Pichi Francesco, Srvivastava Sunil K, Chexal Saradha, Lembo Andrea, Lima Luiz H, Neri Piergiorgio, Saitta Andrea, Chhablani Jay, Albini Thomas A, Nucci Paolo, Freund K Bailey, Chung Hyewon, Lowder Careen Y, Sarraf David
*San Giuseppe Hospital, University Eye Clinic, Milan, Italy; †Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, Ohio; ‡Retina Consultants of Austin, Texas; §Federal University of Sao Paulo (UNIFESP), Sao Paulo, Brazil; ¶The Ocular Immunology Service, The Eye Clinic, Polytechnic University of Marche, Ancona, Italy; **Smt. Kanuri Santhamma Retina Vitreous Centre, L. V. Prasad Eye Institute, Hyderabad, India; ††Bascom Palmer Eye Institute, Miller School of Medicine, University of Miami, Miami, Florida; ‡‡Vitreous Retina Macula Consultants of New York, New York, New York; §§Department of Ophthalmology, Konkuk University School of Medicine, Seoul, Republic of Korea; ¶¶Stein Eye Institute, University of California, Los Angeles, Los Angeles, California; and ***Greater Los Angeles VA Healthcare Center, Los Angeles, California.
Retina. 2016 Dec;36 Suppl 1:S178-S188. doi: 10.1097/IAE.0000000000001255.
To localize the various levels of abnormalities in multiple evanescent white dot syndrome by comparing "en face" optical coherence tomography (OCT) and OCT angiography with various conventional imaging modalities.
In this retrospective case series, multimodal imaging was performed in 9 retinal centers on 36 patients with multiple evanescent white dot syndrome and included widefield fundus autofluorescence (FAF), fluorescein angiography (FA), and indocyanine green angiography, and B-scan and "en face" C-scan enhanced depth imaging and spectral domain OCT. Optical coherence tomography angiography was also performed at the level of the superficial and deep retinal capillary plexus and choroid.
Multiple evanescent white dot syndrome lesions were more numerous and more easily detectable with FA and FAF. Two types of lesions were identified with FAF, FA, and indocyanine green angiography: larger widely scattered "spots" (approximately 200 μ in diameter) that were hyperfluorescent with FA, hyperautofluorescent with FAF, and hyporeflective in indocyanine green angiography, representing abnormalities primarily at the retinal pigment epithelium/photoreceptor junction; and punctate "dots" (less than 100 μ in diameter) that were hyperfluorescent with FA, hyperautofluorescent, or isoautofluorescent with FAF, and hypofluorescent with indocyanine green angiography and that localized to the outer nuclear layer. These lesions colocalized with "en face" OCT. The larger confluent "spots" were hyporeflective and colocalized to the level of the ellipsoid zone, whereas smaller hyperreflective "dots" colocalized to the outer nuclear layer. The location of the "dots" in the outer nuclear layer was further confirmed by structural spectral domain optical coherence tomography which showed coalescence of the dots into hyperreflective lines extending from the external limiting membrane to the outer plexiform layer in certain cases. Optical coherence tomography angiography analysis of the retinal microvasculature and choriocapillaris and choroid were entirely unremarkable in 100% of our patients.
By combining multimodal imaging, the authors propose that multiple evanescent white dot syndrome is primarily the result of inflammation at the outer photoreceptor level leading to a "photoreceptoritis" and causing loss of the inner and outer segments. Its evanescent nature suggests that the photoreceptor cell bodies remain intact ensuring complete recovery of the photoreceptor inner and outer segments in most cases, compatible with the clinical course of spontaneous resolution of white spots and dots.
通过将“正面”光学相干断层扫描（OCT）和OCT血管造影与各种传统成像方式进行比较，定位多发性一过性白点综合征中不同层次的异常。
在这个回顾性病例系列中，9个视网膜中心对36例多发性一过性白点综合征患者进行了多模态成像，包括广角眼底自发荧光（FAF）、荧光素血管造影（FA）、吲哚菁绿血管造影、B超以及“正面”C扫描增强深度成像和光谱域OCT。还在视网膜浅、深层毛细血管丛和脉络膜水平进行了OCT血管造影。
FA和FAF能发现更多且更容易检测到的多发性一过性白点综合征病变。通过FAF、FA和吲哚菁绿血管造影可识别出两种类型的病变：较大的广泛散在的“斑点”（直径约200μm），FA表现为高荧光，FAF表现为高自发荧光，吲哚菁绿血管造影表现为低反射，主要代表视网膜色素上皮/光感受器交界处的异常；点状“小点”（直径小于100μm），FA表现为高荧光，FAF表现为高自发荧光或等自发荧光，吲哚菁绿血管造影表现为低荧光，位于外核层。这些病变与“正面”OCT定位一致。较大的融合性“斑点”表现为低反射，与椭圆体带水平定位一致，而较小的高反射“小点”与外核层定位一致。结构光谱域光学相干断层扫描进一步证实了“小点”在外核层的位置，在某些情况下显示小点融合成从外界膜延伸至外丛状层的高反射线。100%的患者视网膜微血管、脉络膜毛细血管和脉络膜的OCT血管造影分析均无明显异常。
通过结合多模态成像，作者提出多发性一过性白点综合征主要是外光感受器水平炎症导致“光感受器炎”并引起内、外节段丢失的结果。其一过性性质表明光感受器细胞体保持完整，确保在大多数情况下光感受器内、外节段完全恢复，这与白点和小点自发消退的临床过程相符。
