James Nickleson, RNAIJames Nickleson, RNAI

Master of Anesthesiology EducationMaster of Anesthesiology Education

Gonzaga UniversityGonzaga University

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Physiology and epidemiology

Clinical manifestations

Differential diagnosis and treatment

Case review

MH resources

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Inherited disorder of skeletal muscleInherited disorder of skeletal muscle

Problem w/ reuptake of intracellular CaProblem w/ reuptake of intracellular Ca2+2+

Exact cause uncertainExact cause uncertain Ryanodine receptorRyanodine receptor

Disease inheritance is autosomal dominantDisease inheritance is autosomal dominant

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Definite association: central core diseaseDefinite association: central core disease

Possible association: Duchenne, Becker, Possible association: Duchenne, Becker, King-Denborough, other myopathiesKing-Denborough, other myopathies

Coincidental association: neuroleptic Coincidental association: neuroleptic malignant syndrome, SIDS, Lymphomas, malignant syndrome, SIDS, Lymphomas, and Heat Strokeand Heat Stroke

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Frequency 1:3,000 - 1:60,000 anesthetic Frequency 1:3,000 - 1:60,000 anesthetic casescases

Approximately 600 cases per year in U.SApproximately 600 cases per year in U.S11

Increased incidence in young adult malesIncreased incidence in young adult males

1:100,000 hospital discharges complicated 1:100,000 hospital discharges complicated by MHby MH

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Muscle Contracture Muscle Contracture TestTest

Caffeine Halothane Caffeine Halothane Contracture Contracture Test(CHCT)Test(CHCT)

Gold StandardGold Standard

MH Muscle Biopsy MH Muscle Biopsy CenterCenter

Genetic TestingGenetic Testing

Ryanodine receptor Ryanodine receptor (RYR1 Gene)(RYR1 Gene)

Primary genetic focusPrimary genetic focus

DNA blood test or DNA blood test or biopsybiopsy

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MH TriggersMH Triggers Potent volatile Potent volatile

anestheticsanesthetics SevofluraneSevoflurane DesfluraneDesflurane IsofluraneIsoflurane

Depolarizing muscle Depolarizing muscle relaxantsrelaxants SuccinylcholineSuccinylcholine

NOTNOT MH Triggers MH Triggers Nitrous oxideNitrous oxide IV induction agents IV induction agents Non-depolarizing Non-depolarizing

muscle relaxantsmuscle relaxants OpioidsOpioids

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SpecificSpecific Muscle rigidity*Muscle rigidity* Increased COIncreased CO2

productionproduction Marked temperature Marked temperature

elevationelevation RhabdomyolysisRhabdomyolysis

Non-SpecificNon-Specific TachycardiaTachycardia TachypneaTachypnea Acidosis (respiratory; Acidosis (respiratory;

metabolic)metabolic) HyperkalemiaHyperkalemia

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Insufficient anesthesia Insufficient anesthesia or analgesiaor analgesia

Insufficient ventilation Insufficient ventilation or FGFor FGF

Anaphylactic reaction Anaphylactic reaction

PheochromocytomaPheochromocytoma

Thyroid crisisThyroid crisis

Cerebral ischemiaCerebral ischemia

Neuromuscular Neuromuscular disordersdisorders

Procedural causesProcedural causes

Malignant neuroleptic Malignant neuroleptic syndromesyndrome

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Stop triggering inhalation Stop triggering inhalation agents/succinylcholineagents/succinylcholine

Hyperventilate high flow 100% OHyperventilate high flow 100% O22

Dantrolene 2.5mg/kg push, repeat prnDantrolene 2.5mg/kg push, repeat prn Continue monitoring & interventionsContinue monitoring & interventions Treat hyperthermia, acidosis, and Treat hyperthermia, acidosis, and

arrhythmiasarrhythmias

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Muscle relaxantMuscle relaxant

Indications Indications The The onlyonly specific and effective treatment for MHspecific and effective treatment for MH Neuroleptic malignant syndrome, muscle spasticity, Neuroleptic malignant syndrome, muscle spasticity,

serotonin syndrome, and 2,4-dinitrophenol poisoningserotonin syndrome, and 2,4-dinitrophenol poisoning

Drug InteractionsDrug Interactions CCBs, NDNMB, CNS depressants & benzodiazepinesCCBs, NDNMB, CNS depressants & benzodiazepines

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Shut down/disable vaporizersShut down/disable vaporizers

Flow OFlow O22 > 10L/min for 20 minutes through > 10L/min for 20 minutes through

machine and ventilatormachine and ventilator

Change COChange CO22 absorbent absorbent

Use Use non-triggernon-trigger agents and methods agents and methods

Monitor for early signs of MHMonitor for early signs of MH

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34 year old male

Right ankle ORIF with iliac bone graft

Past medical history & medications HTN, GERD, shoulder reconstruction Lisinopril, hydrocodone, marijuana

Anesthesia plan = GA + regional

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Site specific policy Site specific policy

Malignant Hyperthermia CartMalignant Hyperthermia Cart

MHAUSMHAUS Malignant Hyperthermia Association of the Malignant Hyperthermia Association of the

United States @ 1-800-MH-HYPERUnited States @ 1-800-MH-HYPER www.mhaus.orgwww.mhaus.org

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Disorder with intracellular Ca2+ effecting skeletal muscle

Triggered by inhaled anesthetics & succinylcholine

Specific and non-specific clinical signs

Definitive treatment with Dantrolene

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Brady, J.E., Lena, S.S., Rosenberg, H., Li, G. (2009). Prevalance of malignant hyperthermia du to anesthesia in new york state, 2001-2005. Aneshtesia & Analgesia. 109:1162-1166.

Glahn, K.P.E, Ellis, F.R., Halsall, P.J., Muller, C.R., Snoeck, M.M.J., Urwyler, A., & Wappler, F. (2010) Recognizing and managing a malignant hypthermia

crisis: guidelines from the European Malignant Hyperthermia Group. British Journal of Anaesthesia. 105 (4):417-420.

Rosenburg, H., Brandom, B.W., & Sambuughin, N. (2009). Malignant Hyperthermia and Other Inherited Disorders. In P.G. Barash, B.F. Cullen, & R.K.

Stoelting. (Eds.) Clinical Anesthesia (6th ed., pp. 598-619). Philadelphia: Lippincott Williams & Wilkins

Stoelting, R., & Hiller, S. (2006). Handbook of Pharmacology & Physiology in Anesthetic Practice (2nd Ed). Philadelphia: Lippincott Williams & Wilkins

Torpy, J., Lynm, C., Glass, R.M. (2005). Malignant Hyperthermia. JAMA; 293 (23):2958

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