Freeman-Sheldon Syndrome: Difficult Intubation A case report / 대한마취과학회지

ABSTRACT

The Freeman-Sheldon syndrome (FSS) is a rare congenital myopathy. Main manifestations are "whistling face", camptodactyly with ulnar deviation of the finger and talipes equinovarus. Myopathic fibrotic circumoral musculature result in microstomia with the characteristic protruding pursed "whistling lip" and mandibular and laryngeal development may also be abnormal. Thus the patients with FSS are expected to difficult intubation and the use of muscle relaxant should not be expected to improve intubating condition. We report a children with the Freeman-Sheldon syndrome who was intubated with fiberoptic laryngoscope and discuss anesthetic consideration.