Zhai Jiliang, Weng Xisheng, Zhang Baozhong, Liu Yong, Gao Peng, Bian Yan-Yan
1Department of Orthopaedic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
J Bone Joint Surg Am. 2017 Jun 7;99(11):947-953. doi: 10.2106/JBJS.16.01299.
Hemophilic pseudotumor (HPT) is a rare disease with many challenges. Only a few reports on surgical treatment for HPT have been published.
The cases of 23 patients with HPT who had surgical treatment from July 1996 to December 2014 were retrospectively reviewed. Demographic data, blood loss and transfusion during surgery, outcomes, and complications after surgery were analyzed.
Eleven patients underwent HPT resection; 4 underwent HPT excision, allograft transplantation, and absorbable screw fixation; 3 had HPT resection and metallic internal fixation; 2 had HPT resection, autogenous fibular grafting, and absorbable screw fixation; 2 underwent curettage and bone-grafting; and 1 patient received above-the-knee amputation. The average age (and standard deviation) of the patients at the time of surgery was 31.9 ± 12.8 years (range, 6 to 54 years) with an average follow-up of 5.3 ± 4.7 years (range, 1.1 to 19.6 years). The median duration of the surgery was 157 minutes (range, 90 to 315 minutes). The median amount of blood loss during surgery was 800 mL (range, 100 to 4,000 mL). Three patients (13%) had a postoperative infection, 2 (8.7%) had recurrence of HPT, and another 2 patients had fracture nonunion.
Surgical treatment of HPT with a modified protocol of coagulation factor replacement is safe and effective. It should be recommended for patients with HPT who have progressive enlargement of the mass, recurrent and massive bleeding, spontaneous perforation, bone erosion, or compression of surrounding tissues or who have had failure of conservative treatment.
Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.
血友病性假肿瘤（HPT）是一种存在诸多挑战的罕见疾病。关于HPT手术治疗的报道仅有少数几篇。
回顾性分析1996年7月至2014年12月期间接受手术治疗的23例HPT患者的病例。分析人口统计学数据、手术期间的失血量和输血量、手术结果以及术后并发症。
11例患者接受了HPT切除术；4例接受了HPT切除、同种异体移植和可吸收螺钉固定；3例进行了HPT切除和金属内固定；2例进行了HPT切除、自体腓骨移植和可吸收螺钉固定；2例接受了刮除术和骨移植；1例患者接受了膝上截肢术。患者手术时的平均年龄（及标准差）为31.9±12.8岁（范围为6至54岁），平均随访时间为5.3±4.7年（范围为1.1至19.6年）。手术的中位持续时间为157分钟（范围为90至315分钟）。手术期间的中位失血量为800毫升（范围为100至4000毫升）。3例患者（13%）发生术后感染，2例（8.7%）HPT复发，另外2例患者出现骨折不愈合。
采用改良的凝血因子替代方案对HPT进行手术治疗是安全有效的。对于肿块进行性增大、反复大量出血、自发性穿孔、骨质侵蚀、周围组织受压或保守治疗失败的HPT患者，应推荐手术治疗。
治疗性四级证据。有关证据水平的完整描述，请参阅作者指南。
