Review
Complications of anaesthesia in neuromuscular disorders

https://doi.org/10.1016/j.nmd.2004.10.017Get rights and content

Abstract

The purpose of this review is to alert non-anaesthesiologists to the various complications from which patients with neuromuscular disorders and those susceptible to malignant hyperthermia can suffer during anaesthesia. The patient's outcome correlates with the quality of consultation between anaesthesiologists, surgeons, neurologists and cardiologists. Special precautions must be taken, since many anaesthetics and muscle relaxants can aggravate the clinical features or trigger life-threatening reactions. Complications frequently occur in these patients, although anaesthetic procedures have become safer by the reduced administration of suxamethonium and the use of total intravenous anaesthesia, new volatile anaesthetics and non-depolarising relaxants. This review provides a synopsis of pre-operative anaesthetic considerations and adverse drug effects on skeletal, cardiac and smooth muscle tissue. It describes the pathogenetic aspects of typical complications and introduces anaesthetic procedures for the various neuromuscular disorders, including regional anaesthesia for patients in whom a restriction of respiratory and/or cardiac function is predicted.

Introduction

Anaesthesia in patients with neuromuscular diseases is a concern for anaesthesiologists, surgeons, neurologists, pediatricians, cardiologists, pulmonologists and sometimes also for geneticists. It is desirable to discuss with the patient and family members the risks and benefits of the various treatment options. Often the anesthesiologist is not left with a single absolute risk, but in many cases must balance conflicting management strategies, fully bearing in mind the possible deleterious outcomes even with the chosen course of action. In order to reduce patient risk to a minimum, pre-operative considerations in respect of these circumstances and a precise diagnosis (often per biopsy) in advance are essential. It is also important to identify and treat potential anaesthetic complications promptly. Lastly, adverse drug effects or specific risks associated with certain neuromuscular diseases should be taken into account.

This review is an overview of these elements. It deals with the pre-operative anaesthetic considerations, the typical anaesthesia-related exacerbation of skeletal, cardiac and smooth muscle weakness resulting in respiratory distress, cardiac complications and autonomic dysregulation. Adverse drug effects such as rhabdomyolysis, muscle spasms, malignant hyperthermia and similar reactions are discussed with respect to their pathogenesis. Afterwards, typical anaesthetic complications and their prevention and management are described for specific neuromuscular disorders: motoneuron diseases, peripheral neuropathies, neuromuscular transmission disorders, progressive muscular dystrophies, metabolic and mitochondrial myopathies, myotonias and periodic paralyses, and congenital myopathies (Table 1). Lastly, areas are indicated for non-anaesthesiologists to turn to, for more insight into the care of their patients. Further details may be found in [1].

Section snippets

Pre-operative considerations

The most obvious pre-operative question is whether the benefit of surgery justifies the anaesthetic risk. Decisions are based on a clear diagnosis with a full workup including histopathological findings, genetic data, or at least family history, and a wider variety of metabolic tests. Since patients with neuromuscular diseases are very challenging and may appear deceptively healthy, it is important to document all choices and their rationale on the medical record for subsequent care-givers,

Rhabdomyolysis

Depolarizing muscle relaxants such as suxamethonium can lead to rhabdomyolysis in almost all neuromuscular disease, but especially in muscle that is denervated, progressively dystrophic or metabolically altered [9], [10]. Suxamethonium activates nicotinic acetylcholine receptors. Therefore, either an ectopic increase of nicotinic acetylcholine receptors extrajunctionally as in denervated or immobilized muscle [11] or an increase of the fetal γ isoforms as in many neuromuscular diseases [12]

Motoneuron diseases

This group of mainly degenerative, hereditary and rarely infectious origin (e.g. post-polio syndrome) encompasses impairment of the motor nervous without affecting the sensory system. Depending on whether the upper, or, as in spinal muscular atrophy, only the lower motor neuron is affected, the main clinical feature is spasticity or atrophy, or a combination of both as in amyotrophic lateral sclerosis. Loss of innervation ultimately leads to muscle atrophy with extra-junctional and

