Diagnosis and rescue of malignant hyperthermia induced by anesthesia during radical surgery in a cervical cancer patient using the National Remote Emergency System: A case report.

RATIONALE: Malignant hyperthermia (MH) is a rare yet serious medical complication that typically arises following general anesthesia or the administration of specific anesthetics. Due to the infrequency of MH, anesthesiologists often lack sufficient expertise in identifying and managing it, leading to misdiagnosis and inappropriate treatment. There is an urgent need to enhance the diagnosis and management of MH through the utilization of relevant tools. PATIENT CONCERNS: In this case, a 52-year-old woman underwent radical cervical cancer surgery under general anesthesia, with no family or significant medical history. She experienced a gradual increase in end-tidal carbon dioxide (ETCO2) to a maximum of 75 mm Hg and a rise in body temperature from 36.5 to 37.5 °C in a very short period, as well as a blood gas analysis showing a pH of 7.217. DIAGNOSIS: The anesthesiologist immediately used The WeChat applet-based National Remote Emergency System for Malignant Hyperthermia (MH-NRES), and the score was 40, which indicated that the patient was very likely to have MH. INTERVENTIONS: We immediately discontinued sevoflurane and switched total intravenous anesthesia to maintain general anesthesia, with a rapid intravenous infusion of dantrolene sodium. OUTCOMES: The ETCO2 and the temperature quickly dropped to normal, followed by successful completion of the surgery, and the patient was discharged 8 days after surgery. LESSONS: The experience can provide a basis use of MH-NRES and improve the ability of anesthesiologists to deal with intraoperative MH as well as increase the survival probability of patients.

Malignant hyperthermia in maxillofacial surgery: Literature review supported by case presentation.

OBJECTIVE: Malignant hyperthermia (MH) is characterized by a state of hypermetabolism after exposure to halogenated inhalational anesthetics or succinylcholine. The aims of this study were to carry out an updated review on the subject and report an illustrative case of MH in urgent maxillofacial surgery. MATERIAL AND METHODS: A search of the PubMed/MEDLINE database using the keyword "malignant hyperthermia" was performed including articles published over the last 11 years in English, Spanish or Portuguese. Exclusion criteria were similar presentations but not associated with MH and cases not related to the use of anesthetic drugs as a trigger of the condition. CASE REPORT: A 45-year-old man (75 kg, ASA status IE) with a negative family history for neuromuscular diseases, victim of a car accident with a facial fracture, underwent surgery under balanced general anesthesia and developed signs of MH 4 h after anesthesia induction. In our patient, the causative agent was sevoflurane and the diagnosis of MH was confirmed, subsequently, by muscle biopsy. RESULTS/DISCUSSION: Overall, 44 cases of MH were found. According to the recent literature, MH shows a male predilection (3:1) and the mean age of patients is 32.2 ± 22.2 years. The most frequently cited causative agents were sevoflurane (30.5%), isoflurane (22.2%), and sevoflurane + succinylcholine (13.8%). The most common clinical indicators included hypercarbia (88.8%), hyperthermia (86.1%), and tachycardia (63.8%). Dantrolene was administered in 24 cases. The outcome was favorable in 31 cases (86.1%). The in vitro muscle contracture test (IVCT) was performed in only 15 patients and all of them tested positive. In our patient, the causative agent was sevoflurane and the diagnosis of MH was confirmed by muscle biopsy. CONCLUSION: The mortality from MH is still high and an early clinical diagnosis and specific treatment with dantrolene are necessary for a favorable outcome. A complete understanding will allow better management of patients with MH. At present, the best management is to identify susceptible patients and to avoid triggering agents, combined with vigilant monitoring.

Management of patients susceptible to malignant hyperthermia: A surgeon's perspective.

