Anesthetic Management of A Patient with Charcot-Marie-Tooth Disease for 2-stage Revision of Total Knee Replacement.

ABSTRACT

Charcot-Marie-Tooth disease is characterised by hereditary motor and sensory neuropathy. Its anaesthetic management is challenging owing to the unpredictable response observed in patients, especially to non-depolarising neuromuscular blocking drugs, and the risk of malignant hyperthermia and cardiorespiratory complications. A 66-year-old woman underwent anaesthesia for 2 different surgical procedures, a 2-stage revision of total knee replacement over a 4-month period. She presented with severe anatomic disorders, accompanied by severe motor and sensory impairment. An anaesthetic plan without neuromuscular blocking drugs or volatile anaesthetics, using a clean ventilator, with dantrolene available, was successfully used both times. There were no complications during the administration of general anaesthesia or postoperatively at the post-anaesthesia care unit, and the patient did not complain of pain at any time. General anaesthesia with a careful selection of anaesthetic drugs proved to be a safe option for the management of a patient with Charcot-Marie-Tooth disease.