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MULTIPLE EVANESCENT WHITE DOT SYNDROME WITH CENTRAL VISUAL LOSS

Barile, Gaetano R., MD*,†; Harmon, Sarah A., CRA

Retinal Cases and Brief Reports: December 2017 - Volume 11 - Issue - p S219–S225
doi: 10.1097/ICB.0000000000000467
Case Report
Open

Purpose: To report the clinical case of a 64-year-old man who developed several features of multiple evanescent white dot syndrome (MEWDS) but with central visual loss that persisted because of significant structural macular disease.

Methods: A case report was generated by a review of the clinical course and imaging investigations of one patient, along with review of the literature.

Results: A 64-year-old man noted decreased vision in the right eye for 2 weeks, associated with a central scotoma with shimmering, gauze effect of the vision. He had a good vision with normal ophthalmic examinations in the past. Ophthalmic examination revealed acuity of 20/400 with central scotoma and trace pupillary defect in the right eye, normal anterior segment, no evidence of intraocular inflammation, and fundus findings of unilateral MEWDS associated with a central zone of macular pigmentary atrophy. Autofluorescence imaging revealed reflectance changes of MEWDS as well as prominent central hypofluorescence and a zone of hyperfluorescence in inferior macula. Spectral-domain optical coherence tomography identified mild disruptions of outer retina in the regions of the MEWDS lesions and disorganization of the outer retina in the macular region overlying a shallow irregular retinal pigment epithelial detachment. The choroid demonstrated increased thickness compared with the fellow eye with suggestions of dilated outer vessels/pachyvessels. Fluorescein angiography and indocyanine green angiography revealed window defects and staining and hypocyanescence, respectively, of the central macular lesion. The MEWDS lesions resolved without evolution to chorioretinal scars, and the central vision and lesions did not benefit from a systemic steroid course or intravitreal anti–vascular endothelial growth factor therapy.

Conclusion: This case illustrates an unusual presentation of MEWDS characterized by unilateral retinal disease presenting with symptoms and signs of profound central macular dysfunction. The clinical course did not evolve into other inflammatory retinal phenotypes, such as multifocal choroiditis or AZOOR (acute zonal occult outer retinopathy) that can sometimes develop MEWDS-like features or central disease. The central structural disease resembles some features of the pachychoroid clinical spectrum, which may have represented a superimposed diagnosis unrelated to the inflammatory phenotype.

An acute, unilateral, inflammatory maculopathy with features of multiple evanescent white dot syndrome presented with symptoms and signs of profound central macular structural disease. The clinical course did not evolve into other inflammatory retinal phenotypes, such as multifocal choroiditis that can sometimes cause central atrophy, and the central disease may have represented superimposed pachychoroid clinical spectrum disease.

*Hofstra Northwell School of Medicine, Hempstead, New York; and

Department of Ophthalmology, Manhattan Eye, Ear, and Throat Hospital, New York, New York.

Reprint requests: Gaetano R. Barile, MD, Department of Ophthalmology, Manhattan Eye, Ear, and Throat Hospital, 210 East 64 Street, New York, NY 10065; e-mail: gaetanobarilemd@gmail.com

Presented at the Atlantic Coast Conference, New York, NY, January 8, 2016.

None of the authors has any financial/conflicting interests to disclose.

This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

Multiple evanescent white dot syndrome (MEWDS) is an inflammatory disorder of the retina condition that typically affects young, healthy, myopic women.1 Patients present with symptoms of visual loss in the setting of photopsia, dyschromatopsia, and scotomas. A characteristic feature of MEWDS is the presence of multiple discrete white spots at the level of the deep retina, and the fovea often has a granular appearance that may persist. The structural features of these white spots have been confirmed to be at the level of photoreceptors, with autofluorescence imaging revealing hyperautofluorescent lesions in MEWDS, probably related to photoreceptor loss unmasking normal underlying retinal pigment epithelial fluorescence.2,3 The lesions of MEWDS usually resolve with visual recovery, although patients with MEWDS may develop other inflammatory diseases including AZOOR (acute zonal occult outer retinopathy) and multifocal choroiditis (MFC).4

