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虹膜劈裂症:高频超声成像。是否存在基因缺陷的证据?

Iridoschisis: high frequency ultrasound imaging. Evidence for a genetic defect?

作者信息

Danias J, Aslanides I M, Eichenbaum J W, Silverman R H, Reinstein D Z, Coleman D J

机构信息

Mt Sinai School of Medicine, New York, USA.

出版信息

Br J Ophthalmol. 1996 Dec;80(12):1063-7. doi: 10.1136/bjo.80.12.1063.

DOI:10.1136/bjo.80.12.1063
PMID:9059271
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC505704/
Abstract

AIMS

To elucidate changes in the anatomy of the anterior chamber associated with iridoschisis, a rare form of iris atrophy, and their potential contribution to angle closure glaucoma.

METHODS

Both eyes of a 71-year-old woman with bilateral iridoschisis and fibrous dysplasia and her asymptomatic 50-year-old daughter were scanned with a very high frequency (50 MHz) ultrasound system.

RESULTS

The symptomatic patient exhibited diffuse changes in the iris stoma with an intact posterior iris pigmented layer in both eyes. These changes were clinically compatible with the lack of iris transillumination defects. Additionally, iris bowing with a resultant narrowing of the angle occurred. The asymptomatic daughter showed discrete, but less severe iris stromal changes.

CONCLUSION

This is the first detailed study of high frequency ultrasonic imaging of the iris in iridoschisis. The observed structural changes suggest angle narrowing by forward bowing of the anterior iris stroma may be a mechanism of IOP elevation in this condition. The ultrasonic detection of iris changes in the asymptomatic daughter of the symptomatic patient and the association of iridoschisis with fibrous dysplasia suggest a possible genetic component in the pathogenesis of this condition.

摘要

目的

阐明与虹膜劈裂症(一种罕见的虹膜萎缩形式)相关的前房解剖结构变化及其对闭角型青光眼的潜在影响。

方法

使用超高频率(50兆赫)超声系统对一名患有双侧虹膜劈裂症和骨纤维发育不良的71岁女性及其无症状的50岁女儿的双眼进行扫描。

结果

有症状的患者双眼虹膜小孔出现弥漫性变化,而后部虹膜色素层完整。这些变化在临床上与无虹膜透照缺损相符。此外,虹膜前凸导致房角变窄。无症状的女儿表现出离散但较轻的虹膜基质变化。

结论

这是首次对虹膜劈裂症患者的虹膜进行高频超声成像的详细研究。观察到的结构变化表明,前房虹膜基质向前凸出导致房角变窄可能是这种情况下眼压升高的一种机制。对有症状患者无症状女儿的虹膜变化进行超声检测以及虹膜劈裂症与骨纤维发育不良的关联表明,这种疾病的发病机制可能存在遗传因素。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3cdf/505704/48c140a26594/brjopthal00012-0035-c.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3cdf/505704/a9a4adb4d87d/brjopthal00012-0034-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3cdf/505704/ea7d74b98cc7/brjopthal00012-0034-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3cdf/505704/168d746608e4/brjopthal00012-0035-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3cdf/505704/72120a8c958c/brjopthal00012-0035-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3cdf/505704/48c140a26594/brjopthal00012-0035-c.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3cdf/505704/a9a4adb4d87d/brjopthal00012-0034-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3cdf/505704/ea7d74b98cc7/brjopthal00012-0034-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3cdf/505704/168d746608e4/brjopthal00012-0035-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3cdf/505704/72120a8c958c/brjopthal00012-0035-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3cdf/505704/48c140a26594/brjopthal00012-0035-c.jpg

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