White Dot Syndromes Series Part 1: Multiple Evanescent White Dot Syndrome

Figure 1. Case 1: A 23-year-old White woman with a recent history of sinus infection, but who was otherwise healthy, presented with a complaint of flashes on the temporal side of her vision for approximately 1 week, and a blind spot on the temporal side for 2 days, and visual acuities of 20/20. On these fundus exams, multiple, flat 100 to 200 µm creamy color and greyish-white lesions are present in the posterior pole and midperiphery of the right eye. Changes to the retinal pigment epithelium and vitreous cells are visible on the optical coherence tomography (OCT) scan of the right eye. OCT of the left eye, not shown here, was normal.
Figure 2. Case 1: These fundus autofluorescence (FAF) images (top left) of the patient’s right eye shows areas of hyper autofluorescence, while her left eye (top right) is normal. Fluorescein angiography (FA) images (bottom left and middle) reveals an area of hyperfluorescence with late staining and optic nerve head leakage, while her left eye (bottom right) is a normal study.
Figure 3. Case 2: A 42-year-old White woman presented with complaints of “seeing a flash of light in the temporal quadrant last week, then seeing multiple floaters, and finally, seeing a stream of floaters and slow flashes in the shape of a "C". She also noted that her vision was becoming more blurred. Visual acuities were 20/20 in each eye. Fundus examination, OCT and FAF are comparable with what was described in Case 1.
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Figure 4. Case 2: The top images are typical FAs, as previously noted. The indocyanine green (ICG) images at the bottom show the patient’s distinct hypocyanescent lesions.
Figure 5. Case 2: Three weeks later, the patient returned with clearance of the previously noted lesion. Her vision remained 20/20. She reports an episode of severe headache since her first visit. She reported that she was no longer experiencing “floaters,” but continued to experience “flashes.”
Figure 6. Case 3: A 23-year-old White woman presented with a recent onset flashes in the temporal quadrant of her right eye. The demographic presentation, fundus examination, FA, and FAF, were all typical of MEWDs. Examination and OCT imaging revealed a juxtafoveal lesion. Due to the presence of this lesion, the patient was prescribed oral prednisone 10 mg 4 times daily with a daily over-the-counter H2 antagonist (ranitidine). On follow-up examination 10 days later, the juxtafoveal lesion had significant resolution, at which time the patient was placed on a taper off regimen.
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Figure 7. Case 3: Over the next 3 years, the patient has been disease free as evident from these images. On OCT, an area focal choroidal excavation associated with previously noted lesions is present with no evidence of choroidal neovascular membranes or subretinal fluid.
Figure 8. Case 3: A year later, the patient returned for a second opinion. She reported a recurrence of her disease. At this visit, a subfoveal choroidal neovascular membrane was noted. She also reported having had a number of intravitreal anti-VEGF and steroid injections by a retinal specialist.

White dot syndromes (WDS) are a group of posterior segment inflammatory disorders, primarily affecting the outer retina and the choroid. These conditions can effectively be considered posterior uveitic in nature. The etiology of most of these conditions is elusive and not well understood. Specific disease entities under WDS include multiple evanescent white dot syndrome (MEWDS), multifocal choroiditis (MFC), acute posterior multifocal placoid pigment epitheliopathy (APMPPE), serpiginous choroiditis, multifocal choroiditis and panuveitis (MCP), punctate inner choroidopathy (PIC), acute zonal occult outer retinopathy (AZOOR), birdshot chorioretinopathy, diffuse subretinal fibrosis (DSF), and relentless placoid chorioretinitis (ampiginous choroiditis). There is also an overlapping of these conditions, which creates a diagnostic conundrum.^1,2

Multiple evanescent white dot syndrome (MEWDS), a more common form of WDS, was first observed in the 1980s.³ Women, aged 15 to 50 years who have myopia, are most prone to the disease.^3,4 Many patients experience symptoms associated with viral diseases, such as fever, headaches, and malaise.⁴ Ocular symptoms include photopsia, dyschromatopsia, and scotomas which are often located on the temporal side.⁴ Presentation is commonly unilateral (80%).² However, asymmetrical bilateral presentation is also possible.

