Unusual Manifestations of Susceptibility to Malignant Hyperthermia

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Unusual Manifestations of Susceptibility
to Malignant Hyperthermia
K. Lebedinski, A. Triadski St. Petersburg
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MH Epidemiology
Adults in Europe and US 150.000
1150.000 Children and Adolescents 115.000 Susp
ected MH (atypical forms) up to 14.200 !
Wappler F. Eur J Anaesth 2001 18 632-52 Gronert
GA, Antognini JF In Anesthesia, Ed. by RD
Miller, 1996
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The 1st case F., male, 44 yrs. Leiomyoma of the
Stomach with Perforation of the Cyst, Peritonitis
and Hypovolemic Shock Short pre-operating volume
replacement Urgent Surgery atypical resection
of the Stomach, lavage and drainage of the
abdomen
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The 1st case F., male, 44 yrs.

• Succinylcholine 3 mg/kg for tracheal intubation
• Hypotension required Dopamine 5 mkg/kg?min

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• The 1st case F., male, 44 yrs.
• Immediately after the Anaesthesia
• Respiratory weakness
• Prolonged ventilation
• Remarkable Shivering
• Warming
• Stable Circulation

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• The 1st case F., male, 44 yrs.
• Further Events
• In 10 h adequate breathing
• For 5 h breathing via ETT
• Sudden decompensation
• Ventilation again
• Progressive respiratory weakness

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• The 1st case F., male, 44 yrs.
• 24 h after the anaesthesia
• Refractory body temperature rise
• Max point 40,1 ?C (rectal probe)
• Physical cooling
• Reverse in 5 h

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• The 1st case F., male, 44 yrs.
• 48 h after the anaesthesia
• High grade ventricular extrasystoli
• Xylocaine
• Progressive oliguria
• Saluretics

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• The 1st case F., male, 44 yrs.
• At the same time
• Progressive muscle weakness
• Diffuse muscle tenderness
• Polimyosistis, myastenia gravis?
• Negative Neostigmin test
• CPK rise 2387 IU/l
• MH diagnosis was made

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• The 1st case F., male, 44 yrs.
• Final events
• Progressive renal failure
• Cardiac rhythm disturbances
• Refractory vasogenic shock
• Death 4 days after anaesthesia
• PA dignosis Lyell disease

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The 2nd case S., male, 53 yrs. Acute simple
appendicitis Laparoscopic appendectomy Succinylch
oline 1 mg/kg to relief appendix
removal Immediate tachycardia (110 min-1)
Immediate diffuse muscle rigidity Adequate
breathing and voluntary motions (!)
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The 2nd case S., male, 53 yrs. ICU monitoring
for 12 h MgSO4 100 mg/kg IV infusion Mild muscle
rigidity for 3 days Mild T rise (37,6 ?C) for 3
days CPK-MM rise up to 755 IU/l No renal failure!
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• The 2nd case S., male, 53 yrs.
• Anamnesis
• High fever for any flu
• Fever, coffee and physical exertion
• Paresthesias and rigidity in the back and hands
• Senior brother has similar signs

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The 2nd case S., male, 53 yrs. Further
examination Unusual echo-structure of the
muscles CPK-MM rise after veloergometry EchoCG
small and thick heart, LV hypertrophia without
hypertension
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The 3rd case T., male, 32 yrs. Acute simple
appendicitis Routine appendectomy Succinylcholine
2 mg/kg for tracheal intubation Masseter muscle
spasm (MMS) MHS was suspected (!) TIVA with
spontaneous breathing
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The 3rd case T., male, 32 yrs. Cola coloured
urine Generalized muscle tenderness Complete
inability to walk Recovery only in a week
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The 3rd case T., male, 32 yrs. Anamnesis
Remarkable muscle rigidity for coffee, fever and
hunger (not CRAMPS!) At the age of 5 typical
spontaneous MH crisis Examined by neurologist
EEG MRI without any result
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• Conclusions
• Unusual MH episodes is not less dangerous than
  typical ones!
• MH-education of anaesthetists and neurologists
  could reduce the risks!

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Thank you for the attention!