Hipertermia maligna en paciente sometida a mastectomía radical tipo Madden / Malignant hyperthermia in a patient undergoing Madden type radical mastectomy

Resumen: El objetivo del presente estudio es dar a conocer el manejo anestésico que se proporcionó ante un evento crítico hipertermia maligna (HM) en una mastectomía radical Madden programada de manera electiva, la cual fue manejada con lo que se contaba en ese momento por no tener el fármaco específico (dantroleno) para este tipo de evento HM. El cáncer de mama es una enfermedad compleja, es la primera causa de muerte en la mujer a nivel mundial, ocurre en 70% en países desarrollados. México se encuentra en un nivel intermedio, representa un problema de salud con tendencia a la alta debido al envejecimiento de la población y a mayor prevalencia en factores de riesgo. La HM es un trastorno farmacogenético desencadenado por anestésicos que liberan una masiva acumulación de calcio en el sarcoplasma, que conduce a un metabolismo acelerado y a un incremento en la actividad contráctil del musculoesquelético, llevando a un estado hipermetabólico que genera un incremento en la temperatura corporal llegando a tener secuelas importantes y una alta mortalidad. Se trató de paciente femenino 40 años sin antecedentes relevantes para procedimientos anestésicos, se aplicó anestesia general balanceada, a los 60 minutos presentó datos clínicos que nos sugerían hipertermia maligna, fue manejada con los medios disponibles y se obtuvo un resultado favorable desde el punto de vista de morbimortalidad.

Abstract: The objective of the present is to present the anesthetic management that occurred before a critical event malignant hyperthermia HM) in an electively scheduled Madden radical mastectomy and which was managed with what was available at that time for not having the drug specific (dantrolene) for this type of event HM. Breast cancer is a complex disease, being the leading cause of death in women worldwide, with 70% occurring in developed countries. Mexico is at an intermediate level, being a health problem with a tendency to rise due to the aging of the population. population and higher prevalence of risk factors. Malignant hyperthermia (MH) is an anesthetic-triggered pharmacogenetic disorder that triggers a massive accumulation of calcium in the sarcoplasm, leading to accelerated metabolism and increased skeletal muscle contractile activity. Leading to a hypermetabolic state showing an increase in body temperature, leading to significant sequelae and high mortality. It was a 40-year-old female with no relevant history for anesthetic procedures, being managed with balanced general anesthesia at 60 minutes present data clinicians that they suggested malignant hyperthermia, being managed. With the available means, obtaining a favorable result from the point of view of morbidity and mortality.

Hipertermia maligna / Malignant hyperthermia

Resumen: La hipertermia maligna es un raro desorden farmacogenético potencialmente mortal, que se presenta como una respuesta hipermetabólica a los anestésicos volátiles y relajantes musculares despolarizantes en individuos susceptibles. Esta susceptibilidad se asocia a mutaciones en tres genes: RYR1, CACNA1S y STAC3. Puede manifestarse con síntomas leves o como una crisis fulminante, con rabdomiólisis severa, fibrilación ventricular e insuficiencia renal y circulatoria aguda, por lo tanto, el pronóstico depende de qué tan pronto se sospeche del diagnóstico y qué tan rápido se inicie el tratamiento. El diagnóstico definitivo se basa en una prueba de sensibilidad en biopsia muscular fresca y en pruebas genéticas. La mejor manera de prevenir un evento es la detección precoz de los pacientes susceptibles así como contar con el equipo para responder ante una crisis en cada centro donde se administren anestésicos volátiles y la capacitación del personal. Esta revisión sintetiza los conceptos actuales clínicos y biomédicos para detección, prevención, diagnóstico y manejo de la hipertermia maligna.

Abstract: Malignant hyperthermia is a rare, life-threatening pharmacogenetic disorder which presents as a hypermetabolic response to volatile anesthetics and depolarizing muscle relaxants in susceptible individuals. This susceptibility is associated with mutations in three genes: RYR1, CACNA1S and STAC3. Can manifest with mild symptoms or as a fulminant crisis, with severe rhabdomyolysis, ventricular fibrillation and acute renal and circulatory failure, therefore the prognosis depends on how soon the diagnosis is suspected and how fast treatment is started. The definitive diagnosis is based on a fresh muscle biopsy sensitivity test and genetic testing. The best way to prevent an event is the early detection of susceptible, as well as have equipment to respond to a crisis in each center where volatile anesthetics are administered and the training of staff. This review synthesizes current clinical and biomedical concepts for detection, prevention, diagnosis and management of malignant hyperthermia.

