Comparative observation of clinical features between primary and secondary multiple evanescent white dot syndrome_Chinese Journal of Ocular Fundus Diseases

Authors：

Qi Huihui , Li Xuejing , Zhang Peng , Chai Qiannan , Duan Jialiang ,  Shang Qingli

Department of Ophthalmology, The Second Hospital of Hebei Medical University, Shijiazhuang 050000, China S1Huihui now is working in the Hebei Eye Hospital, Xingtai 054001, China;

Corresponding?author：

Shang Qingli, Email: qinglishang2013@sina.cn

Keywords：

Multiple evanescent white dot syndrome; Primary; Secondary; Multifocal choroiditis; Punctate inner choroidopathy; Multimodal imaging

DOI：

10.3760/cma.j.cn511434-20250506-00198

Video：

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Objective To compare the clinical characteristics of primary multiple evanescent white dot syndrome (MEWDS) and secondary MEWDS. Methods A retrospective case-control study. A total of 27 patients 29 eyes diagnosed with MEWDS at the Eye Center of the Second Hospital of Hebei Medical University from January 2022 to January 2024 were enrolled in this study. All affected eyes underwent best corrected visual acuity (BCVA), fundus color photography, fundus autofluorescence (FAF), ultra-widefield FAF, fluorescein fundus angiography (FFA), indocyanine green angiography (ICGA), and optical coherence tomography (OCT) examinations. Among them, 9 eyes (all primary MEWDS) were examined separately by ultra-wide-angle swept-frequency source OCT angiography (WF SS-OCTA). BCVA was measured using a standard logarithmic visual acuity chart and was converted to the logarithmic minimum angle of resolution (logMAR) visual acuity during statistics. Distribution patterns of MEWDS lesions were categorized into optic disc, macular, and sectoral based on ultra-widefield FAF. Based on whether it was complicated by other chorioretinal diseases or whether other chorioretinal diseases appeared before the resolution of MEWDS lesions, the affected eyes were divided into a primary MEWDS group and a secondary MEWDS group, comprising 14 cases 16 eyes and 13 cases 13 eyes, respectively. Among the 13 eyes in the secondary MEWDS group, 8 were complicated by multifocal choroiditis, 3 by punctate inner choroidopathy, and 2 by idiopathic choroidal neovascularization. The clinical and multimodal imaging features were compared between the two groups. Comparison of two sets of count data was performed using Fisher's exact probability test. Results The mean logMAR BCVA was 0.20±0.17 in the primary MEWDS group and 0.44±0.19 in the secondary MEWDS group. The primary group comprised 10 eyes with the optic disc pattern and 6 with the macular pattern, whereas the secondary group had 2 and 11 eyes, respectively. An intact retinal pigment epithelium (RPE)-Bruch's membrane (BM)-choroidal capillary plexus (CC) complex was observed in 16 eyes and 13 eyes of the primary and secondary groups, respectively. The choroidal thickness was 229.00 (110.75) μm and 250.00 (117.50) μm in the primary and secondary groups, respectively. Statistically significant differences were observed between the two groups in logMAR BCVA (t=-3.64), distribution patterns of MEWDS lesions, and the number of eyes with intact RPE-BM-CC complex (P＜0.05). In contrast, no statistically significant differences were found in choroidal thickness, multimodal imaging features, or inflammatory scores (P＞0.05). WF SS-OCTA was performed in 9 eyes, the superficial capillary plexus, deep capillary plexus, outer retina and choriocapillaris layer appeared normal. En-face OCT at the layer of the outer retina showed hyperreflective dots and spots disseminated at the optic disc and posterior pole, corresponding roughly to spots on color fundus photography, hyperfluorescence in the early phase of FFA, hypofluorescence in the late phase of ICGA, hyperautofluorescence on ultra-widefield FFA and disruption of outer retina in OCT. Conclusions The RPE-BM-CC complex in primary MEWDS was intact and predominantly manifested in the optic disc pattern. In secondary MEWDS, the RPE-BM-CC complex was mostly incomplete and predominantly manifested in the macular pattern.

