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骨肿瘤

骨纤维结构不良 2015-04-07 程瑞林 骨纤

发表者:程瑞林 1137人已读

骨纤维结构不良

2015-04-07 程瑞林 山东大学第二医院足踝外科程瑞林

骨纤维结构不良

【概述】

骨纤维结构不良(osteofibrous dysplasia)是一种骨髓和网状骨被纤维结缔组织和不规则骨所代替的纤维骨性病变,过去称为骨纤维异常增殖症,并非真性肿瘤【2】。

Osteofibrous dysplasia is a rare, nonneoplastic condition of unknown etiology that affects the long bones. It frequently is asymptomatic.Most lesions of osteofibrous dysplasia affect the cortex of the tibia, predominantly the middle third of the diaphysis. The cortex often is expanded and thinned, with multiple radiolucencies mixed with intervening areas of sclerosis. The second most common site of involvement is the fibula.【4】

【临床表现】

多见于青少年。好发部位为肋骨、股骨和胫骨,足踝部发病较少。常见的症状是局部疼痛,常由于病理性骨折而是引起。部位表浅者可发现在病骨膨胀变形。累及负重下肢者,可合并各种畸形。它可以是单骨性或多骨性。多骨病损伴有皮肤色素沉着和内分泌紊乱,特别是性早熟,称为Albright综合征。它主要发生在儿童,特别是女孩。偶尔纤维结构不良可恶变成纤维肉瘤或骨肉瘤,恶变率为0.4%。

【辅助检查】

X线表现:典型的“磨砂玻璃”样X线表现是死骨更肉主要诊断依据。病变可位于长骨干骺端或骨干的髓腔内,可呈囊状膨胀、磨砂玻璃样、丝瓜瓤状或虫蛀样表现,皮质往往膨胀变薄,病损边缘有致密硬化,称为“橘皮”。


【病理】

Typical histologic appearance of the lesion under 100X magnification. Note the zonal architecture with a periphery of active osteoblasts surrounding bone trabeculae.【4】


Histologic section under 100X magnification demonstrating vascular channels within the lesion, which has been proposed as the etiologic factor in the development of the lesion.【4】


【治疗】

Osteofibrous dysplasia is treated with marginal resection with or without bone grafting, depending on the size of the lesion and the extent of bony involvement. However, due to the high rate of recurrence in skeletally immature individuals, this procedure is usually postponed until skeletal maturity.【3】

Due to the high recurrence rate, many authors advocate nonoperative treatment of the lesion until after skeletal maturity is reached, at which time marginal resection and bone grafting may be performed without increased risk of recurrence. For patients of any age, surgical correction of associated deformities may be required. Surgery may be indicated if the lesion is aggressive or if the patient experiences multiple pathologic fractures. Resection of large portions of the lesion usually is not necessary and only increases susceptibility to recurrent fractures.【4】

Contraindications:

Though nonoperative management is recommended in patients who are skeletally immature, there are no absolute contraindications to surgical intervention in children, with the exception of any underlying medical or anesthetic issues. Operative management is not recommended in patients who are skeletally immature, because of the high recurrence rate following resection and curettage and because of the predisposition to fracturing after the bone has been weakened by biopsy. Pathologic fracture does not necessarily require surgical management, since cast immobilization frequently results in good healing.【4】

治疗目的是为了防止畸形和骨折发生,治疗涉及增强骨强度,纠正畸形。有报道使用双磷酸盐治疗骨纤维结构不良,可以减小疼痛,预防骨折,减小骨纤维结构不良范围。单纯的刮除植骨术后,很多病例复发,尤其在儿童阶段,复发率很高。

对较大的单骨型,可考虑作病骨切除假体置换或自体大块骨移植替代;使用皮质骨植骨,很难被吸引,还可以提供永久的结构支撑。

对多发性病损,特别对儿童以保护患肢,防止畸形发生或发展发生疲劳骨折,可按骨折处理。

【并发症】

术后骨折,感染,肿瘤复发,肿瘤恶变。


——————

参考文献:

1、王正义 主编《足踝外科学》(第2版),人民卫生出版社,2014年

2、https://dict.youdao.com/search?q=%E9%AA%A8%E7%BA%A4%E7%BB%B4%E7%BB%93%E6%9E%84%E4%B8%8D%E8%89%AF&keyfrom=dict.index

3、https://en.wikipedia.org/wiki/Osteofibrous_dysplasia

4、https://emedicine.medscape.com/article/1256595-overview

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来源: <https://mp.weixin.qq.com/s?__biz=MjM5MzY4MzM4Ng==&mid=206736229&idx=1&sn=ac0e2d230e04cb9e022910c7bfcb70c8#rd>

 


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发表于:2015-04-07 22:53

患者评价
  • 默认头像
    h***f 2017-02-12 09:29:03

    本人患有骨纤维,希望病友添加微信共同讨论,或是给点意见957841978

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