Case report
Anaesthetic management of a paediatric patient with congenital fibre type disproportion myopathy
Tratamiento anestésico en una paciente pediátrica con miopatía congénita por desproporción del tipo de fibras
F. Buisán
, O. de la Varga, M. Flores, J. Sánchez-Ruano
Corresponding author
Servicio de Anestesiología y Reanimación, Hospital Clínico Universitario de Valladolid, Valladolid, Spain
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Buisán, O. de la Varga, M. Flores, J. Sánchez-Ruano" "autores" => array:4 [ 0 => array:4 [ "nombre" => "F." "apellidos" => "Buisán" "email" => array:1 [ 0 => "felix.buisan@gmx.es" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "O." "apellidos" => "de la Varga" ] 2 => array:2 [ "nombre" => "M." "apellidos" => "Flores" ] 3 => array:2 [ "nombre" => "J." "apellidos" => "Sánchez-Ruano" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Anestesiología y Reanimación, Hospital Clínico Universitario de Valladolid, Valladolid, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Tratamiento anestésico en una paciente pediátrica con miopatía congénita por desproporción del tipo de fibras" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Congenital fibre-type disproportion (CFTD) is a rare type of non-progressive or slowly progressive myopathy that presents with muscle weakness and hypotonia during childhood. Clinical features include motor delay, feeding difficulties, limb weakness, joint contractures and scoliosis.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,2</span></a> The prevalence is unknown, and incidence is estimated at less than 1/50,000 live births.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Diagnosis is based on clinical signs, muscle biopsy and a genetic study. Histologically, type 1 and 2 muscle fibres are similar in size in normal muscle tissue. In CFDT, type 1 muscle fibres are consistently smaller than type 2 fibres by at least 35–40%.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> Several specific genetic mutations, namely (in order or frequency) TPM3, RYR1 and ACTA1 genes,<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> have been found in patients with CFDT myopathy. Most cases of CFDT are de novo (sporadic), but it can also be passed through families in a recessive, autosomal dominant, or X-linked manner.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">An important factor shared by all the different genetic forms of CFDT is a predisposition to respiratory muscle weakness. Mutations in the TPM3 gene (α-tropomyosin), in particular, are associated with nocturnal hypoventilation in childhood, which responds well to non-invasive ventilation.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> Cardiac anomalies are rare.</p><p id="par0020" class="elsevierStylePara elsevierViewall">A previous study reported the case of a 3-year-old girl with dilated cardiomyopathy associated with CFDT,<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> who underwent open surgery for congenital hip dislocation. We were only able to read the abstract in English (the article is in Japanese), which describes the anaesthesia used (ketamine, fentanyl and isoflurane), but does not specify whether it was a case of CFDT myopathy or a genetic diagnosis. It must be borne in mind that the difference in size between types of fibres can also occur in other congenital myopathies and in other neuromuscular diseases. These must be considered and ruled out before making a definitive diagnosis of CFDT myopathy.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,2</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">We describe the anaesthetic management of a paediatric patient with CFDT myopathy scheduled for adenotonsillectomy due to adenotonsillar hypertrophy and obstructive sleep apnea–hypopnea syndrome (OSAHS). Parental consent to publish this case report was obtained.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0030" class="elsevierStylePara elsevierViewall">This was a 3-year-old girl, weight 12<span class="elsevierStyleHsp" style=""></span>kg, with CFDT myopathy who had been scheduled for adenotonsillectomy for severe OSAHS. At birth, she was hypotonic with little spontaneous mobility. CFDT myopathy was diagnosed by muscle biopsy in another hospital at 9 months of age (we have no information on the anaesthetic technique used). Following the biopsy, genetic testing was performed that revealed a spontaneous mutation in the TPM3 gene. The patient's history included OSAHS (attributed to both the absence of tone in the oropharyngeal musculature resulting from hypotonia, and to tonsillar hypertrophy) with the need for continuous positive airway pressure (CPAP) at night and during daytime naps, occasional sialorrhea, and some difficulty swallowing fluids.</p><p id="par0035" class="elsevierStylePara elsevierViewall">The patient presented typical myopathic facies, arched palate and hypertrophic, cryptic grade III tonsils. She presented no mental impairment, and no cardiac abnormalities.