1.	Jampol LM, Sieving PA, Pugh D, et al. Multiple evanescent white dot syndrome. I. clinical findings[J]. Arch Ophthalmol, 1984, 102(5): 671-674. DOI: 10.1016/j.preteyeres.2023.101207.
2.	劉杏, 崔琨明, 田玉枝, 等. 多發性一過性白點綜合征和多發性一過性白點綜合征樣改變[J]. 中華眼底病雜志, 2023, 39(5): 430-436. DOI: 10.3760/cma.j.cn511434-20211228-00726.Liu X, Cui KM, Tian YZ, et al. Multiple evanescent white dot syndrome and multiple evanescent white dot syndrome-like change[J]. Chin J Ocul Fundus Dis, 2023, 39(5): 430-436. DOI: 10.3760/cma.j.cn511434-20211228-00726.
3.	Mathis T, Delaunay B, Cahuzac A, et al. Choroidal neovascularisation triggered multiple evanescent white dot syndrome (MEWDS) in predisposed eyes[J/OL]. Br J Ophthalmol, 2018, 102(7): 971-976[2017-09-28]. https://pubmed.ncbi.nlm.nih.gov/28972027/. DOI: 10.1136/bjophthalmol-2017-311005.
4.	Cicinelli MV, Hassan OM, Gill MK, et al. A multiple evanescent white dot syndrome-like reaction to concurrent retinal insults[J]. Ophthalmol Retina, 2021, 5(10): 1017-1026. DOI: 10.1016/j.oret.2020.12.007.
5.	Kang HG, Kim TY, Kim M, et al. Expanding the clinical spectrum of multiple evanescent white dot syndrome with overlapping multifocal choroiditis[J]. Ocul Immunol Inflamm, 2022, 30(1): 81-89. DOI: 10.1080/09273948.2020.1795206.
6.	Essilfie J, Bacci T, Abdelhakim AH, et al. Are there two forms of multiple evanescent white dot syndrome?[J]. Retina, 2022, 42(2): 227-235. DOI: 10.1097/IAE.0000000000003288.
7.	Serrar Y, Cahuzac A, Gascon P, et al. Comparison of primary and secondary forms of multiple evanescent white dot syndrome[J]. Retina, 2022, 42(12): 2368-2378. DOI: 10.1097/IAE.0000000000003609.
8.	Marsiglia M, Gallego-Pinazo R, Cunha de Souza E, et al. Expanded clinical spectrum of multiple evanescent white dot syndrome with multimodal imaging[J]. Retina, 2016, 36(1): 64-74. DOI: 10.1097/IAE.0000000000000685.
9.	Russell JF, Pichi F, Scott NL, et al. Masqueraders of multiple evanescent white dot syndrome (MEWDS)[J]. Int Ophthalmol, 2020, 40(3): 627-638. DOI: 10.1007/s10792-019-01223-4.
10.	Ong AY, Birtel J, Agorogiannis E, et al. Topographic patterns of retinal lesions in multiple evanescent white dot syndrome[J]. Graefe's Arch Clin Exp Ophthalmol, 2023, 261(8): 2257-2264. DOI: 10.1007/s00417-023-06032-1.
11.	Meng Y, Zhang Q, Li L, et al. Primary multiple evanescent white dot syndrome and multiple evanescent white dot syndrome secondary to multifocal choroiditis/punctate inner choroidopathy: a comparative study[J]. Retina, 2023, 43(7): 1122-1131. DOI: 10.1097/IAE.0000000000003776.
12.	Bosello F, Westcott M, Casalino G, et al. Multiple evanescent white dot syndrome: clinical course and factors influencing visual acuity recovery[J]. Br J Ophthalmol, 2022, 106(1): 121-127. DOI: 10.1136/bjophthalmol-2020-317357.
13.	Veronese C, Maiolo C, Morara M, et al. Bilateral multiple evanescent white dot syndrome[J]. Int Ophthalmol, 2018, 38(5): 2153-2158. DOI: 10.1007/s10792-017-0673-5.
14.	Finn AP, Khurana RN. Multiple evanescent white dot syndrome: bilateral disease may be silent and asymmetric[J/OL]. Am J Ophthalmol Case Rep, 2021, 21: 101004[2020-12-19]. https://pubmed.ncbi.nlm.nih.gov/33376834/. DOI: 10.1016/j.ajoc.2020.101004.
