L'hyperthermie maligne comme on ne l'attend pas

Malignant hyperthermia (MH) is a rare disease diagnosed in genetically predisposed patients, that expresses as a crisis when patients are exposed to pharmacological agents used in anesthesia and intensive care (halogenated agents and/or depolarizing curare). The symptomatology is explosive but tends to be delayed with the more frequent use of sevoflurane and the rarer use of suxamethonium. A patient undergoing esophagectomy who developed a malignant hyperthermia crisis illustrates this point. This crisis began at the end of the surgical procedure, eight hours after the start of exposure to sevoflurane. Dantrolene administration was life-saving, enabling hemodynamic stabilization and rapid correction of metabolic disorders. A genetic work-up was performed and revealed a heterozygous mutation in the RYR1 gene. The rarity of this pathology, the use of halogenated agents such as sevoflurane and desflurane, and the large number of mutations potentially responsible for malignant hyperthermia, sometimes make diagnosis difficult.