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Peixoto, C. Dourado, H. Santos, P. Roberto" "autores" => array:4 [ 0 => array:4 [ "nombre" => "C." "apellidos" => "Peixoto" "email" => array:1 [ 0 => "claudiapeixoto_950@hotmail.com" ] "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" => "C." "apellidos" => "Dourado" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "H." "apellidos" => "Santos" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "P." "apellidos" => "Roberto" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Medical Degree, Department of Anesthesiology, Centro Hospitalar de Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Medical Degree, Department of Pediatrics, Centro Hospitalar de Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Manejo anestésico perioperatorio de la deficiencia de acil-coenzima A deshidrogenasa de cadena muy larga" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCADD) is an autosomal recessive disorder with more than 800 reported cases worldwide.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> It is caused by pathogenic variants in the ACADVL gene (17p13.1), which causes a defect in the mitochondrial β-oxidation of very long chain fatty acids.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> After prolonged fasting or intense exercise, the body substitutes carbohydrates for fatty acids to produce energy. In VLCADD, gluconeogenesis is impaired and ketone bodies are not produced, leading to energy deficiency with hypoglycaemia, metabolic acidosis, and rhabdomyolysis. The accumulation of undecomposed fatty acids leads to liver, brain, and heart damage.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> The clinical presentation and onset of symptoms varies depending on the severity and degree of enzyme deficiency. When it manifests in the neonatal period it has higher mortality, with associated cardiomyopathy, metabolic acidosis, and rhabdomyolysis, whereas when it appears later in childhood, with hypoketotic hypoglycaemia without cardiac involvement, it has a more favourable outcome. It can also occur in young adults, in the context of intense exercise and subsequent rhabdomyolysis.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Although this disease is rare, perioperative fasting, and anesthesia pose a difficulty for patients with VLCADD, as they may cause shortage of energy supply from glucose, potentially triggering metabolic decompensation.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,3</span></a> The literature is scarce on anesthetic management, but suggests avoiding the use of propofol and volatile anesthetics, which limits the options for safe delivery of anesthesia.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2–4</span></a> In this clinical case, we highlight the difficulties encountered, and review the available literature on the perioperative management of patients with VLCADD. The patient’s father gave their written consent for publication of this report.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical case</span><p id="par0015" class="elsevierStylePara elsevierViewall">A 10-year-old boy with VLCADD was admitted due to rhabdomyolysis. He was diagnosed with VLCADD through neonatal screening, his first rhabdomyolysis crisis appearing at 13 months of age. Since then, he was repeatedly hospitalised due to similar episodes, triggered by fasting, infection, or exercise. When he was admitted, he had severe low back pain, with no history of trauma or fever. Blood tests revealed a glucose value of 150 mg/dl, creatine kinase (CK) of 34,029 U/l, myoglobin of 10,270 ng/ml, and lactate dehydrogenase (LHD) of 1233 U/l.</p><p id="par0020" class="elsevierStylePara elsevierViewall">An internal jugular central venous catheter was placed because peripheral venous access was difficult. Glucose 10% in .45% NaCl solution was started at 4.3 mg/kg/min and progressively reduced to 3 mg/kg/min over the 8 days of hospitalisation. Analgesia was provided with paracetamol 1000 mg and ketorolac 15 mg as needed.</p><p id="par0025" class="elsevierStylePara elsevierViewall">He was scheduled for placement of a totally implanted central venous catheter (CVC-TI) on the 8th day of hospitalisation after favourable progress, in which no triggering factors were discovered. This intervention was decided due to multiple previous hospitalisations requiring the placement of a temporary central venous catheter, to allow more rapid start of treatment in future decompensations, while minimising the risks and stress associated with the insertion of a new catheter.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Preoperative assessment showed a patient with a body mass index of 27.7 kg/m<span class="elsevierStyleSup">2</span> (weight 56 kg, height 142 cm), American Society of Anesthesiologists (ASA) III. Preoperative blood test results showed a CK value of 564U/l, DHL of 798U/l and elevated alanine transaminase: 188U/l. Transthoracic echocardiogram and electrocardiogram were normal. After consultation with the patient's paediatrician, a 6 h solid fast was planned, increasing the 10% glucose infusion to 3.5 mg/kg/min from the last meal, which was maintained during the perioperative period. An oral 20% maltodextrin solution was given up to one hour preoperatively. The patient was premedicated with 3 mg intravenous midazolam to minimise stress, and was accompanied to the operating theatre by his mother. Anesthetic depth, core temperature and blood glucose were monitored in addition to standard ASA monitoring. General anesthesia was