II. Definition

  1. Rare, inhalational anesthetic related rapid rise in Body Temperature, muscle rigidity, Hypertension and Tachypnea

III. Epidemiology

  1. Rare general anesthesia complication
  2. Autosomal Dominant inherited defect

IV. Causes

  1. Similar to Neuroleptic Malignant Syndrome (due to Antipsychotics)
  2. Anesthetic agents in combination with genetic predisposition
    1. Inhalational anesthetics
    2. Succinylcholine

V. Pathophysiology

  1. Ryanodine receptor gene (RYR1) defect effects the calcium release channel in muscle
  2. Results in an unregulated increase in skeletal muscle oxidative metabolism

VI. Signs

  1. Hyperthermia
  2. Muscle Rigidity
  3. Hypertension
  4. Tachypnea

VII. Lab: Abnormalities

VIII. Complications

  1. Disseminated Intravascular Coagulation (DIC)
  2. Respiratory Failure
  3. Renal Failure
  4. Cardiovascular Collapse
  5. Rhabdomyolysis

IX. Management

  1. Dantrolene (specific treatment of choice)
    1. Adult: 1 to 3 mg/kg IV
    2. Child: 0.5 to 1 mg/kg IV
  2. Supportive measures (especially until Dantrolene available)
    1. Benzodiazepines
    2. Cooling

X. References

  1. (2016) CALS, 14th Ed, 1:126

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Ontology: Malignant hyperpyrexia due to anesthesia (C0024591)

