UPPER AIRWAY-OBSTRUCTION IN STURGE-WEBER AND KLIPPEL-TRENAUNAY-WEBER SYNDROMES

Sturge-Weber syndrome is a rare congenital angiomatosis of unknown cau se that is defined by the following triad: facial port-wine stain, lep tomeningeal vascular anomalies, and choroidal vascular lesions associa ted with glaucoma. Klippel-Trenaunay-Weber syndrome is a related disea se with questionable hereditary factors diagnosed by the following tri ad: superficial nevus of the lower extremity, ipsilateral varicose vei ns, and hypertrophy of the soft and bony tissues of the lower limb. Th e two conditions rarely have been reported to coexist. Upper airway ob struction 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.