Anesthetic characteristics and airway evaluation of patients with Weill-Marchesani syndrome.
نویسندگان
چکیده
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.
منابع مشابه
A Case of Weill-Marchesani Syndrome with Inversion of Chromosome 15
PURPOSE To present a case of Weill-Marchesani syndrome with corneal endothelial dysfunction due to anterior dislocation of a spherophakic lens and corneolenticular contact. METHODS A 17-year-old woman presented with high myopia and progressive visual disturbance. She was of short stature and had brachydactyly. Her initial Snellen best corrected visual acuity (BCVA) was 20/50 (-sph 20.50 -cyl ...
متن کامل[Weill-Marchesani syndrome].
Weill-Marchesani syndrome is a rare systemic connective tissue disease characterized by short stature, brachydactyly, ectopia lentis and spherophakia. This was first reported by Weill in 1932 and subsequently well characterized by Marchesani in 1939(1). Genetically this syndrome often shows autosomal recessive inheritance. Frequent consanguinity between parents support this model. There have be...
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ورودعنوان ژورنال:
- Middle East journal of anaesthesiology
دوره 18 4 شماره
صفحات -
تاریخ انتشار 2006