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SM: Serratus muscle; pm: pectoralis minor muscle; PM: pectoralis major muscle.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "M. Campos, J. Azevedo, L. Mendes, H. Rebelo" "autores" => array:4 [ 0 => array:2 [ "nombre" => "M." "apellidos" => "Campos" ] 1 => array:2 [ "nombre" => "J." "apellidos" => "Azevedo" ] 2 => array:2 [ "nombre" => "L." "apellidos" => "Mendes" ] 3 => array:2 [ "nombre" => "H." 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Pacreu, S. Martínez, E. Vilà, L. Moltó, J. Fernández-Candil" "autores" => array:5 [ 0 => array:4 [ "nombre" => "S." "apellidos" => "Pacreu" "email" => array:1 [ 0 => "94397@parcdesalutmar.cat" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "S." "apellidos" => "Martínez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "E." "apellidos" => "Vilà" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "L." "apellidos" => "Moltó" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 4 => array:3 [ "nombre" => "J." "apellidos" => "Fernández-Candil" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Anestesiología, Reanimación y Terapia del dolor, Parc de Salut Mar, Barcelona, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anestesiología, Reanimación y Terapia del Dolor, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Dexmedetomidina en el manejo de la vía aérea difícil con fibrobroncoscopio en paciente despierto afecto de síndrome de Klippel-Feil" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1218 "Ancho" => 1500 "Tamanyo" => 193600 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">NMR showing platybasia and syringomyelia caused by C2.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Klippel–Feil syndrome (KFS) is a familial disease inherited in an autosomal dominant pattern. It is characterised by the triad of short neck, limitation of neck motion, and low occipital hairline secondary to congenital fusion of 2 or more spinal bones in the neck. It is associated with other congenital malformations, including: heart disease (4%–14%), most frequently ventricular septal defect; musculoskeletal, urinary, genital, pulmonary, gastrointestinal and neurological disorders, such as encephalocele, meningocele and hydrocephalus. It is more frequent in women, with a male to female ratio of 1.5/1.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">KFS has an incidence of 1:42,000 births, and is one of the leading congenital causes of difficult airway.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">2,3</span></a> Short neck with limited movement and cervical instability can lead to neurological damage during laryngoscopy, intubation and position during surgery. Because of their limited neck mobility, anaesthesiologists must consider awake fibreoptic intubation (FOI) in these patients. Ideally, the patient should be sufficiently sedated to tolerate the procedure, but awake enough to cooperate. Dexmedetomidine (DEX), an imidazoline compound, is an α2 adrenoceptor agonists which works by binding to the α2 adrenergic receptor. It exerts its hypnotic-sedative action at the level of the locus coeruleus<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">4</span></a> by binding to the α2A receptors of this cell group, causing a dose-dependent decrease in noradrenaline levels. This reduces activity in the noradrenergic ascending pathway which, together with a decrease in serotonergic neurotransmission, is associated with the transition from the waking state to sleep, and facilitates easy arousal, thus allowing the patient to cooperate and respond, despite the sedation.</p><p id="par0015" class="elsevierStylePara elsevierViewall">We present a case of difficult airway in a patient with KFS scheduled for extension of a previous craniectomy under general anaesthesia. The patient was sedated with DEX infusion to facilitate awake FOI. We obtained the patient's consent to publish this case report.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0020" class="elsevierStylePara elsevierViewall">A 43-year-old man, ASA II, with known KFS, who presented congenital fusion of cervical vertebral, low occipital hairline, short neck and facial dysmorphism, associated with syringomyelia (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). He also presented paresthesia of the left side of the tongue and soft palate, right rotational nystagmus, facial hemianaesthesia, dysphonia due to paralysis of the left recurrent laryngeal nerve that shifted the left vocal cord to the paramedial position, with normal glottic passage, and dysphagia. His surgical history included suboccipital decompression, C1 laminectomy and resection of the atlantooccipital membrane due to Chiari's disease. In the foregoing surgery, awake FOI was performed. Due to clinical progression and enlargement of syringomyelia (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>), the patient was admitted to our unit for extension of craniectomy and duraplasty.