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一例淀粉样变性相关性脉络膜视网膜病变的长期随访

Long-term follow-up of a case of amyloidosis-associated chorioretinopathy.

作者信息

Nagura Koichi, Inoue Tatsuya, Ching Jared, Sato Akinori, Kitahata Shohei, Maruyama-Inoue Maiko, Takeuchi Masaru, Kadonosono Kazuaki

机构信息

Department of Ophthalmology and Micro-Technology, Yokohama City University, Kanagawa, Japan.

Department of Ophthalmology, National Defense Medical College, Saitama, Japan.

出版信息

Am J Ophthalmol Case Rep. 2020 Aug 2;19:100846. doi: 10.1016/j.ajoc.2020.100846. eCollection 2020 Sep.

Abstract

PURPOSE

To describe the findings of long-term follow-up of a case of amyloidosis-associated chorioretinopathy by multimodal imagings, including optical coherence tomography (OCT).

OBSERVATIONS

A 47-year-old woman who had been diagnosed as having systemic amyloidosis was found to have a best corrected visual acuity of 20/13 in both eyes at the age of 41, which subsequently decreased to 20/100 in the left eye and 20/20 in the right eye at age 47. Visual field examination demonstrated worsening of the central scotoma during the follow-up period. Funduscopic examination revealed bilateral white deposits along the choroidal vessels, which became more pronounced over time, along with diffuse pigmentary changes. The fluorescein angiography and indocyanine green angiography findings were consistent not only with atrophic lesions, but also with amyloid deposition (i.e., staining of the vessels).At the baseline, macula OCT revealed a thick hyporeflective band at the choriocapillaris, however, at the last follow-up, it revealed an absent ellipsoid zone, and bilateral progressive retinal pigment epithelium irregularities in both eyes.

CONCLUSIONS

Patients diagnosed as having amyloidosis-related chorioretinopathy may have maintained visual function at the first detection of retinal amyloid deposition, and a number of years may elapse before the patient manifests visual deterioration.

摘要

目的

通过多模态成像,包括光学相干断层扫描(OCT),描述一例淀粉样变性相关性脉络膜视网膜病变的长期随访结果。

观察结果

一名47岁女性被诊断为患有系统性淀粉样变性,在41岁时双眼最佳矫正视力为20/13,随后在47岁时左眼降至20/100,右眼降至20/20。视野检查显示随访期间中心暗点恶化。眼底检查发现沿脉络膜血管有双侧白色沉积物,随时间推移变得更加明显,同时伴有弥漫性色素改变。荧光素血管造影和吲哚菁绿血管造影结果不仅与萎缩性病变一致,也与淀粉样沉积一致(即血管染色)。基线时,黄斑OCT显示脉络膜毛细血管层有一条厚的低反射带,然而,在最后一次随访时,显示椭圆体带缺失,且双眼均有双侧进行性视网膜色素上皮不规则改变。

结论

被诊断为淀粉样变性相关性脉络膜视网膜病变的患者在首次发现视网膜淀粉样沉积时可能保持了视觉功能,且在患者出现视力恶化之前可能会经过数年时间。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a34c/7453112/ada1f4aaa1d7/gr1.jpg

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