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J Clin Anesth. 2003 May;15(3):214-6.

Airway management of a patient with Weill-Marchesani syndrome.

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Faculty of Medicine, Department of Anesthesiology and Reanimation, Gazi University, Ankara, Turkey.


Weill-Marchesani syndrome is a genetically determined rare systemic connective tissue disorder consisting of brachymorphy, brachydactyly, aortic stenosis, and ophthalmologic abnormalities, such as ectopia lentis, microspherophakia, and secondary glaucoma. The primary lesion is fibrous tissue hyperplasia. Airway control and intubation may be difficult in patients with WMS because of stiff joints, poorly aligned teeth, and maxillary hypoplasia with a narrow palate. We describe the successful airway management of a patient with WMS and laryngeal stenosis, using an intubating laryngeal mask following failed conventional laryngoscopic intubation.

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