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Evaluation of Suspected Malignant Hyperthermia Events During Anesthesia Frank Schuster*, Stephan Johannsen, Daniel Schneiderbanger and Norbert Roewer

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Evaluation of Suspected Malignant Hyperthermia Events During Anesthesia Frank Schuster*, Stephan Johannsen, Daniel Schneiderbanger and Norbert Roewer Schuster et al. BMC Anesthesiology 2013, 13:24 http://www.biomedcentral.com/1471-2253/13/24 RESEARCH ARTICLE Open Access Evaluation of suspected malignant hyperthermia events during anesthesia Frank Schuster*, Stephan Johannsen, Daniel Schneiderbanger and Norbert Roewer Abstract Background: Malignant hyperthermia (MH), a metabolic myopathy triggered by volatile anesthetics and depolarizing muscle relaxants, is a potentially lethal complication of general anesthesia in susceptible patients. The implementation of modern inhalation anesthetics that research indicates as less potent trigger substances and the recommended limitations of succinylcholine use, suggests there may be considerable decline of fulminant MH cases. In the presented study, the authors analyzed suspected MH episodes during general anesthesia of patients that were referred to the Wuerzburg MH unit between 2007 and 2011, assuming that MH is still a relevant anesthetic problem in our days. Methods: With approval of the local ethics committee data of patients that underwent muscle biopsy and in vitro contracture test (IVCT) between 2007 and 2011 were analyzed. Only patients with a history of suspected MH crisis were included in the study. The incidents were evaluated retrospectively using anesthetic documentation and medical records. Results: Between 2007 and 2011 a total of 124 patients were tested. 19 of them were referred because of suspected MH events; 7 patients were diagnosed MH-susceptible, 4 MH-equivocal and 8 MH-non-susceptible by IVCT. In a majority of cases masseter spasm after succinylcholine had been the primary symptom. Cardiac arrhythmias and hypercapnia frequently occurred early in the course of events. Interestingly, dantrolene treatment was initiated in a few cases only. Conclusions: MH is still an important anesthetic complication. Every anesthetist must be aware of this life- threatening syndrome at any time. The rapid onset of adequate therapy is crucial to avoid major harm and possibly lethal outcome. Dantrolene must be readily available wherever MH triggering agents are used for anesthesia. Keywords: Malignant hyperthermia, In vitro contracture test, Succinylcholine, Volatile anesthetics Background [2,3]. In contrast to fulminant MH episodes, abortive Malignant hyperthermia (MH) is mostly an inherited courses might occur more frequently, but are difficult to subclinical myopathy triggered by volatile anesthetics diagnose due to the alleviated symptoms. and depolarizing muscle relaxants in susceptible individ- Recent developments in anesthesiology apparently uals, leading to a potentially lethal hypermetabolic reaction have lead to a decrease in severe MH crisis over the last of skeletal muscle due to a disturbance of myoplasmic cal- years: Halothane, a potent MH triggering agent, is no cium homeostasis. Characteristic clinical signs of MH dur- longer used in clinical routine in western countries [4] ing a general anesthesia include hypoxemia, hypercapnia, and currently applied volatile anesthetics, e.g. isoflurane, tachycardia, muscular rigidity, acidosis, hyperkalemia and sevoflurane or desflurane, in some cases significantly hyperthermia [1]. While expected genetic predisposition for decelerate the onset of an MH reaction compared to MH is stated to be as frequent as 1:2.000, the prevalence of halothane [5,6] and are more likely to lead to abortive MH MH episodes varies regionally from 1:10.000 to 1:220.000 with eased symptoms. Furthermore, the recommended indications for succinylcholine, another possible MH trig- gering agent, have been limited by international anesthesia * Correspondence: [email protected] Department of Anesthesia and Critical Care, University of Wuerzburg, societies [7]. Oberduerrbacher Str. 6, D-97080 Wuerzburg, Germany © 2013 Schuster et al.; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Schuster et al. BMC Anesthesiology 2013, 13:24 Page 2 of 7 http://www.biomedcentral.com/1471-2253/13/24 Considering all these facts, the aim of the present University of Wuerzburg. Overall 19 of these patients had study was to investigate, whether MH is still a relevant been referred to the MH unit because of a suspected MH anesthetic problem in our days. event during general anesthesia on the basis of estimation of the attending anesthesiologists. In the remaining pa- Methods tients MH diagnostics were initiated due to MH suscepti- With approval of the local ethics committee (application bility in the family