Caso de hipertermia maligna en paciente intervenida de apendicectomía laparoscópica / Malignant Hyperthermia after a laparoscopic appendicectomy: Case report

Malignant hyperthermia is an hypermetabolic syndrome in which the intracellular calcium receptors are altered. Patients who present compatible symptoms with this symdrome are genetically predisposed. Many of the cases have been described in the surgical area, due to the use of anesthesic drugs and neuromuscular blockers. However, there are some cases with an unknown trigger. The prevalence of the syndrome is very difficult to estimate. The diagnosis and early treatment are pronostic factors predicting the evolution of the syndrome.

La hipertermia maligna es conocida como un síndrome de estimulación masiva del metabolismo, debido a la alteración en la liberación de calcio intracelular. Muchos pacientes que presentan clínica compatible con este síndrome están genéticamente predispuestos. La mayoría de los casos se describen en el ámbito quirúrgico, con el empleo de fármacos anestésicos y determinados relajantes neuromusculares, aunque también existen casos en los que no se halla desencadenante. De prevalencia difícil de estimar, la sospecha diagnóstica y el tratamiento precoz marcan el pronóstico evolutivo de estos pacientes.

Síndrome de hipertermia maligna: una patología infrecuente / Malignant hyperthermia syndrome: A rare entity

Resumen Introducción: El síndrome de hipertermia maligna es un trastorno farmacogenético del músculo esquelético de carácter hereditario, que se caracteriza por un estado hipermetabólico relacionado con la exposición a anestésicos inhalatorios o relajantes musculares despolarizantes. Se trata de una afección infrecuente en individuos genéticamente predispuestos, con una incidencia muy baja en pediatría (1 de cada 10,000-15,000 procedimientos anestésicos). Caso clínico: Se presenta un caso de hipertermia maligna relacionado con la exposición a sevoflurano durante una cirugía de adenoidectomía en un paciente de sexo femenino de 6 años de edad. La paciente presentó taquicardia, hipercapnia e hipertermia, que precisaron la administración de dos dosis sucesivas de dantroleno sódico. La evolución posterior fue buena. Conclusiones: El síndrome de hipertermia maligna es un cuadro poco frecuente en la edad pediátrica. Se debe sospechar de forma precoz, ya que es fundamental su detección temprana para iniciar el tratamiento.

Abstract Background: Malignant hyperthermia syndrome is a hereditary pharmacogenetic disorder of skeletal muscle characterized by hypermetabolic state related to the exposure of volatile anesthetic gases or depolarizing muscle relaxants. It is an infrequent entity that occurs in genetically predisposed individuals, with a very low incidence in pediatrics (1 in 10,000-15,000 anesthetic procedures). Case report: We report a case of malignant hyperthermia related to exposure to sevoflurane during adenoidectomy surgery in a 6-year-old female. The patient presented with tachycardia, hypercapnia, and hyperthermia, requiring two successive doses of dantrolene sodium administration, with an adequate response to the treatment. Conclusions: Malignant hyperthermia syndrome is a rare condition in pediatric patients that should be detected in early stages since it is essential to initiate the treatment as soon as possible.

Cost-benefit relationship of keeping dantrolene stocks from the point of view of healthcare institutions / Relación costo-efectividad del abastecimiento de dantroleno desde el punto de vista del prestador de servicios de salud

Abstract Introduction: Malignant hyperthermia (MH) is an acute syndrome triggered by certain anesthetic medications. Dantrolene is the only specific treatment for MH crises. Without treatment, lethality may be as high as 80%. In Colombia, it is not mandatory to keep dantrolene supplies in stock. Objective: To establish the cost-benefit ratio, from the perspective of healthcare institutions, of keeping dantrolene supplies in stock in the operating theater. Methods: Using a decision tree, a Monte Carlo simulation was run with 10,000 scenarios to determine the median annual cost of keeping full or partial stocks (36 or 12 vials x 20 mg, respectively) of dantrolene. For the option of not keeping supplies in stock, the cost threshold was calculated where the expected value of both alternatives of the decision tree is equalized. Indifference curves were constructed for complete and partial supplies. Results: The median annual cost was estimated at 6.6 million Colombian pesos (COP) for full dantrolene supplies, and at COP 2.2 million for partial supplies. The median economic consequence threshold for 1 death due to the unavailability of dantrolene was estimated at COP 18.5 million for full supplies, and at COP 57.0 million for partial supplies. Conclusion: If, as a result of the unavailability of dantrolene, the economic consequences of a death due to MH exceed the threshold of COP 57.0 or COP 18.5 million, the purchase of full or partial stocks, respectively, is justified.

