Clinical and morphological features of malignant hyperthermia: a rare case from practice
- Authors: Anichkov N.M.1, Kalinina E.Y.1, Davydova Z.V.1, Shcherbakova E.V.2, Yagmurov O.D.3
-
Affiliations:
- Saint-Petersburg State Pediatric Medical University
- Bureau of Forensic Medical Examination of the Leningrad region
- St. Petersburg Bureau of Forensic Medical Examination
- Issue: Vol 8, No 4 (2022)
- Pages: 97-104
- Section: CASE REPORTS
- URL: https://journals.rcsi.science/2411-8729/article/view/122482
- DOI: https://doi.org/10.17816/fm733
- ID: 122482
Cite item
Full Text
Abstract
Malignant hyperthermia, is one of the most serious complications of modern anesthesia, which is a pharmacogenetic disease phenotypically manifested by skeletal muscle hypermetabolism and rhabdomyolysis during or after general anesthesia using inhalation anesthetics. An unexpected increase in CO2 concentration at the end of exhalation is the most common initial sign of malignant hyperthermia. Atypical forms of this pharmacogenetic disease are observed much more often than lightning or fulminant. In Russia, malignant hyperthermia currently remains a problem.
We present a case of malignant hyperthermia in a 19-year-old girl who underwent surgery for nasal breathing disorders and received Sevoran anesthesia as support. The patient died 1 h 25 min after the surgery during withdrawal from anesthesia caused by a clinically confirmed syndrome of malignant hyperthermia. Forensic autopsy confirmed this diagnosis. The morphological changes in the skeletal muscles using both plain and elective histological stains were described.
The presented observation is valuable owing to the rarity of lightning-fast forms of malignant hyperthermia and the high lethality accompanying these pathologies. This expert case demonstrate how a competent and comprehensive histological examination, including the use of genetic testing, allows correct formulation of a diagnosis and, if necessary, reasonably answer questions from law enforcement agencies.
Full Text
##article.viewOnOriginalSite##About the authors
Nikolai M. Anichkov
Saint-Petersburg State Pediatric Medical University
Email: anichkov@bk.ru
ORCID iD: 0000-0003-1834-7881
SPIN-code: 5222-7003
MD, Dr. Sci. (Med.), Professor
Russian Federation, 2, Litovskay street, Saint-Peterburg, 194100Elena Yu. Kalinina
Saint-Petersburg State Pediatric Medical University
Email: drkalinina@yandex.ru
ORCID iD: 0000-0001-7077-3584
SPIN-code: 1176-5739
MD, Cand. Sci. (Med.)
Russian Federation, 2, Litovskay street, Saint-Peterburg, 194100Zlata V. Davydova
Saint-Petersburg State Pediatric Medical University
Author for correspondence.
Email: zlata.davydova@rambler.ru
ORCID iD: 0000-0002-6673-8230
SPIN-code: 7016-7086
MD, Cand. Sci. (Med.)
Russian Federation, 2, Litovskay street, Saint-Peterburg, 194100Ekaterina V. Shcherbakova
Bureau of Forensic Medical Examination of the Leningrad region
Email: maestrovody@mail.ru
ORCID iD: 0000-0002-3818-1535
SPIN-code: 7685-0130
Russian Federation, Saint Petersburg
Oraz D. Yagmurov
St. Petersburg Bureau of Forensic Medical Examination
Email: oraz.yagmurov@gmail.com
ORCID iD: 0000-0003-1822-6043
SPIN-code: 7765-8978
MD, Dr. Sci. (Med.)
Russian Federation, Saint PetersburgReferences
- Lawal TA, Wires ES, Terry NL, et al. Preclinical model systems of ryanodine receptor 1-related myopathies and malignant hyperthermia: a comprehensive scoping review of works published 1990–2019. Orphanet J Rare Dis. 2020;15(1):113. doi: 10.1186/s13023-020-01384-x
- Gong X. Malignant hyperthermia when dantrolene is not readily available. BMC Anesthesiol. 2021;21(1):119. doi: 10.1186/s12871-021-01328-3
- Yang L, Tautz T, Zhang S, et al. The current status of malignant hyperthermia. J Biomed Res. 2020;34(2):75–85. doi: 10.7555/JBR.33.20180089
- Knuiman GJ, Küsters B, Eshuis L, et al. The histopathological spectrum of malignant hyperthermia and rhabdomyolysis due to RYR1 mutations. J Neurol. 2019;266(4):876–887. doi: 10.1007/s00415-019-09209-z
- Chang L, Daly C, Miller DM, et al. Permeabilised skeletal muscle reveals mitochondrial deficiency in malignant hyperthermia-susceptible individuals. Br J Anaesth. 2019;122(5):613–621. doi: 10.1016/j.bja.2019.02.010
- Larach MG, Localio AR, Allen GC, et al. A clinical grading scale to predict malignant hyperthermia susceptibility. Anesthesiology. 1994;80(4):771–779. doi: 10.1097/00000542-199404000-00008
- Lie JT, Holley KF, Kampa WR, et al. New histochemical method for morphologic diagnosis of early stages of myocardial ischemia. Proc Mayo Clin.1971;46(5):319–327.
Supplementary files
