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Korean Journal of Anesthesiology 2000;39(6):901-904.
DOI: https://doi.org/10.4097/kjae.2000.39.6.901   
Total Intravenous Anesthesia for Freeman-Shelden Syndrome.
Byung Dal Lee, Hyun Joo Ahn, Nam Ki Park, Ji Yeon Kim
Department of Anesthesiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Freeman-Sheldon syndrome (FSS) is a rare congenital myopathy and dysplasia characterized by a whistling mouth and hand-foot anomalies. There are several anesthetic challenges like difficult airway and intravenous access, and the possibility of malignant hyperthermia. We controlled the anesthetic management of a patient with FSS undergoing contractured thumb correction. Intubation was done with a tracheal tube by direct laryngoscopy after three failed attempts. An anesthetic technique triggering malignant hyperthermia was not used. Anesthetic agents used were ketamine, propofol, fentanyl and nitrous oxide under spontaneous respiration. The child had an uneventful anesthetic course and no sign or symptoms of malignant hyperthermia.
Key Words: Anesthesia: pediatric, Freeman-Sheldon Syndrome; Complication: malignant hyporthermia; Intubation, tracheal: difficult


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