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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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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author Kailai Zhu
Shuangwei Wu
Xianglin Hao
Chuanguang Wang
author_facet Kailai Zhu
Shuangwei Wu
Xianglin Hao
Chuanguang Wang
author_sort Kailai Zhu
collection DOAJ
description 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.
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series BMC Anesthesiology
spelling doaj-art-0e20dd0827344b709c6bfe8a06ddbe3c2025-08-20T03:53:32ZengBMCBMC Anesthesiology1471-22532025-04-012511410.1186/s12871-025-03076-0Successful sedation with remimazolam and alfentanil in a child susceptible to malignant hyperthermia: a case reportKailai Zhu0Shuangwei Wu1Xianglin Hao2Chuanguang Wang3Department of Anesthesia, The Fifth Affiliated Hospital of Wenzhou Medical University, Lishui Municipal Central HospitalDepartment of Anesthesia, The Fifth Affiliated Hospital of Wenzhou Medical University, Lishui Municipal Central HospitalDepartment of Anesthesia, The Fifth Affiliated Hospital of Wenzhou Medical University, Lishui Municipal Central HospitalDepartment of Anesthesia, The Fifth Affiliated Hospital of Wenzhou Medical University, Lishui Municipal Central HospitalAbstract 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.https://doi.org/10.1186/s12871-025-03076-0Malignant hyperthermiaRemimazolamPediatric anesthesiaRYR1 mutationSedation
spellingShingle Kailai Zhu
Shuangwei Wu
Xianglin Hao
Chuanguang Wang
Successful sedation with remimazolam and alfentanil in a child susceptible to malignant hyperthermia: a case report
BMC Anesthesiology
Malignant hyperthermia
Remimazolam
Pediatric anesthesia
RYR1 mutation
Sedation
title Successful sedation with remimazolam and alfentanil in a child susceptible to malignant hyperthermia: a case report
title_full Successful sedation with remimazolam and alfentanil in a child susceptible to malignant hyperthermia: a case report
title_fullStr Successful sedation with remimazolam and alfentanil in a child susceptible to malignant hyperthermia: a case report
title_full_unstemmed Successful sedation with remimazolam and alfentanil in a child susceptible to malignant hyperthermia: a case report
title_short Successful sedation with remimazolam and alfentanil in a child susceptible to malignant hyperthermia: a case report
title_sort successful sedation with remimazolam and alfentanil in a child susceptible to malignant hyperthermia a case report
topic Malignant hyperthermia
Remimazolam
Pediatric anesthesia
RYR1 mutation
Sedation
url https://doi.org/10.1186/s12871-025-03076-0
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AT shuangweiwu successfulsedationwithremimazolamandalfentanilinachildsusceptibletomalignanthyperthermiaacasereport
AT xianglinhao successfulsedationwithremimazolamandalfentanilinachildsusceptibletomalignanthyperthermiaacasereport
AT chuanguangwang successfulsedationwithremimazolamandalfentanilinachildsusceptibletomalignanthyperthermiaacasereport