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Multiple evanescent white dot syndrome associated with retinal vasculitis.

Takahashi A, Saito W, Hashimoto Y, Ishida S - Int Med Case Rep J (2015)

Bottom Line: One month later, these white dots and retinal sheathing spontaneously resolved in both cases.Three months later, impairments of the outer retinal morphology and the visual acuity were restored.These results suggest that retinal vasculitis possibly plays a role in the pathogenesis of thickened inner retinal layers in acute stage of MEWDS.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.

ABSTRACT

Purpose: A recent study revealed thickening of the inner retinal layers in acute stage of multiple evanescent white dot syndrome (MEWDS); however, the pathogenesis is still unknown. We report two cases with MEWDS whose funduscopy showed obvious retinal vasculitis.

Methods: Case reports.

Results: Healthy myopic 16- and 27-year-old women were the cases under study. In both cases, funduscopic examination revealed multiple, faint, small, subretinal white dots at the posterior pole to the midperiphery and macular granularity oculus dexter. Retinal vascular sheathing was also observed at midperiphery. Late-phase fluorescein angiography revealed leakages corresponding to the vascular sheathing. Enhanced depth imaging optical coherence tomography revealed the discontinuity of the ellipsoid zone corresponding to the white dots and increased macular choroidal thickness. One month later, these white dots and retinal sheathing spontaneously resolved in both cases. Three months later, impairments of the outer retinal morphology and the visual acuity were restored.

Conclusion: These results suggest that retinal vasculitis possibly plays a role in the pathogenesis of thickened inner retinal layers in acute stage of MEWDS.

No MeSH data available.


Related in: MedlinePlus

Photographs of the right eye in a patient with multiple evanescent white dot syndrome (Case 1) at the initial visit (A–E) and 3 months later (F).Notes: (A and B) Funduscopy showing multiple, faint, subretinal white dots extending from the posterior pole to the midperiphery (A and B, white arrowheads), with foveal granularity. Retinal vascular sheathing was also observed in the temporal midperiphery (B, arrows). (C) Late-phase fluorescein angiography showing faint hyperfluorescence corresponding to the white dots (arrowheads) and retinal vascular wall staining with leakage corresponding to the retinal sheathing (arrows). (D) On late-phase indocyanine green angiography, multiple hypofluorescent spots scattered over a wider area than the white dots were seen (arrowheads). (E) A horizontal EDI-OCT image through the fovea revealed the discontinuity of ellipsoid zone at macular area corresponding to the white dots (arrows). SCT was 295 µm. (F) The ellipsoid zone at the macula spontaneously restored, and the SCT decreased to 237 µm.Abbreviations: EDI-OCT, enhanced depth imaging optical coherence tomography; SCT, subfoveal choroidal thickness.
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f1-imcrj-8-209: Photographs of the right eye in a patient with multiple evanescent white dot syndrome (Case 1) at the initial visit (A–E) and 3 months later (F).Notes: (A and B) Funduscopy showing multiple, faint, subretinal white dots extending from the posterior pole to the midperiphery (A and B, white arrowheads), with foveal granularity. Retinal vascular sheathing was also observed in the temporal midperiphery (B, arrows). (C) Late-phase fluorescein angiography showing faint hyperfluorescence corresponding to the white dots (arrowheads) and retinal vascular wall staining with leakage corresponding to the retinal sheathing (arrows). (D) On late-phase indocyanine green angiography, multiple hypofluorescent spots scattered over a wider area than the white dots were seen (arrowheads). (E) A horizontal EDI-OCT image through the fovea revealed the discontinuity of ellipsoid zone at macular area corresponding to the white dots (arrows). SCT was 295 µm. (F) The ellipsoid zone at the macula spontaneously restored, and the SCT decreased to 237 µm.Abbreviations: EDI-OCT, enhanced depth imaging optical coherence tomography; SCT, subfoveal choroidal thickness.

