Department of Orthopaedics and Traumatology, Nanfang Hospital, Southern Medical University, Guangzhou, China.
Department of Orthopaedic Surgery, Zhongnan Hospital, Wuhan University, Wuhan, China.
Haemophilia. 2020 Sep;26(5):873-881. doi: 10.1111/hae.14109. Epub 2020 Jul 22.
Haemophilic pseudotumor (HPT) is a rare but challenging complication of haemophilia. This study was intended to provide our experience about clinical characteristics and surgical treatment of HPT.
Clinical medical records were retrieved from the Hemophilia Center, Nanfang Hospital, to identify the patients who had been surgically treated from 1 January 2006 to 31 December 2017 with a definite diagnosis of HPT. Their clinical features, surgical management, outcomes and complications after surgery were analysed.
We identified 34 patients with HPT who had surgical treatment over a 12-year period and five of them had multiple HPTs. The incidence of HPT at this centre was 2.3% over the dozen years. A previous trauma leading to the development of HPT was reported in 18 cases (52.9%). The HPT affected only soft tissue in 7 patients, bone and soft tissue in 25 ones and joint in 2 ones. Preoperative infection and fistula formation happened in ten patients, two of whom were related to abdominal HPTs. Enterococcus faecalis was cultured in five cases with fistula formation. HPT associated with pathological fracture was observed in five cases, two of whom were treated by external fixation and 3 by HPT resection and metallic internal fixation. Amputation was performed for nine patients, 6 of whom had preoperative infection and fistula formation. Their follow-up duration averaged 4.2 ± 2.9 years (range, from 1 to 13.5 years) after surgery. Of all our cases, three suffered from postoperative infection, five from recurrence of HPT and two with external fixation from fracture non-union.
HPT patients with preoperative infection had worse prognosis than those without. Surgical treatment plus intensive replacement therapy was effective for HPT but with a high rate of complications. HPT resection and metallic internal fixation rather than external fixation should be recommended for HPT patients with pathological fracture.
血友病性假瘤（HPT）是血友病罕见但极具挑战性的并发症。本研究旨在提供我们在 HPT 的临床特征和手术治疗方面的经验。
从南方医院血友病中心检索临床病历，以确定自 2006 年 1 月 1 日至 2017 年 12 月 31 日期间接受手术治疗且明确诊断为 HPT 的患者。分析其临床特征、手术管理、治疗效果及术后并发症。
我们确定了 12 年间接受手术治疗的 34 例 HPT 患者，其中 5 例有多个 HPT。12 年间该中心 HPT 的发病率为 2.3%。18 例（52.9%）有导致 HPT 发展的既往创伤史。7 例 HPT 仅影响软组织，25 例影响骨和软组织，2 例影响关节。10 例患者术前存在感染和瘘形成，其中 2 例与腹部 HPT 相关。5 例瘘形成患者培养出粪肠球菌。5 例 HPT 合并病理性骨折，其中 2 例采用外固定治疗，3 例采用 HPT 切除和金属内固定治疗。9 例患者截肢，其中 6 例术前存在感染和瘘形成。术后随访时间平均为 4.2±2.9 年（1~13.5 年）。所有患者中，3 例发生术后感染，5 例 HPT 复发，2 例外固定后骨折不愈合。
术前感染的 HPT 患者预后较差。手术治疗加强化替代治疗对 HPT 有效，但并发症发生率较高。对于合并病理性骨折的 HPT 患者，建议行 HPT 切除和金属内固定，而非外固定。
