Malignant hyperthermia: Difference between revisions

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==Work-Up==
==Work-Up==
#Core temperature
*Core temperature
#CBC
*CBC
#Chem 7
*Chem 7
#Total CK
*Total CK
#PT/PTT
*PT/PTT
#ABG
*ABG


==Diagnosis==
==Diagnosis==
#Muscle contraction
*Muscle contraction
#Fever
*Fever


#First signs
*First signs
##Hypercarbia
**Hypercarbia
##Sinus tachycardia
**Sinus tachycardia
##Masseter spasm
**Masseter spasm
##Temperature abnormalities (may be early)
**Temperature abnormalities (may be early)
#Most common pattern
*Most common pattern
##Respiratory acidosis and muscular abnormalities
**Respiratory acidosis and muscular abnormalities


===Presentations===
===Presentations===
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===Types===
===Types===
#Fulminant MH
*Fulminant MH
##muscle rigidity, high fever, increased HR shortly after induction of anesthesia
**muscle rigidity, high fever, increased HR shortly after induction of anesthesia
#Masseter muscle rigidity
*Masseter muscle rigidity
##jaw muscle rigidity after succinylchoine
**jaw muscle rigidity after succinylchoine
##More common in children
**More common in children
##Presages MH in 20-30% cases
**Presages MH in 20-30% cases
##All patients demonstrate elevated CK and often gross myoglobinuria
**All patients demonstrate elevated CK and often gross myoglobinuria
##CK >20,000IU = high likelihood of MH  
**CK >20,000IU = high likelihood of MH  
# Late onset MH
* Late onset MH
##Uncommon, may begin shortly after anesthesia termination (usually within first hour)
**Uncommon, may begin shortly after anesthesia termination (usually within first hour)


==Differential Diagnosis==
==Differential Diagnosis==
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==Treatment==
==Treatment==
#Initial
*Initial
##Declare MH Emergency: (call OR for anesthesia to bring MH cart)
**Declare MH Emergency: (call OR for anesthesia to bring MH cart)
## Discontinue Triggering Agents
** Discontinue Triggering Agents
## 100% Oxygen at High Flow
** 100% Oxygen at High Flow
## Give Dantrolene
** Give Dantrolene
###Designate 2 or 3 people to mix sterile water into Dantrolene \
***Designate 2 or 3 people to mix sterile water into Dantrolene \
####60ml sterile water into each vial of dantrolene; may need up to 36 vials
****60ml sterile water into each vial of dantrolene; may need up to 36 vials
####If using Dantrium® (20 mg/vial), each vial contains 3 g of mannitol (renal vasodilation); newer nanocrystalline dantrolene (250 mg/vial) has 250 mg mannitol per vial and requires mannitol supplementation
****If using Dantrium® (20 mg/vial), each vial contains 3 g of mannitol (renal vasodilation); newer nanocrystalline dantrolene (250 mg/vial) has 250 mg mannitol per vial and requires mannitol supplementation
### '''2.5 mg/kg IV push'''
*** '''2.5 mg/kg IV push'''
### Titrate to effect; may need more than 10 mg/kg
*** Titrate to effect; may need more than 10 mg/kg
##Bicarb for metabolic acidosis
**Bicarb for metabolic acidosis
### 1-2 mEQ/kg if blood gas values not yet available
*** 1-2 mEQ/kg if blood gas values not yet available
## Cool the patient if core temp >39 deg C (102.2 deg F)
** Cool the patient if core temp >39 deg C (102.2 deg F)
### Stop cooling when temp reaches 100.4
*** Stop cooling when temp reaches 100.4
## Dysrhythmias usually respond to treatment of acidosis and hyperkalemia
** Dysrhythmias usually respond to treatment of acidosis and hyperkalemia
### Standard therapy EXCEPT NO CA CHANNEL BLOCKERS:
*** Standard therapy EXCEPT NO CA CHANNEL BLOCKERS:
####may cause hyperkalemia or cardiac arrest in presence of dantrolene
****may cause hyperkalemia or cardiac arrest in presence of dantrolene
## Treat hyperkalemia: standard treatment, remember to check glucose levels q1h after treatment with insulin/glucose
** Treat hyperkalemia: standard treatment, remember to check glucose levels q1h after treatment with insulin/glucose
## Call MHAUS Hotline if needed: 1-800-644-0737
** Call MHAUS Hotline if needed: 1-800-644-0737
#Continued Care
*Continued Care
##Dantrolene 1 mg/kg every 4-6 hours for 24–48 hours
**Dantrolene 1 mg/kg every 4-6 hours for 24–48 hours
##Monitor for recrudescence (rate is 25%)
**Monitor for recrudescence (rate is 25%)
##Follow electrolytes, blood gases, CK, core temperature, urine output and color, coagulation studies
**Follow electrolytes, blood gases, CK, core temperature, urine output and color, coagulation studies


