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点状骨骺发育异常近端股骨发育不全中椎体冠状裂的研究

Studies of vertebral coronal cleft in rhizomelic chondrodysplasia punctata.

作者信息

Wells T R, Landing B H, Bostwick F H

机构信息

Department of Pathology, Childrens Hospital, Los Angeles, California 90054-0700.

出版信息

Pediatr Pathol. 1992 Jul-Aug;12(4):593-600. doi: 10.3109/15513819209024210.

Abstract

Dissection and serial section-reconstruction preparations from vertebrae with coronal cleft of a 2 5/12-year-old girl with rhizomelic chondrodysplasia punctata are described. The cartilage plate between the dorsal and ventral vertebral ossification centers, abnormal thickness of which is the explanation of the radiologic coronal cleft, shows differences in thickness, with dorsal and ventral midline extensions and with small foci of communication of the cancellous bone of the dorsal and ventral ossification centers of the vertebral body bilaterally. Although the process in coronal cleft is in some ways similar to that of radiologic "retarded epiphyseal closure," the explanation of the coronal clefts in rhizomelic chondrodysplasia punctata appears to be overproduction of cartilage in the zone between the dorsal and ventral vertebral body ossification centers, plus reduced removal of cartilage cells from this zone. No evidences of persistence of notochord cells were seen microscopically in sections of the coronal clefts.

摘要

本文描述了一名患有肢根型点状软骨发育不良的2又5/12岁女孩的椎骨解剖及连续切片重建标本,其椎骨存在冠状裂。背侧和腹侧椎体骨化中心之间的软骨板厚度异常,这就是影像学冠状裂的原因,该软骨板厚度存在差异,在背侧和腹侧中线处有延伸,且椎体背侧和腹侧骨化中心的松质骨在双侧有小的连通灶。尽管冠状裂的过程在某些方面类似于影像学上的“骨骺闭合延迟”,但肢根型点状软骨发育不良中冠状裂的原因似乎是背侧和腹侧椎体骨化中心之间区域的软骨过度生成,以及该区域软骨细胞清除减少。在冠状裂切片的显微镜检查中未发现脊索细胞残留的证据。

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