</p><p id="par0040" class="elsevierStylePara elsevierViewall">Surgery was scheduled for early morning. The patient was pre-medicated with 0.5<span class="elsevierStyleHsp" style=""></span>mg<span class="elsevierStyleHsp" style=""></span>kg<span class="elsevierStyleSup">−1</span> oral midazolam (1.2<span class="elsevierStyleHsp" style=""></span>ml of a 3<span class="elsevierStyleHsp" style=""></span>ml ampoule of midazolam diluted in 5<span class="elsevierStyleHsp" style=""></span>ml apple juice). The patient was calm and cooperative in the operating room, and standard monitoring was performed with ECG, NIBP, SpO<span class="elsevierStyleInf">2</span> and axillary temperature. A 24G venous catheter was placed in a peripheral vein.</p><p id="par0045" class="elsevierStylePara elsevierViewall">Anaesthesia was induced with propofol (25<span class="elsevierStyleHsp" style=""></span>mg) and fentanyl (25<span class="elsevierStyleHsp" style=""></span>μg), with no muscle relaxants, after which a flexible number 2 laryngeal mask airway (LMA) was inserted. Ventilation was delivered in pressure control mode (15<span class="elsevierStyleHsp" style=""></span>cmH<span class="elsevierStyleInf">2</span>O peak pressure, respiratory rate of 35<span class="elsevierStyleHsp" style=""></span>min<span class="elsevierStyleSup">−1</span>, FiO<span class="elsevierStyleInf">2</span> of 0.5, in an O<span class="elsevierStyleInf">2</span>/air mixture), maintaining an EtCO<span class="elsevierStyleInf">2</span> of 38–40<span class="elsevierStyleHsp" style=""></span>mmHg and oxygen saturation of 100% throughout the procedure. Anaesthesia was maintained with 10<span class="elsevierStyleHsp" style=""></span>mg<span class="elsevierStyleHsp" style=""></span>kg<span class="elsevierStyleSup">−1</span><span class="elsevierStyleHsp" style=""></span>h<span class="elsevierStyleSup">−1</span> of propofol. Once the surgery was started, leaks were observed in the LMA, so we decided to replace it with a number 4 wire-reinforced balloon tube after intravenous administration of 20<span class="elsevierStyleHsp" style=""></span>μg fentanyl and 7<span class="elsevierStyleHsp" style=""></span>mg rocuronium. Acetaminophen (15<span class="elsevierStyleHsp" style=""></span>mg<span class="elsevierStyleHsp" style=""></span>kg<span class="elsevierStyleSup">−1</span>), metamizol (40<span class="elsevierStyleHsp" style=""></span>mg<span class="elsevierStyleHsp" style=""></span>kg<span class="elsevierStyleSup">−1</span>), dexamethasone (0.15<span class="elsevierStyleHsp" style=""></span>mg<span class="elsevierStyleHsp" style=""></span>kg<span class="elsevierStyleSup">−1</span>) and ondansetron (0.1<span class="elsevierStyleHsp" style=""></span>mg<span class="elsevierStyleHsp" style=""></span>kg<span class="elsevierStyleSup">−1</span>) were administered for analgesic and antiemetic prophylaxis. At the end of surgery, muscle relaxation was reversed with sugammadex (4<span class="elsevierStyleHsp" style=""></span>mg<span class="elsevierStyleHsp" style=""></span>kg<span class="elsevierStyleSup">−1</span>) and naloxone (0.04<span class="elsevierStyleHsp" style=""></span>mg) was administered to (partially) reverse the effects of fentanyl.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Surgery lasted 45<span class="elsevierStyleHsp" style=""></span>min and was uneventful. The patient was extubated in the operating room, and then transferred to the paediatric intensive care unit (ICU), where she remained with CPAP during periods of sleep, and supplementary oxygen for a few hours (2<span class="elsevierStyleHsp" style=""></span>bpm, FiO<span class="elsevierStyleInf">2</span> of 0.3). There were no signs of residual paralysis or recurarisation. No episodes of apnoea were observed during her stay in the paediatric ICU. She started oral intake at 6<span class="elsevierStyleHsp" style=""></span>h, which was well tolerated, so she was discharged to the ward and then home 24<span class="elsevierStyleHsp" style=""></span>h after surgery. She was evaluated 1 month later, with the mother reporting the persistence of respiratory symptoms and the need to continue with CPAP.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0055" class="elsevierStylePara elsevierViewall">CFDT myopathy is one of a rare and heterogeneous group of disorders known as congenital myopathies. Other disorders in this group include central core disease, multiminicore myopathy, nemaline myopathy and myotubular myopathy.</p><p id="par0060" class="elsevierStylePara elsevierViewall">Our main concerns in this patient were potential susceptibility to malignant hyperthermia (MH), the risk of anaesthesia-induced rhabdomyolysis (AIR) and hyperkalaemia, increased sensitivity to nondepolarizing muscle relaxants, and the presence of severe OSAHS.