OBJECTIVES: Malignant hyperthermia (MH) susceptibility caries broad implications for the care of pediatric surgical patients. While precautions must often be taken for only a vague family history, two options exist to assess MH-susceptibility. We evaluate the use of MH precautions and susceptibility testing at a freestanding children's hospital. METHODS: This single institution retrospective cohort study identified patients of any age who received general anesthetics utilizing MH precautions over a five-year period. The electronic medical record was further queried for patients diagnosed with MH. The indication for MH precautions and uses of susceptibility testing are assessed. Secondary outcomes included a diagnosis of bona fide MH. RESULTS: A total of 125 patients received 174 anesthetics with MH precautions at a mean age of 114 months (0-363 months). Otolaryngology was the procedural service most frequently involved in the care of the cohort (n = 45; 26%). A reported personal or family history of MH (n = 102; 59%) was the most common indication for precautions, followed by muscular dystrophy (n = 29; 17%). No MH events occurred in the cohort and further review of ICD-9 and -10 diagnosis codes found no MH diagnoses. No study subjects received muscle biopsy and contracture testing and only 5 (4%) underwent genetic testing for genomic variants known to cause MH susceptibility. A case example is given to highlight the implications of a reported MH history. CONCLUSION: Otolaryngologists should maintain a familiarity with the precautions necessary to manage patients at risk for MH and MH-like reactions. Without an accessible test to rule out susceptibility, surgeons must rely on a careful history to appropriately utilize precautions. An inappropriate label of "MH-susceptible" may result in decreased access to care and treatment delays.

Malignant hyperthermia in Mazovia Province - are we adequately prepared?

INTRODUCTION: Malignant hyperthermia (MH) is a life-threatening syndrome caused by sudden skeletal muscle hypermetabolism in response to inhalation anaesthetics and depolarising relaxants. The estimated incidence of MH is between 1 : 10,000 and 1 : 250,000 anaesthetic procedures. In Poland the incidence of MH is unknown. Dantrolene is imported as a life-saving drug and temporally authorised for sale. The aim of the study is to assess the incidence of MH and access to dantrolene in the Mazovia Province. METHODS: Anonymous questionnaires were sent to anaesthesia departments in the Mazovia Province after prior contact by phone and e-mail. The survey was approved by the local ethical review board. RESULTS: Completed surveys were received from 60 respondents which represents 72% of anaesthesiology departments in Mazovia. In the last 5 years there have been 4 episodes of MH in the Mazovia Province. Three patients survived the MH crisis. In a centre that did not have access to dantrolene, the patient died. Dantrolene is found only in 11 (18.3%) anaesthesiology departments in Mazovia. Only 6 (10%) hospitals are able to administer dantrolene within 5 minutes of suspecting MH crisis, while 5 centres may receive it after a few days. Only 38% of units have an algorithm for dealing with MH crisis in the operating theatres. CONCLUSIONS: MH is rare, but if untreated, it can be fatal. Therefore prompt diagnosis and treatment are crucial to avoid fatal outcome. Every centre using inhalational anaesthetics and/or succinylcholine should have dantrolene. To ensure the safety of our patients, we must be better prepared.

Anaesthetic management of a parturient patient with a malignant hyperthermia partner.

Malignant hyperthermia (MH) is a rare but deleterious anaesthetic emergency that has an autosomal dominant inheritance. Successful management of the MH-susceptible fetus hinges on early suspicion and preparation. This case highlights the importance of knowing paternal anaesthetic risk as well as maternal in the parturient population. Paternal anaesthetic history is paramount in this situation, especially with a normal maternal risk, and preparation of the patient, staff and equipment is at the centre of the peripartum management of these patients.

Malignant Hyperthermia: A Killer If Ignored.

