Klippel-Trenaunay syndrome with a life-threatening thromboembolic event

Abstract

Klippel-Trenaunay syndrome is a congenital disorder characterised by the triad of cutaneous vascular nevi, soft tissue or bony hypertrophy, and varicose veins or venous malformations involving one or more extremities. An incidence of venous thromboembolism of up to 22% has been reported in this disorder. Also reported but rare is the development of trophic changes. Herein, we report the case of a male with Klippel-Trenaunay syndrome, deep vein thrombosis, venous ulceration, and death due to recurrent pulmonary embolism.