Anaesthesia in surgical emergency

Even in a surgical emergency it is often possible for the anaesthesiologist to search the OMIM database (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=OMIM) to get a regularly updated, concise and reliable summary on the disease in the shortest possible time. With this information the anaesthesiologist is at least alerted to adverse reactions during anaesthesia. Another key point is registration with MedicAlert or a similar service, and use of the bracelets, necklaces etc. as a life-saving

Conclusions

Anaesthetic procedures in neuromuscular disorders have become safer. The reduced administration of suxamethonium as a muscle relaxant has led to a decreased incidence of hyperkalaemic induced cardiac arrest in patients with pre-symptomatic muscular dystrophies. Total intravenous anaesthesia using newer short-acting anaesthetic agents, opioids and non-depolarising muscle relaxants, as well as the more recently introduced volatile anaesthetics sevoflurane and desflurane, accelerate recovery and

Acknowledgements

We thank the two referees of Neuromuscular Disorders for their very valuable suggestions. This work was supported by the German Research Foundation (DFG) (JU470/1) and the network on Excitation-contraction coupling and calcium signaling in health and disease of the IHP Program funded by the European Community.

References (113)

  • R.J. Telford et al.

    The myasthenic syndrome: anaesthesia in a patient treated with 3.4 diaminopyridine

    Br J Anaesth

    (1990)
  • E.M. Hoogerwaard et al.

    Cardiac involvement in carriers of Duchenne and Becker muscular dystrophy

    Neuromuscul Disord

    (1999)
  • J. Forst et al.

    Platelet function deficiency in Duchenne muscular dystrophy

    Neuromuscul Disord

    (1998)
  • L. Grain et al.

    Cardiac abnormalities and skeletal muscle weakness in carriers of Duchenne and Becker muscular dystrophies and controls

    Neuromuscul Disord

    (2001)
  • M.A. Nolan et al.

    Cardiac assessment in childhood carriers of Duchenne and Becker muscular dystrophies

    Neuromuscul Disord

    (2003)
  • H. Speedy

    Exaggerated physiological responses to propofol in myotonic dystrophy

    Br J Anaesth

    (1990)
  • Y. Tzabar et al.

    Myotonic dystrophy and target-controlled propofol infusions

    Br J Anaesth

    (1995)
  • K. Talbot et al.

    Reduction in excess daytime sleepiness by modafinil in patients with myotonic dystrophy

    Neuromuscul Disord

    (2003)
  • M. Funakoshi et al.

    Emerin and cardiomyopathy in Emery–Dreifuss muscular dystrophy

    Neuromuscul Disord

    (1999)
  • J.M. Moundras et al.

    Anesthetic management of obstetrical labor in a parturient with muscular carnitine palmitoyl transferase deficiency

    Ann Fr Anesth Reanim

    (2000)
  • S.H. Russell et al.

    Anaesthesia and myotonia

    Br J Anaesth

    (1994)
  • J.D. Miller et al.

    Muscle diseases

  • M.I. Polkey et al.

    Respiratory aspects of neurological disease

    J Neurol Neurosurg Psychiatry

    (1999)
  • J. Finsterer et al.

    The heart in human dystrophinopathies

    Cardiology

    (2003)
  • P.R. Hambly et al.

    Anaesthesia for chronic spinal cord lesions

    Anaesthesia

    (1998)
  • R.F. Grace et al.

    Caesarean section in a patient with paramyotonia congenital

    Anaesth Intensive Care

    (1999)
  • R. Mann et al.

    Preanesthetic train-of-four fade predicts the atracurium requirement of myasthenia gravis patients

    Anesthesiology

    (2000)
  • J.W. Albers et al.

    Neuroanesthesia and neuromuscular diseases

  • S.N. Piper et al.

    Nefopam and clonidine in the prevention of postanaesthetic shivering

    Anaesthesia

    (1999)
  • J.A. Martyn et al.

    Up-and-down regulation of skeletal muscle acetylcholine receptors. Effects on neuromuscular blockers

    Anesthesiology

    (1992)
  • E. Breucking et al.

