European Neuromuscular Centre consensus statement on anaesthesia in patients with neuromuscular disorders

Luuk R van den Bersselaar; Luc Heytens; Helga C A Silva; Jens Reimann; Giorgio Tasca; Óscar Díaz-Cambronero; Nicoline Løkken; Anna Hellblom; Philip M Hopkins; Henrik Rueffert; Börge Bastian; Juan Jesus Vilchez; Robyn Gillies; Stephan Johannsen; Francis Veyckemans; Tino Muenster; Andrea Klein; Ron Litman; Heinz Jungbluth; Sheila Riazi; Nicol C Voermans; Marc M J Snoeck

doi:10.1111/ene.15526

European Neuromuscular Centre consensus statement on anaesthesia in patients with neuromuscular disorders

Eur J Neurol. 2022 Dec;29(12):3486-3507. doi: 10.1111/ene.15526. Epub 2022 Sep 14.

Authors

Luuk R van den Bersselaar^{1

2}, Luc Heytens³, Helga C A Silva⁴, Jens Reimann⁵, Giorgio Tasca⁶, Óscar Díaz-Cambronero⁷, Nicoline Løkken⁸, Anna Hellblom⁹, Philip M Hopkins¹⁰, Henrik Rueffert¹¹, Börge Bastian¹¹, Juan Jesus Vilchez¹², Robyn Gillies¹³, Stephan Johannsen¹⁴, Francis Veyckemans¹⁵, Tino Muenster¹⁶, Andrea Klein^{17

18}, Ron Litman¹⁹, Heinz Jungbluth^{20

21}, Sheila Riazi²², Nicol C Voermans², Marc M J Snoeck¹

Affiliations

¹ Malignant Hyperthermia Investigation Unit, Department of Anaesthesiology, Canisius Wilhelmina Hospital Nijmegen, Nijmegen, The Netherlands.
² Department of Neurology, Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, The Netherlands.
³ Malignant Hyperthermia Research Unit, Departments of Anaesthesiology and Neurology, University Hospital Antwerp, University of Antwerp and Born Bunge Institute, Antwerp, Belgium.
⁴ Malignant Hyperthermia Unit, Department of Surgery, Discipline of Anaesthesia, Pain, and Intensive Care, São Paulo Federal University, São Paulo, Brazil.
⁵ Department of Neurology, University of Bonn Medical Centre, Bonn, Germany.
⁶ UOC of Neurology, A. Gemelli University Polyclinic Foundation, Scientific Institute for Research and Health Care, Rome, Italy.
⁷ Malignant Hyperthermia Unit, Department of Anaesthesiology, Perioperative Medicine Research Group, La Fe University and Polytechnic Hospital, Valencia, Spain.
⁸ Copenhagen Neuromuscular Centre, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.
⁹ Department of Intensive and Perioperative Care, Skåne University Hospital Lund, Lund, Sweden.
¹⁰ Leeds Institute of Medical Research at St James's, University of Leeds and Malignant Hyperthermia Investigation Unit, St James's University Hospital, Leeds, UK.
¹¹ Schkeuditz Helios Clinic, Malignant Hyperthermia Investigation Unit, Department of Anaesthesiology, Intensive Care, Pain Therapy, University Hospital Leipzig, Leipzig, Germany.
¹² Neuromuscular Centre, La Fe Hospital UIP and ERN EURO-NMD, Neuromuscular Research Group at La Fe IIS and CIBERER, Valencia, Spain.
¹³ Malignant Hyperthermia Diagnostic Unit, Department of Anaesthesia and Pain Management, Royal Melbourne Hospital, Parkville, Victoria, Australia.
¹⁴ Department of Anaesthesiology, Intensive Care, Emergency, and Pain Medicine, Centre for Malignant Hyperthermia, University Hospital Würzburg, Würzburg, Germany.
¹⁵ Paediatric Anaesthesia Clinic, Jeanne de Flandre Hospital, Lille University Hospital Centre, Lille, France.
¹⁶ Department of Anaesthesia and Intensive Care Medicine, Hospital of the Order of St John of God, Regensburg, Germany.
¹⁷ Department of Paediatric Neurology, University Children's Hospital UKBB, Basel, Switzerland.
¹⁸ Division of Neuropaediatrics, Development, and Rehabilitation, Department of Paediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
¹⁹ Department of Anaesthesiology and Critical Care, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
²⁰ Department of Paediatric Neurology, Neuromuscular Service, Evelina's Children Hospital, Guy's and St Thomas' Hospital National Health Service Foundation Trust, London, UK.
²¹ Randall Centre for Cell and Molecular Biophysics, Muscle Signalling Section, Faculty of Life Sciences and Medicine, King's College London, London, UK.
²² Malignant Hyperthermia Investigation Unit, Department of Anaesthesiology and Pain Medicine, University Health Network, University of Toronto, Toronto, Ontario, Canada.

Abstract

Background and purpose: Patients with neuromuscular conditions are at increased risk of suffering perioperative 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, the main findings of which 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 AGREE II (Appraisal of Guidelines for Research & Evaluation) reporting checklist. The level of evidence has been adapted according to the SIGN (Scottish Intercollegiate Guidelines Network) 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 (i) neuromuscular junction disorders, (ii) muscle channelopathies (nondystrophic myotonia and periodic paralysis), (iii) myotonic dystrophy (types 1 and 2), (iv) muscular dystrophies, (v) congenital myopathies and congenital dystrophies, and (vi) mitochondrial and metabolic myopathies.

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

Keywords: anaesthesia; malignant hyperthermia; myopathy; neuromuscular disorders; perioperative care.

MeSH terms

Anesthesia*
Anesthetics*
Child
Humans
Muscular Diseases*
Neuromuscular Diseases*
Neuromuscular Junction Diseases*

Substances

Anesthetics