Yoshida Arisa, Shibuya Masayuki, Shimada Yoshiaki, Igawa Yuro, Tachibana Midori, Shinoda Kei
Department of Ophthalmology, Saitama Medical University Faculty of Medicine, 38 Moro-Hongo Moroyama-machi, Iruma-gun, Saitama, 350-0495, Japan.
Department of Ophthalmology, Daiyukai Dai-ichi Hospital, Hagoromo1-6-12, Ichinomiya, Aichi, 491-8551, Japan.
Doc Ophthalmol. 2025 Sep 5. doi: 10.1007/s10633-025-10046-x.
To report a rare case of bilateral idiopathic multifocal retinal pigment epithelial detachments (imfPEDs) and to describe the long-term morphological and functional changes observed over a 16-year follow-up period.
A 49-year-old woman was diagnosed with imfPEDs based on multimodal imaging, including optical coherence tomography (OCT), fluorescein angiography (FA), and fundus photography. Full-field electroretinograms (ffERGs) and multifocal ERGs (mfERGs) were recorded to assess retinal function. The patient voluntarily discontinued follow-up but returned 16 years later due to cataract progression. Retinal morphology and function were re-evaluated using comparable multimodal imaging and electrophysiological methods.
At the initial visit, multiple bilateral pigment epithelial detachments (PEDs) were identified. OCT showed hyporeflective, dome-shaped PEDs with smooth borders, and ERG responses were within normal limits. Sixteen years later, some PEDs had resolved, others had newly developed or fused, and geographic atrophy was observed, particularly in the peripheral retina. Fundus autofluorescence (FAF), performed in place of FA, revealed hyperautofluorescent PEDs and numerous peripheral hypofluorescent spots. ffERGs remained normal, while mfERGs showed localized attenuation with relatively preserved macular function. These findings were consistent with large colloid drusen and cuticular drusen.
This case demonstrates the slow morphological progression and relative functional preservation in bilateral imfPEDs over 16 years. Comparable multimodal imaging and electrophysiological testing were valuable in monitoring the long-term clinical course and support the classification of this phenotype as a variant of large colloid or cuticular drusen.
报告一例罕见的双侧特发性多灶性视网膜色素上皮脱离(imfPEDs)病例,并描述在16年随访期内观察到的长期形态学和功能变化。
一名49岁女性基于多模态成像被诊断为imfPEDs,多模态成像包括光学相干断层扫描(OCT)、荧光素血管造影(FA)和眼底摄影。记录全视野视网膜电图(ffERGs)和多焦视网膜电图(mfERGs)以评估视网膜功能。患者自愿中断随访,但16年后因白内障进展而复诊。使用类似的多模态成像和电生理方法重新评估视网膜形态和功能。
初诊时,发现双侧多处色素上皮脱离(PEDs)。OCT显示边界光滑的低反射、圆顶形PEDs,ERG反应在正常范围内。16年后,一些PEDs已消退,另一些则新出现或融合,且观察到地图样萎缩,尤其是在外周视网膜。代替FA进行的眼底自发荧光(FAF)显示高自发荧光的PEDs和众多外周低荧光斑点。ffERGs仍正常,而mfERGs显示局部衰减,黄斑功能相对保留。这些发现与大的玻璃膜疣和表皮玻璃膜疣一致。
该病例显示了双侧imfPEDs在16年内缓慢的形态学进展和相对的功能保留。类似的多模态成像和电生理检测对于监测长期临床病程很有价值,并支持将该表型分类为大的玻璃膜疣或表皮玻璃膜疣的一种变体。