About congenital myopathies and neuromuscular monitorization

González, A.M.

doi:10.1016/j.redare.2018.10.001

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Álvarez-Fuente, A. Fadrique Fuentes, R. Poves-Álvarez, E. Gómez-Pesquera, A. Hernández Lozano" "autores" => array:5 [ 0 => array:2 [ "nombre" => "E." "apellidos" => "Álvarez-Fuente" ] 1 => array:2 [ "nombre" => "A." "apellidos" => "Fadrique Fuentes" ] 2 => array:2 [ "nombre" => "R." "apellidos" => "Poves-Álvarez" ] 3 => array:2 [ "nombre" => "E." "apellidos" => "Gómez-Pesquera" ] 4 => array:2 [ "nombre" => "A." "apellidos" => "Hernández Lozano" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S0034935618301166" "doi" => "10.1016/j.redar.2018.06.004" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0034935618301166?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2341192918301689?idApp=UINPBA00004N" "url" => "/23411929/0000006500000010/v1_201812070615/S2341192918301689/v1_201812070615/en/main.assets" ] "en" => array:13 [ "idiomaDefecto" => true "cabecera" => "Letter to the Editor" "titulo" => "About congenital myopathies and neuromuscular monitorization" "tieneTextoCompleto" => true "saludo" => "Dear Editor," "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "601" "paginaFinal" => "602" ] ] "autores" => array:1 [ 0 => array:3 [ "autoresLista" => "A.M. González" "autores" => array:1 [ 0 => array:3 [ "nombre" => "A.M." "apellidos" => "González" "email" => array:2 [ 0 => "anrgga@humv.es" 1 => "d927@humv.es" ] ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Anestesia, Hospital Universitario Marqués de Valdecilla, Santander, Cantabria, Spain" "identificador" => "aff0005" ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Acerca de miopatías congénitas y monitorización neuromuscular" ] ] "textoCompleto" => "After reading the interesting clinical case submitted by Buisan et al.<a class="elsevierStyleCrossRef" href="#bib0035">1</a> on the anaesthetic management of a paediatric patient with congenital myopathy, I would like to add my personal reflection on the points discussed, in the hope they will be of interest.Because of the low incidence of this type of disease and the steady progress made in molecular genetics, which has complicated rather than simplified the situation by revealing the existence of 16 groups of neuromuscular disorders involving 321 genes (Available in: <a id="intr0010" class="elsevierStyleInterRef" href="http://www.musclegenetable.fr/4DACTION/Blob_groupe3">http://www.musclegenetable.fr/4DACTION/Blob_groupe3</a>), reports of these cases are extremely useful.To my mind, the authors’ theoretical approach to congenital fibre-type disproportion myopathy (CFTDM) from the physiopathological perspective is correct, given the involvement of various different genes: TMP3 (20–25%), RyR1 (10–20%), ACTA1 (6%), MYH7, SEPN1, TPM2, which determine a different functional expression (Ravenscroft et al.<a class="elsevierStyleCrossRef" href="#bib0040">2</a>).In this regard, it is important to determine the associated risk or “susceptibility to malignant hyperthermia (MH), the risk of anaesthesia-induced rhabdomyolysis (AIR) and hyperkalaemia [and] increased sensitivity to nondepolarizing muscle relaxants”, as well as other comorbidities that might be associated with myopathic symptoms in order to choose the best anaesthetic technique.In this case, the patient's genetic study associated the DCTF with the TPM3 mutation, not the RyR1; and, as all congenital myopathies have an increased risk of rhabdomyolysis, the authors decided to avoid inhalational anaesthetic and succinylcholine. Instead, anaesthesia was induced with propofol and maintained with TIVA in combination with an opioid. Muscle relaxants were avoided, a laryngeal mask (LMA) was inserted, and the simple tonsillectomy was performed according to plan.Up to this point, the anaesthetic approach seems perfectly acceptable, although instead of fentanyl I would have chosen remifentanil. Given the uncertainty of this case and the particular pharmacokinetics of remifentanil, it would have been a safer choice and could have avoided the need to antagonize with naloxone at the end of surgery. I would also have used BIS to adjust propofol levels.Questions arise, however, with regard to the absence of a plan B to deal with the hypothetical, though not impossible, failure of the LMA. As it happened, the LMA did indeed fail, and the authors were forced to resort to the use of non-depolarizing neuromuscular relaxants (NFMR), without the aid of muscle relaxant monitoring.As the authors themselves point out, “Given the high risk of respiratory failure and other postoperative complications, myopathic patients, especially children, should be closely monitored after surgery”. Following the same line of reasoning, such close monitoring should be started at the beginning of surgery, using quantitative neuromuscular techniques. In their article on anaesthetic management of myopathic patients, Schieren et al.