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一名患有北卡罗来纳黄斑营养不良的患者出现继发性多发性一过性白点综合征。

Secondary Multiple Evanescent White Dot Syndrome in a patient with North Carolina Macular Dystrophy.

作者信息

Bommakanti Nikhil, Procopio Rebecca, Pulido Jose S

机构信息

Wills Eye Hospital, Mid Atlantic Retina, Philadelphia PA 19017.

出版信息

Retin Cases Brief Rep. 2024 Oct 7. doi: 10.1097/ICB.0000000000001667.

Abstract

PURPOSE

To describe a novel case of secondary multiple evanescent white dot syndrome in a patient with North Carolina Macular Dystrophy (NCMD).

METHODS

The patient was evaluated with ultra-widefield color and autofluorescence imaging, fluorescein angiography, and spectral-domain optical coherence tomography.

RESULTS

A 43-year-old man with longstanding blurred vision in both eyes acutely developed glare in the right eye after an upper respiratory illness. He had chorioretinal atrophy in both eyes consistent with North Carolina Macular Dystrophy, which was later confirmed by genetic testing. He alsyo had white spots in the macula of the right eye consistent with secondary MEWDS. The diagnosis of secondary MEWDS was confirmed with fundus autofluorescence and fluorescein angiography, as well as a negative infectious workup. His symptoms and the retinal lesions had resolved by 11 weeks and after a short course of oral corticosteroids.

CONCLUSION

Prior studies have suggested a link between disruption of the RPE/Bruch's membrane complex and secondary MEWDS. This report describes the first case of secondary MEWDS after NCMD and provides further support for this association.

摘要

目的

描述一例患有北卡罗来纳黄斑营养不良(NCMD)患者的继发性多发性一过性白点综合征的新病例。

方法

对该患者进行了超广角彩色和自发荧光成像、荧光素血管造影以及光谱域光学相干断层扫描评估。

结果

一名43岁男性,双眼长期视力模糊,在上呼吸道疾病后右眼急性出现眩光。他双眼存在与北卡罗来纳黄斑营养不良相符的脉络膜视网膜萎缩,后来经基因检测证实。他右眼黄斑区也有白点,符合继发性多发性一过性白点综合征(MEWDS)。通过眼底自发荧光、荧光素血管造影以及阴性的感染性检查结果确诊为继发性MEWDS。在短期口服皮质类固醇后,他的症状和视网膜病变在11周时已消退。

结论

先前的研究表明视网膜色素上皮(RPE)/布鲁赫膜复合体破坏与继发性MEWDS之间存在联系。本报告描述了NCMD后首例继发性MEWDS病例,并为这种关联提供了进一步支持。

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