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.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Driessen J (2018) StüveWiedemann syndrome. Anästh Intensivmed 59:S637–S643. https://doi.org/10.19224/ai2018.S637
Gaspar IM, Saldanha T, Cabral P et al (2008) Long-term follow-up in Stuve–Wiedemann syndrome. Am J Med Genet A 146A:1748–1753. https://doi.org/10.1002/ajmg.a.32325
Kurdi MS, Theerth KA, Deva RS (2014) Ketamine: current applications in anesthesia, pain, and critical care. Anesth Essays Res 8(3):283–290. https://doi.org/10.4103/0259-1162.143110
Mikelonis D, Jorcyk CL, Tawara K et al (2014) Stüve-Wiedemann syndrome: LIFR and associated cytokines in clinical course and etiology. Orphanet J Rare Dis 9:34. https://doi.org/10.1186/1750-1172-9-34
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors declare that they have no conflict of interest.
Informed consent
Informed consent was obtained for publication.
Additional information
Communicated by: Michal Witt
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
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.
Rights and permissions
About this article
Cite this article
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
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s13353-020-00581-5