Pulmonary thromboembolism in Klippel-Trenaunay-Weber syndrome - should all cases be anticoagulated?

Klippel-Trenaunay-Weber syndrome (KTWS) is a congenital disorder characterized by a triad of varicose veins and venous malformation involving one or more extremities, cutaneous haemangioma, and hypertrophy of soft tissue and bone. The syndrome is rare and it usually presents as varicose veins or cutaneous naevi. We report a patient with KTWS who developed superficial thrombophlebitis in her varicose veins and died from a pulmonary embolism. To optimize management it is important to recognize the syndrome, and the patient should ideally be referred for multidisciplinary vascular care and all such patients should be anticoagulated in the long term.