Fundus fluorescein angiography and optical coherence tomographic characteristics in acute multiple evanescent white dot syndrome

Priya Rasipuram Chandrasekaran

doi:10.4103/kjo.kjo_14_21

PHOTO ESSAY

Year : 2021 | Volume : 33 | Issue : 2 | Page : 214-216

Fundus fluorescein angiography and optical coherence tomographic characteristics in acute multiple evanescent white dot syndrome

Priya Rasipuram Chandrasekaran
Department of Medical Retina and Neuro-ophthalmology, Lotus Eye Hospital, Salem, Tamil Nadu, India

Date of Submission	15-Jan-2021
Date of Acceptance	25-Jan-2021
Date of Web Publication	21-Aug-2021

Correspondence Address:
Dr. Priya Rasipuram Chandrasekaran
Lotus Eye Hospital, Salem - 636 016, Tamil Nadu
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/kjo.kjo_14_21

Abstract

A 38-year-old female presented with a best-corrected visual acuity of 20/60 N18 and 20/20 N6. Fundus examination showed multiple yellow-white dots and spots at the posterior pole with disc edema and orange-yellow granularity at fovea. Fundus fluorescein angiography showed dots showing punctate hyperfluorescence and spots showing late staining and disc leakage. Optical coherence tomographic macula showed sub foveal hyperreflective elevated lesion on the retina pigment epithelium extending into the inner retina as far as the outer nuclear layer through disruption of inter digitation zone, ellipsoid zone (EZ), external limiting membrane and hyporeflectivity, and disruption of ELM and EZ corresponding to parafoveal lesions (predominantly spots).

Keywords: Fundus fluorescein angiography characteristics, multiple evanescent white dot syndrome, optical coherence tomographic characteristics

How to cite this article:
Chandrasekaran PR. Fundus fluorescein angiography and optical coherence tomographic characteristics in acute multiple evanescent white dot syndrome. Kerala J Ophthalmol 2021;33:214-6

How to cite this URL:
Chandrasekaran PR. Fundus fluorescein angiography and optical coherence tomographic characteristics in acute multiple evanescent white dot syndrome. Kerala J Ophthalmol [serial online] 2021 [cited 2022 Jan 23];33:214-6. Available from: http://www.kjophthal.com/text.asp?2021/33/2/214/324195

Case Report

A 38-year-old female presented with best-corrected visual acuity of 20/60 N 18 and 20/20 N6 after a flu-like illness. She had photopsia, scotoma, and decreased vision in the right eye. Fundus photo and fundus photo montage showed multiple yellow-white dots nasal to macula and close to disc and spots at the posterior pole with disc edema and orange-yellow granularity at fovea [Figure 1] and [Figure 2]. Fundus fluorescein angiography (FFA) showed dots revealing punctate hyperfluorescence starting in the early choroidal phase in an incomplete wreath pattern and continuing in the arterial phase [Figure 3] and spots revealing hyperfluorescence of the margins in the early phase which stain in the late phase. Disc leakage was evident [Figure 4]. Optical coherence tomographic macula showed sub foveal hyper reflective material extending into the outer nuclear layer through disruption of external limiting membrane (ELM), ellipsoid zone (EZ), and inter digitation zone [Figure 5]. Parafoveal region showed disruption and mashed up ELM and EZ corresponding to the spots [Figure 6].

Figure 1: Fundus photo showing dots nasal to macula and close to optic disc (yellow arrow), spots in the para macular and posterior pole (white arrow) and orange-yellow granularity at the fovea (black arrow)

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Figure 2: Fundus photo montage showing dots nasal to macula and close to optic disc (yellow arrow), spots in the para macular and posterior pole (white arrow), orange-yellow granularity at the fovea (black arrow) and optic disc edema (pink arrow)

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Figure 3: Dots showing punctate hyperfluorescence starting in the early phase and extending into the arterial phase nasal to macula and close to disc (yellow arrows)

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Figure 4: Late phase of fundus fluorescein angiography showing staining of spots temporal to macula (yellow arrow) and disc leakage (white arrow)

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Figure 5: Optical coherence tomographic macula showing subfoveal hyperreflective lesion extending into the outer nuclear layer through disruption of ellipsoid zone, inter digitation zone and external limiting membrane (yellow arrow) and disruption of external limiting membrane and ellipsoid zone in the parafoveal region (white arrow)

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Figure 6: Optical coherence tomographic macula showing hyporeflective lesions corresponding to spots (yellow arrow), disruption of retina pigment epithelium (white arrow) and discontinuity of external limiting membrane, inter digitation zone, and ellipsoid zone (pink arrow)

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Discussion

Unilateral presentation, female preponderance, flu-like illness and clinical findings at the level of retina pigment epithelium (RPE), not going beyond major vascular arcades in multiple evanescent white dot syndrome (MEWDS) are consistent with the lines of Jampol et al.^[1] Gross et al. classified MEWDS into dots (approximately 100 μm) localized to deep retina and RPE and spots (≥200 μm) localized to RPE and inner choroid. Dots show punctate hyperfluorescence in the choroidal as well as in the retinal artery perfusion phase. This is explained by the window defects generated by perfusion of choriocapillaris through the inflammatory lesions causing disruption of RPE and retinal capillary microangiopathy.^[2] Spots are believed to be due to infiltration within RPE and inner choroid with inflammatory cells without significant ischemia.^[2] The normal appearance of choriocapillaris points the possible injury at the level of outer retina and photoreceptors. The inhomogeneity, attenuation of signal, and disruption of EZ may be due to inflammatory swelling of photoreceptors or RPE-photoreceptor complex and corresponded to spots resting on the RPE and dots if they extended into the inner retina and this could be correlated to FFA.^[3],[4],[5]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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2.	Gross EN, Yannuzzi YA, Freund BK, Spaide FR, Amato PG, Sigal R. Multiple evanescent white dot syndrome. Arch Ophthalmol 2006;124:493-500.
3.	Sikorski BL, Wojtkowski M, Kaluzny JJ, Szkulmowski M, Kowalczyk A. Correlation of spectral optical coherence tomography with fluorescein and indocyanine green angiography in multiple evanescent white dot syndrome. Br J Ophthalmol 2008;92:1552-7.
4.	Marsiglia M, Gallego-Pinazo R, De Souza EC, Munk RM, Yu S, Mrejen S, et al. Expanded clinical spectrum of multiple evanescent white dot syndrome with multimodal imaging. Retina 2016;36:64-74.
5.	Pereir F, Lima HL, de Azevedo AG, Zett C, Farah ME, Belfort R Jr. Swept-source OCT in patients with multiple evanescent white dot syndrome. J Ophthalmic Inflamm Infect 2018;8:16.