Watchful Eye Prevents Catastrophe in Case of Malignant Hyperthermia: A Case Report.

CASE: A 49-year-old woman presented with a displaced left posterior wall acetabular fracture-dislocation and a left comminuted intra-articular distal radius fracture sustained in a motor vehicle accident. Fifteen minutes after anesthesia induction for operative fixation of the fractures, the patient experienced severe hypertension, increased end-tidal carbon dioxide, and fever. A diagnosis of malignant hyperthermia (MH) was made before incision. The patient was medically stabilized, and her acetabular fracture-dislocation was placed in skeletal traction until she was deemed fit for surgery 4 days later. CONCLUSION: This case report highlights the importance of early recognition of MH and perioperative management.

Creation and Implementation of a Preoperative Malignant Hyperthermia Screening Tool.

BACKGROUND: Malignant hyperthermia (MH) is a potentially lethal pharmacogenetic disorder triggered by certain anesthetic agents. There is currently no standardized preoperative screening tool utilized to identify MH-susceptible individuals. LOCAL PROBLEM: This quality improvement (QI) project aimed to enhance preoperative screening for MH susceptibility (MHS) by implementing an evidence-based screening tool for surgical patients at 2 sites. METHODS: This prospective descriptive QI project evaluated the success of implementing an MHS screening tool preoperatively and its impact on the anesthesia plan. INTERVENTIONS: Anesthesia professionals included the screening tool in their preoperative interview for surgical patients so that positively screened patients could receive MH prevention measures. RESULTS: A total of 95 patients at site A and 234 patients at site B were screened using the MH tool, a cumulative total of 21 patients were positively screened, and 1 anesthetic plan was altered. CONCLUSIONS: This MHS screening tool has the potential to prevent MH episodes when used consistently by staff.

Preparation of Dräger Atlan A350 and General Electric Healthcare Carestation 650 anesthesia workstations for malignant hyperthermia susceptible patients.

BACKGROUND: Patients at risk of malignant hyperthermia need trigger-free anesthesia. Therefore, anesthesia machines prepared for safe use in predisposed patients should be free of volatile anesthetics. The washout time depends on the composition of rubber and plastic in the anesthesia machine. Therefore, new anesthesia machines should be evaluated regarding the safe preparation for trigger-free anesthesia. This study investigates wash out procedures of volatile anesthetics for two new anesthetic workstations: Dräger Atlan A350 and General Electric Healthcare (GE) Carestation 650 and compare it with preparation using activated charcoal filters (ACF). METHODS: A Dräger Atlan and a Carestation 650 were contaminated with 4% sevoflurane for 90 min. The machines were decontaminated with method (M1): using ACF, method 2 (M2): a wash out method that included exchange of internal parts, breathing circuits and soda lime canister followed by ventilating a test lung using a preliminary protocol provided by Dräger or method 3 (M3): a universal wash out instruction of GE, method 4 (M4): M3 plus exchange of breathing system and bellows. Decontamination was followed by a simulated trigger-free ventilation. All experiments were repeated with 8% desflurane contaminated machines. Volatile anesthetics were detected with a closed gas loop high-resolution ion mobility spectrometer with gas chromatographic pre-separation attached to the bacterial filter of the breathing circuits. Primary outcome was time until < 5 ppm of volatile anesthetics and total preparation time. RESULTS: Time to < 5 ppm for the Atlan was 17 min (desflurane) and 50 min (sevoflurane), wash out continued for a total of 60 min according to protocol resulting in a total preparation time of 96-122 min. The Carestation needed 66 min (desflurane) and 24 min (sevoflurane) which could be abbreviated to 24 min (desflurane) if breathing system and bellows were changed. Total preparation time was 30-73 min. When using active charcoal filters time to < 5 ppm was 0 min for both machines, and total preparation time < 5 min. CONCLUSION: Both wash out protocols resulted in a significant reduction of trace gas concentrations. However, due to the complexity of the protocols and prolonged total preparation time, feasibility in clinical practice remains questionable. Especially when time is limited preparation of the anesthetic machines using ACF remain superior.