induced by inhalational technique with 8% sevoflurane in 50%/50% O<span class="elsevierStyleInf">2</span>/N<span class="elsevierStyleInf">2</span>O, with a minimum alveolar concentration (MAC) of 2, and a 3 i-gel® supraglottic airway device was inserted. Ventilation was started with pressure support adjusted to a tidal volume of 6−8 ml/kg and normocapnia. Anesthesia was maintained with MAC of 1–1.2 sevoflurane in 40%/60% O<span class="elsevierStyleInf">2</span>/air and remifentanil infusion (.5–1.5 g/kg/min). Blood glucose levels were measured at baseline and then every 15 min, and were 100 mg/dl, 119, and 118 mg/dl, respectively. No intraoperative adjustments of the glucose infusion rate were made. Normothermia was maintained with forced air and intravenous fluid warmers. At the end of the procedure the blood sample showed normal values for electrolytes, acid-base lab, blood glucose, and lactate. Paracetamol 1000 mg was administered for analgesia. After an uneventful 50 min procedure, the supraglottic airway device was removed and the patient was transferred to the post-anesthesia care unit (PACU) breathing spontaneously with adequate oxygenation. Postoperative CK was 322U/l. He was discharged from PACU after one hour, resuming oral intake shortly afterwards. Glucose infusion was reduced to 3 mg/kg/min/every 8 h after the procedure and stopped the following day. The patient was discharged 2 days after the procedure.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0035" class="elsevierStylePara elsevierViewall">The primary goal of managing a patient with VLCADD for anesthesia is to avoid an episode of metabolic decompensation. These patients are unable to utilise their fatty acids when their glucose stores are depleted. Calorie deficit from fasting and emotional stress prior to surgery can precipitate catabolism and rhabdomyolysis and should be avoided with minimal stress and fasting. Glucose infusions, based on age and weight, should be started preoperatively. Glucose requirements depend on several factors, such as residual enzyme activity and the type of procedure, and decrease with age.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> The release of cortisol and endogenous catecholamines during perioperative stress can lead to insulin resistance in peripheral tissue, enabling rhabdomyolysis even with normoglycaemia.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> Hyperglycaemia should therefore be treated with insulin administration and not by reducing glucose infusion. <span class="elsevierStyleSup">2</span> Emotional stress prior to surgery is another known trigger, and therefore preventing it by using pre-medication rather than non-pharmacological techniques may help. The safety of the most commonly used pre-medication has been reported for VLCADD patients.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> However, Kleemann et al. investigated free fatty acid levels, and concluded that premedication did not prevent a stress-induced increase in free fatty acids.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> Maintaining an adequate body temperature is essential to avoid shivering, and the consequent increase in energy demand of skeletal muscles.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">In terms of anesthetic technique, general anesthesia appears to protect against stress-induced catabolism,<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> and the choice of anesthetic agent may interfere with glucose homeostasis. Some anesthetics, such as gamma-aminobutyric acid (GABA) agonists or high doses of benzodiazepines or opioids reduce the hyperglycaemic response to surgery by inhibiting the secretion of adrenocorticotropic hormone (ACTH), cortisol, or catabolic hormones.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> Regional anesthesia appears to have a benefit in reducing the degree of insulin resistance in the early postoperative period<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> and has been recommended as an alternative or combined technique whenever possible.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">9,10</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Propofol is contraindicated by many authors due to its lipid emulsion formulation which may cause accumulation of toxic fat oxidation intermediaries in tissue. It may also lead to impairment of mitochondrial function and inhibition of the respiratory chain at various points.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,9,10</span></a> Etomidate may have a potential beneficial effect by reducing catecholamines through suppression of adrenocortical function. However, due to its formulation, it may also increase fatty acid load. Although anesthetic induction with these drugs is unlikely to cause immediate metabolic decompensation, the available literature recommends giving preference to drugs low in fat such as thiopental.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> To our knowledge, there are no reports on the use of ketamine in VLCADD, although it has been used in other disorders in which mitochondrial fatty acid oxidation occurs.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">There have been conflicting results regarding the use of volatile anesthetics. A number of clinical cases were against their use due to concerns about their impact on free fatty acid levels.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> However, there are several published reports that have described the safe use of volatile anesthetics.