Macular serous retinal detachment (MSRD) or posterior SRD is a rare manifestation in myopic patients with an inferior staphyloma, tilted disc, or dome-shaped macula.1–4 Its pathogenesis is unclear. Multiple evanescent white dot syndrome (MEWDS) is a self-limiting inflammatory disorder entailing multiple small, white or yellowish dots scattered through the posterior pole at the level of the outer retina, retinal pigment epithelium (RPE), and inner choroid.5 MEWDS predominantly occurs in healthy young myopic individuals, particularly women, and it is preceded by a flu-like illness in about one-third of cases. Symptoms include acute unilateral visual impairment with enlarged scotoma and concurrent photopsia. The lesions and vision loss resolve within several weeks, with minimal sequelae.
In this case report, we describe the development and spontaneous resolution of MSRD accompanied by MEWDS in a highly myopic patient with a dome-shaped macula.
A healthy 32-year-old woman presented with acute vision loss in her right eye. Her vision was 20/70 in the right eye and 20/40 in the left eye. She had undergone LASIK 10 years prior. Axial length was 27.95 mm in her right eye and 27.42 mm in her left eye. Fundus examination revealed multiple yellowish dots around the macula and pigmentary change of the RPE in the right eye. Macular detachment and posterior staphyloma with dome-shaped macula were also present (Figure 1A, page 254). The RPE atrophic changes were visible in the both eyes. Spectral-domain optical coherence tomography (Cirrus HD-OCT; Carl Zeiss Meditec, Dublin, CA) revealed subretinal fluid at the top of macular bulge and disruption of the inner segment/ outer segment (IS/OS) junction of the photoreceptor (Figure 1B). Mid-phase fluorescein angiography (FA) (HRA2; Heidelberg Engineering, Heidelberg, Germany) showed increased transmission without active leakage at the macula (Figure 1C). Late-phase indocyanine green angiography (ICGA) revealed multiple hypofluorescent spots at the macula and an annular hypofluorescent area surrounding the optic disc (Figure 1D). An enlarged blind spot was detected in her visual field (Humphrey visual field analyzer; Carl Zeiss Meditec, Dublin, CA) (Figure 1E). The patient was diagnosed with MEWDS accompanied by MSRD.
(A) Fundus photograph at presentation showing multiple white dots around the macula and a myopic crescent. (B) OCT scan revealing shallow serous retinal detachment (arrowhead) at the top of the macular bulge with loss of inner segment/outer segment line of photoreceptors (between the arrows) and a thin choroid (111 μm) at the upper border of the macular bulge (between long arrowheads). (C) Fluorescein angiography showing increased transmission at the fovea (arrow). (D) Late-phase indocyanine green angiography demonstrating multiple hypofluorescent spots (arrow) at the macula and around the optic disc. (E) An enlarged blind spot detected in the automated visual field test.
Two weeks later, the white dots had faded and the patient’s vision had returned but was limited to 20/40 because of the RPE atrophic changes in the fovea (Figure 2A). OCT showed spontaneous resolution of the SRF and restoration of the IS/OS line (Figure 2B). Although the increased transmission was reduced only slightly on FA (Figure 2C), the multiple hypofluorescent lesions disappeared on ICGA. A hyperfluorescent spot of unknown origin was detected at the fovea on ICGA (Figure 2D).
(A) Fundus photograph showing resolution of the white dots 2 weeks later. (B) OCT scan images revealing the spontaneous disappearance of subretinal fluid, with restoration of the inner segment/outer segment line of the photoreceptors (between the arrows). (C) The increased transmission was reduced but remained on fluorescein angiography (arrow). (D) Multiple hypofluorescent spots were decreased on indocyanine green angiography (arrow), and a hyperfluorescent spot was detected. (E) Resolution of the enlarged blind spot was observed in an automated visual field test.
This patient showed development and spontaneous resolution of SRF accompanied by MEWDS in a highly myopic eye with a dome-shaped macula. Variations of MEWDS, such as MEWDS without a white dot,6 concurrence of MEWDS and acute zonal occult outer retinopathy,7 and secondary choroidal neovascularization in MEWDS,8 have been described. However, MEWDS with MSRD has not previously been reported.
In the acute attack of MEWDS, ICGA displays multiple hypofluorescent dots, which usually outnumber the lesions visible on fundus examination or FA.9 Multiple hypofluorescent spots on late-phase ICGA are not specific to MEWDS. They have also been reported in other choroid-involving inflammatory disorders, such as Vogt-Koyanagi-Harada disease10 and lupus choroidopathy.11 Previous results of OCT and electro-physiology studies showed that MEWDS might be a transient disturbance at the level of the RPE and photo-receptor outer segment complex.12–14 MEWDS could be an inflammatory disease, with varying degrees of retinal outer segment and choroidal involvement. However, the pathogenesis of MEWDS has been unclear.
