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一名患有蛋白C缺乏症的年轻患者出现的中心旁急性黄斑病变与非缺血性视网膜中央静脉阻塞

Paracentral Acute Middle Maculopathy and Nonischemic Central Retinal Vein Obstruction in a Young Patient with Protein C Deficiency.

作者信息

Abrishami Mojtaba, Hosseini Seyedeh Maryam, Shoeibi Nasser, Heidarzadeh Hamid Reza

机构信息

Eye Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.

出版信息

Case Rep Ophthalmol Med. 2022 Jun 6;2022:1237148. doi: 10.1155/2022/1237148. eCollection 2022.

Abstract

PURPOSE

To report a case of protein C deficiency with paracentral acute middle maculopathy (PAMM) and nonischemic central retinal vein obstruction (NI-CRVO). . A previously healthy twenty-one-year-old male was referred with sudden-onset, painless, unilateral paracentral scotomata in the right eye for a week. His uncorrected visual acuity was 20/20 in both eyes. In fundus examination of the right eye, mild venous tortuosity, retinal hemorrhages, and a gray-white opacity in the papillomacular bundle were observed. In the macular spectral-domain optical coherence tomography images, a hyperreflective band was noticed at the level of the inner nuclear layer, indicating PAMM. Fundus appearance and fluorescein angiography findings indicated NI-CRVO diagnosis made. The systemic and laboratory evaluations disclosed a protein C deficiency.

CONCLUSION

In this report, in a healthy young male, combined PAMM and NI-CRVO showed to be the cause of blurred vision in the setting of protein C deficiency.

摘要

目的

报告一例伴有黄斑中心凹旁急性黄斑病变(PAMM)和非缺血性视网膜中央静脉阻塞(NI-CRVO)的蛋白C缺乏症病例。一名此前健康的21岁男性因右眼突发、无痛性、单侧黄斑中心凹旁暗点一周前来就诊。他双眼的裸眼视力均为20/20。在右眼眼底检查中,观察到轻度静脉迂曲、视网膜出血以及乳头黄斑束处的灰白色混浊。在黄斑区光谱域光学相干断层扫描图像中,在内核层水平发现一条高反射带,提示为PAMM。眼底外观和荧光素血管造影结果表明诊断为NI-CRVO。全身和实验室评估发现存在蛋白C缺乏症。

结论

在本报告中,一名健康年轻男性中,PAMM和NI-CRVO合并出现是蛋白C缺乏情况下视力模糊的原因。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d598/9192315/2dc3d06ce0a0/CRIOPM2022-1237148.001.jpg

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