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血友病性假肿瘤的外科治疗:一个发展中国家的经验

Surgical Management of Haemophilic Pseudotumors: Experience in a Developing Country.

作者信息

He Yu, Zhou Xi, Cui Haomin, Qiu Guixing, Weng Xisheng, Zhang Baozhong, Liu Yong

机构信息

a Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College , Department of Orthopaedics , Beijing , China.

b Shanghai Jiaotong University Affiliated Sixth People's Hospital , Department of Orthopaedics , Shanghai , China.

出版信息

J Invest Surg. 2019 Mar;32(2):127-136. doi: 10.1080/08941939.2017.1386737. Epub 2017 Nov 2.

Abstract

AIM

Hemophilic pseudotumors result from repeated episodes of bleeding into bone, subperiosteum, and soft tissue. Since clotting factors became available, uncontrolled perioperative bleeding is a less significant problem for surgeons in developed countries. However, they are more difficult to come by in China. Additionally, patients often have to undergo surgery for giant masses and suffer complications. We wanted to present our experience in the surgical management of hemophilic pseudotumors over a 40-year period.

METHODS

We retrospectively reviewed 429 hemorrhagic coagulopathy patients between 1983 and 2015. Diagnosis of hemophilic pseudotumor was confirmed following clinical, radiological, and pathological criteria. The data were recorded and analyzed: type and severity of hemophilia, presence of inhibitor, etiological antecedent, localization of pseudotumors, clinical signs, surgical management and outcomes.

RESULTS

Eighteen pseudotumor patients underwent surgical treatment. All of them were male, with mean age of 34.3 years. Fifteen patients had hemophilia A and three patients had hemophilia B. There were twelve proximal and two distal pseudotumor patients. The mean follow-up was 51.9 months. For pseudotumors in the extremities, complete surgical resection was achieved. For four patients with pelvic or abdominal pseudotumors, complete surgical resection was only achieved in two patients because of preventing potential vital organs injuries. Delayed healing of the incision, allergic reactions, and ureteral injury were the major complications.

CONCLUSION

Surgery is an alternative method with safety and efficacy. Careful and individual treatment is required by the hematologist, orthopedic surgeon and other members of the team who collaborate and participate in hemophilic surgery.

摘要

目的

血友病性假肿瘤是由于反复发生骨、骨膜下及软组织出血所致。自从有了凝血因子后,围手术期出血难以控制在发达国家已不再是外科医生面临的重大问题。然而,在中国凝血因子较难获得。此外,患者常因巨大肿物而需接受手术并遭受并发症。我们想介绍我们在40年期间对血友病性假肿瘤进行外科治疗的经验。

方法

我们回顾性分析了1983年至2015年间429例出血性凝血障碍患者。根据临床、影像学和病理学标准确诊血友病性假肿瘤。记录并分析数据:血友病的类型和严重程度、抑制物的存在情况、病因、假肿瘤的部位、临床体征、外科治疗及结果。

结果

18例假肿瘤患者接受了手术治疗。所有患者均为男性,平均年龄34.3岁。15例为甲型血友病,3例为乙型血友病。有12例近端假肿瘤患者和2例远端假肿瘤患者。平均随访51.9个月。对于肢体假肿瘤,实现了完整的手术切除。对于4例盆腔或腹部假肿瘤患者,因防止潜在重要器官损伤,仅2例实现了完整的手术切除。切口愈合延迟、过敏反应及输尿管损伤是主要并发症。

结论

手术是一种安全有效的治疗方法。血液科医生、骨科医生及参与血友病手术协作的团队其他成员需要进行仔细的个体化治疗。

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