Unusual Manifestations of Susceptibility to Malignant Hyperthermia

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

2. MH Epidemiology 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 ! Wappler F. Eur J Anaesth 2001; 18: 632-52 Gronert GA, Antognini JF In: Anesthesia, Ed. by RD Miller, 1996

3. 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

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

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

6. 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

7. 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

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

9. 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

10. 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

11. The 2nd 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 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!

13. 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

14. 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

15. 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

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

17. 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

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

19. Thank youfor the attention!