Resumen Introducción: La hipertermnia maligna (HM) es un síndrome agudo desencadenado por algunos medicamentos anestésicos. El dantroleno es el único tratamiento específico para las crisis de HM. Sin tratamiento puede tener una letalidad cercana al 80%. En Colombia, el abastecimiento de dantroleno no es obligatorio. Objetivo: Establecer la relación costo-efectividad, desde el punto de vista de las instituciones prestadoras de servicios de salud, de mantener un abastecimiento de dantroleno en quirófanos. Métodos: Mediante un árbol de decisiones se realizó una simulación Monte Carlo con 10 mil escenarios para determinar la mediana del costo anual de un abastecimiento completo o parcial (36 o 12 viales de 20 mg, respectivamente) de dantroleno. Para la alternativa de no tener abastecimiento, se calculó el umbral de costo, donde se iguala el valor esperado de ambas alternativas del árbol de decisiones. Se construyeron curvas de indiferencia para el abastecimiento completo y parcial. Resultados: La mediana del costo anual del abastecimiento completo de dantroleno se estimó en $6.6 millones de pesos colombianos (COP), y el del abastecimiento parcial en $2.2 millones COP. La mediana del umbral de consecuencias económicas por una muerte sin disponibilidad de dantroleno se estimó en 18.5 millones COP para el abastecimiento completo, y en 57.0 millones COP para el abastecimiento parcial. Conclusión: Si por no disponer de dantroleno las consecuencias económicas de una muerte por HM superan el umbral de $57.0 o $18.5 millones COP, se justifica la compra de un abastecimiento completo o parcial, respectivamente.

Malignant hyperthermia in a child after magnetic resonance imaging: a case report / Hipertermia maligna em criança após exame de ressonância magnética: relato de caso

ABSTRACT Objective: To report on a case of malignant hyperthermia in a child after a magnetic resonance imaging of the skull was performed using sevoflurane anesthesia. Case description: A 3-year-old boy admitted to the pediatric intensive care unit after presenting clinical and laboratory findings consistent with unspecified viral meningoencephalitis. While the patient was sedated, a magnetic resonance imaging of the skul was performed using propofol followed by the administration of sevoflurane through a laryngeal mask in order to continue anesthesia. Approximately three hours after the start of the procedure, the patient presented persistent tachycardia, tachypnea, generalized muscular stiffness and hyperthermia. With a diagnostic hypothesis of malignant hyperthermia, dantrolene was then administered, which immediately induced muscle stiffness, tachycardia, tachypnea and reduced body temperature. Comments: Malignant hyperthermia is a rare pharmacogenetic syndrome characterized by a severe hypermetabolic reaction after the administration of halogenated inhalational anesthetics or depolarizing muscle relaxants such as succinylcholine, or both. Although it is a potentially fatal disease, the rapid administration of continued doses dantrolene has drastically reduced the morbidity and mortality of the disease.

RESUMO Objetivo: Relatar um caso de hipertermia maligna em criança após exame de ressonância magnética de crânio realizada sob efeito anestésico de sevoflurano. Descrição do caso: Menino de três anos de idade, admitido na Unidade de Terapia Intensiva Pediátrica (UTIP) após apresentar quadros clínico e laboratorial compatíveis com meningoencefalite viral não especificada. Foi realizado um exame de ressonância magnética de crânio com sedação utilizando, na indução anestésica, o propofol seguido pela administração de sevoflurano por meio de máscara laríngea para manutenção anestésica. Aproximadamente três horas após o início do procedimento, o paciente apresentou taquicardia, taquipneia, rigidez muscular generalizada e hipertermia persistentes. Com hipótese diagnóstica de hipertermia maligna, foi então administrado dantrolene, que fez ceder de forma imediata a rigidez muscular, a taquicardia, a taquipneia e reduziu a temperatura corporal. Comentários: A hipertermia maligna é uma síndrome farmacogenética rara, que se caracteriza por reação hipermetabólica grave após administração de anestésicos inalatórios halogenados e/ou relaxantes musculares despolarizantes, como a succinilcolina. Apesar de ser uma doença potencialmente fatal, a rápida administração de dantrolene, junto às doses de manutenção, tem reduzido drasticamente a morbimortalidade da doença.