15.	Mathis T, Delaunay B, Favard C, et al. Hyperautofluorescent spots in acute ocular toxoplasmosis: a new indicator of outer retinal inflammation[J]. Retina, 2020, 40(12): 2396-2402. DOI: 10.1097/IAE.0000000000002759.
16.	Fung AT, Sorenson JA, Freund KB. An atypical white dot syndrome after traumatic subretinal hemorrhage[J]. Retin Cases Brief Rep, 2012, 6(4): 339-344. DOI: 10.1097/ICB.0b013e318260af4a.
17.	Gliem M, Birtel J, Müller PL, et al. Acute retinopathy in pseudoxanthoma elasticum[J]. JAMA Ophthalmol, 2019, 137(10): 1165-1173. DOI: 10.1001/jamaophthalmol.2019.2910.
18.	Bryan RG, Freund KB, Yannuzzi LA, et al. Multiple evanescent white dot syndrome in patients with multifocal choroiditis[J]. Retina, 2002, 22(3): 317-322. DOI: 10.1097/00006982-200206000-00010.
19.	Ramtohul P, Cicinelli MV, Dolz-Marco R, et al. The chrysanthemum phenotype of idiopathic multifocal choroiditis[J]. Retina, 2023, 43(8): 1377-1385. DOI: 10.1097/IAE.0000000000003815.
20.	閆淑, 杜敏, 邵玲, 等. 多發性一過性白點綜合征光相干斷層掃描圖像特征及脈絡膜厚度觀察[J]. 中華眼底病雜志, 2015, 31(6): 549-552. DOI: 10.3760/cma.j.issn.1005-1015.2015.06.009.Yan S, Du M, Shao L, et al. Characteristic of optical coherence tomography image and choroidal thickness in patients with multiple evanescent white dot syndrome[J]. Chin J Ocul Fundus Dis, 2015, 31(6): 549-552. DOI: 10.3760/cma.j.issn.1005-1015.2015.06.009.
21.	劉洪濤, 張軍軍, 曾志冰, 等. 多發性一過性白點綜合征多種眼底影像特征分析[J]. 中華眼底病雜志, 2016, 32(3): 257-260. DOI: 10.3760/cma.j.issn.1005-1015.2016.03.007.Liu HT, Zhang JJ, Zeng ZB, et al. Characteristics of multiple evanescent white dot syndrome with multimodal imaging[J]. Chin J Ocul Fundus Dis, 2016, 32(3): 257-260. DOI: 10.3760/cma.j.issn.1005-1015.2016.03.007.
22.	陳如, 俞素勤, 鄒文軍, 等. 多發性一過性白點綜合征患眼超廣角短波長眼底自身熒光特征[J]. 中華眼底病雜志, 2016, 32(3): 252-256. DOI: 10.3760/cma.j.issn.1005-1015.2016.03.006.Chen R, Yu SQ, Zou WJ, et al. Imaging features of ultra-wide field fundus autofluorescence in multiple evanescent white dot syndrome[J]. Chin J Ocul Fundus Dis, 2016, 32(3): 252-256. DOI: 10.3760/cma.j.issn.1005-1015.2016.03.006.
23.	Pereira F, Lima LH, de Azevedo AGB, et al. Swept-source OCT in patients with multiple evanescent white dot syndrome[J/OL]. J Ophthalmic Inflamm Infect, 2018, 8(1): 16[2018-10-13]. https://pubmed.ncbi.nlm.nih.gov/30317398/. DOI: 10.1186/s12348-018-0159-2.
24.	Cicinelli MV, Ramtohul P, Marchese A, et al. Latest advances in white spot syndromes: new findings and interpretations[J/OL]. Prog Retin Eye Res, 2023, 97: 101207[2023-08-12]. https://pubmed.ncbi.nlm.nih.gov/37574123/. DOI: 10.1016/j.preteyeres.2023.101207.
25.	Pradas M, Rodriguez-Merchante MP, Estébanez N, et al. Navigating the white dot syndromes with optical coherence tomography (OCT) and OCT angiography (OCT-A)[J]. Ocul Immunol Inflamm, 2022, 30(3): 664-674. DOI: 10.1080/09273948.2022.2046798.
26.	Pichi F, Srvivastava SK, Chexal S, et al. En face optical coherence tomography and optical coherence tomography angiography of multiple evanescent white dot syndrome: new insights into pathogenesis[J]. Retina, 2016, 36(Suppl 1): S178-188. DOI: 10.1097/IAE.0000000000001255.

