Successful 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 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.