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一例记录多区域外层视网膜病变和视网膜色素上皮病变(MORR)独特自然病程并对其进展进行纵向多模式记录的病例。

A case documenting distinct natural history of multizonal outer retinopathy and retinal pigment epitheliopathy (MORR) with longitudinal multi-modal documentation of progression.

作者信息

Klejn Alexandra, Sheard Richard, Grigg John, McCluskey Peter J

机构信息

Royal Hobart Hospital, Uveitis Clinic, Hobart, Tasmania, Australia.

Save Sight Institute, University of Sydney, Specialty of Ophthalmology, Faculty of Medicine and Health, Sydney, Australia.

出版信息

Am J Ophthalmol Case Rep. 2024 Nov 29;36:102222. doi: 10.1016/j.ajoc.2024.102222. eCollection 2024 Dec.

Abstract

PURPOSE

To describe the clinical and imaging characteristics of the acute progressive phase of a recently proposed clinical entity, Multizonal Outer Retinopathy and Retinal Pigment Epitheliopathy (MORR), a variant of Acute Zonal Occult Outer Retinopathy (AZOOR).

METHODS

Single observational case report.

RESULTS

We present the case of a 49-year-old myopic female with progressive outer retinopathy most consistent with a diagnosis of MORR. Through multimodal imaging and longitudinal follow-up, we delineate the clinical course and imaging findings of asymmetrical episodic progressive centrifugal extension of retinal pigment epithelial disturbance in both eyes, highlighting the features of an acute progressive episode not previously described.

CONCLUSIONS

Clinicians should be aware of the active clinical and multimodal imaging features of MORR and its distinction from other outer retinopathies due to its sight-threatening distinct clinical course, bilateral involvement with peripapillary lesions, and episodic progression into the macula. Additionally, we describe a "grass-fire" and "spot-fire" progression pattern during acute exacerbation, highlighting the need for vigilant monitoring and early intervention in MORR.

摘要

目的

描述一种最近提出的临床实体——多区域外层视网膜病变和视网膜色素上皮病变(MORR)的急性进展期的临床和影像学特征,MORR是急性区域性隐匿性外层视网膜病变(AZOOR)的一种变体。

方法

单例观察性病例报告。

结果

我们报告了一例49岁近视女性患者,其外层视网膜病变呈进行性,最符合MORR的诊断。通过多模态成像和纵向随访,我们描绘了双眼视网膜色素上皮紊乱不对称发作性进行性离心性扩展的临床过程和影像学表现,突出了此前未描述的急性进展期特征。

结论

临床医生应了解MORR的活跃临床和多模态成像特征,以及它与其他外层视网膜病变的区别,因为它具有威胁视力的独特临床过程、累及双眼伴视乳头周围病变,以及发作性进展至黄斑区。此外,我们描述了急性加重期的“野火”和“点火”进展模式,强调了对MORR进行密切监测和早期干预的必要性。

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