</p><p id="par0065" class="elsevierStylePara elsevierViewall">The RYR1 gene (ryanodine receptor) is primarily, though not exclusively, responsible for developing susceptibility to MH. Although MH has not been described in CFDT, a subtype of this myopathy is linked to RYR1, so precautions should be taken to prevent MH<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> (no halogenated gases and/or succinylcholine).</p><p id="par0070" class="elsevierStylePara elsevierViewall">Our patient had CFDT with TPM3 mutation, not RYR1, so theoretically an inhalational anaesthetic could have been used. Volatile anaesthetics have certain advantages in both the induction of paediatric anaesthesia and in the maintenance of spontaneous breathing in patient with difficult airway. In addition, the need for venous access can determine the anaesthetic technique used. However, halogenates are associated with an increased risk of AIR, sometimes severe. According to a recent review, all congenital myopathies, including CFDT, have an increased risk of rhabdomyolysis.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a> AIR, which is often confused with MH, can lead to acute renal failure, hyperkalaemia, and cardiac arrest. Succinylcholine<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> is the anaesthetic drug most frequently associated with AIR, so its use is contraindicated. Volatile anaesthetics have also been implicated in cases of AIR, especially in patients with muscular dystrophy, although their mechanism of action is not fully understood.<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">5,6</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Following the “principle of beneficence” (maximising benefit and minimising harm), therefore, it is best to avoid volatile agents. Until these agents are fully understood, it is probably safer to use volatile anaesthetics with caution or sparingly (e.g., difficult intubation or venipuncture) in patients with CFDT, and monitor for signs of AIR. If locoregional anaesthesia has been ruled out, total intravenous anaesthesia (TIVA), except in patients with suspected mitochondrial myopathy (e.g., high lactate, neurological symptoms), is considered the safest option, especially in high-risk patients.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a> There is no evidence that CFDT substantial alters the mitochondrial metabolism, and no cases of propofol infusion syndrome or severe acidosis have been reported. We therefore used propofol for induction and maintenance, at the doses usually used for TIVA in children.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">In CFDT, as in all myopathies, there is also the possibility of increased sensitivity to non-depolarizing muscle relaxants. In this clinical scenario, flexible LMAs for adenotonsillectomy have several advantages: avoidance of muscle relaxants and better recovery with fewer episodes of hoarseness, coughing and oxygen desaturation.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> However, in our patient leaks and obstructions when placing the mouth gag were observed, and the LMA was exchanged for a reinforced endotracheal tube. We decided to use rocuronium to facilitate laryngoscopy and intubation. No muscle relaxation monitoring devices are available in our centre. Although most guidelines recommend neuromuscular monitoring in patients with myopathies, there is no evidence that this reduces the incidence of residual muscular paralysis or recurarisation.<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a> In this context, it is important to note that rocuronium has the advantage that any residual muscle paralysis, which could easily exacerbate an existing depletion of respiratory reserves, is effectively reversed with sugammadex. The recommended dose of sugammadex depends on the depth of neuromuscular block (2<span class="elsevierStyleHsp" style=""></span>mg<span class="elsevierStyleHsp" style=""></span>kg<span class="elsevierStyleSup">−1</span> [moderate blockade]; 4<span class="elsevierStyleHsp" style=""></span>mg<span class="elsevierStyleHsp" style=""></span>kg<span class="elsevierStyleSup">−1</span> [deep blockade]). The lower dose was administered empirically in the hope of neutralising any residual muscle blockade without the need for confirmation. However, it is important to bear in mind that sugammadex may not be effective when downdosed.<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a> In the opinion of some authors, sugammadex eliminates the need to monitor muscle paralysis and reversion in patients who have received rocuronium.<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a> Either way, the rocuronio/sugammadex combination can be beneficial in myopathies and other neuromuscular disorders.<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">Given the high risk of respiratory failure and other postoperative complications, myopathic patients, especially children, should be closely monitored after surgery.