An unusual variant of Klippel-Trenaunay-Weber syndrome

Abstract

The association of soft tissue and bony overgrowth, varicose veins and port-wine nevus is known as Klippel-Trenaunay-Weber syndrome. An unusual case of a young woman with this syndrome is presented. In addition to the classic features she had face swelling, bilateral limb involvement and an infected cutaneous lymphangioma. No evidence of arteriovenous fistula was demonstrated. In contrast to an abnormal lymphatic system the deep veins of the limbs were normal. Unilateral lung lesions were demonstrated which have not been described previously in such cases.