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How to Avoid This Medical Emergency Gregory L. Rose, MD; Thomas McLarney, MD; Raeford E. Brown, MD How to avoid this University of Kentucky College of Medicine, Lexington medical emergency [email protected] Avoiding an episode of malignant hyperthermia requires The authors reported no potential confl ict of interest relevant to this article. that you look for certain clues in a patient’s history. ou are asked to perform a preoperative evaluation of a PRACTICE 6-year-old boy who is due to have a tonsillectomy. Th e RECOMMENDATIONS Y family history reveals that his father had an episode that › Suspect malignant hyper- sounds like malignant hyperthermia (MH). Also, a paternal thermia (MH) if a patient uncle experienced a high fever and almost died after undergo- has night sweats, cramping, ing anesthesia. Th e boy’s parents tell you they took the boy to mottled skin, low-grade fever, a pediatrician, who did a “blood test” for MH. Th ey hand you and excessive sweating, or has a written report of an enzyme-linked immunosorbent assay, elevated creatine kinase and rhabdomyolysis on lab which tested negative for an allergy to succinylcholine. studies. B Has this child been adequately screened for MH? If you answered No, you are correct. Th e review that fol- › Make sure patients and lows explains why. their family members know that MH is life threatening, familial, and can even occur in patients whose previous The “disease of anesthesia” experiences with anesthesia MH is a pharmacogenetic disease process that occurs when have been uneventful. A predisposed individuals are exposed to certain triggering agents—specifi cally, anesthetics. Succinylcholine and all Strength of recommendation (SOR) potent inhalational anesthetic agents have been implicated A Good-quality patient-oriented (TABLE 1). Most episodes occur in the intraoperative period. evidence B Inconsistent or limited-quality MH is a familial disease and follows an autosomal domi- patient-oriented evidence nant pattern, but with incomplete penetrance. Surprisingly, C Consensus, usual practice, the disease was not described until 1961, when Denborough opinion, disease-oriented evidence, case series et al reported a string of anesthetic-related deaths in a fam- ily.1,2 A similar condition was described in pigs in 1966.3 Th is condition, porcine stress syndrome, was noted during re- search in which pigs had received succinylcholine. Th is syn- drome has become the animal model for the study of MH.4,5 Over time, this condition came to be known as malignant hyperthermia because a rapid rise in temperature was a com- mon feature in all reported cases. Additional possible signs and symptoms include skin mottling, arrhythmias, elevated creatine kinase (CK), and rhabdomyolysis, among others (TABLE 2). ❚ Associated conditions. MH may occur with any condition requiring intervention with anesthesia. It was once believed that strabismus and MH were linked, but this assumption was based 30 THE JOURNAL OF FAMILY PRACTICE | JANUARY 2011 | VOL 60, NO 1 TABLE 1 Triggering and nontriggering anesthetic agents Triggering agents Nontriggering agents Succinylcholine (most common) Barbiturates Desfl urane Benzodiazepines Halothane Ketamine Isofl urane Local anesthetics Sevofl urane Nitrous oxide Nondepolarizing muscle relaxants Opioids Propofol Adapted from: Barash PG, et al, eds. Clinical Anesthesia. 6th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2009. Malignant on a statistical error related to an increased TABLE 2 hyperthermia number of surgical procedures in children with Signs and symptoms is a medical strabismus. Currently, a propensity toward MH emergency and seems associated only with rare myopathic con- of malignant hyperthermia requires that the ditions such as central core disease, hypokale- patient receive mic periodic paralysis, Evans myopathy, and Arrhythmias dantrolene King-Denborough syndrome.6 Precise genetic Coagulopathy sodium. mapping will determine what, if any, relation- Elevated creatine kinase ship there is between these processes and MH. Elevated temperature Hypercarbia Awake triggering: Similar disorder Hyperkalemia without anesthesia Increased oxygen consumption Since 1980 there have been several reports of Masseter muscle spasm “awake triggering” in genetically predisposed Metabolic acidosis individuals, whereby stressful conditions alone Muscle rigidity unrelated to general anesthesia cause MH.7–10 Rhabdomyolysis Often, the presenting condition has been heat- Skin mottling stroke, but other symptoms are also common, Tachycardia such as rhabdomyolysis, increased CK, muscle Tachypnea pain, and cardiovascular collapse.11 Relatives Adapted from: Barash PG, et al, eds. Clinical Anesthesia. 