Total Intravenous Anesthesia for Freeman-Shelden Syndrome / 대한마취과학회지

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.