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[颅骨纤维异常增殖症所致视觉障碍的理论解释:筛板压力失衡]

[Theoretic explanation about visual disorder induced by fibrous dysplasia of skull: pressure disequilibrium between lamina cribrosa].

作者信息

Zhao Jing-wu, Wang Dong-sheng, Chang Qing-lin, Dong Hao, Shi Jing, Bai Feng-ge, Zhang Tian-ming

机构信息

Department of Neurosurgery, Beijing Tongren Hospital Affiliated to Capital Medical University, Beijing 100730, China.

出版信息

Zhonghua Yi Xue Za Zhi. 2010 May 18;90(19):1317-21.

Abstract

OBJECTIVE

To investigate the causes for changes in optic nerve head and visual impairment caused by fibrous dysplasia (FD) of optic canal stenosis.

METHODS

A total of 12 FD patients, diagnosed by CT, received the fundus and optical coherence tomography (OCT). Those with FD involving optic canal underwent decompression. The examination of OCT showed that lamina cribrosa located at the top was the exposure factor for retinal pigment epithelium (RPE). There were decreased vision or edematous optic nerve and atrophic lesion. Odds ratio was calculated by Fisher's exact test.

RESULTS

The examination of CT showed the results of optic canal stenosis caused by lesions involving 18 sides: 8-sides with normal vision. Among which, 6 showing that lamina cribrosa was located below RPE and a lamina cribrosa plate near RPE at follow-up, 1 side OCT showing lamina cribrosa above RPE with normal optic nerve; a lateral lamina cribrosa was located above RPE with edematous optic nerve. Visual impairment at 10 sides, normal papillary 2 sides, atrophic papillary 8 sides, OCT showed that lamina cribrosa was located above RPE, postoperatively OCT showed that lamina cribrosa below with 7 sides having improved visual acuity. Fisher's exact test was performed (P = 0.000, odds ratio = infinity).

CONCLUSION

The optic canal stenosis causes a rising pressure of lamina cribrosa zone to shift above RPE. The channel becomes distorted so as to squeeze and cut the ganglion cell axons of optic nerve, block the axoplasmic transport and result in blood circulation disorder. The above factors are the etiologies of visual impairments. Postoperatively lamina cribrosa zone pressure drops so that depressed lamina cribrosa and channel deformation recover and visual acuity improves.

摘要

目的

探讨视神经管狭窄性骨纤维发育不良(FD)导致视神经乳头改变及视力损害的原因。

方法

对12例经CT诊断的FD患者进行眼底及光学相干断层扫描(OCT)检查。对视神经管受累的FD患者行减压术。OCT检查显示位于顶部的筛板是视网膜色素上皮(RPE)的暴露因素。存在视力下降或视神经水肿及萎缩性病变。采用Fisher精确检验计算比值比。

结果

CT检查显示18侧病变导致视神经管狭窄的结果:8侧视力正常。其中,6侧随访时显示筛板位于RPE下方且RPE附近有筛板层,1侧OCT显示筛板位于RPE上方且视神经正常;1侧筛板位于RPE上方且视神经水肿。10侧视力损害,2侧视乳头正常,8侧视乳头萎缩,OCT显示筛板位于RPE上方,术后OCT显示筛板位于下方,7侧视力提高。进行Fisher精确检验(P = 0.000,比值比 = ∞)。

结论

视神经管狭窄导致筛板区压力升高,使筛板移位至RPE上方。管腔变形,从而挤压和切断视神经的神经节细胞轴突,阻断轴浆运输并导致血液循环障碍。上述因素是视力损害的病因。术后筛板区压力下降,使凹陷的筛板和管腔变形恢复,视力提高。

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