Acute postoperative sepsis mimicking symptomology suspicious for malignant hyperthermia: case report / Sepse aguda pós-operatória simulando sintomatologia suspeita para hipertermia maligna: relato de caso

Abstract Background: Sepsis is a life-threatening organ dysfunction with non-specific clinical features that can mimic other clinical conditions with hyper metabolic state such as malignant hyperthermia. Perioperatively anesthesia providers come across such scenarios, which are extremely challenging with the need for urgent intervention. Objective: To illustrate the need for early intervention and consultation for added assistance to approach and rule out malignant hyperthermia and other possible causes during such a scenario. Case report: A 63-year-old male underwent an uneventful elective flexible cystoscopy and transrectal ultrasound-guided prostate biopsy. Postoperatively he developed symptoms raising suspicion for malignant hyperthermia. Immediately malignant hyperthermia protocol was initiated that included administration of dantrolene and consultation of malignant hyperthermia association hotline along with other diagnostic and interventional management aimed at patient optimization. While early administration of dantrolene helped in hemodynamically stabilizing the patient, the consultation with other providers and malignant hyperthermia association hotline along with repeated examinations and lab works helped in ruling out malignant hyperthermia as the possible diagnosis. The patient later recovered in the intensive care unit where he was treated for the bacteremia that grew in his blood cultures. Conclusions: Sepsis shares clinical symptoms that mimic malignant hyperthermia. While sepsis rapidly progresses to secondary injuries, malignant hyperthermia is life threatening. Providing ideal care requires good clinical judgment and a high level of suspicion where timely and appropriate care such as early administration of dantrolene and consultation of malignant hyperthermia association hotline for added assistance can influence positive outcomes.

Resumo Justificativa: A sepse é uma disfunção orgânica fatal com características clínicas inespecíficas que podem imitar outras condições clínicas com quadro hipermetabólico, como a hipertermia maligna. Os cenários são extremamente desafiadores para a anestesia perioperatória e requerem intervenção urgente. Objetivo: Ilustrar a necessidade de intervenção e consulta precoces para uma assistência adicional na abordagem e exclusão de hipertermia maligna e outras possíveis causas durante tal cenário. Relato de caso: Paciente do sexo masculino, 63 anos, submetido à cistoscopia eletiva com cistoscópio flexível e biópsia transretal da próstata guiada por ultrassom sem intercorrências. No pós-operatório, o paciente desenvolveu sintomas que levantaram a suspeita de hipertermia maligna. O protocolo de hipertermia maligna foi imediatamente iniciado, inclusive a administração de dantrolene e uma consulta pela linha direta da associação de hipertermia maligna, juntamente com outros diagnósticos e manejos intervencionistas com vistas ao aprimoramento do paciente. Enquanto a administração precoce de dantrolene ajudou na estabilização hemodinâmica do paciente, a consulta com outros anestesistas e com a Associação de Hipertermia Maligna, juntamente com repetidos exames físicos e laboratoriais, ajudou a excluir a hipertermia maligna como o possível diagnóstico. O paciente recuperou-se mais tarde na unidade de terapia intensiva, onde recebeu tratamento para a bacteremia detectada em suas hemoculturas. Conclusões: A sepse compartilha sintomas clínicos que mimetizam a hipertermia maligna. Enquanto a sepse progride rapidamente para lesões secundárias, a hipertermia maligna é uma ameaça à vida. Proporcionar o tratamento ideal requer um bom julgamento clínico e um alto nível de suspeita quanto aos cuidados oportunos e apropriados, como a administração precoce de dantrolene e a consulta pela linha direta da Associação de Hipertermia Maligna para assistência adicional, que podem resultar em desfechos positivos.

Reconstitución del dantrolene descripción de un modelo de simulación en hipertermnia maligna / Dantrolene reconstitution: description of a simulation model in malignant hyperthermia

Abstract Malignant hyperthermia is a potentially lethal condition triggered by succinylcholine exposure or exposure to halogenated anesthetic agents. Early identification of the crisis, as well as the timely administration of the specific treatment, impact the patient's outcomes. Any potential difficulties for dandrolene reconstitution are critical and may cause delays or preventable errors, if the operator is not familiar with the preparation and administration of the drug. The development of a simulation model to learn the process offers the possibility to acquire one of the skills required when facing a crisis of malignant hyperthermia. The high cost of the vials and sometimes the difficult access to the medication due to lack of availability in many institutions makes the option of simulating dantrolene reconstitution attractive, in order to familiarize the surgical team with the management of the drug. In this scenario, we submit a simulation model to learn the process of preparation of dandrolene in a controlled environment.