Consensus guidelines on perioperative management of malignant hyperthermia suspected or susceptible patients from the European Malignant Hyperthermia Group.

Malignant hyperthermia is a potentially fatal condition, in which genetically predisposed individuals develop a hypermetabolic reaction to potent inhalation anaesthetics or succinylcholine. Because of the rarity of malignant hyperthermia and ethical limitations, there is no evidence from interventional trials to inform the optimal perioperative management of patients known or suspected with malignant hyperthermia who present for surgery. Furthermore, as the concentrations of residual volatile anaesthetics that might trigger a malignant hyperthermia crisis are unknown and manufacturers' instructions differ considerably, there are uncertainties about how individual anaesthetic machines or workstations need to be prepared to avoid inadvertent exposure of susceptible patients to trigger anaesthetic drugs. The present guidelines are intended to bundle the available knowledge about perioperative management of malignant hyperthermia-susceptible patients and the preparation of anaesthesia workstations. The latter aspect includes guidance on the use of activated charcoal filters. The guidelines were developed by members of the European Malignant Hyperthermia Group, and they are based on evaluation of the available literature and a formal consensus process. The most crucial recommendation is that malignant hyperthermia-susceptible patients should receive anaesthesia that is free of triggering agents. Providing that this can be achieved, other key recommendations include avoidance of prophylactic administration of dantrolene; that preoperative management, intraoperative monitoring, and care in the PACU are unaltered by malignant hyperthermia susceptibility; and that malignant hyperthermia patients may be anaesthetised in an outpatient setting.

Successful management of hypothermic cardiopulmonary bypass in a malignant hyperthermia susceptible patient.

Malignant hyperthermia (MH) is a potentially lethal reaction in those that are genetically predisposed, frequently triggered by inhaled anesthetics. MH is often difficult to diagnose because it is accompanied by signs and symptoms that are shared with other disorders. The diagnosis is further obscured in cardiac surgical patients, as the signs of MH can be masked by the cardiopulmonary bypass circuit (CPB) and the use of induced hypothermia. In this case-report, we describe the successful anesthetic management of a 65-year-old MH-susceptible female, confirmed via caffeine halothane contracture test, with aortic regurgitation and ascending aortic dilatation who underwent a Bentall procedure. We have also identified certain key measures for the safe anesthetic management of these patients.

Residual volatile anesthetics after workstation preparation and activated charcoal filtration.

BACKGROUND: Volatile anesthetics potentially trigger malignant hyperthermia crises in susceptible patients. We therefore aimed to identify preparation procedures for the Draeger Primus that minimize residual concentrations of desflurane and sevoflurane with and without activated charcoal filtration. METHODS: A Draeger Primus test workstation was primed with 7% desflurane or 2.5% sevoflurane for 2 hours. Residual anesthetic concentrations were evaluated with five preparation procedures, three fresh gas flow rates, and three distinct applications of activated charcoal filters. Finally, non-exchangeable and autoclaved parts of the workstation were tested for residual emission of volatile anesthetics. Concentrations were measured by multicapillary column-ion mobility spectrometry with limits of detection/quantification being <1 part per billion (ppb) for desflurane and <2.5 ppb for sevoflurane. RESULTS: The best preparation procedure included a flushing period of 10 minutes between removal and replacement of all parts of the ventilator circuit which immediately produced residual concentrations <5 ppm. A fresh gas flow of 10 L/minute reduced residual concentration as effectively as 18 L/minute, whereas flows of 1 or 5 L/minute slowed washout. Use of activated charcoal filters immediately reduced and maintained residual concentrations <5 ppm for up to 24 hours irrespective of previous workstation preparation. The fresh gas hose, circle system, and ventilator diaphragm emitted traces of volatile anesthetics. CONCLUSION: In elective cases, presumably safe concentrations can be obtained by a 10-minute flush at ≥10 L/minute between removal and replacement all components of the airway circuit. For emergencies, we recommend using an activated charcoal filter.

Don't stress: a case report of regional anesthesia as the primary anesthetic for gynecologic surgery in a patient with mitochondrial myopathy and possible malignant hyperthermia susceptibility.

