• DOI:10.1093/bja/aet142
• Corpus ID: 5233298

Anaesthetic management of a patient with Charcot-Marie-Tooth disease for staged diaphragmatic plication.

@article{Pasha2013AnaestheticMO,
  title={Anaesthetic management of a patient with Charcot-Marie-Tooth disease for staged diaphragmatic plication.},
  author={Tina Pasha and A Knowles},
  journal={British journal of anaesthesia},
  year={2013},
  volume={110 6},
  pages={
          1061-3
        }
}

• T. Pasha, A. Knowles
• Published 1 June 2013
• Medicine
• British journal of anaesthesia

Editor—With great interest, we have read the article by Pasha and Knowles. We found it to be a very interesting article as it offers more information on these patients’ anaesthetic management. The authors describe two types of anaesthesia in the same patient and used vecuronium in the first. Under anaesthesia, they describe a possible residual effect with vecuronium. As they mention in their article, there is considerable dispute about the use of neuromuscular blocking agents in these patients. No consensus has been reached on the management of these patients. However, after considering the potential prolonged neuromuscular block that may appear in these patients, we wish to ask about the possibility of using rocuronium, given its greater selectivity with sugammadex. Moreover, we would like to ask the authors if it was possible for them to monitor the neuromuscular response in the adductor pollicis or corrugator supercilii, and if so, what the results were. The literature describes some cases in which it has been possible to monitor the neuromuscular response with the adductor pollicis or corrugator supercilii in patients with Charcot–Marie–Tooth (CMT) syndrome and severe generalized polyneuropathy. 4 The authors do not describe if a neurological examination was done before surgery, or any previous surgery and anaesthesia in the patient. CMTsyndrome is a peripheral polyneuropathy that produces autonomic denervation and muscle atrophy until patients become immobilized, as in this case. Pasha and Knowles indicate that the use of succinylcholine in these patients could be safe. A study by Martyn and Richtsfeld explained the phenomenon of proliferation of acetylcholine receptors on the muscle membrane in neuropathies, which can cause massive release of potassium on exposure to succinylcholine. In addition, prolonged immobilization may also increase the risk. Hence, we should exercise care, perhaps avoid succinylcholine, and consider rocuronium for rapid sequence induction in these patients. We had a patient with CMTsyndrome for an emergency Caesarean section in whom we undertook rapid sequence induction with rocuronium. We reversed it with sugammadex and saw no residual effect. In our case, there was no possibility of neuromuscular monitoring because it was an emergency Caesarean section and there was no monitor in our operating theatre. After an extensive literature search, we found only two studies where rocuronium was used without any residual paralysis, 7 although there was one report of prolonged effect, possibly secondary to liver dysfunction. Finally, Pasha and Knowles considered the risk of malignant hyperthermia (MH) in these patients, which is unfounded because this is a neuropathy, not a myopathy. CMTsyndrome is a peripheral demyelinating polyneuropathy that can evolve progressively and severely to produce chronic muscular dystrophy. As described in the literature, the relationship between CMT syndrome and MH is not clear; thus, one could consider that any patient with muscle disease is at increased risk of MH. Pasha and Knowles have quoted the study by Antognini for an improbable relationship between MH and CMT syndrome. In that study, MH-inducing agents were administered in more than 100 surgical procedures, and no cases of MH were found. The authors mentioned the small sample size, so they were unable to exclude a potential risk in these patients. Furthermore, there is one case which describes MH in CMT syndrome. Therefore, we must be careful and avoid anaesthetic agents that can cause MH as much as possible.