Klippel-Trenaunay syndrome( KTS) is a complex congenital anomaly characterized by lower extremity varix and venous malformations, port-wine stains, and soft tissue and bone hypertrophy. We present a case of a 35-year-old man of KTS with pulmonary embolism (PE). The patient was referred to our hospital complaining of the leg pain and sudden onset of dyspnea. Enhanced computed tomography (CT) showed lower extremity varices, superficial thrombophlebitis and bilateral pulmonary thrombi. No venous malformations except for varicose veins were revealed in the limbs or pelvis. The patient received adequate anticoagulant therapy with edoxaban, followed by intravenous radiofrequency ablation for lower extremity varices. Two months after the operation, the patient suffered from thrombophlebitis in the leg again, however, CT showed no pulmonary thrombus. This case report suggests that intravenous ablation for lower extremity varices in KTS prevented recurrent PE.
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