|Year : 2022 | Volume
| Issue : 3 | Page : 272-274
Multiple evanescent white dot syndrome
M Arthi1, Chokkahalli K Nagesha2, Harshal P Gondhale3
1 Department of Retina and Vitreous, Rajan Eye Care Hospital, Chennai, Tamil Nadu, India
2 Department of Ophthalmology, Vitreo Retinal Consultant, B W Lions Superspeciality Eye Hospital, Bengaluru, Karnataka, India
3 Vitreo Retinal Consultant, Department of Ophthalmology, Gondhale Hospital, Nashik, Pune, Maharashtra, India
|Date of Submission||14-Jan-2022|
|Date of Decision||02-Jul-2022|
|Date of Acceptance||08-Jul-2022|
|Date of Web Publication||26-Sep-2022|
Rajan Eye Care Hospital, Vidyodhaya Second Street, T. Nagar, Chennai - 600 017, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Multiple evanescent white dot syndrome (MEWDS) was described by Jampol et al. in 1984 in 11 young patients who had unilateral transient retinal pigment epithelium (RPE) granularity and white dots at the level of RPE with vitreous cells. It is predominantly a disease of the photoreceptors with occasional involvement of the outer nuclear layer. Inflammatory microglial activation and changes in deeper retinal circulation contribute to the features of MEWDS. Patients are usually young females and may have a preceding history of flu-like illness. This reversible condition involves the photoreceptors causing reduced amplitudes in the full field and multifocal electroretinogram (mfERG), which recovers with the resolution of the disease. We present a case of MEWDS in a young female with typical clinical features with spontaneous improvement in symptoms with observation.
Keywords: Fundus autofluorescence, multiple evanescent white dot syndrome, white dot syndrome
|How to cite this article:|
Arthi M, Nagesha CK, Gondhale HP. Multiple evanescent white dot syndrome. TNOA J Ophthalmic Sci Res 2022;60:272-4
| Case Details|| |
A 32-year-old female patient presented with defective vision and photopsia in the right eye (RE). She had no history of viral prodrome. On examination, RE best-corrected visual acuity (BCVA) was 6/24 with a normal anterior segment. Fundus showed foveal granularity and white dots nasal to disc and temporal to the fovea [Figure 1]a. Fundus autofluorescence (AF) revealed peripapillary hyper autofluorescence extending along the arcades and fovea [Figure 1]d. Optical coherence tomography (OCT) showed disruption of the ellipsoid zone (EZ) [Figure 2]a. Fluorescein angiography showed window defects in the early phase [Figure 3]a and disc hyperfluorescence with punctate hyperfluorescent lesions in late phases [Figure 3]b, [Figure 3]c, [Figure 3]d. The left eye (LE) was normal. A diagnosis of MEWDS was made. One week later, the fundus revealed spontaneous resolution of the white dots, reduction in foveal granularity [Figure 1]b and hyper autofluorescence [Figure 1]e, and reformation of the EZ in the subfoveal region with persisting defects in other areas [Figure 2]b. In the final visit 4 weeks later, BCVA was 6/6 with complete resolution of dots and foveal granularity [Figure 1]c with faint hyperautofluorescence temporal to the fovea [Figure 1]f and persisting EZ defects [Figure 2]c.
|Figure 1: Showing colour fundus and corresponding fundus autofluorescence images taken during each visit. (a) showing the foveal granularity (indicated by a black arrow) and white dots seen temporal to the fovea and nasal to the disc (indicated by white circles). (b and c) showing the resolution of the white dots at 1 week and 4 weeks, respectively. (c) shows hyperautofluorescence, which resolves at 1 and 4 weeks shown by images (d,e and f) respectively|
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|Figure 2: Optical coherence tomography. (a) showing an area of disruption of ellipsoid zone indicated by red arrows and subfoveal EZ disruption denoted by yellow arrows. (b) showing the formation of the ellipsoid zone in the subfoveal region but the persistence of defects in other areas indicated by red arrows. (c) shows the imperfect reformation of the ellipsoid zone at 4 weeks indicated by red arrows|
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|Figure 3: Fundus fluorescein angiography figure a shows window defects seen corresponding to the areas of hyperautofluorescence in early phases (a) with areas of punctate hyperfluorescence arranged in a wreath-like pattern centered around the fovea in late phases (b-d)|
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MEWDS patients present with defective vision, photopsia, and scotoma. Clinical features include anterior vitreous cells, disc edema, foveal granularity, dots (100 m in size) or larger spots (200 m or larger) aligned along the retinal vessels with patchy sheathing. FFA findings include window defects, retinal vascular abnormalities, and punctate areas of early hyperfluorescence, whereas Indocyanine green angiography (ICGA) may show hypofluorescent spots more extensive than FFA, which correspond to disruption of EZ in OCT. Transiently increased choroidal thickness may be noted. Blue autofluorescence shows areas of hyperfluorescence corresponding to areas of hypofluorescence in ICG and EZ loss in OCT. Also, mfERG shows the supernormal response in the first-order kernels, which later become blunted. Differential diagnoses include other white dot syndromes and ocular syphilis. Though an overlap of clinical features of MEWDS with other inflammatory choriocapillaropathies such as multifocal choroiditis and punctate inner choroidopathy make it difficult to differentiate from these conditions, advent multimodal imaging FFA, ICG, and autofluorescence have made it easier to distinguish from these conditions. Carcinoma-associated retinopathy and primary vitreoretinal lymphoma are known to masquerade MEWDS.Age of presentation and duration of symptoms may help us clinch the diagnosis in thesis situations in addition to multimodal imaging. A high degree of suspicion and multimodal imaging are required in older atypical populations to differentiate from masqueraders. The presentation of a younger age group, fleeting nature of the lesions, viral prodrome, and characteristic imaging findings coupled with thorough systemic evaluation to rule out masquerading infections such as tuberculosis and syphilis may aid the clinician in making a diagnosis.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
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Cahuzac A, Wolff B, Mathis T, Errera M-H, Sahel J-A, Mauget-Faÿsse M. Multimodal imaging findings in 'hyper-early' stage MEWDS. Br J Ophthalmol 2017;101:1381-5.
Russell JF, Pichi F, Scott NL, Hartley MJ, Bell D, Agarwal A, et al
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[Figure 1], [Figure 2], [Figure 3]