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成骨不全症患者骨折手术治疗的中期疗效。

Mid-term outcomes of surgical treatment in fractures in patients with osteopetrosis.

机构信息

Department of Orthopaedic Surgery, Dicle University, Faculty of Medicine, Diyarbakır, Turkey.

出版信息

Bone Joint J. 2020 Aug;102-B(8):1082-1087. doi: 10.1302/0301-620X.102B8.BJJ-2020-0431.R1.

Abstract

AIMS

Osteopetrosis (OP) is a rare hereditary disease that causes reduced bone resorption and increased bone density as a result of osteoclastic function defect. Our aim is to review the difficulties, mid-term follow-up results, and literature encountered during the treatment of OP.

METHODS

This is a retrospective and observational study containing data from nine patients with a mean age of 14.1 years (9 to 25; three female, six male) with OP who were treated in our hospital between April 2008 and October 2018 with 20 surgical procedures due to 17 different fractures. Patient data included age, sex, operating time, length of stay, genetic type of the disease, previous surgery, fractures, complications, and comorbidity.

RESULTS

The mean follow-up period was 92.5 months (25 to 140). Bony union was observed in all of our patients. Osteomyelitis developed in two patients with femoral shaft fractures, and two patients had peri-implant stress fractures.

CONCLUSION

Treatment of fractures in OP patients is difficult, healing is protracted, and the risk of postoperative infection is high. In children and young adults with OP who have open medullary canal and the epiphyses are not closed, fractures can be treated with surgical techniques such as intramedullary titanium elastic nail (TENS) technique or fixation with Kirschner (K)-wire. Cite this article: 2020;102-B(8):1082-1087.

摘要

目的

成骨不全症(OP)是一种罕见的遗传性疾病,由于破骨细胞功能缺陷,导致骨吸收减少和骨密度增加。我们旨在回顾治疗 OP 时遇到的困难、中期随访结果和文献。

方法

这是一项回顾性和观察性研究,包含了 2008 年 4 月至 2018 年 10 月期间在我院接受治疗的 9 例 OP 患者(3 名女性,6 名男性)的数据,平均年龄为 14.1 岁(9 至 25 岁),因 17 处不同部位骨折共进行了 20 次手术。患者数据包括年龄、性别、手术时间、住院时间、疾病的遗传类型、既往手术、骨折、并发症和合并症。

结果

平均随访时间为 92.5 个月(25 至 140 个月)。所有患者均观察到骨愈合。2 例股骨干骨折患者发生骨髓炎,2 例患者发生植入物周围应力性骨折。

结论

OP 患者骨折的治疗较为困难,愈合时间较长,术后感染风险较高。对于患有 OP 的儿童和青少年,若干骺端未闭合且骨髓腔未闭合,可以采用髓内钛弹性钉(TENS)技术或克氏针(K)固定等手术技术进行治疗。

引用本文

2020;102-B(8):1082-1087.

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