1. Jampol LM, Sieving PA, Pugh D, et al. Multiple evanescent white dot syndrome. I. clinical findings[J]. Arch Ophthalmol, 1984, 102(5): 671-674. DOI: 10.1016/j.preteyeres.2023.101207.
2. 劉杏, 崔琨明, 田玉枝, 等. 多發性一過性白點綜合征和多發性一過性白點綜合征樣改變[J]. 中華眼底病雜志, 2023, 39(5): 430-436. DOI: 10.3760/cma.j.cn511434-20211228-00726.Liu X, Cui KM, Tian YZ, et al. Multiple evanescent white dot syndrome and multiple evanescent white dot syndrome-like change[J]. Chin J Ocul Fundus Dis, 2023, 39(5): 430-436. DOI: 10.3760/cma.j.cn511434-20211228-00726.
3. Mathis T, Delaunay B, Cahuzac A, et al. Choroidal neovascularisation triggered multiple evanescent white dot syndrome (MEWDS) in predisposed eyes[J/OL]. Br J Ophthalmol, 2018, 102(7): 971-976[2017-09-28]. https://pubmed.ncbi.nlm.nih.gov/28972027/. DOI: 10.1136/bjophthalmol-2017-311005.
4. Cicinelli MV, Hassan OM, Gill MK, et al. A multiple evanescent white dot syndrome-like reaction to concurrent retinal insults[J]. Ophthalmol Retina, 2021, 5(10): 1017-1026. DOI: 10.1016/j.oret.2020.12.007.
5. Kang HG, Kim TY, Kim M, et al. Expanding the clinical spectrum of multiple evanescent white dot syndrome with overlapping multifocal choroiditis[J]. Ocul Immunol Inflamm, 2022, 30(1): 81-89. DOI: 10.1080/09273948.2020.1795206.
6. Essilfie J, Bacci T, Abdelhakim AH, et al. Are there two forms of multiple evanescent white dot syndrome?[J]. Retina, 2022, 42(2): 227-235. DOI: 10.1097/IAE.0000000000003288.
7. Serrar Y, Cahuzac A, Gascon P, et al. Comparison of primary and secondary forms of multiple evanescent white dot syndrome[J]. Retina, 2022, 42(12): 2368-2378. DOI: 10.1097/IAE.0000000000003609.
8. Marsiglia M, Gallego-Pinazo R, Cunha de Souza E, et al. Expanded clinical spectrum of multiple evanescent white dot syndrome with multimodal imaging[J]. Retina, 2016, 36(1): 64-74. DOI: 10.1097/IAE.0000000000000685.
9. Russell JF, Pichi F, Scott NL, et al. Masqueraders of multiple evanescent white dot syndrome (MEWDS)[J]. Int Ophthalmol, 2020, 40(3): 627-638. DOI: 10.1007/s10792-019-01223-4.
10. Ong AY, Birtel J, Agorogiannis E, et al. Topographic patterns of retinal lesions in multiple evanescent white dot syndrome[J]. Graefe's Arch Clin Exp Ophthalmol, 2023, 261(8): 2257-2264. DOI: 10.1007/s00417-023-06032-1.
11. Meng Y, Zhang Q, Li L, et al. Primary multiple evanescent white dot syndrome and multiple evanescent white dot syndrome secondary to multifocal choroiditis/punctate inner choroidopathy: a comparative study[J]. Retina, 2023, 43(7): 1122-1131. DOI: 10.1097/IAE.0000000000003776.
12. Bosello F, Westcott M, Casalino G, et al. Multiple evanescent white dot syndrome: clinical course and factors influencing visual acuity recovery[J]. Br J Ophthalmol, 2022, 106(1): 121-127. DOI: 10.1136/bjophthalmol-2020-317357.
13. Veronese C, Maiolo C, Morara M, et al. Bilateral multiple evanescent white dot syndrome[J]. Int Ophthalmol, 2018, 38(5): 2153-2158. DOI: 10.1007/s10792-017-0673-5.
14. Finn AP, Khurana RN. Multiple evanescent white dot syndrome: bilateral disease may be silent and asymmetric[J/OL]. Am J Ophthalmol Case Rep, 2021, 21: 101004[2020-12-19]. https://pubmed.ncbi.nlm.nih.gov/33376834/. DOI: 10.1016/j.ajoc.2020.101004.
