KLIPPEL-TRENAUNAY-WEBER SYNDROME COMPLICATED WITH CEREBRAL VENOUS THROMBOSIS

摘要

Klippel-Trenaunay-Weber syndrome is a rare and little known congenital disease among physicians, characterized by vascular stains, soft tissue and bone asym-metric hypertrophy, varicose veins, lymphedemas, and arteriovenous fistulas. Alterations in the vascularization of the central nervous system are rare in these cases. This article presents the case of a 32-year-old puerperal black woman, with a history of recurrent lymphangitis, chronic right lower limb lymphedema since childhood, and treatment with oral contraceptives, who came to the Emergency Room presenting left-side hemiparesis and hypoesthesia. Physical examination showed motor and sensory defect, as well as flat hemangioma in the plantar face of the left foot, and right hemi-body hypertrophy. Computed axial tomography and magnetic resonance imaging evidenced the presence of cerebral venous throm-bosis and arteriovenous malformation in the right parieto-occipital region.