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血友病中的假性动脉瘤

Pseudoaneurysms in haemophilia.

作者信息

Rodriguez-Merchan E Carlos

机构信息

Department of Orthopaedic Surgery, La Paz University Hospital, Paseo de la Castellana 261, Madrid, Spain.

出版信息

Blood Coagul Fibrinolysis. 2013 Jul;24(5):461-4. doi: 10.1097/MBC.0b013e32835e42aa.

Abstract

The incidence of bleeding as a result of a pseudoaneurysm in haemophilia is very low. The diagnosis should be suspected if the patient has a history of arterial trauma. Pseudoaneurysms can appear anywhere where trauma occurs. This can include arterial access for catheterization, blunt trauma or penetrating trauma. The diagnosis should be confirmed using Duplex ultrasonography, computed tomography (CT) angiogram or conventional angiogram. Many options exist for the treatment of pseudoaneurysms. Although surgery was the gold standard treatment in the past (surgical ligation with or without distal bypass), several less invasive treatment options are popular today. They include covered stent, ultrasound probe compression and ultrasound-guided thrombin injection. So far, only 14 pseudoaneurysms have been reported in patients with haemophilia: nine were located in the musculoskeletal system (four in the hand, four in the knee, one in the ankle), whereas five were non-musculoskeletal. Early diagnosis and treatment of this complication is vital. Endovascular treatment offers a minimally invasive treatment option. If arterial embolization fails to solve the pseudoaneurysm, open vascular surgery with surgical ligation with or without distal bypass should be performed. Whatever the procedure, a correct surgical haemostasis must be achieved by the infusion of factor concentrate (recombinant or plasma-derived) at the right dose and tranexamic acid. The advent of activated prothrombin complex concentrates and recombinant factor VIII (rFVIIa) has made invasive procedures possible in haemophilia patients with high-titre inhibitors.

摘要

血友病患者因假性动脉瘤导致出血的发生率非常低。如果患者有动脉创伤史,应怀疑有此诊断。假性动脉瘤可出现在任何发生创伤的部位。这包括导管插入术的动脉通路、钝性创伤或穿透性创伤。应使用双功超声、计算机断层扫描(CT)血管造影或传统血管造影来确诊。假性动脉瘤的治疗有多种选择。虽然手术在过去是金标准治疗方法(有或无远端搭桥的手术结扎),但如今一些侵入性较小的治疗选择很受欢迎。它们包括覆膜支架、超声探头压迫和超声引导下注射凝血酶。到目前为止,血友病患者中仅报告了14例假性动脉瘤:9例位于肌肉骨骼系统(4例在手,4例在膝,1例在踝),而5例为非肌肉骨骼系统。早期诊断和治疗这种并发症至关重要。血管内治疗提供了一种微创治疗选择。如果动脉栓塞未能解决假性动脉瘤,应进行有或无远端搭桥的手术结扎的开放血管手术。无论采用何种手术,都必须通过输注正确剂量的因子浓缩物(重组或血浆源性)和氨甲环酸来实现正确的手术止血。活化凝血酶原复合物浓缩物和重组凝血因子VIII(rFVIIa)的出现使有高滴度抑制剂的血友病患者能够进行侵入性手术。

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