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