BACKGROUND: We aim to describe the evaluation and management of a patient with the uncommon combination of both mitochondrial myopathy and possible malignant hyperthermia susceptibility as an important source of information and as a valuable example of the role of regional anesthesia for patients with these diagnoses. CASE PRESENTATION: A 24 year old woman with a history of possible mitochondrial myopathy and possible malignant hyperthermia susceptibility presented for gynecologic surgery. Surgery was well tolerated with combined spinal epidural anesthesia as well as sedation with midazolam, ketamine, and fentanyl. CONCLUSIONS: Anesthetic management of patients with mitochondrial myopathy is challenging, made even more so with concurrent malignant hyperthermia susceptibility. This case adds an example to the literature of employing regional anesthesia as a safe approach to this complex care.

Preparation of anaesthesia workstation for trigger-free anaesthesia: An observational laboratory study.

BACKGROUND: Trigger-free anaesthesia is required for patients who are susceptible to malignant hyperthermia. Therefore, all trace of volatile anaesthetics should be removed from anaesthetic machines before induction of anaesthesia. Because the washout procedure is time consuming, activated charcoal filters have been introduced, but never tested under minimal flow conditions. OBJECTIVE: To investigate performance of activated charcoal filters during long duration (24 h) simulated ventilation. DESIGN: A bench study. SETTING: A Primus anaesthesia machine (Dräger) was contaminated with either 4% sevoflurane or 8% desflurane by ventilating a test lung for 90 min. The machine was briefly flushed according to manufacturer instructions, activated charcoal filters were inserted and a test lung was ventilated in a 24 h test. Trace gas concentrations were measured using a closed gas loop high-resolution ion mobility spectrometer with gas chromatographic preseparation. During the experiment reduced fresh gas flows were tested. At the end of each experiment the activated charcoal filters were removed and the machine was set to standby for 10 min to test for residual contamination within the circuit. The activated charcoal filters were reconnected into the circuit to test their ability to continue removing volatile anaesthetics (functional test) from the gas. Control experiments were conducted without activated charcoal filters. MAIN OUTCOME MEASURES: Absolute concentrations of desflurane and sevoflurane. RESULTS: The concentration of volatile anaesthetics dropped to less than 5 ppm (parts per million) following insertion of activated charcoal filters. In the desflurane experiments at least 1 l min FGF was needed to keep the concentration below an acceptable level (<5 ppm): 0.5 l min fresh gas flow was required in sevoflurane experiments. While activated charcoal filters in the sevoflurane tests passed the functional test after 24 h, activated charcoal filters in the desflurane tests failed. CONCLUSION: Activated charcoal filters meet the requirements for trigger-free low flow (1 l min) ventilation over 24 h. Minimal flow (0.5 l min) ventilation may be possible for sevoflurane contaminated machines.

[Profile of malignant hyperthermia susceptibility reports confirmed with muscular contracture test in Brazil]. / Perfil dos relatos de suscetibilidade à hipertermia maligna confirmados com teste de contratura muscular no Brasil.

BACKGROUND AND OBJECTIVES: Malignant hyperthermia is an autosomal dominant hypermetabolic pharmacogenetic syndrome, with a mortality rate of 10%-20%, which is triggered by the use of halogenated inhaled anesthetics or muscle relaxant 10%-20% succinylcholine. The gold standard for suspected susceptibility to malignant hyperthermia is the in vitro muscle contracture test in response to halothane and caffeine. The determination of susceptibility in suspected families allows the planning of safe anesthesia without triggering agents for patients with known susceptibility to malignant hyperthermia by positive in vitro muscle contracture test. Moreover, the patient whose suspicion of malignant hyperthermia was excluded by the in vitro negative muscle contracture test may undergo standard anesthesia. Susceptibility to malignant hyperthermia has a variable manifestation ranging from an asymptomatic subject presenting a crisis of malignant hyperthermia during anesthesia with triggering agents to a patient with atrophy and muscle weakness due to central core myopathy. The aim of this study is to analyze the profile of reports of susceptibility to malignant hyperthermia confirmed with in vitro muscle contracture test. METHOD: Analysis of the medical records of patients with personal/family suspicion of malignant hyperthermia investigated with in vitro muscle contracture test, after given written informed consent, between 1997 and 2010. RESULTS: Of the 50 events that motivated the suspicion of malignant hyperthermia and family investigation (sample aged 27±18 years, 52% men, 76% white), 64% were investigated for an anesthetic malignant hyperthermia crisis, with mortality rate of 25%. The most common signs of a malignant hyperthermia crisis were hyperthermia, tachycardia, and muscle stiffness. Susceptibility to malignant hyperthermia was confirmed in 79.4% of the 92 relatives investigated with the in vitro muscle contracture test. CONCLUSION: The crises of malignant hyperthermia resembled those described in other countries, but with frequency lower than that estimated in the country.

