Mapping the current evidence on the anesthetic management of adult patients with neuromuscular disorders-a scoping review.

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.

RéSUMé: OBJECTIF: Les patients atteints de maladies neuromusculaires (MNM) courent un risque accru de développer des complications périopératoires. L'objectif de cette étude de portée est de résumer les connaissances émergentes tirées des études, présentations de cas et comptes rendus publiés portant sur l'anesthésie des patients atteints de MNM, tout en suivant le cadre méthodologique d'une étude de portée. CONSTATATIONS PRINCIPALES: ont été incluses trois études prospectives et 21 études rétrospectives comprenant des patients atteints de MNM chez lesquels les myorelaxants ont eu des propriétés pharmacocinétiques et pharmacodynamiques modifiées. En outre, 168 présentations / séries de cas portant sur 212 gestes d'anesthésie chez 197 patients ont été incluses. Ces études ont démontré qu'un suivi neuromusculaire peut être utilisé en pré-anesthésie pour ajuster les doses de myorelaxant chez les patients atteints de myasthénie grave. En postopératoire, un taux plus faible de crises de myasthénie grave a été observé avec le sugammadex. Aucune relation entre les anesthésiques et les complications périopératoires n'a été détectée. Dans les présentations / séries de cas, les patients ayant eu au moins une complication représentaient 67 (32 %) des cas. L'admission non programmée en réanimation est une complication fréquemment rapportée. Les patients dont la maladie s'est dégradée plus rapidement ont possiblement reçu des doses plus fortes de succinylcholine (risque relatif non ajusté 0,13, intervalle de confiance [IC] 95 %, 0,20 à 0,86) et d'agents volatils (rapport de cotes [RC] ajusté, 0,38 (IC 95 %, 0,20 à 0,73), P = 0.004). SOURCES: Les articles sont issus des bases de données PubMed et EMBASE (articles publiés entre le 1er janvier 2000 et le 14 juillet 2021). CONCLUSION: Les données probantes sur la prise en charge anesthésique et les complications périopératoires affectant les patients atteints de MNM sont principalement fondées sur de petites études rétrospectives et des cas cliniques. Des études cliniques ou rétrospectives d'envergure sont nécessaires pour orienter le choix de la technique d'anesthésie optimale. Les principaux domaines d'intérêt sont les bienfaits potentiels du monitorage neuromusculaire et de l'utilisation de sugammadex ainsi que les effets indésirables possibles des anesthésiques volatils et de la succinylcholine.

Anaesthesia and neuromuscular disorders: what a neurologist needs to know.

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.

Cardiac involvement in two rare neuromuscular diseases: LAMA2-related muscular dystrophy and SELENON-related myopathy.

LAMA2-related muscular dystrophy (LAMA2-MD) and SELENON(SEPN1)-related myopathy (SELENON-RM) are rare neuromuscular diseases caused by mutations in the LAMA2 and SELENON (SEPN1) gene, respectively. Systematic reviews on cardiac features in both neuromuscular diseases are lacking. This scoping review aims to elucidate the cardiac involvement in LAMA2-MD or SELENON-RM. Three electronic databases (PubMed, Embase and Cochrane) were searched. All studies, case reports and case series with information on cardiac features in LAMA2-MD or SELENON-RM patients were included. Study selection and data extraction were performed by two independent reviewers. 31 Articles on LAMA2-MD and 17 articles on SELENON-RM met the inclusion criteria, resulting in the inclusion of 131 LAMA2-MD and 192 SELENON-RM cases. In 41% of LAMA2-RM cases, a cardiac abnormality was present. Left ventricular systolic dysfunction and arrhythmia were most frequently described. In 15% of SELENON-RM cases, a cardiac abnormality was reported, of which pulmonary hypertension, including right ventricular dysfunction secondary to pulmonary failure, was most prevalent. We conclude that in LAMA2-MD primary left ventricular dysfunction and in SELENON-RM secondary right ventricular dysfunction are frequently reported. Optimal cardiorespiratory surveillance by screening of asymptomatic patients every two years with ECG, Holter and echocardiography is necessary for early detection and/or treatment of cardiac manifestations.