A new consensus that summarizes important considerations when administering anesthesia in patients with any kind of neuromuscular disorder, including metabolic and mitochondrial myopathies such as Pompe disease, was recently published in the European Journal of Neurology.
Patients with neuromuscular disorders have a higher risk of perioperative complications than the general population. A notable percentage of patients with these conditions has increased cardiorespiratory morbidity and abnormalities in temperature and glucose regulation. Furthermore, certain neuromuscular disorders can cause changes in the pharmacodynamics of certain drugs, including anesthetics and neuromuscular blocking agents.
Although many guidelines regarding anesthetic considerations for some neuromuscular disorders like Duchenne muscular dystrophy have been published in recent decades, there are no consensus guidelines focusing on neuromuscular disorders as a whole.
Therefore, the authors of this study aimed to provide a comprehensive review to aid both neurologists and anesthesiologists in improving the perioperative management of these patients.
Read more about Pompe disease guidelines
International experts in the fields of neurology and anesthesiology participated in the development of a consensus statement using the Appraisal of Guidelines for Research & Evaluation (AGREE II) reporting checklist. A modified Delphi process was applied before deciding upon the final statement.
The specific preoperative recommendations for muscle glycogen storage diseases, such as Pompe disease, include several elements. For example, any circumstances that increase the metabolic burden, including hypoglycemia or hyperthermia, should be avoided, and a glucose drip should be used after 4 hours of fasting.
Intraoperative recommendations for patients with muscle glycogen storage diseases include the avoidance of tourniquets and positions that favor rhabdomyolysis, as well as close monitoring of lactate, pH, ammonia, body temperature, and myoglobinuria. It is worth noting that volatile anesthetics were considered safe for this group of patients.
“Given the low level of evidence, we expect an update of this consensus statement will be necessary in ten years or earlier in case of new highly relevant evidence outdates the content of this consensus statement,” the authors wrote.
van den Bersselaar LR, Heytens L, Silva HCA, et al. The European Neuromuscular Centre consensus statement on anaesthesia in patients with neuromuscular disorders. Eur J Neurol. Published online August 16, 2022. doi:10.1111/ene.15526