Summary
Data are presented on ten cases of anaesthesia-induced malignant hyperthermia in Norway. Seven of the patients died, three recovered. The fatal cases were all boys in the age group 11-20 years. This age and sex distribution suggests that puberty with the increase in androgens is a precipitating factor in malignant hyperthermia. One of the victims who survived was a 4 1/2-year-old pseudohermaphrodite girl with the adrenogenital syndrome. The coincidence of malignant hyperthermia in a patient with such a rare syndrome points to the excessive formation of androgens in patients with this syndrome as a predisposing factor.
The indications for surgery were traumatic injuries in five cases, congenital abnormalities in three and appendicitis in two cases. These conditions in themselves may cause an increased sensitivity to suxamethonium.
One patient received only hexobarbitone, halothane and suxamethonium. After the last drug jaw rigidity and temperature rise to 41.3° C prompted the anaesthetist to end the anaesthetic. The fact that the patient survived proves that suxamethonium induced jaw rigidity is valuable as a warning.
The absence of cardiovascular depression after procaine 3.5 g in one patient is ascribed to the correction of acidosis at the time of infusion of this drug. It is suggested that procaine should be withheld until other measures such as cooling, correction of acidosis and steroid therapy have been tried.
Résumé
Dix cas d’hyperthermie maligne survenus en Norvège sont rapportés et discutés. Sept des dix patients sont morts et trois ont survécu. Tous les décès sont survenus chez des garçons âgés de onze à vingt ans, ce qui suggère que la période de puberté qui s’accompagne d’une augmentation des androgènes peut représenter un facteur déclenchant. L’un des survivantes, une petite fille de quatre ans et demi, présentait un pseudo-hermaphrodisme avec syndrome adrenogenital. Le fait d’observer une Hyperthermie maligne chez une patiente présentant déjà une pathologie aussi rare nous semble indiquer que la formation excessive d’hormones androgènes que l’on retrouve dans ces cas peut agir comme facteur déclenchant de l’hyperthermie.
Cinq des patients furent opérés pour traumatismes, trois pour des malformations congénitales et deux pour appendicite. Ces trois pathologies sont en soi susceptibles d’augmenter la sensibilité à la Succinylcholine.
L’un des patients reçut seulement de l’Hexobarbitone, de l’Halothane et du Suxa methanium. A la suite de l’injection de Suxamethanium, on observa une rigidité du maxillaire et la température s’éleva à 41.3 degrés Centigrade. L’anesthésie fut arrêtée. La survie du malade illustre que la rigidité du maxillaire observée après l’injection de Suxamethanium est un signe très fiable.
On attribue à la correction préalable d’une acidose métabolique chez un malade, l’absence de dépression cardio-respiratoire notée après une dose de 3.5 grammes de Procaine. Les auteurs suggèrent de corriger l’acidose, d’administrer des Steroides et de tenter de refroidir, avant denvenir à la Procaine.
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Stovner, J., Innes, K.R. & Holen, A. Ten cases of malignant hyperthermia in norway. Canad. Anaesth. Soc. J. 23, 518–526 (1976). https://doi.org/10.1007/BF03005980
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DOI: https://doi.org/10.1007/BF03005980