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Clinicopathologic Reports, Case Reports, and Small Case Series
December 2003

Atypical Manifestation of Multiple Evanescent White Dot Syndrome With Large Peripapillary Lesion

Arch Ophthalmol. 2003;121(12):1794-1796. doi:10.1001/archopht.121.12.1794

Multiple evanescent white dot syndrome (MEWDS) is on the spectrum of diseases that includes acute zonal occult outer retinopathy, acute idiopathic blind spot enlargement syndrome, punctate inner choroidopathy, multifocal choroiditis and panuveitis, and acute macular neuroretinopathy.1 We present an atypical case of MEWDS in which a strikingly large white peripapillary outer retinal lesion evolved into a lesion with a more classical MEWDS appearance.

Report of a Case.

A 32-year-old woman sought treatment because of sudden onset of "spots that grew into circles" in the left eye that were accompanied by "flashes and floaters" with loss of "parts of central vision." She denied any ocular pain or discomfort. She denied any systemic complaints, specifically those of any recent viral or flulike symptoms. Other than the use of levothyroxine sodium (Levoxyl; King Pharmaceuticals Inc, Bristol, Tenn) for thyroid dysfunction, she denied any other illnesses or medication use. At initial examination, her visual acuity was 20/20 OU uncorrected with no afferent pupillary defect. Anterior segment examination results were unremarkable in each eye. Funduscopic examination in the right eye revealed a peripapillary chorioretinal scar adjacent to an area of pigment (Figure 1A). Examination in the left eye showed diffuse outer retina or retinal pigment epithelium peripapillary whitening encompassing approximately 7 disc areas in size (Figure 1B). The posterior pole was otherwise normal in appearance. Fluorescein angiography demonstrated early patchy peripapillary choroidal filling in the left eye and minimal peripapillary hyperfluorescence in the middle of the phase (Figure 1C), as well as late staining of the disc and peripapillary vessels (Figure 1D). The patient underwent intensive workup at another hospital that included routine blood and chemistry examinations, as well as testing for antinuclear antibodies, antineutrophil cytoplasmic autoantibodies, rapid plasma reagin, human immunodeficiency virus, human leukocyte antigen-B27, and erythrocyte sedimentation rate. Results of these tests and that of head magnetic resonance imaging were all unremarkable.

Figure 1.
Fundus photograph obtained at the patient's initial examination demonstrated a peripapillary chorioretinal scar in the right eye (A) and a peripapillary lesion 7 disc areas in size in the outer retina or retinal pigment epithelium layer in the left eye (B). Digital fluorescein angiography in the left eye demonstrated patchy peripapillary choroidal filling and disc hyperfluorescence at 13 seconds (C) and staining of the disc and peripapillary vessels at 15 minutes (D).

Fundus photograph obtained at the patient's initial examination demonstrated a peripapillary chorioretinal scar in the right eye (A) and a peripapillary lesion 7 disc areas in size in the outer retina or retinal pigment epithelium layer in the left eye (B). Digital fluorescein angiography in the left eye demonstrated patchy peripapillary choroidal filling and disc hyperfluorescence at 13 seconds (C) and staining of the disc and peripapillary vessels at 15 minutes (D).

The patient returned 2 weeks later with similar complaints, though stating that the "spots had changed somewhat in appearance." Visual acuity remained 20/20 OU. Findings in the right eye remained unchanged. However, there were dramatic changes in the left eye. There was substantial resolution of the peripapillary whitening and the development of moderate disc edema (Figure 2A). More interestingly, however, small white dots ranging in size from 100 to 400 µm had emerged in the outer retina or retinal pigment epithelium layer throughout the posterior pole and middle of the periphery (Figure 2B and C). The patient declined both electroretinography and visual field testing. She returned 3 weeks later still complaining of a central "fuzzy spot" in the left eye. Amsler grid testing revealed a temporal defect. Octopus(Interzeag AG, Schlieren, Switzerland) visual field testing revealed a somewhat enlarged blind spot measuring 10o in the left eye. Residual disc hyperemia and elevation were noted, with almost complete resolution of the white dots (Figure 3).

Figure 2.
A, Two weeks after initial examination, there was substantial improvement in the peripapillary lesion in the left eye, although there was disc edema. The posterior pole demonstrated the emergence of small white dots in the outer retina or retinal pigment epithelium layer(B) that were also present in the middle of the periphery (C).

A, Two weeks after initial examination, there was substantial improvement in the peripapillary lesion in the left eye, although there was disc edema. The posterior pole demonstrated the emergence of small white dots in the outer retina or retinal pigment epithelium layer(B) that were also present in the middle of the periphery (C).

Figure 3.
Five weeks after initial examination, the patient had almost complete resolution of the peripapillary lesion and white dots from the posterior pole in the left eye. Mild disc edema persisted.

Five weeks after initial examination, the patient had almost complete resolution of the peripapillary lesion and white dots from the posterior pole in the left eye. Mild disc edema persisted.

Comment.

To our knowledge, there was 1 previous report of MEWDS that initially manifested as a transient circumpapillary retinal lesion that preceded the appearance of white dots.2 Results in our article and others in which indocyanine green angiography was used to demonstrate peripapillary hypofluroescence may suggest that this site is important in the early stages of the disease. Although the cause of MEWDS remains a mystery, Gass et al1 proposed that in acute zonal occult outer retinopathy viral introduction in the retina may occur at the optic nerve margin and ora serrata because of the absence of surrounding neuroepithelium at these sites. The initial manifestation of transient discrete peripapillary outer retina or retinal pigment epithelium involvement centered around the optic nerve in our patient would be consistent with such a hypothesis. The funduscopic appearance subsequently evolved into more typical MEWDS findings, including deep retinal white dots surrounding the paramacular area with foveal sparing, blurring of the disc margin, and vascular involvement at angiography.3 The patient's subjective complaints of photopsia and temporal scotoma and the blind spot enlargement at visual field testing are all consistent with the diagnosis of MEWDS.3 Because of their transient nature, these peripapillary findings may be more common in MEWDS than previously appreciated.

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Article Information

Corresponding author: John Loewenstein, MD, Retina Service, Massachusetts Eye and Ear Infirmary, Department of Ophthalmology, Harvard Medical School, 243 Charles St, Boston, MA 02446(e-mail: john_loewenstein@meei.harvard.edu).

References
1.
Gass  JDAgarwal  AScott  IU Acute zonal occult outer retinopathy: a long-term follow-up study. Am J Ophthalmol. 2002;134329- 339
PubMedArticle
2.
Jampol  LMSieving  PAPugh  DFishman  GAGilbert  H Multiple evanescent white dot syndrome: clinical findings. Arch Ophthalmol. 1984;102 ((pt 1)) 671- 674
PubMedArticle
3.
Barile  GRReppucci  VSSchiff  WMWong  DT Circumpapillary chorioretinopathy in multiple evanescent white-dot syndrome. Retina. 1997;1775- 77
PubMed
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