1. Unusual Manifestations of Susceptibility to Malignant Hyperthermia K. Lebedinski, A. Triadski St. Petersburg

2. Adults in Europe and US… 1:50.000 – 1:150.000 Children and Adolescents… 1:15.000 “Suspected MH” (atypical forms)… up to 1:4.200 ! MH Epidemiology Wappler F. Eur J Anaesth 2001; 18: 632-52 Gronert GA, Antognini JF In: Anesthesia, Ed. by RD Miller, 1996

3. The 1 st 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

4. The 1 st case: F., male, 44 yrs. Succinylcholine 3 mg/kg for tracheal intubation Succinylcholine 3 mg/kg for tracheal intubation Hypotension required Dopamine 5 mkg/kg  min Hypotension required Dopamine 5 mkg/kg  min

5. The 1 st case: F., male, 44 yrs. Immediately after the Anaesthesia: Respiratory weakness Respiratory weakness Prolonged ventilationProlonged ventilation Remarkable Shivering Remarkable Shivering WarmingWarming Stable Circulation Stable Circulation

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

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

8. The 1 st case: F., male, 44 yrs. 48 h after the anaesthesia: High grade ventricular extrasystoli High grade ventricular extrasystoli XylocaineXylocaine Progressive oliguriaProgressive oliguria SalureticsSaluretics

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

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

11. The 2 nd case: S., male, 53 yrs. Acute simple appendicitis Laparoscopic appendectomy Succinylcholine 1 mg/kg to relief appendix removal Immediate tachycardia (110 min -1 ) Immediate diffuse muscle rigidity Adequate breathing and voluntary motions (!)

12. The 2 nd case: S., male, 53 yrs. ICU monitoring for 12 h MgSO 4 100 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!

13. The 2 nd case: S., male, 53 yrs. Anamnesis: High fever for any flu Fever, coffee and physical exertion: Paresthesias and rigidity in the back and handsParesthesias and rigidity in the back and hands Senior brother has similar signs

14. The 2 nd 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

15. The 3 rd 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

16. The 3 rd case: T., male, 32 yrs. Cola coloured urine Generalized muscle tenderness Complete inability to walk Recovery only in a week

17. The 3 rd 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

18. Conclusions: Unusual MH episodes is not less dangerous than typical ones! Unusual MH episodes is not less dangerous than typical ones! MH-education of anaesthetists and neurologists could reduce the risks! MH-education of anaesthetists and neurologists could reduce the risks!

19. Thank you for the attention!