颅面部骨纤维异常增殖症的囊性退变
Cystic Degeneration of Craniofacial Fibrous Dysplasia.
作者信息
Holl Dana C, Hardillo Jose A U, Dammers Ruben, van der Schroeff Marc P, van der Lugt Aad
机构信息
Department of Neurosurgery, Erasmus Medical Center and Sophia Children's Hospital, Rotterdam, The Netherlands.
Department of Otolaryngology-Head and Neck Surgery, Erasmus Medical Center and Sophia Children's Hospital, Rotterdam, The Netherlands.
出版信息
World Neurosurg. 2018 Dec;120:159-162. doi: 10.1016/j.wneu.2018.08.175. Epub 2018 Aug 31.
BACKGROUND
Fibrous dysplasia (FD) is most often a slowly progressive benign disease in which the normal bone structure is replaced by fibrous and osteoid tissue.
CASE DESCRIPTION
A 16-year-old adolescent, known with FD in the sphenoid bone, suffered an acute decreased visual acuity with papilledema on the left eye. The radiologic images were best compatible with cystic degeneration of the known FD with optic nerve compression in the optic canal. Decompression of the optic nerve was performed through an endoscopic exploration of the left sphenoid sinus. The visual acuity recovered completely.
CONCLUSIONS
In FD with cystic changes, leading to acute signs of optic nerve compression, early aggressive surgical decompression is strongly recommended. Cystic degeneration of the FD, although rare, should be considered.
背景
骨纤维异常增殖症（FD）通常是一种缓慢进展的良性疾病，其中正常骨结构被纤维组织和类骨质组织所取代。
病例描述
一名16岁青少年，已知患有蝶骨骨纤维异常增殖症，左眼出现急性视力下降并伴有视乳头水肿。放射影像学图像最符合已知骨纤维异常增殖症的囊性退变并伴有视神经管内视神经受压。通过左侧蝶窦的内镜探查对视神经进行减压。视力完全恢复。
结论
对于伴有囊性改变并导致视神经受压急性体征的骨纤维异常增殖症，强烈建议早期积极进行手术减压。骨纤维异常增殖症的囊性退变虽然罕见，但应予以考虑。
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