General Anesthesia Preceded by Awake - Trial of LMA in a Child with Freeman - Sheldon Syndrome

Freeman-Sheldon Syndrome (FSS), or distal arthrogryposis type 2A, is a rare congenital myopathy and dysplasia characterized by multiple contractures, abnormalities of the head and face, defective development of the hands and feet and skeletal malformations. The facial muscle contracture produces the typical “whistling face” appearance. Anesthetic issues include difficult intravenous access, difficult airway, postoperative pulmonary complications, and possibly malignant hyperthermia and unpredictable responses to neuromuscular blocking agents [1].