Resumen La hipertermia maligna es una condición potencialmente mortal desencadenada por la exposición a la succinilcolina o a los anestésicos halogenados. La identificación temprana de la crisis así como la administración oportuna del tratamiento específico tiene implicaciones en el desenlace del paciente, siendo las posibles dificultades en la reconstitución del dantroleno un punto crítico donde se pueden generar retrasos o errores prevenibles si hay familiaridad con el proceso de preparación y administración del medicamento. El desarrollo de un modelo de simulación que permite conocer este proceso ofrece la posibilidad de adquirir una de las destrezas requeridas para afrontar una crisis de hipertermia maligna. El elevado costo de los viales y en ocasiones el difícil acceso a estos debido a la no disponibilidad del medicamento en muchas de las instituciones hace atractiva la opción de simular la reconstitución del dantroleno para familiarizar al equipo quirúrgico con el manejo del medicamento. En este escenario proponemos un modelo de simulación con el cual es posible conocer el proceso de preparación del dantroleno en un ambiente controlado.

Dantrolene e células-tronco mesenquimais promovem melhora funcional em ratos Wistar com trauma espinhal agudo / Dantrolene and mesenchymal stem cells promote functional improvement in Wistar rats with spinal cord injury

Objetivou-se avaliar o efeito do dantrolene (DAN) e das células-tronco mesenquimais (CTM) no trauma espinhal agudo (TEA). Sessenta ratos Wistar foram divididos nos grupos CTM, DAN + CTM, DAN, trauma e placebo (TP) e sem trauma e placebo (STP). Realizou-se laminectomia de T12 em todos os grupos, seguida de TEA contusivo ∕ compressivo, com exceção do grupo STP. Uma hora depois, os grupos DAN + CTM e DAN receberam 10mg/kg de DAN. Após sete dias os grupos CTM e DAN + CTM receberam 1x106 células, por via intravenosa. Testes comportamentais foram realizados para avaliar a recuperação funcional durante 28 dias. Os animais traumatizados apresentaram paraplegia. Houve melhora funcional significativa nos grupos tratados com CTM, DAN ou associação DAN + CTM em comparação ao grupo TP (p<0,05). Conclui-se que o DAN e as CTM para tratamento de TEA em ratos apresentam efeitos neuroprotetores e promovem melhora neurológica funcional.(AU)

This study aimed to evaluate the effects of dantrolene (DAN) and mesenchymal stem cells (MSCs) in acute spinal cord injury (SCI). Sixty Wistar rats were divided into groups MSCs, MSCs + DAN, DAN, trauma and placebo (TP) and no trauma and placebo (STP). Laminectomy was performed at T12 level in all animals, followed by a weight-drop model of SCI, except for the STP group. An hour later, the MSCs + DAN and DAN groups received 10mg/kg of DAN. After seven days, the MSCs and MSCs + DAN groups received 1x106 cells intravenously. Behavioral tests were performed to assess functional recovery for 28 days. Traumatized animals showed paraplegia. There was a significant improvement in groups MSCs, DAN and MSCs + DAN compared to TP (p<0.05). It was concluded that DAN and MSCs for the treatment of SCI in rats have neuroprotection effect and promote functional neurological improvement.(AU)

Dantrolene treatment in a patient with uncontrolled hyperthemia after general anesthesia: a case report of suspected malignant hyperthermia: A case report

Fever (body temperature above 38℃) is relatively common during the first few days after general anesthesia. Postoperative fever is usually caused by the inflammation induced by surgery and resolves spontaneously; however, it can be a manifestation of a serious complication such as malignant hyperthermia. We report a case of postoperative hyperthermia (body temperature > 40℃) that was refractory to conventional anti-pyretic measures and finally resolved with dantrolene administration.

Successful early application of extracorporeal membrane oxygenation to support cardiopulmonary resuscitation for a patient suffering from severe malignant hyperthermia and cardiac arrest: a case report / 대한마취과학회지

Malignant hyperthermia (MH) may lead to metabolic crisis of skeletal muscle in susceptible individuals following exposure to triggering agents such as volatile anesthetics or depolarizing muscle relaxants. MH is a rare and a potentially lethal disease, which can lead to cardiac arrest. We report a case of severe MH, in which the rapidly evolving signs of hypermetabolism eventually resulted in cardiac arrest. Despite conventional treatments following cardiopulmonary resuscitation, the patient's vital signs did not improve. Therefore, we applied extracorporeal membrane oxygenation for providing hemodynamic support.

