AB271. SOH26AB_0103. Anaesthetic management of a patient with myofibrillar myopathy undergoing carotid endarterectomy: a case report

Background: Myofibrillar myopathies are rare neuromuscular disorders associated with progressive muscle weakness, respiratory compromise, and potential cardiac involvement. These conditions carry significant anaesthetic risk, particularly in the context of major vascular surgery, due to susceptibility to rhabdomyolysis, arrhythmias, and malignant hyperthermia-like reactions when exposed to volatile anaesthetic agents or depolarising neuromuscular blocking agents.

Case Description: We present the anaesthetic management of a patient with myofibrillar myopathy undergoing elective carotid endarterectomy following a recent left frontal lobe ischaemic stroke. Preoperative planning involved extensive multidisciplinary discussion between anaesthesia and vascular surgical team members. Preoperative assessment of the patient found dysphagia and mild restrictive lung disease but normal cardiac function. Inhalational agents and suxamethonium were excluded from the theatre to eliminate known triggers. General anaesthesia was induced and maintained using total intravenous anaesthesia (TIVA) with propofol and remifentanil infusions. Intraoperative monitoring included invasive arterial blood pressure and bispectral index. A phenylephrine infusion was titrated to maintain systolic blood pressure within 20% of baseline to ensure adequate cerebral perfusion during carotid cross-clamping. Anaesthesia was delivered safely with no intraoperative haemodynamic instability, arrhythmias, or desaturation. Neuromuscular recovery was confirmed before extubation. The patient was managed postoperatively in a high-dependency unit with close monitoring for early detection of respiratory compromise, rhabdomyolysis, or cardiac arrhythmia. No complications were observed.

Conclusions: This case illustrates the importance of meticulous anaesthetic planning for patients with neuromuscular disorders. Avoidance of volatile agents with TIVA providing a safe, controllable alternative, as well as multidisciplinary collaboration and vigilant perioperative monitoring, were instrumental in optimising outcomes in this high-risk patient.

Keywords: Carotid endarterectomy; malignant hyperthermia; rhabdomyolysis; total intravenous anaesthesia (TIVA); case report