Profile of malignant hyperthermia susceptibility reports confirmed with muscular contracture test in Brazil / Perfil dos relatos de suscetibilidade à hipertermia maligna confirmados com teste de contratura muscular no Brasil

Abstract Background and objectives: Malignant hyperthermia is an autosomal dominant hypermetabolic pharmacogenetic syndrome, with a mortality rate of 10%-20%, which is triggered by the use of halogenated inhaled anesthetics or muscle relaxant succinylcholine. The gold standard for suspected susceptibility to malignant hyperthermia is the in vitro muscle contracture test in response to halothane and caffeine. The determination of susceptibility in suspected families allows the planning of safe anesthesia without triggering agents for patients with known susceptibility to malignant hyperthermia by positive in vitro muscle contracture test. Moreover, the patient whose suspicion of malignant hyperthermia was excluded by the in vitro negative muscle contracture test may undergo standard anesthesia. Susceptibility to malignant hyperthermia has a variable manifestation ranging from an asymptomatic subject presenting a crisis of malignant hyperthermia during anesthesia with triggering agents to a patient with atrophy and muscle weakness due to central core myopathy. The aim of this study is to analyze the profile of reports of susceptibility to malignant hyperthermia confirmed with in vitro muscle contracture test. Method: Analysis of the medical records of patients with personal/family suspicion of malignant hyperthermia investigated with in vitro muscle contracture test, after given written informed consent, between 1997 and 2010. Results: Of the 50 events that motivated the suspicion of malignant hyperthermia and family investigation (sample aged 27 ± 18 years, 52% men, 76% white), 64% were investigated for an anesthetic malignant hyperthermia crisis, with mortality rate of 25%. The most common signs of a malignant hyperthermia crisis were hyperthermia, tachycardia, and muscle stiffness. Susceptibility to malignant hyperthermia was confirmed in 79.4% of the 92 relatives investigated with the in vitro muscle contracture test. Conclusion: The crises of malignant hyperthermia resembled those described in other countries, but with frequency lower than that estimated in the country.

Resumo Justificativa e objetivo: Hipertermia maligna é uma síndrome farmacogenética hipermetabólica, autossômica dominante, com mortalidade entre 10%-20%, desencadeada por uso de anestésico inalatório halogenado ou relaxante muscular succinilcolina. O padrão-ouro para pesquisa de suscetibilidade à hipertermia maligna é o teste de contratura muscular in vitro em resposta ao halotano e à cafeína. A determinação da suscetibilidade nas famílias suspeitas permite planejar anestesias seguras sem agentes desencadeantes para os pacientes confirmados como suscetíveis à hipertermia maligna pelo teste de contratura muscular in vitro positivo. Além disso, o paciente no qual a suspeita de hipertermia maligna foi excluída pelo teste de contratura muscular in vitro negativo pode ser anestesiado de forma convencional. Suscetibilidade à hipertermia maligna tem manifestação variável, desde indivíduo assintomático que apresenta crise de hipertermia maligna durante anestesia com agentes desencadeantes, até paciente com atrofia e fraqueza muscular por miopatia central core disease. O objetivo deste trabalho é analisar o perfil dos relatos de suscetibilidade à hipertermia maligna confirmados com teste de contratura muscular in vitro. Método: Análise das fichas de notificação dos pacientes com suspeita pessoal/familiar de hipertermia maligna investigados com teste de contratura muscular in vitro, após assinatura do termo de consentimento, entre 1997-2010. Resultados: Dos 50 eventos que motivaram a suspeita de hipertermia maligna e a investigação familiar (amostra com 27 ± 18 anos, 52% homens, 76% brancos), 64% foram investigados por crise de hipertermia maligna anestésica, com mortalidade de 25%. Sinais mais comuns da crise de hipertermia maligna foram hipertermia, taquicardia e rigidez muscular. Suscetibilidade à hipertermia maligna foi confirmada em 79,4% dos 92 parentes investigados com teste de contratura muscular in vitro. Conclusão: Crises de hipertermia maligna assemelharam-se às descritas em outros países, porém com frequência inferior à estimada no país.

