Kamal Achmad Fauzi, Waryudi Agus, Kurniawan Aryadi, Lubis Anna Mira, Gatot Djayadiman
Department of Orthopaedic and Traumatology Dr.Cipto Mangunkusumo General Hospita, Faculty of Medicine, University of Indonesia, Jakarta, Indonesia.
Integrated Hamophillia TeamDr. Cipto Mangunkusumo General Hospital, Faculty of Medicine, University of Indonesia, Jakarta, Indonesia.
Arch Bone Jt Surg. 2019 Nov;7(6):514-522.
Bleeding episodes in severe hemophilia may occur more frequently and spontaneously after mild trauma or daily activities. An inadequate treatment of that bleeding in hemophilia may result in pseudotumor, usually in the muscle adjacent to the bone. We reported haemophilic pseudotumor treated with various surgical interventions.
This study was conducted inthe Department of Orthopaedic and Traumatology at a government hospital over a period of 7 years(2010 -2017). Patients Perioperative management was done in accordance with the Integrated Hemophilia Team of our institution protocol.Diagnosis and management planning of hemophilic pseudotumor was confirmed via Integrated HemophiliaTeam meeting. After the surgery, all patients were asked to come for routine follow up.
We reported six Haemophilia-A patients with pseudotumor in the pelvis, proximal femur and lower leg. One case in pelvic bone underwent hematoma evacuation, acetabular reconstruction using the Harrington procedure, and total hip arthroplasty.Two cases, a case in the proximal femur and another case in the distal fibula, were treated with amputation, other two cases, one was soft tissue psedotumor in the pelvic region and was treated by hematoma evacuation, and the remaining casewas managed with wide excision and followed by defect closure.
Surgery is a preferable treatment for pseudotumors that have been present for years.It's associated with the best outcomes especially when selected as the primary line ofwith preventable and manageable bleeding complication. As previously published by many authors, this paper confirms that surgical excision is the treatment of choice but should only be carried out in major hemophilia centers by a multidisciplinary surgical team.
严重血友病患者在轻度创伤或日常活动后可能更频繁地自发出现出血事件。血友病患者出血治疗不当可能导致假肿瘤,通常发生在靠近骨骼的肌肉中。我们报告了采用各种手术干预治疗血友病性假肿瘤的情况。
本研究在一家政府医院的骨科和创伤科进行,为期7年(2010 - 2017年)。患者围手术期管理按照我们机构综合血友病团队的方案进行。血友病性假肿瘤的诊断和管理计划通过综合血友病团队会议确认。手术后,要求所有患者前来进行常规随访。
我们报告了6例患有骨盆、股骨近端和小腿假肿瘤的甲型血友病患者。1例骨盆骨患者接受了血肿清除、使用哈灵顿手术进行髋臼重建以及全髋关节置换术。2例患者,1例股骨近端和1例腓骨远端患者接受了截肢治疗,另外2例患者,1例是骨盆区域的软组织假肿瘤,接受了血肿清除治疗,其余1例患者接受了广泛切除并随后进行缺损闭合。
手术是治疗已存在多年的假肿瘤的首选方法。它与最佳预后相关,尤其是当被选为主要治疗方法并伴有可预防和可管理的出血并发症时。正如许多作者之前发表的那样,本文证实手术切除是首选治疗方法,但应仅由多学科手术团队在主要血友病中心进行。