Findings

In the acute phase, clinical and diagnostic findings include multiple white, cream-colored lesions of various sizes in the central and mid-peripheral retina³ (Figures 1 and 3). These lesions are usually at the level of the retinal pigment epithelium (RPE), as detected by optical coherence tomography (OCT).^4,5 Posterior vitreous cells may also be present and are easily observed on optical coherence tomography (OCT).^4,5 Discrete or diffuse plaque-like hyper autofluorescent areas are often detected upon fundus autofluorescence (FAF) analysis (Figures 2 and 3).^4,5 Early-phase fluorescein angiography (FA) may reveal punctate hyperfluorescence lesions of different sizes and leakage of the disc may reveal optic nerve involvement (Figures 2 and 4).^4,5 Indocyanine green angiography (ICG) highlights these lesions more effectively in the late phase as a hyperfluorescent area, sometimes coalesced together (Figure 4).^4,5

Prognosis

MEWDS is a self-limiting condition that may resolve within 2 to 10 weeks (Figure 5).² However, a patient’s symptoms may last for several months. Recurrence is possible in 10% of cases.⁴ Since etiology is undetermined, clinicians have yet to establish a standard prognosis or viable treatment options for the disease. The use of systemic steroids may be considered in cases where lesions invade the macular region, but the treatment method remains controversial due to the idiopathic nature of the disease (Figures 6 and 7).^1,2

Choroidal neovascular membranes (CNVM) are also possible as a sequela of the disease (Figures 7 and 8). This complication requires intravitreal anti-vascular endothelial growth factor (VEGF) or steroid injections (Figure 8).⁴ White dot syndromes including MEWDS are not common, with a reported incidence of 0.45 per 100,000 in community-based population reports.² However, optometrists should be familiar with the signs and symptoms of these conditions for proper management of the disease.

Mohammad Rafieetary, OD, FAAO, FORS, Dipl ABO, and Jessica Haynes, OD, FAAO, FORS, Dipl ABO, both practice at the Charles Retina Institute in Tennessee.

References

1. Mount GR, Kaufman EJ. White dot syndromes. StatPearls Publishing; 2021. http://www.ncbi.nlm.nih.gov/pubmed/32491777. Accessed December 28, 2021.

2. Abu-Yaghi NE, Hartono SP, Hodge DO, Pulido JS, Bakri SJ. White dot syndromes: A 20-year study of incidence, clinical features, and outcomes. Ocul Immunol Inflamm. 2011;19(6):426-430. doi:10.3109/09273948.2011.624287

3. Jampol LM, Sieving PA, Pugh D, Fishman GA, Gilbert H. Multiple evanescent white dot syndrome: I. clinical findings. Arch Ophthalmol. 1984;102(5):671-674. doi:10.1001/archopht.1984.01040030527008

4. Papasavvas I, Mantovani A, Tugal-Tutkun I, Herbort CP. Multiple evanescent white dot syndrome (MEWDS): update on practical appraisal, diagnosis and clinicopathology; a review and an alternative comprehensive perspective. J Ophthalmic Inflamm Infect. 2021;11(1):45. doi:10.1186/s12348-021-00279-7

5. Zicarelli F, Mantovani A, Preziosa C, Staurenghi G. Multimodal imaging of multiple evanescent white dot syndrome: a new interpretation. Ocul Immunol Inflamm. 2020;28(5):814-820. doi:10.1080/09273948.2019.1635169

Slideshow

Figure 4. Case 2: The top images are typical FAs, as previously noted. The indocyanine green (ICG) images at the bottom show the patient’s distinct hypocyanescent lesions.

Figure 7. Case 3: Over the next 3 years, the patient has been disease free as evident from these images. On OCT, an area focal choroidal excavation associated with previously noted lesions is present with no evidence of choroidal neovascular membranes or subretinal fluid.

Figure 8. Case 3: A year later, the patient returned for a second opinion. She reported a recurrence of her disease. At this visit, a subfoveal choroidal neovascular membrane was noted. She also reported having had a number of intravitreal anti-VEGF and steroid injections by a retinal specialist.

Findings

Prognosis

References

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