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International Ophthalmology
Published in: International Ophthalmology 3/2020

01-03-2020 | Lymphoma | Original Paper

Masqueraders of multiple evanescent white dot syndrome (MEWDS)

Authors: Jonathan F. Russell, Francesco Pichi, Nathan L. Scott, Matthew J. Hartley, Dugald Bell, Aniruddha Agarwal, Belinda Leong, Gary N. Holland, K. Bailey Freund, David Sarraf

Published in: International Ophthalmology | Issue 3/2020

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Abstract

Purpose

To describe disorders that can masquerade as multiple evanescent white dot syndrome (MEWDS).

Design

Retrospective, multicenter case series.

Participants

Patients who presented with clinical findings compatible with a diagnosis of MEWDS but were ultimately diagnosed with an alternative inflammatory, infectious, or neoplastic disorder.

Methods

Clinical records and multimodal imaging findings including fundus photography, fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), optical coherence tomography (OCT), and OCT angiography (OCTA) were analyzed.

Main outcome measures

Inclusion criteria to be defined as a masquerade syndrome for MEWDS included the presence of disseminated grayish-white outer retinal spots that were hyperautofluorescent on FAF and associated with ellipsoid zone (EZ) disruption on OCT.

Results

Twenty-two eyes of 13 patients were identified. All patients presented with the classic findings of MEWDS listed above. A MEWDS-like presentation was bilateral in nine of 13 patients (69%). Final diagnosis was determined on the basis of additional investigations including serologies and biopsy. These diagnoses included syphilis (three patients), lymphoma (three patients), idiopathic multifocal choroiditis (two patients), idiopathic retinal phlebitis (one patient), idiopathic acute zonal occult outer retinopathy (one patient), sarcoidosis (one patient), tuberculosis (one patient), and cancer-associated retinopathy (one patient). The outer retinal lesions and imaging findings resolved with treatment for the associated systemic disorders.