Malignant hypothermia (MH) is a potentially fatal hypermetabolic reaction of skeletal muscle. It is an autosomal dominant disorder that generally occurs in people with RYR1, CACNA1S, or STAC3 mutations. And these genetic abnormalities often cause the imperfection of calcium release channels of skeletal muscle. The incidence of MH among different racial groups across the world ranges from approximately 1:5,000-1:250,000, but there is no national statistic MH incidence in China. It is not clear whether there are racial or regional differences in the incidence, but patients under 18 years old may be more affected. MH can be triggered by anesthetics, or other stimuli, such as strenuous exercise, heat-stroke, and emotional stress. While viral infection, statins, hyperglycemia, and muscle metabolic dysfunctions might accelerate the onset of MH. The onset of MH is insidious and rapid, with the preclinical stage characterized by rigidity of the masseter muscle, a high level of end-tidal carbon dioxide, and a sharp and persistent increase in body temperature. Medical history, family history, clinical presentation, in vitro caffeine-halothane contracture testing (IVCT/CHCT) and genetic testing are commonly diagnostic methods of MH. As soon as the onset of MH is suspected, immediate cessation of exposure to stimuli, call for professional support, and access to dantrolene are the highest priorities. For symptomatic treatment, "5C principles" were summarized as an algorithm to guide clinicians.

Malignant Hyperthermia Preparedness Training: Using Cognitive Aids and Emergency Checklists in the Perioperative Setting.

PURPOSE: The purpose of this project was to prepare perioperative staff at a military ambulatory surgical center to respond to a malignant hyperthermia crisis effectively with the assistance the Stanford emergency manual cognitive aid. DESIGN: This is an evidence-based practice improvement project. METHODS: Multidisciplinary perioperative staff at a military ambulatory surgical center participated in an in-service about the Stanford emergency manual cognitive aid tool, and a simulation exercise was conducted to practice using the tool during a malignant hyperthermia crisis. Preproject and postproject implementation surveys were to used to measure staff perceptions of and willingness to use cognitive aids/emergency checklists during perioperative emergencies. FINDINGS: Staff positive perception of and willingness to use the Stanford emergency manual cognitive aid improved after implementation of an education session and simulation exercise with the checklist tool. CONCLUSIONS: Introduction of the Stanford emergency manual cognitive aid to multidisciplinary perioperative staff with an in-service and simulated malignant hyperthermia scenario improved staff perception of cognitive aid use during emergencies. Use of cognitive aid checklists during simulated perioperative emergencies was shown to reduce missed critical treatment steps.

Total Intravenous Anesthesia in a Malignant Hyperthermia-Susceptible Patient with Klinefelter Syndrome Undergoing Orthognathic Surgery.

ABSTRACT: A patient with Klinefelter syndrome and skeletal Class III malocclusion experienced a malignant hyperthermia-like reaction while undergoing orthognathic surgery. The patient fully recovered after prompt diagnosis and management, and surgery was reattempted under total intravenous anesthesia. The patient was discharged without any anesthetic complications and was satisfied with the surgical results. This is the first described case of a malignant hyperthermia-like event in a patient with Klinefelter syndrome. Total intravenous anesthesia may be safely administered in malignant hyperthermia-susceptible patients who require orthognathic surgery.

A case report of malignant hyperthermia in a patient with myotonic dystrophy type I: A CARE-compliant article.

RATIONALE: Several hereditary myopathies that can predispose to malignant hyperthermia (MH) are reported. However, the risk of MH in myotonic dystrophy type I (DM1) has been suggested equal to general population, although the evidence is limited to only a few case reports. PATIENT CONCERNS: We encountered a rare case of MH during anesthesia induction with sevoflurane in a male adolescent with previously undiagnosed DM1. DIAGNOSES: After the event, genetic testing revealed the presence of a previously unknown heterozygous missense mutation in ryanodine receptor 1 (RYR1) associated with MH (c.6898T > C; p.ser2300Pro). Concomitantly, the patient was diagnosed with DM1 with abnormal cytosine-thymine-guanine triplet expansion in the DMPK gene. INTERVENTIONS: Dantrolene was administered to treat the hypermetabolic manifestations in 20 minutes after the identification of MH. OUTCOMES: The patient was successfully treated and discharged without any complications. Laboratory abnormalities were recovered to baseline at postoperative 4 days. LESSONS: The authors suggest that possible MH susceptibility in DM1 patients may be refocused. Genetic testing can be a screening tool for MH susceptibility in these population, prior to receiving general anesthesia.

