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眼眶颅面骨纤维发育不良

Craniofacial fibrous dysplasia of orbit.

作者信息

Tabrizi Reza, Ozkan Birkan Taha

机构信息

Craniomaxillofacial Surgery Research Center, University of Shiraz, Medical Science, Shiraz, Iran.

出版信息

J Craniofac Surg. 2008 Nov;19(6):1532-7. doi: 10.1097/SCS.0b013e31818ac270.

Abstract

Fibrous dysplasia is a benign, rare, and idiopathic skeletal disorder characterized by replacement and expansion of medullary bone by disorganized fibro-osseous tissue. Treatment for these lesions is usually sought for cosmetic or functional reasons. The surgical approaches vary from watchful expectancy for the lesion to stop growing after puberty to conservative resection to radical resection. In this case reports, 3 patients with craniofacial fibrous dysplasia involving orbital region underwent surgery. In these primary cases, resection and immediate reconstruction were performed to regain facial contour, dental occlusion, and masticatory function. Significant improvement was seen in facial appearance, and all patients also felt that there had been improvement aesthetically. Therefore, conservative treatment for fibrous dysplasia, unless there is involvement of vital structure as orbital region, can be suggested. Early surgery to address progressive sensory disturbance is recommended so as to avoid the hazards of late-stage decompression.

摘要

骨纤维异常增殖症是一种良性、罕见的特发性骨骼疾病,其特征是骨髓骨被杂乱的纤维骨组织替代和扩张。通常出于美容或功能原因对这些病变进行治疗。手术方法从对青春期后病变停止生长的观察期待,到保守切除再到根治性切除不等。在本病例报告中,3例累及眼眶区域的颅面骨纤维异常增殖症患者接受了手术。在这些原发性病例中,进行了切除并立即重建,以恢复面部轮廓、牙列咬合和咀嚼功能。面部外观有显著改善,所有患者也感觉在美学上有所改善。因此,除非累及眼眶区域等重要结构,可建议对骨纤维异常增殖症进行保守治疗。建议早期手术以解决进行性感觉障碍,从而避免晚期减压的风险。

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