Definition (NCI) A rare disorder characterized by rapid rise of the body temperature, accompanied by rhabdomyolysis and, if untreated, by collapse and death. It occurs in susceptible individuals who receive certain drugs for general anesthesia, gas anesthetics, or succinylcholine. It may be inherited in an autosomal dominant pattern.
Definition (MSH) Rapid and excessive rise of temperature accompanied by muscular rigidity following general anesthesia.
Definition (CSP) usually inherited reaction to general anesthesia manifested as a sudden, rapid rise in temperature, hypermetabolism, and skeletal muscle rigidity; mutation is in the calcium release channel of skeletal muscle sarcoplasmic reticulum.
Concepts Disease or Syndrome (T047)
MSH D008305
ICD9 995.86
ICD10 T88.3
SnomedCT 269435009, 111738008, 248453001, 405500008, 157762007, 213026003, 405501007
English Hyperpyrexia, Malignant, Hyperpyrexias, Malignant, Hyperthermia, Malignant, Malignant Hyperpyrexia, Malignant Hyperpyrexias, Malignant Hyperthermia, Malignant Hyperthermias, HYPERPYREXIA MALIGNANT, HYPERTHERMIA MALIGNANT, Malignant hyperpyrexia due to anesthesia, Malignant hyperpyrexia, Malign.hyperpyrexia-anaesthet., MHS, MH, HYPERTHERMIA OF ANESTHESIA, HYPERPYREXIA, MALIGNANT, MALIGNANT HYPERTHERMIA, SUSCEPTIBILITY TO, 1, MHS1, hyperthermia of anesthesia, anesthesia related hyperthermia, malignant hyperthermia due to anesthesia (diagnosis), malignant hyperthermia, malignant hyperthermia due to anesthesia, malignant hyperthermia (diagnosis), Fever malignant, Hyperpyrexia malignant, Malignant hyperthemia, Malignant Hyperthermia Syndrome, Malignant Hyperthermia [Disease/Finding], malignant hyperthemia, hyperpyrexia malignant, hyperthermia malignant, malignant fever, Anesthesia Hyperthermia, Anesthesia Hyperthermias, Hyperthermia of Anesthesia, Related Hyperthermias, Anesthesia, Hyperthermias, Anesthesia Related, Anesthesia Related Hyperthermia, Anesthesia Related Hyperthermias, Hyperthermia, Anesthesia Related, Related Hyperthermia, Anesthesia, Malignant hyperpyrexia -anaest, Malignant hyperpyrexia -anest, Malignant hyperpyrexia due to anesthesia (finding), Malignant hyperpyrexia anesth, Hyperthermia of anesthesia, Malignant hyperpyrexia anaesth, Hyperthermia of anaesthesia, Malignant hyperpyrexia due to anaesthetic, Malignant hyperpyrexia due to anesthetic, Malignant hyperpyrexia due to anesthetic (finding), Malignant hyperthermia, MH - Malignant hyperpyrexia, MHS - Malignant hyperthermia, Malignant hyperthermia (finding), Hyperthermia malignant, complications; anesthesia, hyperthermia, malignant, complications; anesthesia, malignant hyperthermia, hyperpyrexia; malignant, hyperthermia; malignant, malignant; hyperpyrexia, malignant; hyperthermia, anesthesia; complication, hyperthermia, malignant, anesthesia; complication, malignant hyperthermia, Malignant hyperpyrexia due to anaesthesia, Malignant hyperthermia due to anaesthesia, Malignant hyperthermia due to anesthesia, malignant hyperpyrexia
French HYPERTHERMIE MALIGNE, Fièvre maligne, Hyperthémie maligne, Hyperthermie maligne peranesthésique, Hyperpyrexie maligne, HYPERPYREXIE MALIGNE, Hyperthermie maligne
Portuguese HIPERTERMIA MALIGNA, HIPERPIREXIA MALIGNA, Febre maligna, Hiperpirexia maligna, Hipertermia maligna, Hiperpirexia Maligna, Hipertermia Maligna
Spanish HIPERTERMIA MALIGNA, HIPERPIREXIA MALIGNA, Fiebre maligna, Hiperpirexia maligna, hipertermia maligna por anestesia (hallazgo), hipertermia maligna por anestesia, hiperpirexia maligna por anestesia, hiperpirexia maligna secundaria a la administración de anestesia, hipertermia secundaria a la administración de anestesia, hiperpirexia maligna secundaria a la administración de anestesia (hallazgo), hiperpirexia maligna por agente anestésico (hallazgo), hiperpirexia maligna por agente anestésico, Hipertermia maligna, hiperpirexia maligna, hipertermia maligna, hipertermia maligna (hallazgo), hipertermia maligna por agente anestésico, Hiperpirexia Maligna, Hipertermia Maligna
Dutch hyperpyrexie maligne, maligne koorts, maligne hyperthermie, maligne hyperpyrexie, anesthesie; complicatie, hyperthermie, maligne, anesthesie; complicatie, maligne hyperthermie, complicaties; anesthesie, hyperthermie, maligne, complicaties; anesthesie, maligne hyperthermie, hyperpyrexie; maligne, hyperthermie; maligne, maligne; hyperpyrexie, maligne; hyperthermie, Maligne hyperthermie door anesthesie, hyperthermie maligne, Hyperpyraxie, maligne, Hyperthermie, maligne, Maligne hyperpyrexie, Maligne hyperthermie
German boesartige Hyperthermie, Fieber, boesartig, Hyperpyrexie maligne, boesartige Hyperpyrexie, HYPERPYREXIE MALIGNE, HYPERTHERMIE MALIGNE, Maligne Hyperthermie durch Anaesthesie, maligne Hyperthermie, Hyperpyrexie, maligne, Hyperthermie, maligne, Maligne Hyperpyrexie, Maligne Hyperthermie
Italian Febbre maligna, Iperpiressia maligna, Ipertermia maligna
Japanese 悪性発熱, アクセイコウネツ, アクセイハツネツ, 悪性高熱, 悪性高体温, 悪性超高熱, 悪性高体温症, 悪性高熱症, 激症過高体温, 麻酔時悪性高熱, 麻酔時悪性高熱症
Swedish Malign hypertermi
Czech maligní hypertermie, Maligní horečka, Maligní hyperpyrexie, Maligní hypertermie, Zhoubná hyperpyrexie, Zhoubná hypertermie
Finnish Maligni hypertermia
Russian ZLOKACHESTVENNAIA GIPERPIREKSIIA, ZLOKACHESTVENNAIA GIPERTERMIIA, GIPERPIREKSIIA ZLOKACHESTVENNAIA, GIPERTERMIIA ZLOKACHESTVENNAIA, ГИПЕРПИРЕКСИЯ ЗЛОКАЧЕСТВЕННАЯ, ГИПЕРТЕРМИЯ ЗЛОКАЧЕСТВЕННАЯ, ЗЛОКАЧЕСТВЕННАЯ ГИПЕРПИРЕКСИЯ, ЗЛОКАЧЕСТВЕННАЯ ГИПЕРТЕРМИЯ
Korean 마취에 의한 악성 이상고열
Polish Hipertermia złośliwa
Hungarian Láz, malignus, Malignus hyperpyrexia, Malignus láz, Malignus hyperthermia
Norwegian Hypertermi, malign, Malign hyperpyreksi, Hyperpyreksi, malign, Malign hypertermi