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">Physical examination of the airway showed mouth opening of less than 5<span class="elsevierStyleHsp" style=""></span>cm, thyromental distance of less than 6.5<span class="elsevierStyleHsp" style=""></span>cm, severely limited neck flexion and extension, limited mandible subluxation (lower incisors behind the upper), and Mallampati IV. Given the likelihood of difficult intubation, and it was decided to perform awake FOI. In the operating room, monitoring consisted of non-invasive blood pressure, 5-lead electrocardiogram, and oxygen saturation measured by pulse oximetry. After placing an intravenous line, the patient was premedicated with midazolam (2<span class="elsevierStyleHsp" style=""></span>mg) and DEX infusion, starting at 0.6<span class="elsevierStyleHsp" style=""></span>μg/kg/h. Following this, the posterior pharynx was coated with 10<span class="elsevierStyleHsp" style=""></span>ml of 2% lidocaine gel and sprayed with 2 doses of 10% Xylocaine, and 4<span class="elsevierStyleHsp" style=""></span>l/min of oxygen was delivered via nasal prongs. After 10<span class="elsevierStyleHsp" style=""></span>min, the fibreoptic bronchoscope (FOB) was inserted once the vocal cords were visualised, local anaesthetic (5<span class="elsevierStyleHsp" style=""></span>ml 2% lidocaine) was injected via the FOB and the patient was intubated using a no. 7 tracheal tube, without incident. The patient did not cough or move his neck, and oxygen saturation was maintained at between 96% and 97%. After intubation, EtCO<span class="elsevierStyleInf">2</span> was confirmed by capnography and vesicular murmur was heard in both hemithorax. General anaesthesia was induced with propofol (50<span class="elsevierStyleHsp" style=""></span>mg), fentanyl (100<span class="elsevierStyleHsp" style=""></span>μg) and rocuronium (30<span class="elsevierStyleHsp" style=""></span>mg), and maintained with sevoflurane 0.5% MAC to maintain BIS values between 45 and 60, with a mixture of 50% oxygen/air and perfusion of DEX 0.6<span class="elsevierStyleHsp" style=""></span>μg/kg/h to spare anaesthetic drugs. Neuromuscular blockade was monitored intraoperatively by kinemiography, using a Datex-Ohmeda Neuromuscular Transmission monitor (GE Healthcare, Helsinki, Finland). Surgery lasted for around 4<span class="elsevierStyleHsp" style=""></span>hours, and a further bolus of rocuronium was administered to maintain a TOF of 0.</p><p id="par0030" class="elsevierStylePara elsevierViewall">At the end of surgery, sevoflurane and DEX infusion was discontinued, and sugammadex (2<span class="elsevierStyleHsp" style=""></span>mg/kg) was administered to reverse the residual neuromuscular blockade. The patient returned to spontaneous breathing and started to obey orders. He was extubated 5<span class="elsevierStyleHsp" style=""></span>min after the end of surgery, transferred to the postanaesthesia care unit for postoperative monitoring, and from there to the hospital ward 24<span class="elsevierStyleHsp" style=""></span>h later.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0035" class="elsevierStylePara elsevierViewall">KFS is a rare entity that was first described in 1912 by Maurice Klippel and André Feil. The syndrome is characterised by the congenital fusion of the 2nd to the 7th cervical vertebra. There are several degrees of fusion: (a) type <span class="elsevierStyleSmallCaps">I</span>, massive fusion of the cervical spine to the upper dorsal level; (b) type <span class="elsevierStyleSmallCaps">II,</span> fusion of 1 or 2 vertebrae, generally accompanied by occipital–cervical fusion and hemivertibrae. This is the most common, although it is largely asymptomatic; (c) type III, in which cervical fusion is associated with a similar disorder at the dorsal or lumbar level; and (d) type IV, which involves cervical, upper thoracic, lower dorsal or lumbar fusion.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">1</span></a> The syndrome, particularly types I and IV, can be associated with other alterations. Our patient presented type <span class="elsevierStyleSmallCaps">II</span> KFS, with no associated clinical manifestations.</p><p id="par0040" class="elsevierStylePara elsevierViewall">Airway management in patients with this syndrome is challenging for the anaesthesiologist. Different sedative drugs have been used, such as benzodiazepines, opioids (such as iv remifentanil), ketamine, and propofol,<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">5</span></a> but almost all cause respiratory depression. We suggest DEX as an adequate and effective option in these patients with difficult airway, provided it is not contraindicated due to associated cardiac dysfunction, and also as a coadjuvant, since it provides adequate sedation without causing respiratory depression.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">4</span></a> Local anaesthesia of the upper airway is also important to ensure successful completion of the procedure.</p><p id="par0045" class="elsevierStylePara elsevierViewall">Other authors have reported the use of DEX for sedation in awake FOI in patients with difficult airway.<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">6–8</span></a> These included 2 cases of parturients, one with KFS and another with spinal muscular atrophy type <span class="elsevierStyleSmallCaps">III</span> (Kugelberg-Welander disease) in whom neuraxial block was ruled out due to Arnold Chiari malformation and multiple spinal surgeries, and in which DEX was used for sedation in FOI during caesarean section.<a class="elsevierStyleCrossRefs" href="#bib0100"><span class="elsevierStyleSup">8,9</span></a> In these cases, DEX was chosen for its sedative, anxiolytic, hypnotic and analgesic properties, and also as an anaesthetic-sparing strategy, Furthermore, DEX is an antisialogogue that attenuates the neuroendocrine response and protects cardiovascular function without affecting breathing.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">4</span></a> All these properties, together reports of it use as an analgesic during FOI,<a class="elsevierStyleCrossRefs" href="#bib0085"><span class="elsevierStyleSup">5,7</span></a> prompted us to use 0.3<span class="elsevierStyleHsp" style=""></span>g/kg/h iv DEX with midazolam for awake FOI. The recommended loading dose is 1<span class="elsevierStyleHsp" style=""></span>μg/kg for 10<span class="elsevierStyleHsp" style=""></span>min (rapid intravenous bolus is not indicated due to the risk of hypertension) followed by a maintenance infusion of 0.2–0.7<span class="elsevierStyleHsp" style=""></span>μg/kg/h. In our case, we did not administer a loading dose, since the half-life of DEX is 4–10, and during this time the local anaesthetic was instilled in the airway.