Halliday Norman J
Department of Anesthesiology, University of Miami, Miami, FL 33136, USA.
J Craniofac Surg. 2003 Sep;14(5):800-2. doi: 10.1097/00001665-200309000-00039.
Malignant Hyperthermia (MH) has been a recognized complication of general anesthesia after the first case reports in the 1940's. Since then a great deal has been discovered about the genetics, pathophysiology and treatment of this once fatal syndrome. MH is the only clinical entity specifically related to and caused by anesthetic agents. MH once triggered during anesthesia results in a profound hyper metabolic state with rise in the core temperature, increased carbon dioxide production and oxygen consumption. Death will ensue if specific treatment is not started. The incidence of fulminant MH ranges from 1:62,000 to 1: 84,000 of general anesthesia cases if succinylcholine and inhalation agents are used. Massseter muscle spasm on induction of anesthesia, with an incidence of between 1:16,000 and 1:4,000, may be a predromal indication of the development of MH. Anesthetic agents, which may trigger MH in susceptible individuals, are the depolarizing muscle relaxant, succinyl choline and all the volatile anesthetic gasses. Nitrous oxide, intravenous induction agents, benzodiazepines, opioids, and the non-depolarizing relaxants do not trigger MH. MH susceptibility is associated with certain disorders, such as Duchene muscular dystrophy, and triggering agent should not be used in these patients. Inheritance is an autosomal dominant trait with variable penetrance. The pathogenesis of MH involves the loss of control of intracellular calcium ions in skeletal muscle with resultant protracted spasm and hyper metabolism. Clinically this will progress to hypercarbia, hypoxia, hyperthermia, hyperkalemia and death will result if specific treatment is not started. Management involves immediate discontinuation of the triggering anesthetics, hyperventilation with 100% oxygen and most importantly the definitive treatment with intravenous dantrolene.The importance of instigating the use of dantrolene in cases of MH cannot be overemphasized. MH is now treatable when once it would be fatal before the availability of dantrolene. Unless of an emergent nature, surgery should be canceled following the acute phase of MH. The patient should be admitted to intensive care for at least 24 hours and dantrolene continued as recurrence has been described. It is imperative that the patient and their family are counseled, Medalert bracelets provided and registration with the Malignant Hyperthermia Association of the United States (MHAUS), encouraged. The caffeine/halothane testing of muscle biopsies is currently the most definitive test for malignant hyperthermia susceptibility. The routine use in suspected cases or the immediate family of known cases remains a matter of contention.
恶性高热(MH)自20世纪40年代首次报道以来,一直被认为是全身麻醉的一种并发症。从那时起,人们对这种曾经致命的综合征的遗传学、病理生理学和治疗方法有了很多发现。MH是唯一一种与麻醉剂有特定关联并由其引起的临床病症。麻醉期间一旦触发MH,就会导致严重的高代谢状态,核心体温升高,二氧化碳生成增加和耗氧量增加。如果不开始进行特异性治疗,将会导致死亡。如果使用琥珀酰胆碱和吸入性麻醉剂,暴发性MH的发生率在全身麻醉病例中为1:62,000至1:84,000。麻醉诱导时咬肌痉挛的发生率在1:16,000至1:4,000之间,可能是MH发生的前驱指征。可能在易感个体中触发MH的麻醉剂是去极化肌松剂琥珀酰胆碱和所有挥发性麻醉气体。氧化亚氮、静脉诱导剂、苯二氮䓬类、阿片类药物和非去极化肌松剂不会触发MH。MH易感性与某些疾病有关,如杜氏肌营养不良症,这些患者不应使用触发剂。遗传方式为常染色体显性遗传,具有可变的外显率。MH的发病机制涉及骨骼肌细胞内钙离子控制丧失,导致持续性痉挛和高代谢。临床上这将进展为高碳酸血症、低氧血症、高热、高钾血症,如果不开始特异性治疗将会导致死亡。处理措施包括立即停用触发麻醉剂,用100%氧气进行过度通气,最重要的是用静脉注射丹曲林进行确定性治疗。在MH病例中倡导使用丹曲林的重要性无论如何强调都不为过。在丹曲林可用之前,MH一旦发生往往是致命的,而现在它是可治疗的。除非是紧急情况,在MH急性期过后应取消手术。患者应入住重症监护病房至少24小时,并持续使用丹曲林,因为已有复发的报道。必须对患者及其家属进行咨询,提供医疗警示手环,并鼓励他们在美国恶性高热协会(MHAUS)登记。目前,肌肉活检的咖啡因/氟烷试验是诊断恶性高热易感性最具决定性的检测方法。在疑似病例或已知病例的直系亲属中常规使用该检测方法仍存在争议。