We present a case of an acute inflammatory retinopathy that included white spots typically seen in MEWDS that occurred in a middle-aged man. The eye also had a profound central region of zonal outer retinal loss associated with a shallow, irregular retinal pigment epithelial detachment, leading to persistent visual loss. The clinical course did not exhibit features typical of other inflammatory maculopathies and the central disease may have been due to superimposed pachychoroid clinical disease.5

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Case Report

A 64-year-old man complained of a 2-week history of central visual loss with intermittent photopsia in his right eye. The patient reported symptoms of a scotoma, as well as a shimmering, gauze-like effect to the vision. His medical history was unremarkable (no medication usage), and he had a normal eye examination and vision within the previous year according to his referring ophthalmologist. He did not report a distinct viral prodrome but vaguely indicated that he had not felt well recently. Upon examination, the best-corrected visual acuity was 20/400 in the right eye and 20/20–1 in the left eye with mild astigmatism in the left eye (+0.50 −1.00 × 75°). An absolute scotoma with adjacent relative scotomas was present in the right eye on Amsler grid testing. A trace afferent pupillary defect in the right eye was present. The anterior segment was normal with age appropriate nuclear sclerosis and without evidence of intraocular inflammation in the anterior chamber or vitreous cavity.

The fundus examination revealed a confluent region of central hypopigmentation of the macula contiguous with the optic nerve in the right eye (Figure 1A). Very subtle white spot lesions were seen along the arcades, best seen on red-free images (Figure 2A). The left eye fundus revealed a slight hypopigmentary disturbance nasal to the fovea but was otherwise normal (Figure 1B). Autofluorescence (AF), infrared, and high-definition spectral-domain optical coherence tomography (SD-OCT) were performed using confocal scanning laser ophthalmoscopy imaging (Heidelberg Spectralis HRA+OCT version 1.9.1.0; Heidelberg Engineering, Heidelberg, Germany). The AF imaging revealed multifocal hyperautofluorescent spots in the right eye corresponding to white dots seen clinically, as well as a prominent region of hypoautofluorescence corresponding to the region of hypopigmentation seen clinically in the right eye and a zone of hyperautofluorescence inferior to the central macula in the right eye (Figure 3A). Infrared imaging showed subtle changes corresponding to the clinical and AF findings in the right eye (Figure 4A). The AF and infrared imaging was unremarkable in the left eye (Figures 3B and 4B). The SD-OCT showed mild disruptions of inner segment/outer segment junctions in the regions of the white dots and SD-OCT of the central macular lesion in the right eye revealed a shallow, irregular elevation of the retinal pigment epithelium, subretinal hyperreflective material, and disorganization and loss of the outer retina (Figure 5). The SD-OCT in the left eye was unremarkable. Enhanced depth imaging OCT estimated a choroidal thickness of 375 μm in the right eye and 239 μm in the left eye. Dilation of the outer choroidal vessels was seen in several scans in the right eye, particularly when compared with that in the left eye. Fluorescein angiography revealed a peripapillary and central macula window defect in the regions of the central macula lesion and zone of hyperautofluorescence, with gradual staining of the region with progression of the study (Figure 6). Indocyanine green angiography revealed a central region of hypocyanescence without other findings (Figure 7). Fluorescein angiography and indocyanine green angiography studies of the left eye were unremarkable (Figures 6 and 7).

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At the time of presentation, the right eye was felt to have an inflammatory maculopathy with features of MEWDS and a central placoid epitheliopathy. The patient was referred for testing for exposure to tuberculosis, syphilis, lyme, and Bartonella, all of which were negative. Five days after presentation, the patient reported persistent photopsia and visual loss. The findings were unchanged, and a trial of prednisone 60 mg per day was administered. Three weeks after presentation, the patient reported resolved photopsia. The acuity had improved to 20/150 in the right eye, and the white dots best seen on AF imaging were evanescent (Figure 8). Prednisone was tapered, and the acuity declined to 20/250 in the right eye, without a significant change in the clinical and imaging features. Three months after presentation, the SD-OCT demonstrated tubulation and possible cysts within the region of the central outer retinal disorganization in the right eye. An intravitreal injection of aflibercept (2 mg) was administered, and there was no evidence of anatomical or visual benefit on follow-up. The retina has remained unchanged during further follow-up, specifically without evolution to further retinal lesions over 2 years. The right eye subsequently experienced a vitreous separation without sequelae. The acuity has fluctuated between 20/250 and 20/400 during this time.