<a class="elsevierStyleCrossRefs" href="#bib0110"><span class="elsevierStyleSup">10,11</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Although DEX is associated with few adverse effects, the most frequent are hypotension, bradycardia, nausea and dry mouth typically caused by α2 agonists.<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">6,12</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The DEX also facilitates extubation by reducing the need for hypnotic drugs and opioids, which could seriously compromise the airway. In our case, we decided to continue iv DEX intraoperatively to spare anaesthetic drugs and facilitate rapid awakening.</p><p id="par0060" class="elsevierStylePara elsevierViewall">We believe it best to plan the best possible airway management strategy in patients with KFS and known difficult airway. In our case DEX, with its excellent safety profile, provided adequate sedation during intubation.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of interest</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors have no conflict of interest to declare.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:9 [ 0 => array:3 [ "identificador" => "xres1103966" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1044205" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1103965" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1044204" "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" => "2018-03-22" "fechaAceptado" => "2018-05-06" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1044205" "palabras" => array:5 [ 0 => "Klippel–Feil syndrome" 1 => "Difficult airway" 2 => "Fibre-optic intubation" 3 => "Awake patient" 4 => "Dexmedetomidine" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1044204" "palabras" => array:5 [ 0 => "Síndrome de Klippel-Feil" 1 => "Vía aérea difícil" 2 => "Fibrobroncoscopio" 3 => "Paciente despierto" 4 => "Dexmedetomidina" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Klippel–Feil syndrome is a disease characterised by congenital fusion of cervical vertebra, which leads to cervical limitation and instability. In these cases, the best option is the orotracheal intubation with the fibre-optic bronchoscope with the patient awake. The advantage is that cervical movements that could lead to neurological damage are minimised. In these patients, adequate sedation, together with instillation of local anaesthetic in the pharynx and hypopharynx, is the key to reducing patient discomfort and achieving successful orotracheal intubation. Dexmedetomidine is a selective α2-adrenergic receptor agonist that produces sedation and analgesia at the locus coeruleus without producing respiratory depression, as well as maintaining patient collaboration. The case is presented of a patient with Klippel–Feil syndrome and difficult airway management, who was given a dexmedetomidine infusion at 0.6<span class="elsevierStyleHsp" style=""></span>μg/kg/h as sedation for an awake fibre-optic endotracheal intubation.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">El síndrome de Klippel-Feil es una enfermedad que se caracteriza por la fusión congénita de vértebras cervicales, que condiciona una limitación e inestabilidad cervical. En estos casos la mejor opción es la intubación orotraqueal con fibrobroncoscopio con el paciente despierto. La ventaja es que se minimizan los movimientos cervicales que podrían conllevar un daño neurológico. En estos pacientes una sedación adecuada, junto con la instilación de anestésico local en la faringe y la hipofaringe es clave para reducir las molestias del paciente y conseguir la intubación orotraqueal con éxito. La dexmedetomidina es un agonista selectivo de los receptores α-2 adrenérgicos que produce sedación y ansiolisis al nivel del locus coeruleus, sin provocar depresión respiratoria, y preservando la colaboración del paciente. Presentamos el caso de un paciente con síndrome de Klipple-Feil y vía aérea difícil en el que utilizamos una perfusión de dexmedetomidina a dosis de 0,6<span class="elsevierStyleHsp" style=""></span>μg/kg/h como sedación para la intubación orotraqueal con fibrobroncoscopio con el paciente despierto.</p></span>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Pacreu S, Martínez S, Vilà E, Moltó L, Fernández-Candil J. Dexmedetomidina en el manejo de la vía aérea difícil con fibrobroncoscopio en paciente despierto afecto de síndrome de Klippel-Feil. Rev Esp Anestesiol Reanim. 2018;65:537–540.</p>" ] ] "multimedia" => array:2 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 911 "Ancho" => 900 "Tamanyo" => 81250 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Klippel–Feil syndrome with low occipital hairline, short neck and facial dysmorphia.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1218 "Ancho" => 1500 "Tamanyo" => 193600 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">NMR showing platybasia and syringomyelia caused by C2.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:12 [ 0 => array:3 [ "identificador" => "bib0065" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Classification of congenitally fused cervical patterns in Klippel–Feil patients" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "D. 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Case report
Dexmedetomidine in difficult airway management with a fibre-optic bronchoscope in the awake patient with Klippel–Feil Syndrome
Dexmedetomidina en el manejo de la vía aérea difícil con fibrobroncoscopio en paciente despierto afecto de síndrome de Klippel-Feil