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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 3  |  Page : 719-721

Centrifugal propagation of the circumpapillary lesion in a patient with atypical multiple evanescent white dot syndrome


Department of Ophthalmology, Hallym University College of Medicine, Kangdong Sacred Heart Hospital, Seoul, South Korea

Date of Submission14-Feb-2022
Date of Acceptance13-May-2022
Date of Web Publication16-Jul-2022

Correspondence Address:
Dr. Yong-Kyu Kim
Department of Ophthalmology, Hallym University College of Medicine, Kangdong Sacred Heart Hospital, #150 Seongan-ro, Gangdong-gu, Seoul - 05355
South Korea
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_116_22

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  Abstract 


A 20-year-old woman presented with central scotoma in her left eye. Whitish retinal spots were observed mainly in the region nasal to the optic disk outside the major vascular arcade. Optical coherence tomography revealed subfoveal outer retinal disruption with hyperreflective material. Retinal spots coalesced into a larger lesion and progressed peripherally. Nasal retinal spots and foveal outer retinal lesion improved spontaneously, and her vision was completely recovered. The current case suggests that sequential sublethal infection of photoreceptors possibly via blood-borne pathogens and spreading to the nearby cells may be the possible alternative mechanism of multiple evanescent white dot syndrome pathophysiology.

Keywords: Fluorescein Angiography, optical coherence tomography, white dot syndromes


How to cite this article:
Joo CW, Kim YD, Park SP, Kim YK. Centrifugal propagation of the circumpapillary lesion in a patient with atypical multiple evanescent white dot syndrome. Indian J Ophthalmol Case Rep 2022;2:719-21

How to cite this URL:
Joo CW, Kim YD, Park SP, Kim YK. Centrifugal propagation of the circumpapillary lesion in a patient with atypical multiple evanescent white dot syndrome. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Aug 13];2:719-21. Available from: https://www.ijoreports.in/text.asp?2022/2/3/719/351118



Multiple evanescent white dot syndrome (MEWDS) is characterized by multiple white dots observed in the paramacular area. The disease usually affects young females on the unilateral side. The lesion usually subsides spontaneously within a few weeks, and patients get full recovery of vision.[1] The exact etiology of the disease is still elusive. Infectious origin, usually viral, or autoimmune is thought to be the possible mechanism.[2] Gass coined the term “AZOOR complex” for those series of diseases sharing similar clinical characteristics and suggested that they are various spectra of a single disease with the same pathophysiology.[3] These diseases are thought to be due to a disorder in photoreceptors, and the spreading of virus through nearby cells is a possible mechanism of the disease progression.[4] On the other hand, Jampol et al.[2] suggested that MEWDS is a distinctive disorder other than AZOOR or related diseases. They thought that the immune process involving genetic susceptibility and the environmental trigger was expected to be the mechanism of disease.

The white dots that last for several weeks are present in the posterior pole, mostly inside the major vascular arcade.[1] The retinal lesions could be classified as larger “spots” localized to the retinal pigment epithelium and photoreceptors and smaller “dots” localized to the outer nuclear layer.[5] However, data on the disappearance pattern of retinal dots or spots are lacking. Here, we present a case of MEWDS with foveal outer retinal disruption and centrifugal propagation of retinal spots in the nasal retina outside the major vascular arcade.


