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VACTERLS综合征中镫骨畸形:一项组织病理学研究及三维重建

Malformation of stapes in VACTERLS association: a histopathologic study and three-dimensional reconstruction.

作者信息

Chrobok Viktor, Jezek Bruno, Simáková Eva, Northrop Clarindy, Carranza Alfonso

机构信息

Department of Otolaryngology, General Hospital, Pardubice, Czech Republic.

出版信息

Otol Neurotol. 2004 Jan;25(1):46-9. doi: 10.1097/00129492-200401000-00009.

Abstract

OBJECTIVE

To investigate the temporal bone with a malformed stapes in VACTERLS association.

STUDY DESIGN

The study design was a case report, histopathology of the temporal bone, and three-dimensional reconstruction of the ossicles and cochlea.

SETTING

The study was carried out in the Temporal Bone Bank at Charles University, Hradec Králové, on a case from Temporal Bone Foundation, Boston.

PATIENT

A 1540-g, 41-cm female neonate with VACTERLS association, histopathology of the temporal bone, and three-dimensional reconstruction of the middle ear ossicles and cochlea.

RESULTS

The posterior part of the footplate was missing, and the posterior crura of the stapes was not connected to the footplate but found freely suspended in the sinus tympani.

CONCLUSION

Computer-assisted three-dimensional reconstruction was performed from histologic sections of the temporal bone removed from a case of VACTERLS association. The reconstruction included structures such as the bony labyrinth and the middle ear ossicles. The abnormality observed in the reconstruction included the mislocation of the stapes and a shortened footplate.

摘要

目的

研究VACTERLS综合征中镫骨畸形的颞骨情况。

研究设计

本研究设计为病例报告、颞骨组织病理学以及听小骨和耳蜗的三维重建。

研究地点

本研究在布拉格查理大学的颞骨库进行,该病例来自波士顿的颞骨基金会。

患者

一名体重1540克、身长41厘米的女性新生儿,患有VACTERLS综合征,进行了颞骨组织病理学检查以及中耳听小骨和耳蜗的三维重建。

结果

镫骨足板后部缺失,镫骨后脚未与足板相连,而是自由悬浮于鼓窦内。

结论

对一例VACTERLS综合征患者切除的颞骨组织切片进行了计算机辅助三维重建。重建包括骨迷路和中耳听小骨等结构。重建中观察到的异常包括镫骨位置异常和足板缩短。

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