<a class="elsevierStyleCrossRef" href="#bib0045">3</a> observed that “neuromuscular monitoring is strictly necessary to guide muscle relaxant dosing requirements, as the latter is markedly reduced compared with non-myopathic patients”.It is disconcerting to justify the absence of neuromuscular monitoring with the arguement that “there is no evidence that [neuromuscular monitoring] reduces the incidence of residual muscular paralysis or recurarisation”, when numerous articles (Murphy et al.<a class="elsevierStyleCrossRef" href="#bib0050">4</a>) have clearly established that quantitative neuromuscular monitoring significantly reduces postoperative residual curarization (PORC).The rocuronium-sugammadex combination has made modern anaesthetic practice safer, particularly in the context of neuromuscular diseases. However, far from releasing us from the duty to monitor our patients, the correct use of sugammadex as a reversal agent requires quantitative neuromuscular monitoring, in contrast to the notion that “sugammadex eliminates the need to monitor muscle paralysis and reversion in patients who have received rocuronium”. According to Caldwell andMiller<a class="elsevierStyleCrossRef" href="#bib0055">5</a>, there are “two principal uses for neuromuscular monitoring: to guide intra-operative dosing of an agent; and to assess adequacy of reversal”.Personally, I believe this is the best approach, instead of having to administer “the highest dose (4mg/kg), empirically, trusting that the effect of the drug will neutralize the residual muscular blockade, without the need to monitor reversal”. Based on pharmacokinetic criteria, the paediatric dose (0.6mg/kg) should give moderate neuromuscular blockade 45min into the procedure, with 4 TOF responses and a significant percentage of T4/T1. Reversal does not require 4mg/kg sugammadex.The presence of deeper blockade in myopathic patients may be due to increased sensitivity to the action of NDMRs, or perhaps to muscle hypotonia; we cannot be sure. However, without quantitative neuromuscular monitoring it is impossible to ascertain, and resorting to higher doses (4mg/kg) of sugammadex in paediatric patients also raises the legal issue of off-label use. According to the prospectus, sugammandex should only be used for reversal of moderate blockade (2mg/kg) even though its safety profile at higher doses has been documented (Liu et al.<a class="elsevierStyleCrossRef" href="#bib0060">6</a>).Based on the arguments presented, I believe that the correct anaesthetic management of any myopathic patient should include quantitative neuromuscular monitoring, since this will safeguard the quality of the anaesthetic technique and above all ensure patient safety." 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Wang" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1038/s41598-017-06159-2" "Revista" => array:5 [ "tituloSerie" => "Sci Rep" "fecha" => "2017" "volumen" => "7" "paginaInicial" => "5724" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/28720838" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23411929/0000006500000010/v1_201812070615/S2341192918301781/v1_201812070615/en/main.assets" "Apartado" => array:4 [ "identificador" => "66474" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Letter to the Director" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23411929/0000006500000010/v1_201812070615/S2341192918301781/v1_201812070615/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2341192918301781?idApp=UINPBA00004N"]

ISSN: 2341-1929

Revista Española de Anestesiología y Reanimación (REDAR) -the Spanish Journal of Anesthesiology and Resuscitation- is the scientific body of the Spanish Society of Anesthesiology, Resuscitation and Pain Therapy. This is a monthly journal (10 issues) that publishes scientific articles of all areas within its specialty: clinical anesthesia, resuscitation-intensive medicine and critical care, diagnosis and treatment of acute and chronic pain, urgent care and emergencies and studies on basic science and related fields. The journal accepts work in both Spanish and English.

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Vol. 65. Issue 10.