  Case Report Top


A 20-year-old woman visited our clinic due to a central scotoma in her left eye that started 5 days ago. She had no underlying disease and never had any ocular surgery other than laser-assisted subepithelial keratectomy in her both eyes to correct myopia 2 years ago. Best-corrected visual acuities (BCVAs) were 1.0 in her right eye and 0.4 in her left eye. Slit-lamp examination revealed no specific abnormal findings in the anterior segments. Fundus examination showed normal fundus findings in the right eye; however, multiple whitish retinal spots were observed mainly in the nasal retina of the left eye [Figure 1]a. Fluorescein angiography showed hyperfluorescent spots in the nasal retina and some in the temporal perifoveal area. A ring-shaped hyperfluorescent lesion was observed in the fovea [Figure 1]b. No significant abnormalities were observed in the perifoveal fundus autofluorescence image [Figure 1]c. Optical coherence tomography (OCT; Spectralis, Heidelberg Engineering, Heidelberg, Germany) showed diffuse, amorphous hyperreflective material in the outer retinal level of the fovea [Figure 2]a. On Day 5, BCVA of the left eye was 0.5. The nasal retinal spots coalesced into a larger lesion and new spots were observed in the periphery [Figure 1]d. The hyperreflective material that accumulated under the fovea decreased; however, focal ellipsoid zone disruption was found inferior to the foveal lesion [Figure 2]b. On systemic and aqueous viral tests, only immunoglobulin G for varicella-zoster virus and cytomegalovirus was positive in the serum; however, aqueous viral polymerase chain reaction (PCR) revealed negative result. Serology tests for syphilis, toxoplasmosis, and toxocariasis were all negative. On Day 9, BCVA of the left eye was 0.5. The nasal and superior lesions merged further into a larger lesion with an indistinct border and progressed peripherally [Figure 1]e. On OCT, the foveal lesion remained unchanged while the ellipsoid zone was obliterated in the area inferior to the foveal lesion [Figure 2]c. On Day 16, BCVA of the left eye was improved to 0.7. Foveal lesion diminished on OCT [Figure 2]d, and retinal spots were clinically invisible [Figure 1]f. On Day 30, the ellipsoid zone had recovered in the area inferior to the foveal lesion [Figure 2]e. BCVA of the left eye had improved to 1.0, and the patient's subjective visual discomfort was relieved on Day 65. The foveal ellipsoid zone recovered on Day 65 [Figure 2]f, and the foveal outer retinal structure was fully recovered with an intact interdigitation zone on Day 142 [Figure 2]g. There was no recurrence of retinal spots [Figure 1]f, [Figure 1]g, [Figure 1]h. Serial changes in the boundary of retinal spots are illustrated in [Figure 1]i
Figure 1: Serial changes in retinal spots. Serial fundus photography green channel images (a and d–h), initial fluorescein angiography (b), and initial fundus autofluorescence (c) are shown. (a) Baseline fundus photography green channel image (Day 0). Multiple bright spots were observed nasal to the optic disk. Corresponding fluorescein angiography (b) revealed hyperfluorescent spots in the nasal area and some in the perifoveal area. The annular hyperfluorescence was observed in the fovea. (c) No definite hyper- or hypo-autofluorescence was observed in the perifoveal area. (d) On Day 5, nasal retinal spots coalesced into a larger lesion and new spots were observed in the periphery. (e) On Day 9, nasal and superior lesions merged further into a larger lesion and progressed peripherally. (f) On Day 16, retinal spots disappeared and were not visible clinically. (g and h) On days 30 (g) and 65 (h), no recurrence of retinal spots was observed. (i) A cartoon depicting serial changes in the boundary of retinal spots during the initial 10 days. Red, Day 0; blue, Day 5; green, Day 9

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Figure 2: Optical coherence tomography findings for serial changes in foveal lesion. (a) Baseline image (Day 0). Diffuse, amorphous hyperreflective material (arrowheads) was observed in the outer retinal level of the fovea. (b) On Day 5, foveal hyperreflective material decreased with a distinct border (arrowheads). A focal ellipsoid zone disruption was observed inferior to the fovea (arrows). (c) On Day 9, no significant change in the foveal lesion was observed (arrowheads). The ellipsoid zone was totally obliterated in the area inferior to the foveal lesion (arrows). (d) On Day 16, foveal lesion further diminished (arrowheads). No significant changes were observed inferior to the foveal lesion (arrows). (e) On Day 30, the foveal lesion further decreased (arrowheads) and the ellipsoid zone had recovered in the area inferior to the foveal lesion (arrows). (f) On Day 65, the foveal ellipsoid zone had recovered (arrowheads). (g) On Day 142, the foveal interdigitation zone had recovered (arrowheads)

