Diagnosis and treatment of postoperative malignant hyperthermia in a pediatric patient with congenital myopathy due to RyR1 gene mutations
10.3760/cma.j.cn131073.20210320.01112
- VernacularTitle:RyR1基因突变先天性肌肉病患儿术后恶性高热诊疗案例
- Author:
Bin HAN
1
;
Zhengqian LI
;
Yang ZHOU
;
Jing ZHANG
;
Yanbin ZHAO
;
Chao LI
;
Xiaoxiao LI
;
Jun WANG
;
Yu SUN
;
Min YI
;
Yingshuang ZHANG
;
Li YANG
;
Xiangyang GUO
Author Information
1. 北京大学第三医院麻醉科 100191
- Keywords:
Malignant hyperthermia;
Postoperative complications;
Child;
Ryanodine receptor calcium release channel;
Mutation;
Myopathies, structural, congenital
- From:
Chinese Journal of Anesthesiology
2021;41(11):1330-1333
- CountryChina
- Language:Chinese
-
Abstract:
A 14-year-old male pediatric patient was admitted to the hospital mainly because of neck and back deformity, with limited activity for 7 yr, dysphagia and short of breath for more than 10 months.He was diagnosed with cervical lordosis deformity, RyR1 gene-related myopathy, high possibility of multi-minicore disease and being susceptible to malignant hyperthermia.Posterior cervical orthopedic internal fixation surgery was successfully performed under total intravenous anesthesia with propofol.The vital signs were stable during anesthesia and operation which lasted for 10 h. The patient was admitted to intensive care unit after the uneventful operation.When emerging from general anesthesia, the patient suddenly presented with symptoms of muscular fasciculation in the head, face, trunk and limbs, along with elevated body temperature as high as 39.4℃, severe acidosis and hypercapnia, meanwhile, the blood creatine kinase, blood myoglobin and urinary myoglobin gradually increased.The patient was diagnosed with malignant hyperthermia based on the clinical grading scale score of 63.Dantrolene sodium was infused intravenously, combined with multiple treatments such as physical cooling, correction of acidosis and electrolyte disturbance, alkalization of urine, intermittent hemofiltration and plasma exchange.The arrhythmia and delirium were treated symptomatically.The pediatric patient was fully recovered and discharged with good outcomes.
