Successful sedation with remimazolam and alfentanil in a child susceptible to malignant hyperthermia: a case report

Abstract 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 anest...

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Bibliographic Details
Main Authors: Kailai Zhu, Shuangwei Wu, Xianglin Hao, Chuanguang Wang
Format: Article
Language:English
Published: BMC 2025-04-01
Series:BMC Anesthesiology
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Online Access:https://doi.org/10.1186/s12871-025-03076-0
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Summary:Abstract 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 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 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 sedation in genetically confirmed pediatric MH patients, supporting its safety profile in this population. Further multicenter studies are needed to establish standardized protocols.
ISSN:1471-2253