15. Mathis T, Delaunay B, Favard C, et al. Hyperautofluorescent spots in acute ocular toxoplasmosis: a new indicator of outer retinal inflammation[J]. Retina, 2020, 40(12): 2396-2402. DOI: 10.1097/IAE.0000000000002759.
16. Fung AT, Sorenson JA, Freund KB. An atypical white dot syndrome after traumatic subretinal hemorrhage[J]. Retin Cases Brief Rep, 2012, 6(4): 339-344. DOI: 10.1097/ICB.0b013e318260af4a.
17. Gliem M, Birtel J, Müller PL, et al. Acute retinopathy in pseudoxanthoma elasticum[J]. JAMA Ophthalmol, 2019, 137(10): 1165-1173. DOI: 10.1001/jamaophthalmol.2019.2910.
18. Bryan RG, Freund KB, Yannuzzi LA, et al. Multiple evanescent white dot syndrome in patients with multifocal choroiditis[J]. Retina, 2002, 22(3): 317-322. DOI: 10.1097/00006982-200206000-00010.
19. Ramtohul P, Cicinelli MV, Dolz-Marco R, et al. The chrysanthemum phenotype of idiopathic multifocal choroiditis[J]. Retina, 2023, 43(8): 1377-1385. DOI: 10.1097/IAE.0000000000003815.
20. 閆淑, 杜敏, 邵玲, 等. 多發性一過性白點綜合征光相干斷層掃描圖像特征及脈絡膜厚度觀察[J]. 中華眼底病雜志, 2015, 31(6): 549-552. DOI: 10.3760/cma.j.issn.1005-1015.2015.06.009.Yan S, Du M, Shao L, et al. Characteristic of optical coherence tomography image and choroidal thickness in patients with multiple evanescent white dot syndrome[J]. Chin J Ocul Fundus Dis, 2015, 31(6): 549-552. DOI: 10.3760/cma.j.issn.1005-1015.2015.06.009.
21. 劉洪濤, 張軍軍, 曾志冰, 等. 多發性一過性白點綜合征多種眼底影像特征分析[J]. 中華眼底病雜志, 2016, 32(3): 257-260. DOI: 10.3760/cma.j.issn.1005-1015.2016.03.007.Liu HT, Zhang JJ, Zeng ZB, et al. Characteristics of multiple evanescent white dot syndrome with multimodal imaging[J]. Chin J Ocul Fundus Dis, 2016, 32(3): 257-260. DOI: 10.3760/cma.j.issn.1005-1015.2016.03.007.
22. 陳如, 俞素勤, 鄒文軍, 等. 多發性一過性白點綜合征患眼超廣角短波長眼底自身熒光特征[J]. 中華眼底病雜志, 2016, 32(3): 252-256. DOI: 10.3760/cma.j.issn.1005-1015.2016.03.006.Chen R, Yu SQ, Zou WJ, et al. Imaging features of ultra-wide field fundus autofluorescence in multiple evanescent white dot syndrome[J]. Chin J Ocul Fundus Dis, 2016, 32(3): 252-256. DOI: 10.3760/cma.j.issn.1005-1015.2016.03.006.
23. Pereira F, Lima LH, de Azevedo AGB, et al. Swept-source OCT in patients with multiple evanescent white dot syndrome[J/OL]. J Ophthalmic Inflamm Infect, 2018, 8(1): 16[2018-10-13]. https://pubmed.ncbi.nlm.nih.gov/30317398/. DOI: 10.1186/s12348-018-0159-2.
24. Cicinelli MV, Ramtohul P, Marchese A, et al. Latest advances in white spot syndromes: new findings and interpretations[J/OL]. Prog Retin Eye Res, 2023, 97: 101207[2023-08-12]. https://pubmed.ncbi.nlm.nih.gov/37574123/. DOI: 10.1016/j.preteyeres.2023.101207.
25. Pradas M, Rodriguez-Merchante MP, Estébanez N, et al. Navigating the white dot syndromes with optical coherence tomography (OCT) and OCT angiography (OCT-A)[J]. Ocul Immunol Inflamm, 2022, 30(3): 664-674. DOI: 10.1080/09273948.2022.2046798.
26. Pichi F, Srvivastava SK, Chexal S, et al. En face optical coherence tomography and optical coherence tomography angiography of multiple evanescent white dot syndrome: new insights into pathogenesis[J]. Retina, 2016, 36(Suppl 1): S178-188. DOI: 10.1097/IAE.0000000000001255.

Chinese Journal of Ocular Fundus Diseases

Latest ArticlesComparative observation of clinical features between primary and secondary multiple evanescent white dot syndrome

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