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MALIGNANT HYPERTHERMIA AND CONGENITAL VERTEBRAE MALFORMATIONS



Abstract

Malignant hyperthermia (MH) is a pharmacogenetic disorder, potentially lethal, due to the exposure to anesthetic drugs that triggers, a high increase of corporal temperature, progressive muscular stiffness, severe rabdomiolisis and death due to cardiac dysfunction. Many research works relate Malignant Hyperthermia to muscular illnesses or to the King Syndrome. Through this study we present the incidence of MH in patients with congenital vertebrae malformations. (CVM)

The objective is to establish the incidence of the MH in patients who were operated on CVM and to alert about this association.

1029 patients with CVM were treated between 1972 and 2000. 390 with congenital vertebrae malformation were operated on. 3 patients (0.76%) (1 girl and 2 boys) developed MH while they underwent surgical treatment for the CVM. 1 patient presented an isolated congenital vertebrae malformation. 1 patient presented King Syndrome and the other presented Robert Syndrome. Only 1 elevated amount of preoperative CPK was found (the are no reports on the others).

No muscular biopsy was done to test sensitivity. Two of them were biopsied for a post episode study. At the surgical moment, any patients reported personal or familiar antecedents of MH. No deaths were reported, although it is considered as a potentially lethal disorder.

We found no reports in the literature in this subject. Most of the bibliographic data belonged to anesthesiologists or geneticists. Our approach as spine surgeons leaded us to the detailed analysis of this studies and the 0.76% (3 out of 390) incidence suggested us to have an alert attitude when facing patients with surgical MVC and take the necessary precautions.