title: Anesthetic characteristics and airway evaluation of patients with Weill-Marchesani syndrome.
abstract: Weill-Marchesani syndrome (WMS) is a genetic connective tissue disorder associated with fibrous tissue hyperplasia. Weill-Marchesani syndrome is characterized by short stature, broad head and other facial abnormalities such as hypoplastic maxilla and distinctive ocular abnormalities. Joint stiffness is one of the features of this syndrome. We report 5 cases with classical features of WMS who were subjected to different ophthalmic procedures. To the best of our knowledge, this is the first series on the anesthetic management of this rare syndrome. We observe that patients with WMS can present for cataract, glaucoma as well as retinal surgery. Special consideration should be given to difficult intubation, cardiac abnormalities and patient positioning.
authors.affiliation: Department of Anesthesia. King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia. waleed_riad@yahoo.com
authors.forename: Waleed
authors.initials: W
authors.lastname: Riad
authors.forename: Marwan
authors.initials: M
authors.lastname: Abouammoh
authors.forename: Mamdouh
authors.initials: M
authors.lastname: Fathy
mesh_terms: D000015:Abnormalities, Multiple / Q000235:genetics*; D000293:Adolescent; D000328:Adult; D000368:Aged; D000758:Anesthesia / Q000379:methods*; D003240:Connective Tissue Diseases / Q000150:complications* / Q000235:genetics*; D006801:Humans; D008297:Male; D013577:Syndrome