Recurrent pulmonary embolism associated with Klippel-Trenaunay-Weber syndrome

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare, congenital disorder char acterized by the triad of varicose veins, cutaneous hemangiomas, and hypert rophy of soft tissue and bone. We present the case of a woman with KTWS, co r pulmonale, and death due to recurrent pulmonary embolism (PE). The risk o f deep venous thrombosis and PE in patients with KTWS is evaluated, and tre atment recommendations are made with emphasis on the role of early, aggress ive management in the subset of patients with KTWS known to have thromboemb olic disease.