history, an unexplained rhabdomyolysis number: 263/11, ethics committee of the University of or to exclude a myopathic disorder in association with Wuerzburg) data of patients who where referred to the persistently elevated CK levels. MH unit of the Department of Anesthesia and Critical Care of the University of Wuerzburg for diagnostic muscle Diagnostic findings biopsy and subsequent in vitro contracture testing (IVCT) Muscle biopsy and IVCT detected MH susceptibility in 7 between 2007 and 2011 were evaluated. Based on available (37%; 7 male) of the 19 patients. In 8 patients (42%; 3 patient documents and medical records the intraoperative male, 5 female) MH susceptibility could be excluded. events were examined. To confirm the suspicion of an Muscle bundles of 4 patients (21%; 1 male, 3 female) MH crisis, applied triggering agents, clinical symptoms developed a pathologic contracture only after exposure to e.g. cardiac arrhythmia, increase of end-tidal carbon halothane but not after caffeine (MHEh). Interestingly, ini- dioxide ≥ 45 mmHg, rises of patients’ body temperature tial applied CGS rated the probability of an MH crisis as ≥ 38.5°C and possible use of dantrolene were analyzed. “almost certain” (> 50 points) in 2 MHS patients and “very Besides that, the medical records were reviewed for severe likely” (35 – 49 points) in 5 MHS and 1 MHEh patients, postoperative complications, e.g. neurological deficits, dis- while in 3 MHEh patients the likelihood was classified as seminated intravascular coagulation (DIC), acute renal “less than likely” (10 - 19 points). Noteworthy, MH was failure or signs of rhabdomyolysis according to maximum assumed “greater than likely” (20 – 34 points) in 6 MHN creatine kinase (CK) levels. If blood gas analysis was patients and “less than likely” or “almost never” in 1 MHN implemented, pH ≤ 7.2, base excess ≤ -5 mmol/l and patient each by CGS. Genetic screening detected muta- PaCO2 ≥ 50 mmHg defined a severe metabolic response. tions in the ryanodine receptor gene (Gly4037Alafs, Only patients with a suspected MH episode during general Glu2174Ala, Val4234Leu) of 3 MHS patients. In 16 (84%) anesthesia due to the estimation of the responsible anes- patients the suspected MH event occurred between 2006 thesiologist, completed IVCT and genetic analysis of the and 2010 (6 MHS, 3 MHEh, 7 MHN). The remaining 3 ryanodine receptor gene were included in the investigation. patients had been 10 years old or younger at the time of In referred patients a diagnostic IVCT with increasing incident (1992, 1995, 1998) and therefore muscle biopsy in caffeine and halothane concentrations in separated tissue these patients was delayed until the age of 16 years baths was performed according to the guidelines of the according to our hospital standard operating procedures. European MH Group [8]. A contracture ≥ 2mNatcaf- Since the MH diagnostic was performed within the study feine 2 mM and halothane 0.44 mM lead to the diagnosis period, these 3 patients were included in the evaluation, MH susceptible (MHS). If significant contractures oc- even if the applied triggers were halothane or enflurane re- curred after one of the drugs only, patients were classified spectively. Interestingly, 2 MHS individuals with suspected as MH equivocal (MHE, MHE for halothane (MHEh) or MH in their history had undergone at least one uneventful caffeine MHEc). If no significant contracture was observed general anesthesia in the past. The histopathological ex- the patients were rated MH non-susceptible (MHN). aminations revealed a myopathic tissue syndrome in com- In addition, for each patient, the clinical grading scale bination with cell clumps indicating a possibly neurogenic (CGS) by Larach and colleagues, which includes metabolic component in 1 MHEh patient, who had received suc- and muscular parameters as well as changes in cardiac cinylcholine as sole trigger agent. In the other patients rhythm and body temperature, was applied retrospectively. there was no evidence of a muscular pathology (Table 1). According to the grading scale 3 to 15 points were cal- culated for each parameter and added to receive a score. Trigger agents and clinical presentations This score allowed allocation to individual MH-ranks Only 21% of the MH suspected patients solely received (0=MHalmostnever,3-9=MHunlikely,10-19=MH an inhalation anesthetic (sevoflurane: 1 MHS; isoflurane: somewhat less than likely, 20-34 = MH somewhat 1 MHN; desflurane: 2 MHN), while in 47% of the cases greater than likely, 35-49 = MH very likely, > 50 = MH (6 MHS, 1 MHEh, 2 MHN) a combination of succinylcho- almost certain) [9]. line with a volatile anesthetic, e.g. halothane (1 MHEh), en- flurane (1 MHN) isoflurane (3 MHS, 1 MHN), sevoflurane Results (2 MHS) or desflurane (1 MHS) was used. In 28% Between 2007 and
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