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胫腓骨骨纤维发育异常

Osteofibrous dysplasia of the tibia and fibula.

作者信息

Blackwell J B, McCarthy S W, Xipell J M, Vernon-Roberts B, Duhig R E

机构信息

Bone Tumour Registry of Western Australia.

出版信息

Pathology. 1988 Jul;20(3):227-33. doi: 10.3109/00313028809059497.

Abstract

Osteofibrous dysplasia (ossifying fibroma of long bone) is an uncommon disorder of bone affecting the tibia and, rarely, the fibula of children and adolescents. It has been delineated from monostotic fibrous dysplasia, from which it can be distinguished by its radiological and histological characteristics and by its clinical course. We report 11 patients with osteofibrous dysplasia whose ages ranged from 10 months to 22 years. Four presented with a pathological fracture and in 2 a pseudarthrosis developed. A circumscribed area of intracortical lucency and expansion in the diaphysis is the characteristic radiological finding. Histologically there is a similarity to fibrous dysplasia but the trabeculae are lamellar or have a lamellar surface and most show osteoblastic rimming. Treatment may be difficult but more recent experience strongly favours a conservative approach wherever possible.

摘要

骨纤维发育异常(长骨骨化性纤维瘤)是一种罕见的骨疾病,影响儿童和青少年的胫骨,很少累及腓骨。它已从单骨型纤维发育异常中区分出来,可通过其放射学和组织学特征以及临床病程加以鉴别。我们报告了11例骨纤维发育异常患者,年龄从10个月至22岁不等。4例出现病理性骨折,2例发生假关节。骨干皮质内局限性透亮区和膨大是其特征性放射学表现。组织学上它与纤维发育异常相似,但小梁呈板层状或具有板层表面,且大多数有骨母细胞包绕。治疗可能困难,但近期经验强烈支持尽可能采取保守治疗方法。

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