Recurrent pulmonary embolism associated with Klippel-Trenaunay-Weber syndrome

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare, congenital disorder characterized by the triad of varicose veins, cutaneous hemangiomas, and hypertrophy of soft tissue and bone. We present the case of a woman with KTWS, cor pulmonale, and death due to recurrent pulmonary embolism (PE). The risk of deep venous thrombosis and PE in patients with KTWS is evaluated, and treatment recommendations are made with emphasis on the role of early, aggressive management in the subset of patients with KTWS known to have thromboembolic disease.