh syndrome involves impaired energy production and lactate metabolization. Patients with this syndrome experience a progressive neurodegenerative course, with subacute necrotizing encephalomyelopathy. Leigh syndrome usually presents in infancy with developmental delays, seizures, ataxia, dystonia, abnormalities of central control of respiration, and metabolic distraction. Patients with a mitochondrial disease such as Leigh syndrome are susceptible to propofol and volatile agents. We report the successful anesthetic management of an 18-month-old 12-kg boy with Leigh syndrome who underwent a scheduled tracheostomy. The general anesthesia was conducted with remimazolam, remifentanil, and rocuronium and was completed uneventfully.