A patient with neuromuscular disease can’t be anesthetized like others, agreed. But what are the best practices? A Dutch team brought them together in an article published in October 2020. This publication details each step (preoperative assessment, premedication, etc.) and their risks. It also provides specific instructions by disease or group of diseases (including unlabeled myopathies) and by anesthetic agent. The authors have chosen to highlight four key concepts:
- patients with neuromuscular disease may have cardiac and / or pulmonary involvement, which increases the risk of perioperative complications and justifies a multidisciplinary approach involving anesthesiologist, cardiologist, pulmonologist, surgeon and neurologist;
- succinylcholine should be avoided in these patients as it may exacerbate pre-existing muscle weakness and cause potentially fatal hyperkalaemia;
- volatile anesthetic agents and succinylcholine can cause malignant hyperthermia in patients with mutations in the RYR1 gene (central core myopathy, autosomal centronuclear myopathy, etc.) and less often in those carrying mutations in the CACNA1S gene ( normokalemic or hypokalaemic periodic paralysis, or Westphal’s disease ) or of the STAC3 gene (new congenital myopathy also associated with dysmorphia);
- the doses of non-depolarizing muscle relaxants, sedatives and opioids should be reduced and their muscle relaxant effect closely monitored because their effect and duration of action are greater in most patients with neuromuscular disease.