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,6</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">As for neuromuscular blocking agents, succinylcholine is not recommended, due to its potential to trigger rhabdomyolysis and increase CK and potassium release.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> The safety of non-depolarising agents has been reported<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a>; however, given the suspected altered sensitivity of these drugs, due to skeletal muscle involvement, they should be used with caution, and with neuromuscular blockade monitoring.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">9,10</span></a> Benzodiazepines and opioids have also been used without apparent complications.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,6,9</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">Propofol and neuromuscular blocking agents were not used in this case to avoid the risks associated with these drugs. We chose to maintain anesthesia with sevoflurane and remifentanil to maintain the stability of haemodynamic parameters and to allow rapid and gentle emergence.</p><p id="par0065" class="elsevierStylePara elsevierViewall">In conclusion, anesthetic management in these patients should be multimodal and multidisciplinary. Although perioperative metabolic decompensation cannot be predicted, it is a serious complication that should be avoided. We report the uneventful administration of sevoflurane, nitrous oxide, and remifentanil to a child with VLCADD. Glucose infusion administered during the perioperative period was effective in providing the amount of energy needed to prevent metabolic decompensation, with no increase in serum CK and lactate levels. This is in line with more recent literature, which suggests that volatile anesthetics are a safe option and may have been erroneously avoided in the past. Further research is needed to provide validated guidelines to improve outcomes in this population.</p><p id="par0070" class="elsevierStylePara elsevierViewall">All applicable international, national, and institutional ethical guidelines were followed.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Funding</span><p id="par0075" class="elsevierStylePara elsevierViewall">No funding was received for this work.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Conflict of interests</span><p id="par0080" class="elsevierStylePara elsevierViewall">The authors have no conflict of interests to declare.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:10 [ 0 => array:3 [ "identificador" => "xres1900654" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1643801" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1900653" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1643800" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Clinical case" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Funding" ] 8 => array:2 [ "identificador" => "sec0025" "titulo" => "Conflict of interests" ] 9 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2021-08-06" "fechaAceptado" => "2021-12-13" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1643801" "palabras" => array:6 [ 0 => "Anesthesia" 1 => "Very long-chain acyl-coenzyme A dehydrogenase deficiency" 2 => "Lipid metabolism" 3 => "Fatty acids" 4 => "Rhabdomyolysis" 5 => "Metabolic disease" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1643800" "palabras" => array:6 [ 0 => "Anestesia" 1 => "Deficiencia de acil-coenzima A deshidrogenasa de cadena muy larga" 2 => "Metabolismo lipídico" 3 => "Ácidos grasos" 4 => "Rabdomiolisis" 5 => "Enfermedad metabólica" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Very long-chain acyl-coenzyme A dehydrogenase deficiency is a rare disorder of β-oxidation fatty acid metabolism that results in susceptibility to hypoglycemia, liver failure, cardiomyopathy and rhabdomyolysis during catabolic situations. We report the case of a 10-year-old male undergoing a totally implanted central venous catheter placement during hospitalisation for rhabdomyolysis, who was successfully managed with general anesthesia with nitrous oxide, sevoflurane and remifentanil. No hypoglycemia occurred and creatine kinase levels did not increase in the perioperative period. We describe the challenges encountered and the strategies used to avoid further decompensation of the disease due to surgical stress.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">La deficiencia de acil-coenzima A deshidrogenasa de cadena muy larga es un trastorno infrecuente del metabolismo de β-oxidación de los ácidos grasos que origina susceptibilidad a hipoglucemia, fallo hepático, cardiomiopatía y rabdomiólisis durante las situaciones catabólicas. Reportamos el caso de un varón de diez años de edad programado para colocación de catéter venoso central totalmente implantado durante su hospitalización por rabdomiólisis, que fue exitosamente gestionada con anestesia general con óxido nitroso, sevoflurano y remifentanilo. No se produjo hipoglucemia y los niveles de creatina quinasa no se incrementaron durante el periodo perioperatorio. Describimos las dificultades a que nos enfrentamos y las estrategias utilizadas para evitar mayor descompensación de la enfermedad debida al estrés quirúrgico.</p></span>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Very long-chain acyl-coenzyme a dehydrogenase deficiency" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "N. Leslie" 1 => "C. Valencia" 2 => "A. Strauss" 3 => "K. Zhang" 4 => "M. Adam" 5 => "H. 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