Department of Orthopaedic Surgery, La Paz University Hospital-IdiPaz, Paseo de la Castellana 261, 28046-Madrid, Spain.
Cardiovasc Hematol Disord Drug Targets. 2020;20(4):242-248. doi: 10.2174/1871529X20666200415121409.
Prevention is essential for avoiding the complications of muscle hematomas (compartment syndrome, pseudotumors 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. Acute compartment syndrome (ACS) requires emergency surgical treatment (decompression fasciotomy). As for pseudotumors, the biopsy will help us confirm the diagnosis and rule out true tumors (chondrosarcoma, liposarcoma, synovial sarcoma) that sometimes mimic hemophilic pseudotumors. Surgical removal of hemophilic pseudotumors 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. Peripheral nerve injuries, which are rare, almost always occur due to compression of hematomas in the vicinity. In most cases, they usually resolve with hematological treatment only. If such treatment fails, surgery would be indicated.
预防是避免血友病患者发生肌肉血肿（间隔综合征、假性肿瘤和周围神经病变）并发症的关键。这可以通过早期诊断肌肉血肿和适当的长期血液学治疗来实现，直到血肿消退（通过影像学研究确认）。超声引导下经皮引流可能有助于更快更好地缓解症状。急性间隔综合征（ACS）需要紧急手术治疗（筋膜切开减压）。至于假性肿瘤，活检将有助于我们确诊并排除真正的肿瘤（软骨肉瘤、脂肪肉瘤、滑膜肉瘤），这些肿瘤有时会模仿血友病性假性肿瘤。手术切除血友病性假性肿瘤是最佳解决方案。作为替代方案，还有刮除和松质骨填充以及放疗（当手术禁忌时）。术前动脉栓塞（理想情况下在手术前 2 周进行）有助于控制巨大骨盆假性肿瘤手术中的术中出血。周围神经损伤很少见，几乎总是由于血肿在附近的压迫引起。在大多数情况下，仅通过血液学治疗通常就可以解决。如果这种治疗失败，则需要手术。