Conclusions

Widespread grayish-white outer retinal spots associated with hyperautofluorescence on FAF and disruption of the EZ on OCT are not pathognomonic for MEWDS. A high index of suspicion must be maintained for masqueraders of MEWDS, which can include serious inflammatory, infectious, and neoplastic disorders.
Literature
1.
Jampol LM, Sieving PA, Pugh D, Fishman GA, Gilbert H (1984) Multiple evanescent white dot syndrome. I. Clinical findings. Arch Ophthalmol 102(5):671–674CrossRef
2.
Sieving PA, Fishman GA, Jampol LM, Pugh D (1984) Multiple evanescent white dot syndrome. II. Electrophysiology of the photoreceptors during retinal pigment epithelial disease. Arch Ophthalmol 102(5):675–679CrossRef
3.
Yamamoto S, Hayashi M, Tsuruoka M et al (2003) S-cone electroretinograms in multiple evanescent white dot syndrome. Doc Ophthalmol 106:117–120CrossRef
4.
Feigl B, Haas A, El-Shabrawi Y (2002) Multifocal ERG in multiple evanescent white dot syndrome. Graefes Arch Clin Exp Ophthalmol 240:615–621CrossRef
5.
Dell’omo R, Wong R, Marino M et al (2010) Relationship between different fluorescein and indocyanine green angiography features in multiple evanescent white dot syndrome. Br J Ophthalmol 94:59–63CrossRef
6.
Agrawal RV, Biswas J, Gunasekaran D (2013) Indocyanine green angiography in posterior uveitis. Indian J Ophthalmol 61:148–159CrossRef
7.
Pichi F, Srivastava S, Chexal S et al (2016) En face optical coherence tomography and optical coherence tomography angiography of multiple evanescent white dot syndrome. Retina 36:S178–S188CrossRef
8.
Marsiglia M, Gallego-Pinazo R, Cunha de Souza E et al (2016) Expanded clinical spectrum of multiple evanescent white dot syndrome with multimodal imaging. Retina 36:64–74CrossRef
9.
10.
Su D, Xu D, Phasukkijwatana N et al (2017) En face optical coherence tomography of multiple evanescent white dot syndrome. Retin Cases Brief Rep 11(Suppl 1):S121–123CrossRef
11.
Theodore FH (1967) Conjunctival carcinoma masquerading as chronic conjunctivitis. Eye Ear Nose Throat Mon 46(11):1419–1420PubMed
12.
Kubicka-Trzaska A, Romanowska-Dixon B (2008) Malignant uveitis masquerade syndromes. Klin Oczna 110(4–6):199–202PubMed
13.
Saha S, Graham EM (2009) Masquerade syndromes. Uveitis and immunological disorders. In: Singh A (ed) Essential of ophthalmology. Springer, pp 97–115
14.
Kim JA, Park KH, Woo SJ (2014) A case of syphilitic outer retinitis mimicking acute zonal occult outer retinopathy. Korean J Ophthalmol 28(6):497–499CrossRef
15.
Lima BR, Mandelcorn ED, Bakshi N et al (2014) Syphilitic outer retinopathy. Ocul Immunol Inflamm 22(1):4–8CrossRef
16.
Saleh MGA, Campbell JP, Yang P et al (2017) Ultra-wide-field fundus autofluorescence and spectral-domain optical coherence tomography findings in syphilitic outer retinitis. Ophthalmic Surg Lasers Imaging Retina 48:208–215CrossRef
17.
Hussnain SA, Daccache A, Klausner JD et al (2019) Multimodal imaging of atypical acute syphilitic posterior placoid chorioretinitis mimicking a white dot syndrome. Ophthalmic Surg Lasers Imaging Retina 50:e52–e55CrossRef
18.
Joseph A, Rahimy E, Freund KB et al (2013) Fundus autofluorescence and photoreceptor bleaching in multiple evanescent white dot syndrome. Ophthalmic Surg Lasers Imaging Retina 44(6):588–592CrossRef
19.
Onishi AC, Roberts PK, Jampol LM et al (2019) Characterization and correlation of “Jampol dots” on adaptive optics with foveal granularity on conventional fundus imaging. Retina 39(2):235–246CrossRef
20.
Tsui E, Gal-Or O, Ghadiali Q et al (2018) Multimodal imaging adds new insights into acute syphilitic posterior placoid chorioretinitis. Retin Cases Brief Rep 12(Suppl 1):S3–S8CrossRef
21.
Pichi F, Ciardella AP, Cunningham ET Jr et al (2014) Spectral domain optical coherence tomography findings in patients with acute syphilitic posterior placoid chorioretinopathy. Retina 34(2):373–384CrossRef
22.
Jabbarpoor Bonyadi MH, Ownagh V, Rahimy E et al (2019) Giraffe or leopard spot chorioretinopathy as an outstanding finding: case report and literature review. Int Ophthalmol 39(6):1405–1412CrossRef
23.
Davis JL (2013) Intraocular lymphoma: a clinical perspective. Eye (London) 27(2):153–162CrossRef
24.
Dreyer RF, Gass JDM (1984) Multifocal choroiditis and panuveitis: a syndrome that mimics ocular histoplasmosis. Arch Ophthalmol 102:1776–1784CrossRef
25.
Jung JJ, Khan S, Mrejen S et al (2014) Idiopathic multifocal choroiditis with outer retinal or chorioretinal atrophy. Retina 34(7):1439–1450CrossRef
26.
Munk MR, Jung JJ, Biggee K et al (2015) Idiopathic multifocal choroiditis/punctate inner choroidopathy with acute photoreceptor loss or dysfunction out of proportion to clinically visible lesions. Retina 35(2):334–343CrossRef
27.
Gattoussi S, Ghadiali Q, Dolz-Marco R et al (2018) Idiopathic multifocal choroiditis presenting with a transient peripapillary white ring. Retinal Cases Brief Rep 12(Suppl 1):S81–S86CrossRef
28.
Chen KC, Marsiglia M, Dolz-Marco R et al (2017) Foveal exudate and choroidal neovascularization in atypical cases of multiple evanescent white dot syndrome. Retina 37(11):2025–2034CrossRef
29.
Fine HF, Spaide RF, Ryan Jr. EH et al (2009) Acute zonal occult outer retinopathy in patients with multiple evanescent white dot syndrome. Arch Ophthalmol 127(1):66–70CrossRef
30.
Takahashi A, Saito W, Hashimoto Y et al (2015) Multiple evanescent white dot syndrome associated with retinal vasculitis. Int Med Case Rep J 8:209–213PubMedPubMedCentral
31.
Khochtali S, Abroug N, Ksiaa I et al (2018) Atypical white dot syndrome with choriocapillaris ischemia in a patient with latent tuberculosis. J Ophth Inflamm Infect 8(1):20CrossRef
32.
Chen FK, Chew AL, Zhang D et al (2017) Acute progressive paravascular placoid neuroretinopathy with negative-type electroretinography in paraneoplastic retinopathy. Doc Ophthalmol 134(3):227–235CrossRef
33.
Metadata
Title
Masqueraders of multiple evanescent white dot syndrome (MEWDS)
Authors
Jonathan F. Russell
Francesco Pichi
Nathan L. Scott
Matthew J. Hartley
Dugald Bell
Aniruddha Agarwal
Belinda Leong
Gary N. Holland
K. Bailey Freund
David Sarraf
Publication date
01-03-2020
Publisher
Springer Netherlands
Published in
International Ophthalmology / Issue 3/2020
Print ISSN: 0165-5701
Electronic ISSN: 1573-2630
DOI
https://doi.org/10.1007/s10792-019-01223-4

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