Hipertermia maligna: a propósito de un caso / Malignant hyperthermia: a case report

Malignant hyperthermia (MH) is a rare neuromuscular hereditary disorder, triggered in susceptible individuals by exposure to inhalational agents or succinylcholine and manifested as a hypermetabolic state. We report the case of a 22 years old male patient anesthetized with Desflurane in whom MH was suspected because of unexplained increased levels of End-Tidal carbon dioxide. Dantrolene was administered with good response. Respiratory acidosis and hyperkalemia were also detected but could be easily controlled. The postoperative period was uneventful with the exception of a superficial venous thrombosis at the dantrolene’s injection site. The patient was discharged eight days after the episode without sequela. Successful management of a malignant hyperthemia episode must include: early suspicion, asking for help and early treatment with dantrolene.

La Hipertermia Maligna es un trastorno neuromuscular hereditario infrecuente, manifestado por un estado hipermetabólico desencadenado en individuos susceptibles por la exposición a anestésicos halogenados o Succinilcolina. Se reporta el caso de un hombre de 22 años anestesiado con Desflurano, en quien el diagnóstico de Hipertermia Maligna se sospechó por la presencia de elevación inexplicada del CO2 espirado, que respondió a la suspensión del Desflurano y administración de Dantroleno. Se observó además hiperkalemia y acidosis respiratoria, que fueron fácilmente compensadas. La evolución postoperatoria fue satisfactoria, a excepción de una trombosis venosa superficial en el sitio de inyección del Dantroleno. El paciente fue dado de alta al 8avo día postoperatorio, sin secuelas. Las claves del manejo exitoso de un episodio de hipertermia maligna están en: Sospecha precoz, solicitar ayuda e inició rápido del tratamiento con Dantroleno.

Hipertermia maligna: reporte de un caso clínico / Malignant hyperthermia: report of a clinical case / Hipertermia maligna: relatório de um caso clínico

Resumen: La Hipertermia Maligna (HM), es un síndrome clínico que ocurre en pacientes susceptibles tras la exposición a un agente anestésico desencadenante. La mortalidad desciende con tratamiento específico, por lo que es fundamental su diagnóstico y tratamiento precoz. Se presenta el caso clínico de una niña de 3 años y 10 meses, en estudio por hemiparesia derecha de 3 meses de evolución. Se plantea probable enfermedad desmielinizante. Se le realizaron dos resonancias magnéticas (RNM) previas con anestesia sin incidentes. Por mala evolución de su enfermedad, se realiza nueva RNM con anestesia. A las 5 horas presenta episodio de distonías, acidosis, CPK elevada. Se interpreta como HM, se inicia tratamiento para la misma con mejoría del cuadro clínico. Presentamos este caso con el objetivo de exponer un enfoque diagnóstico y terapéutico de esta patología.

Summary: Malignant Hyperthermia (MH)is a clinical syndrome that occurs in susceptible patients after exposure to a triggering anesthetic agent. Associated mortality decreases with specific treatment, which is why its diagnosis and early treatment is essential. We present the case of a 3-year and 10-month-old girl, who presented a right hemiparesis. Possible demyelinating disease was suspected. Two previous magnetic resonance imaging (MRI) were performed under general anesthesia without complications. Due to poor evolution of his disease, new MRI is performed with anesthesia. After the procedure, she presented an episode of dystonias, acidosis, elevated CPK. It was interpreted as MH, and specific treatment was initiated instated. We present this case with the objective of exposing a diagnostic and therapeutic approach to this pathology.

Resumo: A hipertermia maligna (HM), è uma sindrome clinica que ocorre em pacientes sucetíveis devido a exposição a um agente anestésico desencadeante. A mortalidade diminui com tratamento específico, para o qual é fundamental diagnostico e tratamento precoce. Apresenta-se um caso clínico de uma criança de 3 anos e 10 meses de idade, em estudo por hemiparesia direita de 3 meses de evolução. Questiona-se provável doença desmielinizante. Foram realizadas duas ressonância magnéticas (RNM) previas, sob anestesia, sem incidentes. Devido à má evolução de sua doença, realizuo-se nova RNM sob anestesia. Cinco horas após apresentou episódios de rigidez muscular, acidose e CPK elevada. Interpretou-se como HM, iniciando-se tratamento específico, obtém-se melhoria do quadro clínico. Apresentamos este caso clínico com o objetivo de expor um enfoque diagnóstico e terapêutico desta patologia.