[Malignant Hyperthermia and Pregnancy - Guidelines of the European Malignant Hyperthermia Group]. / Maligne Hyperthermie und Schwangerschaft ­ Empfehlungen der Europäischen Malignen Hyperthermie Gruppe.

Malignant hyperthermia is a rare, subclinical pharmacogenetic syndrome leading to potentially life-threatening skeletal muscle hypermetabolism. Providing a safe and trigger-free anesthesia in predisposed individuals is essential to avoid serious harm to the patient. Especially the management of malignant hyperthermia predisposition in the context of pregnancy poses a huge challenge to the attending anesthesiologist. In May 2019 the European Malignant Hyperthermia Group published a guideline on malignant hyperthermia during pregnancy. The article summarizes and discusses the recommendations and provides practical advice for treatment of pregnant women or their fetus with known or suspected susceptibility to malignant hyperthermia.

Pathophysiology and Treatment of Malignant Hyperthermia.

Malignant hyperthermia (MH) is caused by a genetic disorder of the skeletal muscle that induces a hypermetabolic response when patients are exposed to a triggering agent such as volatile inhaled anesthetics or depolarizing neuromuscular blockers. Symptoms of MH include increased carbon dioxide production, hyperthermia, muscle rigidity, tachypnea, tachycardia, acidosis, hyperkalemia, and rhabdomyolysis. Common scenarios for triggering agents are those used are during surgery and rapid sequence intubation. Hypermetabolic symptoms have a rapid onset; hence, prompt recognition and treatment are vital to prevent morbidity and mortality. The first-line treatment agent for an MH response is dantrolene. Further treatment includes managing complications related to a hypermetabolic response such as hyperkalemia and arrhythmias. This review is focused on the recognition and treatment considerations of MH in the emergency department to optimize therapy and improve patient morbidity and mortality.

The Use of Continuous Blood Purification for the Treatment of Malignant Hyperthermia in an Infant.

BACKGROUND: Malignant hyperthermia (MH) is a rare and potentially life-threatening pharmacogenetic disorder encountered during general anesthesia, with the incidence higher in children than in adults. Dantrolene is the specific antagonist of MH, but it is not readily available in China, thus developing alternative treatment protocols is of great practical importance. CASE PRESENTATION: Herein, the authors report a two-month-old infant who underwent holmium laser epiglottis retrofitting through a bronchoscope, but developed limb muscular stiffness, tachypnea, tachycardia, and hyperthermia after sevoflurane exposure. After the diagnosis of MH, corresponding supportive treatment was implemented. Because there was no dantrolene available, continuous blood purification and mechanical ventilation were performed. A few days later, the boy recovered without any complications. CONCLUSION: Based on the authors' successful clinical practice, the authors consider continuous blood purification as a reliable treatment for MH. But its feasibility still needs to be clarified after multicenter clinical observations.

Malignant hyperthermia during pediatric kidney transplantation-A medical and ethical quandary.

Malignant hyperthermia (MH) is a rare life-threatening anesthetic complication with high mortality rates. MH during adult kidney transplant has been reported previously. However, the occurrence of MH after multiple previous uneventful anesthetic exposures in a pediatric kidney transplant recipient is rare. To our knowledge, this is the first reported case of MH in a child undergoing a live donor kidney transplant. The approaches for addressing perioperative challenges and ethical dilemmas to ensure successful outcomes are described. The recipient, a 5-year-old male child, weighing 20 kg, with a history of multiple previous uneventful anesthetic exposures, underwent live donor kidney transplant for end-stage renal disease (ESRD). Post-reperfusion he developed fulminant MH with rapidly progressing hyperthermia, hypercarbia, tachycardia, and muscle rigidity, which in addition to complicating the medical management raised several ethical issues as well. MH was successfully managed with dantrolene and other supportive measures. Judicious use of inotropes and fluids helped maintain stable hemodynamics and graft perfusion. Management of MH is complicated in a pediatric patient with ESRD undergoing live donor kidney transplant. Preference for non-depolarizing muscle relaxants instead of succinylcholine during endotracheal intubation can result in delayed onset of clinical manifestations. However, the metabolic complications may be more severe due to preexisting electrolyte and acid-base disturbances. Maintaining optimal graft perfusion while simultaneously combating MH can be very challenging in a child. Since the allograft is a precious commodity, critical decisions regarding the harvesting of the donor kidney need to be well thought out. Early diagnosis and prompt treatment with dantrolene are critical to preserving graft function and the recipient's life.