There have been several reports of serous retinal detachment associated with an inferior staphyloma and dome-shaped macula.3,15,16 Subretinal fluid reportedly seldom resolves spontaneously.2 In previous reports of patients with serous retinal detachment, increased transmission and late staining due to the RPE depigmentation were revealed above the upper border of the inferior staphyloma on FA, and hypofluorescent lesions were shown on ICGA beyond the area of increased transmission on FA.1,13 RPE dysfunction was suggested as a possible mechanism in the development of serous retinal detachment in the inferior staphyloma.1,17 RPE atrophic changes were also observed in patients with a dome-shaped macula4 or tilted disc syndrome.2
Our patient showed increased transmission on FA, a large hypofluorescent spot on ICGA, and a very thin choroid due to a dome-shaped macula corresponding to the MSRD area on the OCT scan of the foveal lesion. The multiple hypofluorescent lesions on ICGA significantly decreased when the whitish lesions of MEWDS and MSRD resolved, but slightly increased transmission on FA remained. One possible mechanism is that mild or moderate RPE impairment insufficient to induce MSRD was superimposed by the RPE dysfunction resulting from MEWDS and provoked MSRD in this highly myopic patient with a dome-shaped macula. With time, the RPE dysfunction due to MEWDS improved, followed by the disappearance of the MSRD.
In summary, transient MSRD accompanied by MEWDS can develop in highly myopic patients with a dome-shaped macula.
- Yamagishi T, Koizumi H, Yamazaki T, Kinoshita S. Choroidal thickness in inferior staphyloma associated with posterior serous retinal detachment. Retina. 2012;32(7):1237–1242.
- Cohen SY, Quentel G, Guiberteau B, Delahaye-Mazza C, Gaudric A. Macular serous retinal detachment caused by subretinal leakage in tilted disc syndrome. Ophthalmology. 1998;105(10):1831–1834. doi:10.1016/S0161-6420(98)91024-7 [CrossRef]
- Leys AM, Cohen SY. Subretinal leakage in myopic eyes with a posterior staphyloma or tilted disk syndrome. Retina. 2002;22(5):659–665. doi:10.1097/00006982-200210000-00025 [CrossRef]
- Gaucher D, Erginay A, Lecleire-Collet A, et al. Dome-shaped macula in eyes with myopic posterior staphyloma. Am J Ophthalmol. 2008;145(5):909–914. doi:10.1016/j.ajo.2008.01.012 [CrossRef]
- dell’Omo R, Pavesio CE. Multiple evanescent white dot syndrome (MEWDS). Int Ophthalmol Clin. 2012;52(4):221–228. doi:10.1097/IIO.0b013e31826647ed [CrossRef]
- Shelsta HN, Rao RR, Bhatt HK, Jampol LM. Atypical presentations of multiple evanescent white dot syndrome without white dots: a case series. Retina. 2011;31(5):973–976. doi:10.1097/IAE.0b013e31820a67cc [CrossRef]
- Fine HF, Spaide RF, Ryan EH Jr, Matsumoto Y, Yannuzzi LA. Acute zonal occult outer retinopathy in patients with multiple evanescent white dot syndrome. Arch Ophthalmol. 2009;127(1):66–70. doi:10.1001/archophthalmol.2008.530 [CrossRef]
- Rouvas AA, Ladas ID, Papakostas TD, Moschos MM, Vergados I. Intravitreal ranibizumab in a patient with choroidal neovascularization secondary to multiple evanescent white dot syndrome. Eur J Ophthalmol. 2007;17(6):996–999.
- dell’Omo R, Wong R, Marino M, Konstantopoulou K, Pavesio C. Relationship between different fluorescein and indocyanine green angiography features in multiple evanescent white dot syndrome. Br J Ophthalmol. 2010;94(1):59–63. doi:10.1136/bjo.2009.163550 [CrossRef]
- Herbort CP, Mantovani A, Bouchenaki N. Indocyanine green angiography in Vogt-Koyanagi-Harada disease: angiographic signs and utility in patient follow-up. Int Ophthalmol. 2007;27(2–3):173–182. doi:10.1007/s10792-007-9060-y [CrossRef]
- Gharbiya M, Bozzoni-Pantaleoni F, Augello F, Balacco-Gabrieli C. Indocyanine green angiographic findings in systemic lupus erythematosus choroidopathy. Am J Ophthalmol. 2002;134(2):286–290. doi:10.1016/S0002-9394(02)01477-0 [CrossRef]
- Li D, Kishi S. Restored photoreceptor outer segment damage in multiple evanescent white dot syndrome. Ophthalmology. 2009;116(4):762–770. doi:10.1016/j.ophtha.2008.12.060 [CrossRef]
- 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(11):1552–1557. doi:10.1136/bjo.2007.135863 [CrossRef]
- Sieving PA, Fishman GA, Jampol LM, Pugh D. Multiple evanescent white dot syndrome. II. Electrophysiology of the photoreceptors during retinal pigment epithelial disease. Arch Ophthalmol. 1984;102(5):675–679. doi:10.1001/archopht.1984.01040030531009 [CrossRef]
- Tosti G. Serous macular detachment and tilted disc syndrome. Ophthalmology. 1999;106(8):1453–1455. doi:10.1016/S0161-6420(99)90472-4 [CrossRef]
- Caillaux V, Gaucher D, Gualino V, Massin P, Tadayoni R, Gaudric A. Morphologic characterization of dome-shaped macula in myopic eyes with serous macular detachment. Am J Ophthalmol. 2013;156(5):958–967. doi:10.1016/j.ajo.2013.06.032 [CrossRef]
- Nakanishi H, Tsujikawa A, Gotoh N, et al. Macular complications on the border of an inferior staphyloma associated with tilted disc syndrome. Retina. 2008;28(10):1493–1501. doi:10.1097/IAE.0b013e318183589c [CrossRef]