Tiroidectomía total en paciente con síndrome de McArdle: manejo anestésico / Total thyroidectomys in patient with McArdle's syndrome: anesthetic management

La enfermedad de McArdle o glucogenosis de tipo V es una miopatía metabólica rara que consiste en pérdida muscular y debilidad. Los pacientes con esta enfermedad presentan riesgos asociados a la anestesia. Pueden presentar hipoglucemia, rabdomiólisis, fallo renal agudo, alteraciones iónicas y también se ha relacionado con una mayor incidencia de hipertermia maligna durante el procedimiento anestésico. La compresión intermitente debido a la medición de la presión no invasiva, las posturas en la mesa de quirófano que puedan provocar contracturas musculares o el temblor ocasionado por la hipotermia o por la misma anestesia pueden desencadenar rabdomiólisis en estos pacientes. En este artículo exponemos nuestra experiencia con una paciente con síndrome de McArdle bajo anestesia general para tiroidectomía total por bocio multinodular eutiroideo

McArdle disease or type V glycogenosis is a rare metabolic myopathy consisting of muscle loss and weakness. These patients have risks associated with anaesthesia. They can present with hypoglycaemia, rhabdomyolysis, acute renal failure, and electrolyte changes. It has also been associated with a higher incidence of malignant hyperthermia during the anaesthetic procedure. Intermittent compression due to the measurement of non-invasive pressure, postures on the operating table that may cause muscle contractures, or tremor caused by hypothermia or anaesthesia itself, may trigger rhabdomyolysis in these patients. In this article we present our experience in submitting a patient with McArdle's syndrome to general anaesthesia for total thyroidectomy due to multinodular euthyroid goitre

Association between known or strongly suspected malignant hyperthermia susceptibility and postoperative outcomes: an observational population-based study.

PURPOSE: Whether current standards of care management for malignant hyperthermia (MH)-susceptible patients result in acceptable postoperative clinical outcomes at a population level is not known. Our objective was to determine if patients with susceptibility to MH experienced similar outcomes as patients without MH susceptibility after surgery under general anesthesia. METHODS: This was a retrospective, population-based cohort study from 1 April 2009 until 31 March 2016 in the Canadian province of Ontario. Participants were adults who underwent common in- or outpatient surgeries under general anesthesia. The exposure studied was either known or strongly suspected MH susceptibility as determined by usage of a specific physician billing code. The primary outcome was a composite of all-cause death, hospital readmission, or major postoperative complications, all within 30 postoperative days. Separate analyses were employed, based on whether a patient had in- or outpatient surgery. Inverse probability of exposure weighting based on the propensity score was used to estimate adjusted exposure effects. RESULTS: The cohort included 957,876 patients (583,254 in- and 374,622 outpatients). There were 2,900 (0.3%) patients with a known or strong suspicion of MH susceptibility. For inpatients, the primary outcome occurred in 146,192 (25.1%) of the non-MH-susceptible group and in 337 (20.1%) of the MH-susceptible group (unadjusted risk difference [RD], -5.0%; 95% confidence interval [CI], -6.9 to -3.1%; P < 0.001). In outpatients, the primary outcome occurred in 9,146 (2.4%) of the non-MH-susceptible group and in 32 (2.6%) of the MH-susceptible group (RD, 0.2%; 95% CI, -0.7 to 1.1%; P = 0.72). After adjustment, MH susceptibility was not associated with the primary outcome in either the inpatients (adjusted risk difference [aRD], 1.2%; 95% CI, -1.3 to 3.6%; P = 0.35) or outpatients (aRD, -0.1%; 95% CI -1.0 to 0.9%; P = 0.90). CONCLUSIONS: Among adults in Ontario who underwent common surgeries under general anesthesia from 2009 to 2016, known or strongly suspected MH was not associated with a higher risk of adverse postoperative outcomes. These findings support the current standard of care management for MH-susceptible patients.

