Abstract
Stüve-Wiedemann syndrome (SWS) is a rare genetic disorder characterized by skeletal dysplasia and severe dysautonomia, evidencing a difficult airway approach and likely increased malignant hyperthermia susceptibility. Developmental dysmorphism classically worsens with age, therefore translating in a poor prognosis. In this article, we describe a case of a 27-year-old woman diagnosed with SWS proposed for abscess drainage under dissociative anesthesia. This patient has outlived the life expectancy described for SWS, acknowledging the importance of reporting this rare adult clinical case in what SWS anesthetic management is concerned.
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Communicated by: Michal Witt
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Implication statement
Stüve-Wiedemann syndrome (SWS), as a rare progressive dysmorphic disorder, stands particularly unknown regarding adult patient anesthetic management. Therefore, this manuscript introduces a new anesthetic perspective on an adult case report with a difficult airway and increased malignant hyperthermia susceptibility, as the first publication described in the literature.
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Artilheiro, V., Portela, F. & Reis, A.T. Anesthesia for Stüve-Wiedemann syndrome: a rare adult patient case report. J Appl Genetics 61, 571–573 (2020). https://doi.org/10.1007/s13353-020-00581-5
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DOI: https://doi.org/10.1007/s13353-020-00581-5