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Letter to the Editor
About congenital myopathies and neuromuscular monitorization
Acerca de miopatías congénitas y monitorización neuromuscular
A.M. González
Servicio de Anestesia, Hospital Universitario Marqués de Valdecilla, Santander, Cantabria, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">After reading the interesting clinical case submitted by Buisan et al&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> on the anaesthetic management of a paediatric patient with congenital myopathy&#44; I would like to add my personal reflection on the points discussed&#44; in the hope they will be of interest&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">Because of the low incidence of this type of disease and the steady progress made in molecular genetics&#44; which has complicated rather than simplified the situation by revealing the existence of 16 groups of neuromuscular disorders involving 321 genes &#40;Available in&#58; <a id="intr0010" class="elsevierStyleInterRef" href="http://www.musclegenetable.fr/4DACTION/Blob_groupe3">http&#58;&#47;&#47;www&#46;musclegenetable&#46;fr&#47;4DACTION&#47;Blob&#95;groupe3</a>&#41;&#44; reports of these cases are extremely useful&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">To my mind&#44; the authors&#8217; theoretical approach to congenital fibre-type disproportion myopathy &#40;CFTDM&#41; from the physiopathological perspective is correct&#44; given the involvement of various different genes&#58; TMP3 &#40;20&#8211;25&#37;&#41;&#44; RyR1 &#40;10&#8211;20&#37;&#41;&#44; ACTA1 &#40;6&#37;&#41;&#44; MYH7&#44; SEPN1&#44; TPM2&#44; which determine a different functional expression &#40;Ravenscroft et al&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a>&#41;&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">In this regard&#44; it is important to determine the associated risk or &#8220;susceptibility to malignant hyperthermia &#40;MH&#41;&#44; the risk of anaesthesia-induced rhabdomyolysis &#40;AIR&#41; and hyperkalaemia &#91;and&#93; increased sensitivity to nondepolarizing muscle relaxants&#8221;&#44; as well as other comorbidities that might be associated with myopathic symptoms in order to choose the best anaesthetic technique&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">In this case&#44; the patient&#39;s genetic study associated the DCTF with the TPM3 mutation&#44; not the RyR1&#59; and&#44; as all congenital myopathies have an increased risk of rhabdomyolysis&#44; the authors decided to avoid inhalational anaesthetic and succinylcholine&#46; Instead&#44; anaesthesia was induced with propofol and maintained with TIVA in combination with an opioid&#46; Muscle relaxants were avoided&#44; a laryngeal mask &#40;LMA&#41; was inserted&#44; and the simple tonsillectomy was performed according to plan&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Up to this point&#44; the anaesthetic approach seems perfectly acceptable&#44; although instead of fentanyl I would have chosen remifentanil&#46; Given the uncertainty of this case and the particular pharmacokinetics of remifentanil&#44; it would have been a safer choice and could have avoided the need to antagonize with naloxone at the end of surgery&#46; I would also have used BIS to adjust propofol levels&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Questions arise&#44; however&#44; with regard to the absence of a plan B to deal with the hypothetical&#44; though not impossible&#44; failure of the LMA&#46; As it happened&#44; the LMA did indeed fail&#44; and the authors were forced to resort to the use of non-depolarizing neuromuscular relaxants &#40;NFMR&#41;&#44; without the aid of muscle relaxant monitoring&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">As the authors themselves point out&#44; &#8220;Given the high risk of respiratory failure and other postoperative complications&#44; myopathic patients&#44; especially children&#44; should be closely monitored after surgery&#8221;&#46; Following the same line of reasoning&#44; such close monitoring should be started at the beginning of surgery&#44; using quantitative neuromuscular techniques&#46; In their article on anaesthetic management of myopathic patients&#44; Schieren et al&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> observed that &#8220;neuromuscular monitoring is strictly necessary to guide muscle relaxant dosing requirements&#44; as the latter is markedly reduced compared with non-myopathic patients&#8221;&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">It is disconcerting to justify the absence of neuromuscular monitoring with the arguement that &#8220;<span class="elsevierStyleItalic">there is no evidence that</span> &#91;neuromuscular monitoring&#93; <span class="elsevierStyleItalic">reduces the incidence of residual muscular paralysis or recurarisation&#8221;</span>&#44; when numerous articles &#40;Murphy et al&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a>&#41; have clearly established that quantitative neuromuscular monitoring significantly reduces postoperative residual curarization &#40;PORC&#41;&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">The rocuronium-sugammadex combination has made modern anaesthetic practice safer&#44; particularly in the context of neuromuscular diseases&#46; However&#44; far from releasing us from the duty to monitor our patients&#44; the correct use of sugammadex as a reversal agent <span class="elsevierStyleItalic">requires</span> quantitative neuromuscular monitoring&#44; in contrast to the notion that &#8220;<span class="elsevierStyleItalic">sugammadex eliminates the need to monitor muscle paralysis and reversion in patients who have received rocuronium&#8221;&#46;</span> According to Caldwell andMiller<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a>&#44; there are &#8220;<span class="elsevierStyleItalic">two principal uses for neuromuscular monitoring&#58; to guide intra-operative dosing of an agent&#59; and to assess adequacy of reversal&#8221;&#46;</span></p><p id="par0055" class="elsevierStylePara elsevierViewall">Personally&#44; I believe this is the best approach&#44; instead of having to administer &#8220;the highest dose &#40;4<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#41;&#44; empirically&#44; trusting that the effect of the drug will neutralize the residual muscular blockade&#44; without the need to monitor reversal&#8221;&#46; Based on pharmacokinetic criteria&#44; the paediatric dose &#40;0&#46;6<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#41; should give moderate neuromuscular blockade 45<span class="elsevierStyleHsp" style=""></span>min into the procedure&#44; with 4 TOF responses and a significant percentage of T4&#47;T1&#46; Reversal does not require 4<span class="elsevierStyleHsp" style=""></span>mg&#47;kg sugammadex&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">The presence of deeper blockade in myopathic patients may be due to increased sensitivity to the action of NDMRs&#44; or perhaps to muscle hypotonia&#59; we cannot be sure&#46; However&#44; without quantitative neuromuscular monitoring it is impossible to ascertain&#44; and resorting to higher doses &#40;4<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#41; of sugammadex in paediatric patients also raises the legal issue of off-label use&#46; According to the prospectus&#44; sugammandex should only be used for reversal of moderate blockade &#40;2<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#41; even though its safety profile at higher doses has been documented &#40;Liu et al&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a>&#41;&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">Based on the arguments presented&#44; I believe that the correct anaesthetic management of any myopathic patient should include quantitative neuromuscular monitoring&#44; since this will safeguard the quality of the anaesthetic technique and above all ensure patient safety&#46;</p></span>"
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Article information
ISSN: 23411929
Original language: English
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es en pt

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