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  Discussion Top


In our case, the lesions were mainly located in the nasal retina and fovea, sparing most of the perifoveal area. MEWDS seems to be the most likely diagnosis as there was no anterior chamber inflammation and the whitish retinal lesion fully recovered without leaving any atrophic scar and vision had improved spontaneously.[6] Furthermore, early circumpapillary progression of the lesion[7] and subfoveal accumulation of various hyperreflective materials[8] are reported to be one of the various clinical features of MEWDS. Our case is unique in that the propagation of the lesion was biased toward the nasal side with discrete foveal involvement. Although it was not a recent infection, the patient showed a positive result for immunoglobulin G for varicella-zoster and cytomegalovirus. Past infection may have caused an immune response by some trigger. However, sequential spreading of the retinal lesion suggests direct cell infection rather than an autoimmune process. As Gass et al.[4] suggested, optic disk margin might be a possible site for pathogen invasion. Cytopathic effect may have occurred due to cell invasion; however, it does not seem to cause permanent damage. In addition to the nasal retina, a foveal lesion sparing most of the perifoveal area was observed. Hyperreflective material accumulation of various degrees was observed in the outer retina of MEWDS patients.[8] Fovea is the area with the highest metabolic demand,[9] and external pathogens might be delivered to the foveal region through blood flow, causing numerous reactions.


  Conclusion Top


Our case indicates that sequential sublethal infection of photoreceptors possibly via blood-borne pathogens and spreading to the nearby cells may be an alternative possible mechanism of MEWDS pathophysiology. Further research on a detailed study of the pattern of retinal white spot disappearance is needed.

Ethics approval

This study was exempted from review by the Institutional Review Board of the Kangdong Sacred Heart Hospital.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given consent for the use of images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

This research was supported by the Korean Association of Retinal Degeneration and the Basic Science Research Program through the National Research Foundation of Korea (NRF) funded by the Ministry of Science and ICT (2021R1F1A1057121).

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Jampol LM, Sieving PA, Pugh D, Fishman GA, Gilbert H. Multiple evanescent white dot syndrome: I. Clinical findings. Arch Ophthalmol 1984;102:671-4.  Back to cited text no. 1
    
2.
Jampol LM, Becker KG. White spot syndromes of the retina: A hypothesis based on the common genetic hypothesis of autoimmune/inflammatory disease. Am J Ophthalmol 2003;135:376-9.  Back to cited text no. 2
    
3.
Gass JD. Acute zonal occult outer retinopathy. Donders lecture: The Netherlands Ophthalmological Society, Maastricht, Holland, June 19, 1992. J Clin Neuroophthalmol 1993;13:79-97.  Back to cited text no. 3
    
4.
Gass JD, Agarwal A, Scott IU. Acute zonal occult outer retinopathy: A long-term follow-up study. Am J Ophthalmol 2002;134:329-39.  Back to cited text no. 4
    
5.
Pichi F, Srvivastava SK, Chexal S, Lembo A, Lima LH, Neri P, et al. En face optical coherence tomography and optical coherence tomography angiography of multiple evanescent white dot syndrome: New insights into pathogenesis. Retina 2016;36(Suppl 1):S178-88.  Back to cited text no. 5
    
6.
Russell JF, Pichi F, Scott NL, Hartley MJ, Bell D, Agarwal A, et al. Masqueraders of multiple evanescent white dot syndrome (MEWDS). Int Ophthalmol 2020;40:627-38.  Back to cited text no. 6
    
7.
Vela JI, Passabosc C, Buil Calvo JA. Early progressive circumpapillary lesion as atypical presentation of multiple evanescent white dot syndrome: A case report. Case Rep Ophthalmol 2020;11:546-52.  Back to cited text no. 7
    
8.
Marsiglia M, Gallego-Pinazo R, Cunha de Souza E, Munk MR, Yu S, Mrejen S, et al. Expanded clinical spectrum of multiple evanescent white dot syndrome with multimodal imaging. Retina 2016;36:64-74.  Back to cited text no. 8
    
9.
Ingram NT, Fain GL, Sampath AP. Elevated energy requirement of cone photoreceptors. Proc Natl Acad Sci U S A 2020;117:19599-603.  Back to cited text no. 9
    


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