==Prognosis==
==Prognosis==
===Stable to Transfer Criteria===
===Stable to Transfer Criteria===
#ETCO2 is declining or normal
*ETCO2 is declining or normal
#HR is stable or decreasing
*HR is stable or decreasing
#No ominous dysrhythmias
*No ominous dysrhythmias
#Temperature is declining
*Temperature is declining
#Generalized muscular rigidity is resolving (if present)
*Generalized muscular rigidity is resolving (if present)
#IV dantrolene administration has begun
*IV dantrolene administration has begun


===Complications===
===Complications===
#Consciousness Level Change/Coma
*Consciousness Level Change/Coma
#Cardiac Dysfunction
*Cardiac Dysfunction
#Pulmonary Edema
*Pulmonary Edema
#Renal Dysfunction
*Renal Dysfunction
#Disseminated Intravascular Coagulation
*Disseminated Intravascular Coagulation
#Hepatic Dysfunction
*Hepatic Dysfunction
#Relapse
*Relapse
#Death
*Death


==See Also==
==See Also==

Revision as of 18:46, 14 May 2015

Background

  • Inherited disorder of skeletal muscles triggered most often by anesthesia inhalation agents and/or succinylcholine
  • Results in hypermetabolism, skeletal muscle damage, hyperthermia, and death if not treated quickly
  • GENERALIZED rigidity NOT always present; if it occurs, MH is almost certain

Likelihood of Complications

  • Increased time from 1st sign to 1st dantrolene
    • For every 30 minute increase in the interval, complication likelihood increases x 1.6
  • Increased maximal temperature
    • For every 2°C increase in max temp, complication likelihood increases x 2.9

Work-Up

  • Core temperature
  • CBC
  • Chem 7
  • Total CK
  • PT/PTT
  • ABG

Diagnosis

  • Muscle contraction
  • Fever
  • First signs
    • Hypercarbia
    • Sinus tachycardia
    • Masseter spasm
    • Temperature abnormalities (may be early)
  • Most common pattern
    • Respiratory acidosis and muscular abnormalities

Presentations

  • 99% Respiratory Acidosis
  • 26% Metabolic Acidosis
  • 80% Muscular Abnormalities

Watch for it with succinylcholine use.

Types

  • Fulminant MH
    • muscle rigidity, high fever, increased HR shortly after induction of anesthesia
  • Masseter muscle rigidity
    • jaw muscle rigidity after succinylchoine
    • More common in children
    • Presages MH in 20-30% cases
    • All patients demonstrate elevated CK and often gross myoglobinuria
    • CK >20,000IU = high likelihood of MH
  • Late onset MH
    • Uncommon, may begin shortly after anesthesia termination (usually within first hour)

Differential Diagnosis

Fever

Infectious

Non-infectious

Treatment

  • Initial
    • Declare MH Emergency: (call OR for anesthesia to bring MH cart)
    • Discontinue Triggering Agents
    • 100% Oxygen at High Flow
    • Give Dantrolene
      • Designate 2 or 3 people to mix sterile water into Dantrolene \
        • 60ml sterile water into each vial of dantrolene; may need up to 36 vials
        • If using Dantrium® (20 mg/vial), each vial contains 3 g of mannitol (renal vasodilation); newer nanocrystalline dantrolene (250 mg/vial) has 250 mg mannitol per vial and requires mannitol supplementation
      • 2.5 mg/kg IV push
      • Titrate to effect; may need more than 10 mg/kg
    • Bicarb for metabolic acidosis
      • 1-2 mEQ/kg if blood gas values not yet available
    • Cool the patient if core temp >39 deg C (102.2 deg F)
      • Stop cooling when temp reaches 100.4
    • Dysrhythmias usually respond to treatment of acidosis and hyperkalemia
      • Standard therapy EXCEPT NO CA CHANNEL BLOCKERS:
        • may cause hyperkalemia or cardiac arrest in presence of dantrolene
    • Treat hyperkalemia: standard treatment, remember to check glucose levels q1h after treatment with insulin/glucose
    • Call MHAUS Hotline if needed: 1-800-644-0737
  • Continued Care
    • Dantrolene 1 mg/kg every 4-6 hours for 24–48 hours
    • Monitor for recrudescence (rate is 25%)
    • Follow electrolytes, blood gases, CK, core temperature, urine output and color, coagulation studies

Prognosis

Stable to Transfer Criteria

  • ETCO2 is declining or normal
  • HR is stable or decreasing
  • No ominous dysrhythmias
  • Temperature is declining
  • Generalized muscular rigidity is resolving (if present)
  • IV dantrolene administration has begun

Complications

  • Consciousness Level Change/Coma
  • Cardiac Dysfunction
  • Pulmonary Edema
  • Renal Dysfunction
  • Disseminated Intravascular Coagulation
  • Hepatic Dysfunction
  • Relapse
  • Death

See Also