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Published on: November 6, 2019
Anesthesia for Stüve-Wiedemann syndrome: a rare adult patient case report
Vanessa Artilheiro1, Filipa Portela2, Ana Teresa Reis2
1Department of Anesthesiology, Centro Hospitalar de Lisboa Ocidental, Estrada Forte do Alto Duque, 1449-005, Lisbon, Portugal. vanessa.artilheiro@gmail.com.
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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