A 44-year-old man was transferred to our hospital due to the sudden onset of bilateral leg pain. His medical history included type I hereditary antithrombin (AT) deficiency (AT activity; 53%), protein C (PC) deficiency (PC activity; 29%), and protein S (PS) deficiency (PS activity; 41%), treatment with edoxaban for pulmonary embolism, and deep venous thromboembolism (VTE) from 30 years of age. He also had branch retinal vein occlusion at 38 years of age. His father had hereditary AT deficiency. There was no prior history of arterial thrombosis, or risk factors for arterial thrombosis including atrial fibrillation, valvular heart disease, polycythemia vera, and chronic obstructive atherosclerosis with dyslipidemia.
Whole-body CT with a contrast agent revealed a massive thrombus attached from the terminal aortic artery to a site proximal to the bilateral common iliac artery (Picture A-C: arrow), and left upper renal ischemia (Picture A, B: arrowhead). After intravenous heparin sodium administration, emergency endovascular thrombectomy was performed to recanalize this thrombus. However, due to the rigid shape of the thrombus, which was firmly attached to the aortic wall, it was difficult to remove. Based on these findings, this thrombus was suspected to be a subacute or chronic thrombus, rather than an acute thrombus. Conventional balloon angioplasty improved the blood flow and the bilateral lower extremity pain. Then intravenous heparin was administered.
Picture.
Type I heredity of AT, PC, and PS deficiency, which are characterized by reduced activity levels, can be associated with an increased risk of VTE (1); however, arterial thrombosis is relatively rare (2). In the present case, although a histological examination of this specimen was not performed, it was highly likely that the aortic artery thrombosis and left renal ischemia were associated with these inherited thrombophilias. As additional risk factors for these thrombi, we suspected that a hypercoagulative state due to unintentional medication non-adherence, dehydration, or smoking might have played a part in the thrombus formation. Although aortic artery thrombosis is rare in patients with inherited thrombophilia, arterial massive thrombus may be life-threatening.
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The authors state that they have no Conflict of Interest (COI).
References
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