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

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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.

Abstract

This consensus statement summarizes the most important recommendations concerning anaesthesia in patients with neuromuscular disorders.

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Most cited references 102

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M. Brouwers, M E Kho, G P Browman … (2010)

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Diagnosis and management of spinal muscular atrophy: Part 1: Recommendations for diagnosis, rehabilitation, orthopedic and nutritional care

Eugenio Mercuri, Richard Finkel, Francesco Muntoni … (2018)

Spinal muscular atrophy (SMA) is a severe neuromuscular disorder due to a defect in the survival motor neuron 1 (SMN1) gene. Its incidence is approximately 1 in 11,000 live births. In 2007, an International Conference on the Standard of Care for SMA published a consensus statement on SMA standard of care that has been widely used throughout the world. Here we report a two-part update of the topics covered in the previous recommendations. In part 1 we present the methods used to achieve these recommendations, and an update on diagnosis, rehabilitation, orthopedic and spinal management; and nutritional, swallowing and gastrointestinal management. Pulmonary management, acute care, other organ involvement, ethical issues, medications, and the impact of new treatments for SMA are discussed in part 2.

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Muscular dystrophies

Eugenio Mercuri, Carsten Bönnemann, Francesco Muntoni (2019)

Muscular dystrophies are primary diseases of muscle due to mutations in more than 40 genes, which result in dystrophic changes on muscle biopsy. Now that most of the genes responsible for these conditions have been identified, it is possible to accurately diagnose them and implement subtype-specific anticipatory care, as complications such as cardiac and respiratory muscle involvement vary greatly. This development and advances in the field of supportive medicine have changed the standard of care, with an overall improvement in the clinical course, survival, and quality of life of affected individuals. The improved understanding of the pathogenesis of these diseases is being used for the development of novel therapies. In the most common form, Duchenne muscular dystrophy, a few personalised therapies have recently achieved conditional approval and many more are at advanced stages of clinical development. In this Seminar, we concentrate on clinical manifestations, molecular pathogenesis, diagnostic strategy, and therapeutic developments for this group of conditions.

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Author and article information

Contributors

Luuk R. van den Bersselaar:

ORCID: https://orcid.org/0000-0001-7735-3267

luuk.vandenbersselaar@radboudumc.nl

Journal

Journal ID (nlm-ta): Eur J Neurol

Journal ID (iso-abbrev): Eur J Neurol

Journal ID (doi): 10.1111/(ISSN)1468-1331

Journal ID (publisher-id): ENE

Title: European Journal of Neurology

Publisher: John Wiley and Sons Inc. (Hoboken )

ISSN (Print): 1351-5101

ISSN (Electronic): 1468-1331

Publication date (Electronic): 14 September 2022

Publication date (Print): December 2022

Volume: 29

Issue: 12 ( doiID: 10.1111/ene.v29.12 )

Pages: 3486-3507

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

Author notes

[*] [* ] Correspondence

Luuk R. van den Bersselaar, Malignant Hyperthermia Investigation Unit, Department of Anaesthesiology, C40‐01, Canisius Wilhelmina Hospital, Weg door Jonkerbos 100, 6532 SZ Nijmegen, the Netherlands.

Email: luuk.vandenbersselaar@ 123456radboudumc.nl

Author information

Luuk R. van den Bersselaar https://orcid.org/0000-0001-7735-3267

Jens Reimann https://orcid.org/0000-0003-3349-6877

Giorgio Tasca https://orcid.org/0000-0003-0849-9144

Juan Jesus Vilchez https://orcid.org/0000-0002-0532-2872

Heinz Jungbluth https://orcid.org/0000-0002-7159-3427

Article

Publisher ID: ENE15526 Other ID: EJoN-22-1346.R1

DOI: 10.1111/ene.15526

PMC ID: 9826444

PubMed ID: 35971866

SO-VID: 6aff8ccd-c75c-4e44-b3f2-fe32c8ec4581

License:

This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.

History

Date revision received : 05 July 2022

Date received : 01 June 2022

Date accepted : 11 August 2022

Page count

Figures: 2, Tables: 7, Pages: 22, Words: 10047

Funding

Funded by: European Neuromuscular Centre , doi 10.13039/501100011640;

Award ID: NA

Custom metadata

source-schema-version-number 2.0

cover-date December 2022

details-of-publishers-convertor Converter:WILEY_ML3GV2_TO_JATSPMC version:6.2.3 mode:remove_FC converted:08.01.2023

ScienceOpen disciplines: Neurology

Keywords: anaesthesia,malignant hyperthermia,myopathy,neuromuscular disorders,perioperative care

Data availability:

ScienceOpen disciplines: Neurology

Keywords: anaesthesia, malignant hyperthermia, myopathy, neuromuscular disorders, perioperative care

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

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Abstract

Background and purpose

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Abstract

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Karger: Neurology and Neuroscience

Most cited references 102

AGREE II: advancing guideline development, reporting and evaluation in health care.

Diagnosis and management of spinal muscular atrophy: Part 1: Recommendations for diagnosis, rehabilitation, orthopedic and nutritional care

Muscular dystrophies

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