Mentions: The patient’s best-corrected visual acuity (BCVA) was 1.0 OD (oculus dexter) and 1.5 OS (oculus sinister). Relative afferent pupillary defect was negative. There were no abnormal ocular findings OS. Slit-lamp examination revealed no inflammation in the anterior chamber or vitreous. Funduscopic examination showed multiple, faint, subretinal white dots at the posterior pole to the equator and macular granular change (Figure 1A and B, arrowheads). In addition, retinal vascular sheathing was observed at the midperiphery (Figure 1B, arrows). Fluorescein angiography (FA) showed hyperfluorescence continuing from the initial phase corresponding to the white dots (Figure 1C, arrowheads) and the staining of the optic disk. The retinal sheathing sites showed vascular staining with leakages in the late-phase OD (Figure 1C, arrows). Indocyanine green angiography showed no abnormal appearance in the initial phase and multiple hypofluorescence at the wider area than the white dots (Figure 1D, arrowheads). Enhanced depth imaging optical coherence tomography (EDI-OCT) revealed the discontinuity of the ellipsoid zone at macular area OD (Figure 1E, arrows) and that the subfoveal choroidal thickness (SCT) was 295 µm. Humphrey perimetry (30-2 Swedish threshold interactive algorithm standard test) showed a mild blind spot enlargement OU (oculus uterque) (mean deviation [MD] value: −1.83 dB OD and −1.08 dB OS). Multifocal electroretinography (mfERG) showed entirely decreased amplitude, except normal central amplitude OD, and mildly decreased amplitude at the retinal site that corresponded to the peripapillary area OS. Infection screening, including tuberculosis, syphilis, human T-cell leukemia virus type 1, and toxoplasmosis, showed negative results on QuantiFERON and serological testing or with antibodies for these agents. The chest X-ray was normal and serum angiotensin-converting enzyme was within normal limit. The patient received a diagnosis of MEWDS OD and was followed up with no treatment. One month after the first visit, the white dots and retinal vascular sheathing spontaneously resolved. After 3 months, the ellipsoid zone at the macula restored and the SCT decreased to 237 µm, together with increased BCVA of 1.5 OD (Figure 1F). On Humphrey perimetry, the MD value improved to −0.28 dB OD and −0.69 dB OS.


Multiple evanescent white dot syndrome associated with retinal vasculitis.

Takahashi A, Saito W, Hashimoto Y, Ishida S - Int Med Case Rep J (2015)

Photographs of the right eye in a patient with multiple evanescent white dot syndrome (Case 1) at the initial visit (A–E) and 3 months later (F).Notes: (A and B) Funduscopy showing multiple, faint, subretinal white dots extending from the posterior pole to the midperiphery (A and B, white arrowheads), with foveal granularity. Retinal vascular sheathing was also observed in the temporal midperiphery (B, arrows). (C) Late-phase fluorescein angiography showing faint hyperfluorescence corresponding to the white dots (arrowheads) and retinal vascular wall staining with leakage corresponding to the retinal sheathing (arrows). (D) On late-phase indocyanine green angiography, multiple hypofluorescent spots scattered over a wider area than the white dots were seen (arrowheads). (E) A horizontal EDI-OCT image through the fovea revealed the discontinuity of ellipsoid zone at macular area corresponding to the white dots (arrows). SCT was 295 µm. (F) The ellipsoid zone at the macula spontaneously restored, and the SCT decreased to 237 µm.Abbreviations: EDI-OCT, enhanced depth imaging optical coherence tomography; SCT, subfoveal choroidal thickness.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4590319&req=5

