Multiple Evanescent White-Dot Syndrome in a 9-Year-Old Girl

Abstract

Purpose:

This work describes a case of multiple evanescent white-dot syndrome (MEWDS) in a 9-year-old girl.

Methods:

A case report is presented.

Results:

A case of MEWDS in a 9-year-old girl is described.

Conclusions:

To our knowledge this is the youngest presentation of MEWDS discussed in the literature. MEWDS should be considered in the differential diagnosis of ocular inflammation in the first decade of life.

Introduction

Case Report

A 9-year-old White girl presented with acute-onset blurred vision and several “spots with a blue tint” in her left eye. There was no discomfort or redness. About 10 days earlier, she reported a sore throat, headache, fever, and stomachache. Her medical history was unremarkable other than an episode of right periorbital cellulitis treated at age 13 months. Her social history was unremarkable, and her family history was remarkable only for a maternal grandmother with age-related macular degeneration and glaucoma. Review of systems found only the now-resolved flu-like symptoms.

Methods

On initial ophthalmic evaluation, best-corrected visual acuity was 20/25 OD and 20/50 OS. There was no afferent pupillary defect. Intraocular pressures and motility were within normal limits. Slitlamp and funduscopic examination of the right eye was unremarkable. In the left eye, there was at least 1+ cell in the anterior chamber. Examination of the left fundus revealed trace anterior vitreous cell. Optic nerves appeared pink and sharp, with a cup-to-disc ratio of 0.3 bilaterally; in the left eye there were peripapillary retinal pigment epithelium (RPE) changes. Retinal examination of the left eye revealed macular RPE mottling and multiple discrete yellow-white lesions deep to the retina scattered throughout the posterior pole and periphery (Figure 1).

Figure 1.

(A) Normal fundus photograph of the right eye using the Optos panoramic P200Tx imaging system. (B) Fundus photograph of the left eye showing peripapillary retinal pigment epithelium changes, macular retinal pigment epithelium mottling, and scattered discrete yellow/white lesions deep to the retina.

The patient was referred for further management of her unilateral ocular inflammation. On examination 2 weeks later, she felt that her vision was improving, and she reported seeing fewer blue dots with her left eye. Her best-corrected visual acuity was 20/25 OU, with normal pupillary and motility examination findings. Fundus examination again demonstrated multiple yellow-white lesions in the left eye, although they appeared less prominently. The diagnosis of multiple evanescent white-dot syndrome (MEWDS) was proposed. Further evaluation with visual field testing, fundus autofluorescence (FAF), optical coherence tomography (OCT), and angiography was recommended. Her parents declined fluorescein (FA) or indocyanine green angiography (ICGA). A 24-2 static Humphrey visual field test demonstrated blind-spot enlargement in the left eye (Figure 2).

Figure 2.

Humphrey visual field test results of both eyes. (Left) The left eye shows an enlarged blind spot. (Right) The right eye displays a normal visual field.

Results

FAF findings were normal in the right eye; the left eye showed hyperautofluorescence of the lesions (Figure 3). Widefield OCT (Optovue Avanti RTVue XR) also demonstrated the outer retinal abnormalities in the fovea and peripapillary areas in the left eye (Figure 4). At follow-up examination 3 weeks later, the patient was asymptomatic with resolution of the white spots and decreasing foveal RPE mottling in the left eye. Given the classic appearance and self-resolving nature of her retinal lesions, further systemic workup was deferred.

Figure 3.

(A) Normal fundus autofluorescence of the right eye (Optos panoramic P200Tx imaging system). (B) Fundus autofluorescence of the left eye with scattered hyperautofluorescent spots.

Figure 4.

Widefield en face optical coherence tomography with Optovue Avanti RTVue XR Ocular Coherence Tomography. (Top) Right eye with normal retinal architecture. (Bottom) Left eye with diffuse disruption of the ellipsoid layer as well as retinal pigment epithelium irregularity just temporal to the optic nerve.

Conclusions

Jampol et al ¹ first described MEWDS in 1984. They reported acute-onset unilateral vision loss in otherwise healthy young adults that was commonly preceded by viral illness, affected females more often than males, and had the appearance of small, discrete white dots at the level of the RPE. The small (100-200 μM) discrete white dots at the RPE level, along with mild anterior chamber cell and vitritis, are believed to be result of a transient disease of the RPE and outer retina. ² Bilateral cases, often asymmetric, have been described. The course is typically self-limiting, and treatment is usually not required.

There have been numerous reports in the literature describing the imaging characteristics of MEWDS. Since the 1980s, multiple imaging modalities, including FA, ICGA, and more recently, spectral-domain OCT and FAF, have provided a richer description of the natural history and understanding of MEWDS’ pathogenesis. ^{2 -6} It has been established that MEWDS affects both the outer retina and choroid. As was seen in our patient, there is typically a transient disruption of the ellipsoid layer on OCT scan, as described by Nguyen et al. ⁷

Despite the disruption, the RPE usually remains intact and there is no apparent photoreceptor loss, as there tends to be a full visual recovery. FA imaging demonstrates early hyperfluorescent dots that later stain in a “wreath-like” pattern. There may be concomitant optic disc leakage. ICGA typically demonstrates discrete areas of hypofluorescence corresponding to the lesions. There are often more of these hypofluorescent areas on ICGA than areas of hyperfluorescence on FA, indicating there may be a more widespread choroidal ischemia or inflammation. ^{3 -6} On FAF imaging, the lesions visible clinically demonstrate hyperautofluorescence. ³

MEWDS primarily affects young adults aged between 20 and 45 years. In the first description of MEWDS by Jampol and colleagues, ¹ initial presentation ranged between ages 17 and 39 years with a mean age of 28 years. Meyer and Jampol ⁸ later reported a case of asymmetric, bilateral MEWDS in a 14-year-old girl. In 1998, Olitsky ⁹ described the youngest reported case of MEWDS in a 10-year-old boy. As of June 2021, a PubMed literature search of English-language articles using the search terms “multiple evanescent white dot syndrome,” “children,” as well as iterations with “pediatric,” “youngest,” and “MEWDS,” did not reveal a patient younger than the one in our described case.

The history, course, and clinical findings in our patient are consistent with a diagnosis of unilateral MEWDS. To our knowledge, this is the first reported case of MEWDS in the first decade of life. MEWDS should be considered in the differential diagnosis of retinal inflammation in children.