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血友病性假肿瘤:诊断与管理

Hemophilic Pseudotumors: Diagnosis and Management.

作者信息

Rodriguez-Merchan E Carlos

机构信息

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

出版信息

Arch Bone Jt Surg. 2020 Mar;8(2):121-130. doi: 10.22038/abjs.2019.40547.2090.

DOI:10.22038/abjs.2019.40547.2090
PMID:32490041
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7191985/
Abstract

Prevention is essential for avoiding the complications of muscle hematomas (pseudotumors, compartment syndromes and peripheral nerve lesions) in hemophilic patients. This is achieved through early diagnosis of muscle hematomas and proper long-term hematological treatment until they have resolved (confirmed by image studies). Ultrasound-guided percutaneous drainage could be beneficial in terms of achieving better and faster symptom relief. When suspecting a hemophilic pseudotumor, biopsy will help us confirm the diagnosis and rule out true tumors (chondrosarcoma, liposarcoma, synovial sarcoma) that sometimes mimic hemophilic pseudotumor. Surgical removal of hemophilic pseudotumor is the best solution. As alternatives, there are curettage and filling with cancellous bone and radiotherapy (when surgery is contraindicated). Preoperative arterial embolization (ideally 2 weeks before surgery) helps control intraoperative bleeding during surgery for giant pelvic pseudotumors.

摘要

预防对于避免血友病患者发生肌肉血肿并发症(假肿瘤、骨筋膜室综合征和周围神经损伤)至关重要。这可通过早期诊断肌肉血肿并进行适当的长期血液学治疗直至血肿消退(经影像学检查证实)来实现。超声引导下经皮引流在更好、更快缓解症状方面可能有益。当怀疑为血友病假肿瘤时,活检有助于我们确诊并排除有时会模仿血友病假肿瘤的真性肿瘤(软骨肉瘤、脂肪肉瘤、滑膜肉瘤)。手术切除血友病假肿瘤是最佳解决方案。作为替代方案,有刮除并填充松质骨和放射治疗(手术禁忌时)。术前动脉栓塞(理想情况下在手术前2周)有助于控制巨大盆腔假肿瘤手术中的术中出血。

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本文引用的文献

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The role of orthopaedic surgery in haemophilia: current rationale, indications and results.骨科手术在血友病中的作用:当前的理论依据、适应症及结果
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Intraosseous pseudotumour in a child with mild hemophilia B: report of a rare case and brief review of literature.一名患有轻度乙型血友病儿童的骨内假肿瘤:罕见病例报告及文献简要回顾
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