Neurologists are often asked for specific advice regarding patients with neuromuscular disease who require general anaesthesia. However, guidelines on specific neuromuscular disorders do not usually include specific guidelines or pragmatic advice regarding (regional and/or general) anaesthesia or procedural sedation. Furthermore, the medical literature on this subject is mostly limited to publications in anaesthesiology journals. We therefore summarise general recommendations and specific advice for anaesthesia in different neuromuscular disorders to provide a comprehensive and accessible overview of the knowledge on this topic essential for clinical neurologists. A preoperative multidisciplinary approach involving anaesthesiologists, cardiologists, chest physicians, surgeons and neurologists is crucial. Depolarising muscle relaxants (succinylcholine) should be avoided at all times. The dose of non-depolarising muscle relaxants must be reduced and their effect monitored. Patients with specific mutations in RYR1 (ryanodine receptor 1) and less frequently in CACNA1S (calcium channel, voltage-dependent, L type, alpha 1S subunit) and STAC3 (SH3 and cysteine rich domain 3) are at risk of developing a life-threatening malignant hyperthermia reaction.

Purpose Patients with neuromuscular disorders (NMDs) are at increased risk of perioperative complications. The objective of this scoping review was to examine emerging evidence from published studies, case reports, and review articles on anesthetic management of patients with NMDs, following the methodological frame for scoping reviews. Sources We searched PubMed and EMBASE for articles published between 1 January 2000 and 14 July 2021. Principal findings Three prospective and 21 retrospective studies on altered pharmacokinetics and pharmacodynamics of neuromuscular blocking agents (NMBA) in NMD patients were included. Furthermore, 168 case reports/series reporting 212 anesthetics in 197 patients were included. These studies showed that preanesthetic neuromuscular monitoring can be used for precise NMBA dosing in myasthenia gravis patients. Sugammadex was associated with fewer postoperative myasthenic crises. Perioperative complications were not associated with specific anesthetic agents. Case reports/series showed that in 32% (67/212) of anesthetics, at least one complication was reported. Unexpected intensive care unit admission was a frequently reported complication. Patients with a complicated disease course may have had a higher use of succinylcholine (unadjusted relative risk, 0.13; 95% confidence interval [CI], 0.20 to 0.86) and volatile anesthetics (adjusted odds ratio [OR], 0.38; 95% CI, 0.20 to 0.73; P = 0.004). Conclusion Evidence on the anesthetic management and perioperative complications of patients with NMDs is mainly based on small retrospective studies and case reports. Further clinical trials or large retrospective studies are required to investigate the choice of safe anesthetic agents. Main areas of interest are the potential benefits of neuromuscular monitoring and sugammadex and the risks possibly associated with volatile anesthetics and succinylcholine. Supplementary Information The online version of this article contains supplementary material available 10.1007/s12630-022-02230-3.

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Background: Patients with neuromuscular disorders are at increased risk of suffering perioperative complications. Current knowledge concerning this topic is based on small retrospective studies and expert opinion. Therefore, an individualized multidisciplinary approach to perioperative anaesthesia planning is invaluable to anticipate difficulties and to optimize outcomes. Objective: To evaluate current practice regarding preoperative counselling and perioperative care of neuromuscular patients, with the aim to facilitate standardization and improvement of perioperative care for neuromuscular patients. Methods: A questionnaire-based cross-sectional, observational study was conducted between July, 1st 2020 and December, 31st, 2020 in Dutch anaesthesia, neurology and clinical genetics departments. Main outcome measures were 1.) frequency of consultation requests for neuromuscular patients prior to surgery, 2.) current practice, educational activities and departmental approach to this topic and 3.) preoperative counselling of neuromuscular patients. Results: A total of 83 departments participated. Consultations for a neuromuscular patient scheduled for anaesthesia were requested from anaesthesia and neurology department only infrequently. Local guidelines concerning perioperative care of neuromuscular patients were available in 36.4% of the participating departments. Quality of specific training for residents and staff anaesthetists/neurologists covering perioperative care of neuromuscular patients was rated as ‘very good’ or ‘good’ by 42.9%. Neuromuscular patients scheduled for surgery were ‘always’ or ‘often’ discussed in multidisciplinary meetings involving anaesthesiologists and neurologists in 20.8% of the participating departments. Conclusion: Perioperative care for neuromuscular patients in the Netherlands is highly variable and might benefit from guidelines, education of health care professionals and multidisciplinary meetings between anaesthesiologists and neurologists on a regular basis.