6th of those with a history of MH have also exhib- ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2009. ited chronic muscle pain or chronic CK eleva- tion. All of these people, when tested, have had Should such fi ndings—especially elevat- a positive reaction to the caff eine-halothane ed CK and rhabdomyolysis—come to your at- contracture test (CHCT), which is the gold tention by a patient’s report or during physical standard for confi rming MH.12 examination, consider further workup for MH. Th e Malignant Hyperthermia Association of the United States (MHAUS) lists signs and How MH develops symptoms that accompany awake triggering on MH occurs because of a defect in the ry- its Web site, www.mhaus.org. Th ey include heat anodine receptor, RYR1. Th is receptor is re- sensitivity, night sweats, cramping, mottled sponsible for intracellular calcium release by skin, low-grade fever, and excessive sweating. its mediation of the sarcoplasmic reticulum. CONTINUED JFPONLINE.COM VOL 60, NO 1 | JANUARY 2011 | THE JOURNAL OF FAMILY PRACTICE 31 RYR1 is found in all skeletal muscle.13 Dur- ing an episode of MH, exposure to the trigger- Incidence of MH ing agent causes intracellular calcium release is increasing by the sarcoplasmic reticulum and sustained The number of reported cases of malignant skeletal muscle contractions and rigidity. In- hyperthermia (MH) has increased over creased oxygen consumption occurs, and this the last 20 years, but the exact incidence hypermetabolic state leads to hypercarbia, se- is unknown. It varies not only by country, vere metabolic acidosis, tachycardia, arrhyth- but, within the United States, from state to mias, hyperkalemia, and elevated temperature. state, and even within states, presumably due to variations in the genetic pool. Michi- Rhabdomyolysis and elevations in CK also oc- gan, Nebraska, West Virginia, and Wiscon- cur because of skeletal muscle breakdown. sin report especially high incidences.19 MH is more common in men than women (58% vs 42%, respectively, of affected How to treat patients ). The last major survey of MH inci- this medical emergency dence was published in 2009.20 In the survey Only rapid, specifi c treatment can save a years (2000-2005), MH incidence increased from 10.2 to 13.3 patients per million hospi- patient with MH from death. Before inves- tal discharges. Mortality in the same period tigators discovered that dantrolene sodium decreased from 16.1% to 6.5%. is an eff ective treatment for MH, >70% of Malignant patients who developed the disease died.12 hyperthermia Mortality has since dropped to ≤15%.14 With water, cold intravenous fl uid, or ice water la- can occur as a presumptive diagnosis of MH, dantrolene vage at the surgical site. Active cooling should long as 12 hours should be prepared and given immediately. stop when the patient’s temperature reaches after exposure Th e drug inhibits the release of calcium from 100ЊF (so as not to cause hypothermia). Cool- to a presumed the sarcoplasmic reticulum of skeletal muscle ing should not, however, prevent or slow the trigger. by limiting the activation of RYR1. administration of dantrolene. Successful resuscitation of patients with ❚ Treating acidosis, arrhythmias, elec- MH hinges on the following: trolyte disturbances, coagulopathy, excess ❚ Making a prompt diagnosis. While myoglobin, and acute renal failure. Ar- most episodes of MH occur shortly after in- rhythmias should not be treated with calcium duction of anesthesia or during the intraop- channel blockers. erative course, nearly 2% of MH cases occur ❚ Monitoring patients in the ICU for postoperatively15—some as long as 12 hours potential adverse eff ects of dantrolene that after exposure to the presumed trigger. can include sedation and muscle weakness. ❚ Immediately discontinuing triggering Mechanical ventilation may be needed. (Th e anesthetics and starting dantrolene. Th e malignant hyperthermia hotline [800-644- patient should be kept anesthetized using 9737] at MHAUS is available 24 hours a day nontriggering agents (TABLE 1), and surgery to assist clinicians with questions regarding should be concluded as quickly as possible. diagnosis and treatment.) Preparation of dantrolene is a tedious pro- cess because of its poor water solubility. One Keeping patients safe to 2 minutes are needed for the preparation to by properly testing them solubilize. Initial treatment with dantrolene is Th e CHCT is 97% sensitive and 78% specifi c 2.5 mg/kg administered intravenously (via pe- for MH.16 It is an invasive procedure, requir- ripheral or central line). Th is dose (or up to 10 ing the patient to undergo anesthesia. Th e mg/kg) can be repeated every 5 to 10 minutes test costs more than $5,000, which most in- until major symptoms such as hypercarbia, surance companies will cover.17 arrhythmias, hyperpyrexia, and metabolic aci- Th e CHCT is performed only
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