Hipertermia maligna / Malignant huperthermia

Carriers of malignant hyperthermia (MH), a subclinical metabolic myopathy, respond differently to the general population in response to triggering agents, such as volatile anesthetics and succinylcholine. Its incidence ranges from 1:10,000 to 1:250,000 anesthesias. Using etiological treatment, the current mortality rate is around 5%. The biochemical, metabolic, and physiological deterioration generally associated with MH is a direct result of a sudden and progressive increase in intracellular calcium in striated muscle cells. This generates a hypermetabolic state, with a rapid increase in body temperature that can lead to a fatal outcome if not diagnosed and treated in time. The genetic factors that determine susceptibility to MH are complex, with the participation of more than one gene. Its clinical symptoms are highly variable, from mild or moderate to fulminant attacks with severe muscle hypermetabolism and rhabdomyolysis. Capnography and pulse oximetry have great clinical diagnostic value. Other early symptoms of an MH attack may include sinus tachycardia, supraventricular or ventricular arrhythmia, and isolated masterean spasm or generalized muscle stiffness. The rise in temperature is a late sign. After an attack, or in possibly susceptible patients, the laboratory diagnosis is made with the in vitro contracture test, in which the contraction of muscle fibers, obtained through a skeletal muscle biopsy, is studied in the presence of halothane or caffeine. In patients known to be susceptible to MH, neuraxial and regional techniques should be preferred if surgery allows it; otherwise, trigger-free anesthetic methods (TIVA) should be available. Management of the MH crisis is based on three main actions: 1) stopping the administration of halogenates; 2) hyperventilation with 100% oxygen, and 3) administration of intravenous dantrolene.

La hipertermia maligna (HM) es una miopatía metabólica subclínica, cuyos portadores tienen una respuesta diferente a la población general ante la presencia de un agente desencadenante: anestésicos volátiles y succinilcolina. Su incidencia tiene rangos entre 1:10.000 a 1:250.000 anestesias. Su mortalidad actual usando tratamiento etiológico es de 5%. El deterioro bioquímico, metabólico y fisiológico asociado clásicamente al cuadro de HM es el resultado directo de un aumento súbito y progresivo del calcio intracelular de las células musculares estriadas, que genera un estado hipermetabólico, calor y un rápido aumento de la temperatura corporal, que puede llevar a un desenlace fatal si no se diagnostica y se trata a tiempo. Su herencia es complicada: se trata de una transmisión multigénica, en que la susceptibilidad a la HM depende de más de un gen. Los síntomas clínicos son muy variables, desde leves o moderados hasta crisis fulminantes con hipermetabolismo muscular severo y rabdomiólisis. La capnografía y la oximetría tiene un gran valor diagnóstico clínico. Otros síntomas tempranos de una crisis de HM pueden incluir taquicardia sinusal, arritmia supraventricular o ventricular y espasmo de maséteros aislado o rigidez muscular generalizada; el aumento de la temperatura es un signo tardío. Después de la crisis o en los pacientes posiblemente susceptibles, el diagnóstico de laboratorio se hace con el test de contractura , basado en la contracción de fibras musculares tomadas a partir de una biopsia de músculo estriado en presencia de halotano o cafeína. En los pacientes conocidamente susceptibles a HM se debe preferir las técnicas neuroaxiales y regionales si la cirugía lo permite; en caso contrario, debe disponerse de métodos anestésicos libres de agentes desencadenantes (TIVA). El manejo de la crisis de HM está basado en tres medidas principales: 1) la detención de la administración de halogenados; 2) la hiperventilación con oxígeno al 100% y 3) la administración de dantrolene endovenoso.