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Discussion

This case exhibits features of an acute inflammatory retinopathy with some features of MEWDS also associated with significant structural macular disease. The patient presented with photopsia and visual loss that persisted because of the profound disturbance of the central macula. The white dots were evanescent and followed a course typical of MEWDS on autofluorescence imaging coincident with resolution of photopsia. However, the fluorescein and indocyanine green imaging studies did not demonstrate the hyperfluorescent wreath-like changes or hypocyanescent spots, respectively, that are usually present in MEWDS. The central macular findings demonstrated pronounced outer retinal loss and disorganization sometimes seen in cases of MFC.6 However, the white dots seen clinically and on autofluorescent imaging did not evolve into chorioretinal scars typically seen with MFC lesions. Instead, the macula exhibited a shallow, irregular retinal pigment epithelial detachment that often exhibits Type 1 neovascular disease with OCT angiography in the pachychoroid spectrum of macular disorders.7 Unfortunately, OCT angiography was not available at the time of diagnosis, and there were no definitive fluorescein or indocyanine green angiographic features of neovascular disease. However, the hyperfluorescent changes seen in the inferior macula on autofluorescence imaging are suggestive of previous central serous chorioretinopathy, and the choroid was increased in thickness with distended outer choroidal vessels, particularly compared with the fellow eye, consistent with a central serous chorioretinopathy pachychoroid variant.

Inflammatory maculopathies can sometimes overlap in the same eye, as seen in some cases of MEWDS and MFC presenting with concurrent lesions in the same eye or in cases of MEWDS occurring after prior episodes of MFC.8,9 The controversy regarding the susceptibility of these inflammatory phenotypes and whether they have an overlapping pathogenesis persists in the era of multimodal imaging. Our case differs from those of MFC cases with outer retinal atrophy in that no new chorioretinal scars typical of MFC developed during a 2-year follow-up period, and the retinal pigment epithelium appeared to remain intact unlike cases of MFC. In discussion of this case with other colleagues at Macula 2016, the possibility that another maculopathy was present before the development of MEWDS was suggested. The history of acute visual loss and previous normal eye examinations suggest concurrent development of central macular dysfunction with the onset of MEWDS-like features, but the structural and autofluorescent findings are consistent with more longstanding disease of the pachychoroid variant that may have gone undetected by the patient given the unilateral symptoms.

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References

1. Jampol LM, Sieving PA, Pugh D, et al.. Multiple evanescent white dot syndrome. I. Clinical findings. Arch Ophthalmol 1984;102:671–674.
2. Li D, Kishi S. Restored photoreceptor outer segment damage in multiple evanescent white dot syndrome. Ophthalmology 2009;116:762–770.
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4. Fine HF, Spaide RF, Ryan EH Jr, et al.. Acute zonal occult outer retinopathy in patients with multiple evanescent white dot syndrome. Arch Ophthalmol 2009;127:66–70.
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6. Jung JJ, Khan S, Mrejen S, et al.. Idiopathic multifocal choroiditis with outer retinal or chorioretinal atrophy. Retina 2014;34:1439–1450.
7. Dansingani KK, Balaratnasingam C, Klufas MA, et al.. Optical coherence tomography angiography of shallow irregular pigment epithelial detachments in pachychoroid spectrum disease. Am J Ophthalmol 2015;160:1243–1254.
8. Schaal S, Schiff WM, Kaplan HJ, Tezel TH. Simultaneous appearance of multiple evanescent white dot syndrome and multifocal choroiditis indicate a common causal relationship. Ocul Immunol Inflamm 2009;17:325–327.
9. Bryan RG, Freund KB, Yannuzzi LA, et al.. Multiple evanescent white dot syndrome in patients with multifocal choroiditis. Retina 2002;22:317–322.
Keywords:

multiple evanescent white dot syndrome; multifocal choroiditis; pachychoroid spectrum disease

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