TY - CHAP M1 - Book, Section TI - Freeman-Sheldon Syndrome (FSS) A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - Freeman-Sheldon Syndrome, or distal arthrogryposis Type 2A, is a rare genetic malformative disorder characterized by microstomia, 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, and postoperative pulmonary complications. Although an association with malignant hyperthermia has been suggested, this has not been confirmed. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1164070298 ER -