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Background <p>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.</p> Case presentation <p>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.</p> Conclusion <p>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.</p>

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

  • 期刊论文

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  • 发表日期：

    2025年4月23日

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  • Kailai Zhu,
  • Shuangwei Wu,
  • Xianglin Hao,
  • Chuanguang Wang

摘要

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.