Venous Thromboembolism Following Dantrolene Treatment for Neuroleptic Malignant Syndrome

Neuroleptic malignant syndrome (NMS) is one of the most severe iatrogenic emergencies in clinical service. The symptoms including sudden consciousness change, critical temperature elevation and electrolytes imbalance followed by mutli-organ system failure were common in NMS. In addition to aggressive interventions with intravenous fluid resuscitation and antipyretics, several antidotes have been suggested to prevent further progression of the muscle damage. Dantrolene has been reported to be one of the most effective treatments for NMS. However, the adverse effects of dantrolene treatment for NMS have not yet been evaluated thoroughly. Here we report a young male patient with bipolar I disorder who developed NMS after rapid tranquilization with haloperidol. Dantrolene was given intravenously for the treatment of NMS. However, fever accompanied with local tenderness, hardness with clear border and swelling with heat over the patient's left forearm occurred on the sixth day of dantrolene treatment. Venous thromboembolism (VTE) over intravenous indwelling site at the patient's forearm was noted and confirmed by Doppler ultrasound. The patient's VTE recovered after heparin and warfarin thrombolytic therapy. To our knowledge, this is the first case report demonstrating the possible relationship between dantrolene use and VTE in a patient with antipsychotic treatment. Although the causal relationship and the underlying pathogenesis require further studies, dantrolene should be used with caution for patients with NMS.

Sindrome de hipertermia maligna: a propósito de un caso clínico / Malignant hyperthermia syndrome: case report

El síndrome de hipertermia maligna es una miopatía familiar de naturaleza farmacogenética, que se presenta como un síndrome hipercatabólico del músculo esquelético ligado a la anestesia. La incidencia en pediatría es de 1 cada 10 000 cirugías. Puede ser de instalación rápida, asociado a succinilcolina, o tardía, relacionado con agentes inhalatorios. El cuadro ocurre con taquicardia, hipertermia, hipercapnia, acidosis, rigidez muscular, hiperpotasemia, falla renal y arritmia. La mortalidad sin tratamiento específico es del 80% y desciende al 7% con el uso de dantroleno sódico. Reportamos el caso de un paciente de 8 años que se internó para cirugía de fimosis. Por presentar taquicardia, hipercapnia y rigidez muscular, se inició tratamiento con dantroleno sódico en quirófano, el cual se mantuvo durante 72 h. Evolucionó las primeras 12 h con bajo gasto cardíaco, con máximo valor de creatina fosfoquinasa de 155 147 U/L. Permaneció con asistencia mecánica respiratoria por 48 h. Se otorgó alta al sexto día, sin secuelas.

Malignant hyperthermia syndrome is a family myopathy of pharmacogenetic nature, which appears as a skeletal muscle hypercatabolic syndrome linked to anesthesia. The incidence in pediatrics is 1 event per 10 000 surgeries. The clinical picture may have a rapid onset associated with succinylcholine, or a late onset related to inhalation agents. The clinical picture includes tachycardia, hyperthermia, hypercapnia, acidosis, muscle rigidity, hyperkalemia, renal failure and arrhythmia. Mortality without specific treatment is of 80% and drops to 7% with the use of dantrolene sodium. We report an 8-year-old patient admitted for phimosis surgery; having tachycardia, hypercapnia and muscle rigidity, he started treatment with dantrolene sodium in the operating room, which was maintained for 72 hours. He evolved the first 12 hours with low cardiac output and creatine phosphokinase maximum of 155 147 U/L. He remained with mechanical ventilation for 48 hours. Discharge was given on the sixth day without sequelae.

The effect of long-term oral dantrolene on the neuromuscular action of rocuronium: a case report / 대한마취과학회지

Oral dantrolene causes a dose-dependent depression of skeletal muscle contractility. A 52-year-old man treated with oral dantrolene for spasticity after spinal cord injury was scheduled to undergo irrigation and drainage of a thigh abscess under general anesthesia. He had taken 50 mg oral dantrolene per day for 3 years. Under standard neuromuscular monitoring, anesthesia was performed with propofol, rocuronium, and sevoflurane. A bolus dose of ED95 (0.3 mg/kg) of rocuronium could not depress T1 up to 95%. An additional dose of rocuronium depressed T1 completely and decreased the train-of-four (TOF) count to zero. There was no apparent prolongation of the neuromuscular blocking action of rocuronium. The TOF ratio was recovered to more than 0.9 within 40 minutes after the last dose of rocuronium. A small dose of oral dantrolene does not prolong the duration of action and recovery of rocuronium.

