Unusual Manifestations Unusual Manifestations of Susceptibility of Susceptibility

to Malignant to Malignant HyperthermiaHyperthermia

K. Lebedinski, A. TriadskiK. Lebedinski, A. TriadskiSt. PetersburgSt. Petersburg

Adults in Europe and US…Adults in Europe and US…1:50.000 – 1:150.0001:50.000 – 1:150.000

Children and Adolescents…Children and Adolescents…1:15.0001:15.000

““Suspected MH” (atypical forms)…Suspected MH” (atypical forms)…up to 1:4.200 !up to 1:4.200 !

MH EpidemiologyMH Epidemiology

Wappler F. Eur J Anaesth 2001; 18: 632-52Wappler F. Eur J Anaesth 2001; 18: 632-52Gronert GA, Antognini JF In: Anesthesia, Ed. by RD Miller, Gronert GA, Antognini JF In: Anesthesia, Ed. by RD Miller, 19961996

The 1The 1stst case: F., male, 44 yrs. case: F., male, 44 yrs.Leiomyoma of the Stomach with Leiomyoma of the Stomach with Perforation of the Cyst, Perforation of the Cyst, Peritonitis and Hypovolemic Peritonitis and Hypovolemic ShockShock

Short pre-operating volume Short pre-operating volume replacement replacement

Urgent Surgery: atypical Urgent Surgery: atypical resection of the Stomach, resection of the Stomach, lavage and drainage of the lavage and drainage of the abdomenabdomen

The 1The 1stst case: F., male, 44 yrs. case: F., male, 44 yrs.• Succinylcholine 3 mg/kg for tracheal Succinylcholine 3 mg/kg for tracheal intubationintubation• Hypotension required Dopamine 5 Hypotension required Dopamine 5 mkg/kgmkg/kgminmin

The 1The 1stst case: F., male, 44 yrs. case: F., male, 44 yrs.Immediately after the Immediately after the Anaesthesia:Anaesthesia:

• Respiratory weaknessRespiratory weakness• Prolonged ventilationProlonged ventilation

• Remarkable ShiveringRemarkable Shivering• WarmingWarming

• Stable CirculationStable Circulation

The 1The 1stst case: F., male, 44 yrs. case: F., male, 44 yrs.Further Events:Further Events:• In 10 h – adequate breathing In 10 h – adequate breathing • For 5 h – breathing via ETTFor 5 h – breathing via ETT• Sudden decompensationSudden decompensation

• Ventilation againVentilation again• Progressive respiratory Progressive respiratory

weaknessweakness

The 1The 1stst case: F., male, 44 yrs. case: F., male, 44 yrs.24 h after the anaesthesia:24 h after the anaesthesia:• Refractory body temperature Refractory body temperature

rise rise • Max point – 40,1 Max point – 40,1 C (rectal C (rectal

probe)probe)• Physical coolingPhysical cooling• Reverse in 5 hReverse in 5 h

The 1The 1stst case: F., male, 44 yrs. case: F., male, 44 yrs.48 h after the anaesthesia:48 h after the anaesthesia:• High grade ventricular High grade ventricular

extrasystoli extrasystoli • XylocaineXylocaine

•Progressive oliguria Progressive oliguria • SalureticsSaluretics

The 1The 1stst case: F., male, 44 yrs. case: F., male, 44 yrs.At the same time:At the same time:•Progressive muscle weakness Progressive muscle weakness •Diffuse muscle tenderness Diffuse muscle tenderness

• Polimyosistis, myastenia Polimyosistis, myastenia gravis?gravis?

• Negative Neostigmin testNegative Neostigmin test• CPK rise: 2387 IU/lCPK rise: 2387 IU/l

• MH diagnosis was madeMH diagnosis was made

The 1The 1stst case: F., male, 44 yrs. case: F., male, 44 yrs.Final events:Final events:• Progressive renal failure Progressive renal failure • Cardiac rhythm disturbancesCardiac rhythm disturbances• Refractory vasogenic shockRefractory vasogenic shock• Death – 4 days after anaesthesiaDeath – 4 days after anaesthesia• PA dignosis: Lyell diseasePA dignosis: Lyell disease

The 2The 2ndnd case: S., male, 53 yrs. case: S., male, 53 yrs.Acute simple appendicitis Acute simple appendicitis Laparoscopic appendectomyLaparoscopic appendectomySuccinylcholine 1 mg/kg to relief Succinylcholine 1 mg/kg to relief appendix removalappendix removal

Immediate tachycardia (110 minImmediate tachycardia (110 min-1-1) ) Immediate diffuse muscle rigidityImmediate diffuse muscle rigidityAdequate breathing and voluntary Adequate breathing and voluntary motions (!) motions (!)

The 2The 2ndnd case: S., male, 53 yrs. case: S., male, 53 yrs.ICU monitoring for 12 h ICU monitoring for 12 h MgSOMgSO44 100 mg/kg IV infusion 100 mg/kg IV infusionMild muscle rigidity for 3 days Mild muscle rigidity for 3 days Mild T rise (37,6 Mild T rise (37,6 C) for 3 daysC) for 3 daysCPK-MM rise up to 755 IU/lCPK-MM rise up to 755 IU/lNo renal failure! No renal failure!

The 2The 2ndnd case: S., male, 53 yrs. case: S., male, 53 yrs.Anamnesis: Anamnesis: High fever for any fluHigh fever for any fluFever, coffee and physical Fever, coffee and physical exertion:exertion:

• Paresthesias and rigidity in Paresthesias and rigidity in the back and handsthe back and hands

Senior brother has similar signs Senior brother has similar signs

The 2The 2ndnd case: S., male, 53 yrs. case: S., male, 53 yrs.Further examination: Further examination: Unusual echo-structure of the Unusual echo-structure of the musclesmuscles

CPK-MM rise after veloergometryCPK-MM rise after veloergometryEchoCG: small and “thick” heart, EchoCG: small and “thick” heart, LV hypertrophia without LV hypertrophia without hypertension hypertension

The 3The 3rdrd case: T., male, 32 yrs. case: T., male, 32 yrs.Acute simple appendicitis Acute simple appendicitis Routine appendectomyRoutine appendectomySuccinylcholine 2 mg/kg for Succinylcholine 2 mg/kg for tracheal intubationtracheal intubation

Masseter muscle spasm (MMS)Masseter muscle spasm (MMS)MHS was suspected (!)MHS was suspected (!)TIVA with spontaneous breathingTIVA with spontaneous breathing

The 3The 3rdrd case: T., male, 32 yrs. case: T., male, 32 yrs.Cola coloured urine Cola coloured urine Generalized muscle tendernessGeneralized muscle tendernessComplete inability to walkComplete inability to walkRecovery only in a weekRecovery only in a week

The 3The 3rdrd case: T., male, 32 yrs. case: T., male, 32 yrs.Anamnesis: Anamnesis: Remarkable muscle rigidity for Remarkable muscle rigidity for coffee, fever and hunger (not coffee, fever and hunger (not CRAMPS!)CRAMPS!)

At the age of 5 – typical At the age of 5 – typical spontaneous MH crisisspontaneous MH crisis

Examined by neurologist: EEG & Examined by neurologist: EEG & MRI without any resultMRI without any result

Conclusions:Conclusions:• Unusual MH episodes is not Unusual MH episodes is not less dangerous than typical less dangerous than typical ones!ones!

• MH-education of MH-education of anaesthetists and anaesthetists and neurologists could reduce neurologists could reduce the risks!the risks!

Thank youThank you

for the for the attention!attention!