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a> There is usually a greater need for non-invasive ventilation after general anaesthesia, as was the case of our patient. For this reason, our patient was monitored postoperatively in the paediatric ICU.</p><p id="par0090" class="elsevierStylePara elsevierViewall">In conclusion, there is no evidence that patients with CFDT are more susceptible to MH, but the risk cannot be completely excluded (RYR1 gene). Similarly, perioperative AIR can occur in patients with CFDT. Our experience suggests that if regional anaesthesia has been ruled out, TIVA with propofol is a safe alternative in patients with CFDT myopathy. If nondepolarizing muscle relaxants are needed, it may be best to administer rocuronium, which can be reversed with sugammadex. Given the high risk of respiratory failure and other complications, patients should be monitored closely in the postoperative period.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of interest</span><p id="par0095" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:9 [ 0 => array:3 [ "identificador" => "xres1092854" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1035710" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1092853" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1035709" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Case report" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Conflicts of interest" ] 8 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2017-12-03" "fechaAceptado" => "2018-03-05" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1035710" "palabras" => array:6 [ 0 => "Congenital fibre type disproportion" 1 => "Congenital structural myopathies" 2 => "Obstructive sleep apnoea hypopnoea syndrome" 3 => "Intravenous anaesthesia" 4 => "Rocuronium" 5 => "Sugammadex" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1035709" "palabras" => array:6 [ 0 => "Desproporción congénita del tipo de fibras" 1 => "Miopatías estructurales congénitas" 2 => "Apnea obstructiva del sueño" 3 => "Anestesia intravenosa" 4 => "Rocuronio" 5 => "Sugammadex" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Congenital fibre type disproportion (CFTD) is a rare type of myopathy that is characterised by muscle weakness and hypotonia during childhood. Clinical features include motor delay, feeding difficulties, limb weakness, joint contractures, and scoliosis. A report is presented of the anaesthetic management of a 3-year-old girl with CFTD myopathy associated with a mutation of the TPM3 gene, scheduled for adenotonsillectomy because of obstructive sleep apnoea hypopnoea syndrome (OSAHS). The main concerns were the possible susceptibility to malignant hyperthermia, the risk of anaesthesia-induced rhabdomyolysis, a greater sensitivity to non-depolarising muscle relaxants, and the presence of OSAHS. Total intravenous anaesthesia with propofol and the use of rocuronium/sugammadex appear to be safe options. Given the high risk of respiratory compromise and other complications, patients should be closely monitored in the post-operative period.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">La desproporción congénita del tipo de fibras (DCTF) es un raro tipo de miopatía caracterizado por debilidad muscular e hipotonía durante la infancia. Las características clínicas incluyen retraso motor, dificultades en la alimentación, debilidad de las extremidades, contracturas articulares y escoliosis. Se describe el tratamiento anestésico de una paciente de 3 años con miopatía DCTF asociada a mutación del gen TPM3, programada para realización de adenoamigdalectomía por presentar un síndrome de apnea-hipopnea obstructiva del sueño (SAHOS). Nuestras principales preocupaciones fueron la posible susceptibilidad a la hipertermia maligna, el riesgo de rabdomiólisis inducida por anestesia, una mayor sensibilidad a los relajantes musculares no despolarizantes y la presencia de SAHOS. La anestesia total intravenosa con propofol y el empleo de rocuronio/sugammadex parecen ser opciones seguras. Dado el alto riesgo de compromiso respiratorio y otras complicaciones, los pacientes deben controlarse estrechamente en el periodo postoperatorio.</p></span>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Buisán F, de la Varga O, Flores M, Sánchez-Ruano J. Tratamiento anestésico en una paciente pediátrica con miopatía congénita por desproporción del tipo de fibras. Rev Esp Anestesiol Reanim. 2018;65:469–472.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0055" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:3 [ "comentario" => "Available from: <a class="elsevierStyleInterRef" target="_blank" id="intr0010" href="https://www.ncbi.nlm.nih.gov/books/NBK1259/">https://www.ncbi.nlm.nih.gov/books/NBK1259/</a> 12 January 2007 [Updated 11.04.13; accessed 08.10.17]" "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Congenital fiber-type disproportion" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "E.T. DeChene" 1 => "P.B. Kang" 2 => "A.H. 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