Rodriguez-Merchan E C
Service of Traumatology and Orthopaedic Surgery, and Haemophilia Centre, La Paz University Hospital, Madrid, Spain.
Haemophilia. 2002 May;8(3):393-401. doi: 10.1046/j.1365-2516.2002.00609.x.
It is very likely that with the advent of widespread maintenance therapy, pseudotumours will be less common in the future. It is important that they are diagnosed early, and prevention of muscular haematomas is the key to reducing their incidence. There are a number of therapeutic alternatives for this dangerous condition: surgical removal, percutaneous management, exeresis and filling of the dead cavity, irradiation, and embolization. The management of a patient with a haemophilic pseudotumour is complex, with a high rate of potential complications. Surgical excision is the treatment of choice but should only be carried out in major haemophilia centres by a multidisciplinary surgical team. The main postoperative complications are death, infection, fistulization, and pathological fractures (even requiring amputations of affected limbs). Pelvic pseudotumours can even become complicated by fistulization to the large bowel and by obstruction of the ureters. Untreated, proximal pseudotumours will ultimately destroy soft tissues, erode bone, and may produce neurovascular complications.
随着广泛的维持治疗的出现,未来假肿瘤很可能会变得不那么常见。早期诊断很重要,预防肌肉血肿是降低其发病率的关键。对于这种危险情况有多种治疗选择:手术切除、经皮处理、切除并填充死腔、放射治疗和栓塞。血友病性假肿瘤患者的管理很复杂,潜在并发症发生率很高。手术切除是首选治疗方法,但应由多学科手术团队在主要的血友病中心进行。主要的术后并发症包括死亡、感染、形成瘘管和病理性骨折(甚至需要截肢受影响的肢体)。盆腔假肿瘤甚至可能因与大肠形成瘘管和输尿管梗阻而变得复杂。未经治疗的近端假肿瘤最终会破坏软组织、侵蚀骨骼,并可能产生神经血管并发症。