Methods

The Institutional Review Board/Ethics Committee of Gunma University School of Medicine (Gunma, Japan) approved the retrospective review of patient data for multiple evanescent white dot syndrome. This study complied with the tenets of the Declaration of Helsinki. All patients provided informed consent for participation in this research.

The study included 14 eyes of 13 patients (4 men, 9 women; mean age, 35.8 years; range, 17–50 years) with MEWDS at Gunma University Hospital. One patient developed the MEWDS bilaterally at a different time. All patients reported unilateral blurred vision and spotty visual field defects at the first examination. The fundi in the affected eyes all had faint white spots of varying diameters mainly in the midperiphery to equatorial region. The included patients had typical MEWDS with numerous transient white spots and diffuse disruption of the photoreceptor outer segment on SD OCT images, which was later restored. All patients were healthy except for the ocular symptoms. No women were pregnant. The mean refractive error of the affected eyes was −5.3 diopters (D) (range, −0.5 to −11.25 D). The patients were followed untreated for a mean of 13.7 months (range, 3–57 months). In addition to routine ocular examinations, we performed color fundus photography with a 50-degree angle camera (Topcon TRC-50DX; Topcon Corporation, Tokyo, Japan), Goldmann perimetry (GP) or Humphrey field analyzer (HFA), ffERG, mfERG, and SD OCT (Cirrus HD-OCT; Carl Zeiss Meditec, Inc, Dublin, California, USA). We evaluated the FAF using a wide-field scanning laser ophthalmoscope (OPTOS 200Tx; Optos PLC, Dunfermline, UK), which allows nonmydriatic FAF imaging of up to 200 degrees of the fundus. OPTOS fundus photograph with pseudo-color images were obtained at each examination. During the acute 3-week stage, we performed OCT and wide-field FAF weekly. At the 3- to 8-week intermediate stage, we performed those test every 4 weeks. At the late stage after 9 weeks, we performed the tests once or twice in 2-month intervals. Two independent retinal specialists (H.H., S.K.) evaluated all photographs. The agreement in the grading of the FAF, color fundus, and OCT images exceeded 90%.

Results

Ultra-wide-field FAF detected the affected lesions with greater sensitivity than conventional color fundus photography. The affected lesions appeared as diffuse hyper-autofluorescence in the posterior pole and involved the peripapillary retina and macular area and surrounding hyper-autofluorescent spots outside the vascular arcades in all 14 eyes of 13 patients. During the first 2 weeks of observation, diffuse hyper-autofluorescence in the posterior pole expanded to the midperiphery and the peripheral hyper-autofluorescent spots spread farther to their maximal extent. The peripheral hyper-autofluorescent spots faded first and the posterior diffuse autofluorescence then faded. SD OCT showed diffuse disruption or blurring of the IS/OS line in the posterior fundus. The disrupted IS/OS then aggregated in nodules on the RPE and the IS/OS was restored. As the abnormal hyper-autofluorescence in the periphery and the posterior fundus faded to the level of the unaffected fellow eyes, the continuity of the disrupted IS/OS was restored. Table 1 shows the patient characteristics.

Case 1

A 41-year-old woman reported a central scotoma in the left eye 9 days before the first examination. The scotoma expanded gradually and caused generally blurred vision. At the first examination, the vision was 0.1 × −9.5 D in the left eye. Biomicroscopy showed cells in the anterior chamber in the left eye. A mild vitreous opacity was seen in the left eye. The right eye appeared normal. The left fundus had a slight whitish discoloration in the posterior fundus and numerous white spots of 1–2 disc diameters spreading in the midperiphery and equatorial regions ( Figure 1 ). Ultra-wide-field FAF showed diffuse hyper-autofluorescence in the peripapillary and macular regions. A hyper-autofluorescent spot was peripheral to the posterior lesion ( Figure 1 ). SD OCT showed diffuse disruption of the photoreceptor layer including the IS/OS, cone outer segment tips (COST), and external limiting membrane (ELM) in the posterior fundus. The RPE appeared irregular ( Figure 2 ). Cells were seen in the posterior vitreous. The photoreceptor layer was intact in the right eye. GP showed an enlarged blind spot that involved the macula in the left eye and peripheral depression in the right eye ( Figure 3 ). ffERG showed a nonrecordable rod response and low amplitudes of the maximal and cone responses in the affected eye and a normal response in the unaffected fellow eye. One week after the first examination, the whitish spot became faint but ultra-wide-field FAF showed diffuse hyper-autofluorescence that involved the posterior fundus and midperiphery. Multiple hyper-autofluorescent spots fused together in the peripheral fundus ( Figure 1 ). The BCVA of the left eye was 0.15. SD OCT showed multiple drusenoid nodules at the level of the disrupted IS/OS ( Figure 2 ). At 4 weeks, the left fundus appeared normal except for mottling in the equatorial region. Ultra-wide-field FAF showed irregular hyper-autofluorescence in the equatorial region but the retina was otherwise normal. The BCVA was 0.4 in the left eye. SD OCT showed that the IS/OS was restored except at the fovea. At 16 weeks, the BCVA of the left eye was 0.9. The left fundus appeared normal on ultra-wide color fundus photographs and FAF images ( Figure 1 , Bottom right). SD OCT showed that the IS/OS and COST were restored in the posterior pole, but the IS/OS was blurred and the COST was unidentifiable peripheral to the posterior pole ( Figure 2 ). ffERG showed slight recovery of the rod response, but the amplitudes of the maximal and cone responses were low. The GP became normal bilaterally ( Figure 3 ).

Case 1. The patient is a 41-year-old woman with multiple evanescent white dot syndrome in her left eye. (Top left) A color fundus photograph obtained during the first examination shows foveal granularity and scattered yellowish spots in the posterior pole and nasal midperiphery (best-corrected visual acuity [BCVA], 0.1). (Top right) At the first examination, the ultra-wide-field fundus autofluorescence image shows diffuse hyper-autofluorescence in the peripapillary retina and posterior pole and numerous hyper-autofluorescent spots in the periphery. (Bottom left) After 1 week, diffuse hyper-autofluorescence in the posterior fundus expanded and the peripheral hyper-autofluorescent spots fused, resulting in a geographic pattern in the peripheral fundus (BCVA, 0.15). (Bottom right) After 4 months, the fundus autofluorescence is normal (BCVA, 0.9).

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