Effects of dantrolene pretreatment on diaphragmatic function in septic rats / 中华麻醉学杂志

Objective To evaluate the effects of dantrolene pretreatment on diaphragmatic function in septic rats .Methods Thirty adult male Sprague-Dawley rats , weighing 200-220 g , aged 9-10 weeks , were randomized into 3 groups (n=10 each) using a random number table :sham operation group (group S) ,spesis group (group CLP) and dantrolene group (group D) .The animals were anesthetized with pentobarbital sodium . Dantrolene 6 mg/kg was injected intraperitoneally (in dimethyl sulfoxide 500 μl ) .Sepsis was induced by cecal ligation and puncture 1 h later in CLP and D groups .The left and right diaphragm was rapidly excised at 24 h after cecal ligation and puncture . The left diaphragm was used to detect the systolic function including the single stimulation twitch , dmax/dt , dmin/dt , maximal force of tetanic contraction , force-frequency curves , and fatigue index .Results Compared with group S ,the single twitch myopalmus ,dmax/dt ,dmin/dt ,maximum peak tension and fatigue index were significantly decreased in group CLP ,the single stimulation twitch ,dmax/dt ,dmin/dt and maximal force of tetanic contraction were decreased , fatigue index was increased in group D , and the force-frequency curve was shifted downward in CLP and D groups ( P<0.05 or 0.01) .Compared with group CLP ,the single stimulation twitch ,dmax/dt ,dmin/dt ,maximal force of tetanic contraction and fatigue index were significantly increased in group D ( P< 0.05 or 0.01 ) .Conclusion Dantrolene pretreatment can improve diaphragmatic function in septic rats .

A suspicious case of malignant hyperthermia during general anesthesia with desflurane: A case report

Malignant hyperthermia (MH) is an inherited disorder of skeletal muscle manifested as a life threatening hypermetabolic crisis in susceptible individuals following exposure to commonly used inhaled anesthetics and depolarizing muscle relaxants. We experienced a suspicious case of MH in 34-year-old male during transfemoral cerebral angiography embolization under general anesthesia with desflurane. The episode emerged 15 minutes after induction of general anesthesia using propofol, rocuronium, remifentanil, desflurane. Desflurane is a recently developed inhaled anesthetics and there has been no case report of MH related with it in Korea. When we suspected episode, vigorous treatment was carried out, symptoms were resolved without dantrolene administration.

Hipertermia maligna / Malignant hypertermia

La hipertermia maligna es un trastorno muscular hereditario que se manifiesta como un estado hipermetabólico desencadenado después de que individuos susceptibles reciben anestésicos volátiles y/o relajantes musculares depolarizantes. La susceptibilidad se basa en una alteración en la regulación del calcio dentro de la fibra del músculo esquelético causada por un defecto en canales de calcio en el retículo sarcoplásmico. Una vez que la crisis de hipertermia maligna se desencadena, se inicia una liberación anormalmente alta de calcio en el retículo sarcoplásmico, dando lugar a un estado hipermetabólico, con signos clínicos típicos tales como taquicardia, rigidez muscular, hipercapnia e hipertermia. Debido a que la hipertermia maligna es potencialmente letal, es relevante establecer los conceptos de manejo de cuidados perioperatorios durante la crisis inesperada y en los pacientes susceptibles que se someten a cirugía. El objetivo de esta revisión es presentar los conocimientos actuales sobre la fisiopatología y los factores desencadenantes de la hipertermia maligna, así como conceptos para el manejo seguro de la anestesia de estos pacientes.

Malignant hyperthermia is an inherited muscle disorder characterized by hypermetabolism and is usually triggered after susceptible individuals are given volatile anesthetics and/or depolarizing muscle relaxants. Susceptibility is based on an altered regulation of calcium within the skeletal muscle fibre caused by a defective calcium release channel at the sarcoplasmic reticulum. Once malignant hyperthermia is triggered an abnormally high release of calcium from the sarcoplasmic reticulum is initiated resulting in a hypermetabolic state, leading to typicalclinical signs, such as tachycardia, muscle rigidity, hypercapnia as well as hyperthermia. Because malignant hyperthermia is potentially lethal, it is relevant to establish management concepts for perioperative care during unexpected crisis and susceptible patients who require surgery. The aim of this review is to present current knowledge about pathophysiology and triggers of malignant hyperthermia as well as concepts for safe anaesthesiological management of these patients.

A case of malignant hyperthermia during anesthesia induction with sevoflurane: A case report / 대한마취과학회지

We experienced a case of malignant hyperthermia (MH) in 6-year-old boy during anesthesia induction for strabismus surgery. It has been generally reported that sevoflurane can induce the delayed onset of MH in the absence of succinylcholine. Our case of MH was elicited after about 2-3 min of sevoflurane administration with N2O, O2 and rocuronium. However, we successfully treated the patient by early recognition of his condition and administering symptomatic treatment and dantrolene.