Recurrent Venous Thromboembolism in a Patient with Klippel-Trenaunay Syndrome Despite Adequate Anticoagulation with Warfarin

Klippel-Trenaunay syndrome (KTS) is a rare genetic condition defined by capillary malformation, venous malformation, and soft tissue and bony overgrowth. Due to venous malformations, individuals are predisposed to intravascular coagulopathy leading to thrombosis and thromboembolism. However, anticoagulating these patients long-term remains a challenge because of the presence of capillary malformations that increase bleeding risk. We present a rare case of a 30-year-old Caucasian male with KTS and history of gastrointestinal bleeding who has been on anticoagulation since the age of 7 and has had three different inferior vena cava filters placed during his lifetime. At presentation, he had dyspnea with stable vital signs. His prothrombin time/international normalized ratio was 37.3 and 3.2, respectively and chest computed tomography showed bilateral segmental pulmonary embolism (PE). He was treated with heparin drip and his home anticoagulation was switched from warfarin to apixaban at the time of discharge for better anticoagulation optimization. KTS is a condition associated with venous thromboembolic complications that can be difficult to manage. PE should remain on the top of the list of differential diagnoses in patients with KTS presenting with dyspnea even if laboratory findings suggest an alternate diagnosis.