Klippel-Trenaunay-Weber syndrome complicated with cerebral venous thrombosis

Abstract

Klippel-Trenaunay-Weber syndrome is a rare and little known congenital disease among physicians, characterized by vascular stains, soft tissue and bone asymmetric 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 thrombosis and arteriovenous malformation in the right parieto-occipital region.