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KEY POINTS

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KEY POINTS

  1. Neuromuscular disorders (NMDs) are rare, are difficult to study, and have significant anesthetic implications for perioperative management. These include diseases of the motor neurons, peripheral and autonomic nerves, neuromuscular junction, and myopathies.

  2. Patients with NMDs may have life-threatening contraindications to hydrocarbon-based volatile anesthetics and neuromuscular blocking drugs, particularly succinylcholine, with risks of prolonged muscle weakness, hyperkalemia, rhabdomyolysis, or malignant hyperthermia (MH).

  3. The use of regional anesthesia, spinal or epidural, is reported in case series in most types of NMDs. There is concern for local anesthetic neurotoxicity with demyelinating NMDs such as multiple sclerosis and Guillain-Barré disease.

  4. The leading causes of death for patients with NMDs are respiratory failure and aspiration pneumonia. Preoperative consultation with a neurologist or pulmonologist to optimize these patients, depending on severity of disease, may improve outcomes. Coordination of the perioperative care by the patient’s primary care provider should facilitate recovery.

  5. Some patients with NMDs have cardiac muscle affected, leading to dilated cardiomyopathy, arrhythmias, and valvular regurgitation. The degree of skeletal muscle involvement does not necessarily correlate with the severity of cardiac involvement.

  6. Patients with NMDs may have autonomic dysfunction with delayed gastrointestinal emptying, resting tachycardia, and orthostatic hypotension and may exhibit severe hemodynamic instability.

  7. Patients with NMDs, particularly those with advanced disease, should have a discussion of advanced directives.

  8. In patients with myasthenia gravis, the signs and symptoms of toxicity with a cholinergic crisis include salivation, hyperhidrosis, tearing, bradycardia, and miosis, and it can be difficult to differentiate from a myasthenic crisis; however, the vast majority of events are caused by a myasthenic crisis.

  9. The presenting signs of Duchenne muscular dystrophy (MD) in pediatric males might be acute hyperkalemia and asystole with succinylcholine or rhabdomyolysis in Becker MD.

  10. The hyperdynamic reaction noted in some patients with Duchenne or Becker MD has certain aspects that are similar to MH. Some authors call this reaction anesthesia-induced rhabdomyolysis (AIR).

  11. Exacerbations of multiple sclerosis during the perioperative period have been linked to stress, delivery, and temperature fluctuations.

  12. The major anesthetic-related concerns for patients with skeletal disorders are the stability of the cervical spine and airway management and safe positioning.

  13. Patients treated for Parkinson disease have an increased incidence of dysphagia, aspiration pneumonia, and falls. They are at risk for neuroleptic malignant syndrome and serotonin syndrome.

  14. Patients with Marfan syndrome should have an assessment of the ascending aorta and cardiac valves prior to surgery.

  15. Patients with Ehlers-Danlos syndrome may be difficult to intubate because of temporomandibular joint dislocation or atlantoaxial laxity.

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INTRODUCTION

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Patients with neuromuscular, skeletal, and movement disorders pose unique challenges to the anesthesiologist and other perioperative providers because these conditions are relatively uncommon, have varying degrees of disability and systemic manifestations, and frequently have potentially life-threatening considerations for anesthetic care. Understanding the pathophysiology, systemic manifestations, common causes of morbidity and mortality, and anesthetic implications of these disorders is essential for developing a safe perioperative management strategy. Appropriate preoperative preparation requires a ...

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