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骨骼肌系统选择性血管造影栓塞在血友病中的作用。

The role of selective angiographic embolization of the musculo-skeletal system in haemophilia.

机构信息

Department of Orthopaedics and Haemophilia Unit, La Paz University Hospital and University Autónoma, Madrid, Spain.

出版信息

Haemophilia. 2009 Jul;15(4):864-8. doi: 10.1111/j.1365-2516.2009.02015.x. Epub 2009 Mar 23.

Abstract

The incidence of haemarthrosis as a result of a spontaneous periarticular aneurysm in haemophilia is very low. In these circumstances, angiographic embolization might be considered as a promising therapeutic and coagulation factor saving option in joint bleeds not responding to replacement of coagulation factor to normal levels. Moreover, embolization should be considered as a possible treatment for postoperative pseudoaneurysms complicating total knee arthroplasty in haemophilia. However, the pathological process of aneurysmal bleeding and clotting factor replacement is entirely different. While embolization is the treatment of choice for some periarticular complications that may occur, it is by no means a panacea for all resistant periarticular bleeds in haemophilia or for postoperative bleeding which usually settles with clotting factor replacement. Another use of arterial embolization is for the treatment of haemophilic tumours of the pelvis, because they can act as a focus for infection and cause cutaneous fistulas. When they present perforations and infections of endogenous origin, their course is usually fatal. Suitable treatment has been investigated on numerous occasions, most of the literature agreeing that the only curative treatment is surgical resection. However, surgical resection after performing arterial embolization to reduce the vascularization of the pseudotumour is a good alternative, thereby reducing the size of the pseudotumour and the risk of bleeding complications during surgery. It is important to bear in mind that despite its efficacy, arterial embolization is an invasive procedure with a reported rate of complications up to 25% (16% minor, 7% serious, 2% death).

摘要

自发性关节旁动脉瘤导致的关节积血在血友病中发病率非常低。在这种情况下,对于不能通过凝血因子替代治疗恢复正常水平的关节积血,血管造影栓塞可能是一种有前途的治疗方法,同时还可以节约凝血因子。此外,对于血友病全膝关节置换术后假性动脉瘤,栓塞也应被视为一种可能的治疗方法。然而,动脉瘤性出血和凝血因子替代的病理过程完全不同。虽然栓塞是治疗某些关节周围并发症的首选方法,但对于血友病患者的所有难治性关节周围出血或术后出血(通常通过凝血因子替代治疗即可解决),它绝不是万能的。动脉栓塞的另一个用途是治疗骨盆血友病性肿瘤,因为它们可能成为感染的焦点,并导致皮肤瘘管。当出现穿孔和内源性感染时,其病程通常是致命的。已经多次研究了合适的治疗方法,大多数文献都认为唯一的治愈性治疗方法是手术切除。然而,在进行动脉栓塞以减少假性肿瘤的血管化后进行手术切除是一个很好的选择,因为它可以减少假性肿瘤的大小并降低手术过程中出血并发症的风险。需要注意的是,尽管动脉栓塞具有疗效,但它是一种有创性操作,据报道其并发症发生率高达 25%(16%为轻度,7%为严重,2%为死亡)。

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