Successful Deep Sedation with Remimazolam and Alfentanil in a Child Susceptible to Malignant Hyperthermia: A Case Report

Background Malignant hyperthermia (MH) is a life-threatening autosomal-dominant disorder caused by mutations in the ryanodine receptor 1 (RYR1) gene, leading to calcium dysregulation in skeletal muscle. Patients with genetically confirmed MH susceptibility must strictly avoid volatile anesthetics and succinylcholine. Intravenous deep sedation presents a viable alternative, yet evidence supporting remimazolam use in pediatric MH patients remains scarce. Case presentation We report the first case of a 1-year-old male patient with genetically confirmed MH susceptibility undergoing orchidopexy under remimazolam-alfentanil deep sedation combined with caudal block. The patient had no MH manifestations intraoperatively or postoperatively and recovered uneventfully. Conclusion This case demonstrates the feasibility of remimazolam-based deep sedation in genetically confirmed pediatric MH patients, supporting its safety profile in this population. Further multicenter studies are needed to establish standardized protocols.