    Anesthetic complications. The incidence of severe anesthetic complications in patients and families with progressive muscular dystrophy of the Duchenne and Becker types

    Anaesthesist

    (2000)
  • S.M. Yentis

    Suxamethonium and hyperkalaemia

    Anaesth Intensive Care

    (1990)
  • S. Gattenlohner et al.

    Expression of foetal type acetylcholine receptor is restricted to type 1 muscle fibres in human neuromuscular disorders

    Brain

    (2002)
  • W. Klingler et al.

    Deregulation of skeletal muscle Ca2+ homoeostasis is facilitated by ecstasy

    Pflügers Arch—Eur J Physiol

    (2000)
  • J.T. Chang et al.

    Rhabdomyolysis with HMG-CoA reductase inhibitors and gemfibrozil combination therapy

    Pharmacoepidemiol Drug Saf

    (2004)
  • F. Loiodice et al.

    Carboxylic acids and chloride conductance in skeletal muscle: influence on the pharmacological activity induced by the chain substituents and the distance between the phenolic group and the carboxylic function in 4-chloro-phenoxy alkanoic acids

    Farmaco

    (1993)
  • Y. Sonoda et al.

    Electrical myotonia of rabbit skeletal muscles by HMG-CoA reductase inhibitors

    Muscle Nerve

    (1994)
  • M. Evans et al.

    Effects of HMG-CoA reductase inhibitors on skeletal muscle: all statins the same?

    Drug Saf

    (2002)
  • S. Shibata

    A drug over the millennia: pharmacognosy, chemistry, and pharmacology of licorice

    Yakugaku Zasshi

    (2000)
  • R. Vanholder et al.

    Rhabdomyolysis

    J Am Soc Nephrol

    (2000)
  • K.J. Dar et al.

    MDMA induced hyperthermia: report of a fatality and review of current therapy

    Intensive Care Med

    (1996)
  • R. Obata et al.

    Rhabdomyolysis in association with Duchenne's muscular dystrophy

    Can J Anaesth

    (1999)
  • G.A. Gronert et al.

    Malignant hyperthermia

  • J.M. Sauret et al.

    Rhabdomyolysis

    Am Fam Physician

    (2002)
  • G.F. Cox et al.

    Dystrophies and heart disease

    Curr Opin Cardiol

    (1997)
  • P.J. Hanley et al.

    Mechanisms of force inhibition by halothane and isoflurane in intact rat cardiac muscle

    J Physiol

    (1998)
  • R. Huneke et al.

    Effects of volatile anesthetics on cardiac ion channels

    Acta Anaesthesiol Scand

    (2004)
  • Y. Ishikawa et al.

    Cardiovascular considerations in the management of neuromuscular disease

    Semin Neurol

    (1995)
  • H.J. Trappe et al.

    Arrhythmias in the intensive care patient

    Curr Opin Crit Care

    (2003)
  • F. Lehmann-Horn et al.

    Chapter 46: nondystrophic myotonias and periodic paralyses

    • Cited by (102)

    • Identification and Management of Acute Neuromuscular Respiratory Failure in the ICU

      2023, Chest

    • Perioperative Management of Patients With Neuromuscular Disorders

      2021, Neuromuscular Disorders: Treatment and Management

    • Managing pregnancy and anaesthetics in patients with skeletal muscle channelopathies

      2020, Neuromuscular Disorders

    • Essentials of Neurology and Neuromuscular Disorders

      2019, A Practice of Anesthesia for Infants and Children

    • Pregnancy outcomes in women with spinal muscular atrophy: A review

      2018, Journal of the Neurological Sciences
      Citation Excerpt :

      However, there are no specific guidelines about the use of anesthetic agents in this condition. Halogenated agents can be used safely in motor neuron diseases [29,37], while intravenous agents (propofol, thiopental) and opioids have the potential to cause respiratory and cardiac depression, therefore lower doses and careful titration to effect should be used [28]. Postoperative weakness and respiratory failure has been reported as possible complications, thus ventilation and ICU admission might be required.

    • Essentials of Neurology and Neuromuscular Disorders

      2018, A Practice of Anesthesia for Infants and Children
    View all citing articles on Scopus
    View full text
    Copyright © 2004 Elsevier B.V. All rights reserved.