The European Neuromuscular Centre Consensus Statement on Anaesthesia in Patients with Neuromuscular Disorders.

van den Bersselaar, Luuk R; Heytens, Luc; Silva, Helga C A; Reimann, Jens; Tasca, Giorgio; Díaz-Cambronero, Óscar; Løkken, Nicoline; Hellblom, Anna; Hopkins, Philip M; Rueffert, Henrik; Bastian, Börge; Vilchez, Juan Jesus; Gillies, Robyn; Johannsen, Stephan; Veyckemans, Francis; Muenster, Tino; Klein, Andrea; Litman, Ron; Jungbluth, Heinz; Riazi, Sheila; ... (2022). The European Neuromuscular Centre Consensus Statement on Anaesthesia in Patients with Neuromuscular Disorders. European journal of neurology, 29(12), pp. 3486-3507. Wiley 10.1111/ene.15526

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BACKGROUND

Patients with neuromuscular conditions are at increased risk of suffering peri-operative complications related to anaesthesia. There is currently little specific anaesthetic guidance concerning these patients. Here we present the European Neuromuscular Centre (ENMC) consensus statement on anaesthesia in patients with neuromuscular disorders as formulated during the 259th ENMC workshop on Anaesthesia in neuromuscular disorders.

METHODS

International experts in the field of (paediatric) anaesthesia, neurology and genetics were invited to participate in the ENMC workshop. A literature search was conducted in PubMed and EMBASE whose main findings were disseminated to the participants and presented during the workshop. Depending on specific expertise, participants presented the existing evidence and their expert opinion concerning anaesthetic management in six specific groups of myopathies and neuromuscular junction disorders. The consensus statement was prepared according to the Appraisal of Guidelines for REsearch & Evaluation (AGREE II) reporting checklist. The level of evidence has been adapted according to the Scottish Intercollegiate Guidelines Network (SIGN) grading system. The final consensus statement was subjected to a modified Delphi process.

RESULTS

A set of general recommendations valid for the anaesthetic management of patients with neuromuscular disorders in general have been formulated. Specific recommendations were formulated for 1) neuromuscular junction disorders; 2) muscle channelopathies (non-dystrophic myotonia and periodic paralysis); 3) myotonic dystrophy (type 1 and 2); 4) muscular dystrophies; 5) congenital myopathies and congenital dystrophies and 6) mitochondrial and metabolic myopathies.

CONCLUSION

This ENMC consensus statement summarizes the most important considerations for planning and performing anaesthesia in patients with neuromuscular disorders.

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