RéSUMé: OBJECTIF: Nous ignorons si les normes actuelles de gestion des soins de patients susceptibles d'hyperthermie maligne (HM) aboutissent à des résultats cliniques postopératoires acceptables à l'échelle d'une population. Notre objectif a été de déterminer si des patients présentant une susceptibilité à l'HM présentaient une évolution comparable à celle des patients non connus susceptibles après chirurgie sous anesthésie générale. MéTHODES: Il s'agissait d'une étude de cohorte rétrospective, basée sur une population de la province canadienne de l'Ontario allant du 1er avril 2009 au 31 mars 2016. Les participants étaient des adultes, hospitalisés ou ambulatoires, ayant subi des interventions sous anesthésie générale. L'exposition étudiée était une susceptibilité à l'HM connue ou fortement suspectée, déterminée par l'utilisation d'un code de facturation spécifique des médecins. Le critère d'évaluation principal était un critère composite incluant les décès toutes causes confondues, les réadmissions hospitalières ou les complications postopératoires majeures qui étaient survenus dans un délai de 30 jours postopératoires. Des analyses séparées ont été utilisées, selon que les patients avaient été hospitalisés ou opérés en chirurgie d'un jour. La probabilité inverse de la pondération de l'exposition basée sur le score pour la propension a servi à estimer les effets ajustés de l'exposition. RéSULTATS: La cohorte a inclus 957 876 patients (583 254 patients hospitalisés et 374 622 patients ambulatoires). Parmi eux, 2 900 patients (0,3 %) avaient une susceptibilité à l'HM connue ou fortement suspectée. Pour les patients hospitalisés, le critère d'évaluation principal est survenu chez 146 192 (25,1 %) des patients du groupe non susceptible d'HM et chez 337 (20,1 %) patients du groupe susceptible d'HM (différence de risques [DR] non ajustée : −5,0 %; intervalle de confiance [IC] à 95 % : −6,9 % à −3,1 %; P < 0,001). Pour les patients ambulatoires, le critère d'évaluation principal est survenu chez 9 146 (2,4 %) des patients du groupe non susceptible d'HM et chez 32 (2,6 %) patients du groupe susceptible d'HM (différence de risques [DR] non ajustée : 0,2 %; IC à 95 % : −0,7 % à 1,1 %; P = 0,72). Après ajustement, la susceptibilité à l'HM ne s'est pas avérée associée au critère d'évaluation principal dans le groupe de patients hospitalisés (différence de risques ajustée [DRa], 1,2 %; IC à 95 % : −1,3 % à 3,6 %; P = 0,35) ou dans le groupe de patients ambulatoires (DRa : −0,1 %; IC à 95 % : −1,0 % à 0,9 %; P = 0,90). CONCLUSIONS: Parmi les adultes de la province de l'Ontario ayant subi des interventions chirurgicales usuelles sous anesthésie générale entre 2009 et 2016, l'HM connue ou fortement suspectée n'a pas été associée à un plus grand risque d'évolution postopératoire défavorable. Ces constatations sont en faveur du maintien des normes des soins actuels pour la gestion des patients susceptibles d'HM.

Efficacy of Malignant Hyperthermia Association of the United States-Recommended Methods of Preparation for Malignant Hyperthermia-Susceptible Patients Using Dräger Zeus Anesthesia Workstations and Associated Costs.