f1-imcrj-8-209: Photographs of the right eye in a patient with multiple evanescent white dot syndrome (Case 1) at the initial visit (A–E) and 3 months later (F).Notes: (A and B) Funduscopy showing multiple, faint, subretinal white dots extending from the posterior pole to the midperiphery (A and B, white arrowheads), with foveal granularity. Retinal vascular sheathing was also observed in the temporal midperiphery (B, arrows). (C) Late-phase fluorescein angiography showing faint hyperfluorescence corresponding to the white dots (arrowheads) and retinal vascular wall staining with leakage corresponding to the retinal sheathing (arrows). (D) On late-phase indocyanine green angiography, multiple hypofluorescent spots scattered over a wider area than the white dots were seen (arrowheads). (E) A horizontal EDI-OCT image through the fovea revealed the discontinuity of ellipsoid zone at macular area corresponding to the white dots (arrows). SCT was 295 µm. (F) The ellipsoid zone at the macula spontaneously restored, and the SCT decreased to 237 µm.Abbreviations: EDI-OCT, enhanced depth imaging optical coherence tomography; SCT, subfoveal choroidal thickness.
Mentions: The patient’s best-corrected visual acuity (BCVA) was 1.0 OD (oculus dexter) and 1.5 OS (oculus sinister). Relative afferent pupillary defect was negative. There were no abnormal ocular findings OS. Slit-lamp examination revealed no inflammation in the anterior chamber or vitreous. Funduscopic examination showed multiple, faint, subretinal white dots at the posterior pole to the equator and macular granular change (Figure 1A and B, arrowheads). In addition, retinal vascular sheathing was observed at the midperiphery (Figure 1B, arrows). Fluorescein angiography (FA) showed hyperfluorescence continuing from the initial phase corresponding to the white dots (Figure 1C, arrowheads) and the staining of the optic disk. The retinal sheathing sites showed vascular staining with leakages in the late-phase OD (Figure 1C, arrows). Indocyanine green angiography showed no abnormal appearance in the initial phase and multiple hypofluorescence at the wider area than the white dots (Figure 1D, arrowheads). Enhanced depth imaging optical coherence tomography (EDI-OCT) revealed the discontinuity of the ellipsoid zone at macular area OD (Figure 1E, arrows) and that the subfoveal choroidal thickness (SCT) was 295 µm. Humphrey perimetry (30-2 Swedish threshold interactive algorithm standard test) showed a mild blind spot enlargement OU (oculus uterque) (mean deviation [MD] value: −1.83 dB OD and −1.08 dB OS). Multifocal electroretinography (mfERG) showed entirely decreased amplitude, except normal central amplitude OD, and mildly decreased amplitude at the retinal site that corresponded to the peripapillary area OS. Infection screening, including tuberculosis, syphilis, human T-cell leukemia virus type 1, and toxoplasmosis, showed negative results on QuantiFERON and serological testing or with antibodies for these agents. The chest X-ray was normal and serum angiotensin-converting enzyme was within normal limit. The patient received a diagnosis of MEWDS OD and was followed up with no treatment. One month after the first visit, the white dots and retinal vascular sheathing spontaneously resolved. After 3 months, the ellipsoid zone at the macula restored and the SCT decreased to 237 µm, together with increased BCVA of 1.5 OD (Figure 1F). On Humphrey perimetry, the MD value improved to −0.28 dB OD and −0.69 dB OS.

Bottom Line: One month later, these white dots and retinal sheathing spontaneously resolved in both cases.Three months later, impairments of the outer retinal morphology and the visual acuity were restored.These results suggest that retinal vasculitis possibly plays a role in the pathogenesis of thickened inner retinal layers in acute stage of MEWDS.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.

ABSTRACT

Purpose: A recent study revealed thickening of the inner retinal layers in acute stage of multiple evanescent white dot syndrome (MEWDS); however, the pathogenesis is still unknown. We report two cases with MEWDS whose funduscopy showed obvious retinal vasculitis.

Methods: Case reports.

Results: Healthy myopic 16- and 27-year-old women were the cases under study. In both cases, funduscopic examination revealed multiple, faint, small, subretinal white dots at the posterior pole to the midperiphery and macular granularity oculus dexter. Retinal vascular sheathing was also observed at midperiphery. Late-phase fluorescein angiography revealed leakages corresponding to the vascular sheathing. Enhanced depth imaging optical coherence tomography revealed the discontinuity of the ellipsoid zone corresponding to the white dots and increased macular choroidal thickness. One month later, these white dots and retinal sheathing spontaneously resolved in both cases. Three months later, impairments of the outer retinal morphology and the visual acuity were restored.

Conclusion: These results suggest that retinal vasculitis possibly plays a role in the pathogenesis of thickened inner retinal layers in acute stage of MEWDS.

No MeSH data available.


Related in: MedlinePlus