Freeman-Sheldon Syndrome: Difficult Intubation A case report.
10.4097/kjae.1998.34.6.1254
- Author:
Eun Gyung PARK
1
;
Chi Hyo KIM
;
Jong Hak KIM
;
Choon Hi LEE
Author Information
1. Department of Anesthesiology, College of Medicine, Ewha University, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Genetic factor: freeman-sheldon syndrome;
Intubation, tracheal: difficult;
fiberoptic
- MeSH:
Child;
Clubfoot;
Fingers;
Humans;
Intubation*;
Laryngoscopes;
Microstomia;
Muscular Diseases
- From:Korean Journal of Anesthesiology
1998;34(6):1254-1257
- CountryRepublic of Korea
- Language:Korean
-
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.