BACKGROUND: The objective of this study was to assess the efficacy and cost of Malignant Hyperthermia Association of the United States-recommended methods for preparing Dräger Zeus anesthesia workstations (AWSs) for the malignant hyperthermia-susceptible patient. METHODS: We studied washout profiles of sevoflurane, isoflurane, and desflurane in 3 Zeus AWS following 3 preparation methods. AWS was primed with 1.2 minimum alveolar concentration anesthetic for 2 hours using 2 L/min fresh gas flow, 500 mL tidal volume, and 12/min respiratory rate. Two phases of washout were performed: high flow (10 L/min) until anesthetic concentration was <5 parts per million (ppm) for 20 minutes and then low flow (3 L/min) for 20 minutes to identify the rebound effect. Preparation methods are as follows: method 1 (M1), changing disposables (breathing circuit, soda lime, CO2 line, and water traps); method 2 (M2), M1 plus replacing the breathing system with an autoclaved one; and method 3 (M3), M1 plus mounting 2 activated charcoal filters on respiratory limbs. Primary outcomes are as follows: time to obtain anesthetic concentration <5 ppm in the high-flow phase, peak anesthetic concentrations in the low-flow phase, and for M3 only, peak anesthetic concentration after 70 minutes of low-flow phase, when activated charcoal filters are removed. Secondary outcomes are as follows: cost analysis of time and resources to obtain anesthetic concentration <5 ppm in each method and a vapor-free Zeus AWS. Sensitivity analyses were performed using alternative assumptions regarding the costs and the malignant hyperthermia-susceptible caseload per year. RESULTS: Primary outcomes were as follows: M3 instantaneously decreased anesthetic concentration to <1 ppm with minimal impact of low-flow phase. M1 (median, 88 minutes; 95% confidence interval [CI], 69-112 minutes) was greater than M2 (median, 11 minutes; 95% CI, 9-15 minutes). Means of peak rebound anesthetic concentrations in M1, M2, and M3 were 15, 6, and 1 ppm, respectively (P < .001). Anesthetic concentration increased 33-fold (95% CI, 21-50) after removing charcoal filters (from 0.7 to 20 ppm). The choice of anesthetic agents did not impact the results. Secondary outcomes were as follows: M3 was the lowest cost when the cost of lost operating room (OR) time due to washout was included, and M1 was the lowest cost when it was not included. When the cost of lost OR time due to washout was considered the estimated cost/case of M3 was US $360 (M1, US $2670; M2, US $969; and a "vapor-free" Zeus AWS was US $930). The OR time and equipment costs represent the largest differentiators among the methods. CONCLUSIONS: Institutions in which demand for OR time has exceeded capacity should consider M3, and institutions with surplus OR capacity should consider M1.

Oral Management of Steinert's Disease and Role of Anxiolysis.

BACKGROUND: Myotonic dystrophy type I (DM1) is a genetic autosomal dominant disorder; malignant hyperthermia is a possible complication. It may occur following administration of some halogenated general anesthetics, muscle relaxants, or surgical stress. AIM: The purpose of this case report is to evaluate the dental management of patients with Steinert's disease. CASE REPORT: The patient needed dental extraction. A locore-gional paraperiosteal anesthesia was performed using bupiva-caine without vasoconstrictor and sedation with nitrous oxide. The syndesmotomy of the elements 3.1, 4.1, and 4.2 was executed. The elements were dislocated through a straight lever and avulsed with an appropriate clamp. The socket was courted, washing with saline solution, inserting a fibrin sponge, and applying sutures (silk 3-0). CONCLUSION: Dental treatment of the patient with Steinert's dystrophy must be carried out under a hospital environment and the use of local anesthetic without vasoconstrictor and with use of nitrous oxide; anxiolysis is recommended. CLINICAL SIGNIFICANCE: This case report describes the precautions to perform oral surgery in patients with Steinert's disease and emphasizes the role of anxiolysis to avoid episodes of malignant hyperthermia.

Malignant Hyperthermia in the Post-Genomics Era: New Perspectives on an Old Concept.

This article reviews advancements in the genetics of malignant hyperthermia, new technologies and approaches for its diagnosis, and the existing limitations of genetic testing for malignant hyperthermia. It also reviews the various RYR1-related disorders and phenotypes, such as myopathies, exertional rhabdomyolysis, and bleeding disorders, and examines the connection between these disorders and malignant hyperthermia.