Upper Airway Obstruction in Sturge-Weber and Klippel-Trenaunay-Weber Syndromes

Abstract

Sturge-Weber syndrome is a rare congenital angiomatosis of unknown cause that is defined by the following triad: facial port-wine stain, leptomeningeal vascular anomalies, and choroidal vascular lesions associated with glaucoma. Klippel-Trenaunay-Weber syndrome is a related disease with questionable hereditary factors diagnosed by the following triad: superficial nevus of the lower extremity, ipsilateral varicose veins, and hypertrophy of the soft and bony tissues of the lower limb. The two conditions rarely have been reported to coexist. Upper airway obstruction is not a prominent feature of either of these two diseases. We present two patients with both of these angiomatoses in whom severe upper airway obstruction necessitated tracheotomy.