Choroidal nevus is a common benign intraocular tumor that occurs with a frequency of 5% to 6.5% of the adult white population.1 Choroidal nevus can be associated with several retinal and retinal pigment epithelium (RPE) alterations including drusen, RPE hyperplasia, atrophy, detachment and fibrous metaplasia, orange pigment, subretinal fluid (SRF), intraretinal edema, and choroidal neovascular membrane.2 Overlying RPE changes can be observed ophthalmoscopically and are considered signs of chronicity, typically found over long-standing stable choroidal nevi.
Optical coherence tomography (OCT) is a useful tool for visualization of potentially important overlying retinal and RPE changes associated with choroidal nevi. OCT findings such as SRF, RPE detachment, cystoid retinal edema, and RPE and photoreceptor loss can be predictive of visual loss over time.3 Herein, we report a unique case of focal outer retinal damage overlying choroidal nevus that initially simulated solar retinopathy.
A 58-year-old man presented with visual distortion of 2 years duration in the right eye. Past ocular and medical history was noncontributory. He denied sun-gazing, laser pointer-gazing, and alkyl nitrate (popper) medications. Ocular examination revealed best-corrected visual acuity of 20/25 in each eye. He was not myopic in either eye. Funduscopically, the right eye showed a brown macular hue, suggestive of choroidal nevus, measuring approximately 5.0 mm × 5.0 mm in basal diameter with oval central RPE atrophy and drusen (Figure 1a). There was no visible orange pigment or SRF. The left eye showed mild macular drusen consistent with early age-related macular degeneration.
(a) Color photograph of the right eye shows a brown macular lesion with oval central retinal pigment epithelial (RPE) atrophy. (b) Subfoveal enhanced depth imaging optical coherence tomography reveals a slightly elevated choroidal lesion with gentle-sloping anterior border and deep shadowing. Overlying this lesion, there is outer retinal atrophy. (c) Infrared autofluorescence reveals central hypoautofluorescence consistent with loss of RPE. (d, e) Fluorescein angiography shows a predominantly hypofluorescent lesion with a ring of central hyperfluorescence in early (d) and late frames (e), compatible with window defect from RPE loss.
Imaging with enhanced depth imaging OCT (EDI-OCT) of the right eye revealed a slightly elevated solid choroidal lesion with gentle sloping anterior border, choroidal vascular compression, and deep shadowing, suggestive of choroidal nevus. Additional EDI-OCT demonstrated abrupt, focal outer retinal atrophy and trace cystoid retinal edema overlying the apex of the nevus (Figure 1b). Infrared autofluorescence (IRAF) revealed central hypoautofluorescence consistent with loss of RPE (Figure 1c). Fluorescein angiography (FA) showed a hypofluorescent lesion with a ring of central hyperfluorescence in early (Figure 1d) and late (Figure 1e) frames, compatible with window defect from RPE loss. The lesion was unmeasurable by B-mode ultrasonography.
The EDI-OCT findings were compatible with solar, laser, or popper maculopathy, but the history was denied. Based on the clinical, OCT, and IRAF findings, a diagnosis of choroidal nevus with abrupt overlying outer retinal damage was rendered. Conservative observation was advised. Anti-vascular endothelial growth factor treatment was not deemed to be of value in this situation because of the chronicity of the lesion and extent of outer retinal damage.
Choroidal hemangioma and choroidal nevus/small melanoma all can appear as gentle sloping, dome-shaped tumors on EDI-OCT.4 Choroidal hemangioma displays thickened choriocapillaris (CC) with no vascular compression, features that were not present in our case.4 The chronic RPE and retinal loss was not suggestive of choroidal melanoma. In this case, there was abrupt and deep outer retinal atrophy with loss of photoreceptors and ellipsoid layer up to the outer plexiform layer in a “cut out” configuration, reminiscent of solar, laser, or popper maculopathy. However, our patient denied all exposures. Solar retinopathy is usually unilateral except in those individuals sufficiently committed to keeping both eyes open while looking at the sun. The final diagnosis of choroidal nevus associated with severe outer retinal damage was rendered.5,6 Outer retinal damage overlying choroidal nevus to this extent, as demonstrated by EDI-OCT in this case, is unusual. The choroidal nevus, by its compressive effect, perhaps interfered in some way with CC nutrition and oxygen delivery to the RPE and outer retina. Our patient surprisingly had 20/25 visual acuity in the right eye, probably attributable to the remaining photoreceptors on the atrophic RPE.
In the literature, EDI-OCT has proven valuable in the assessment of choroidal nevus.4 The most common EDI-OCT features of choroidal nevus include choroidal findings such as CC compression overlying the nevus (94%) and partial (59%) or complete (35%) shadowing deep to the nevus, and RPE findings such as RPE atrophy (43%), loss (14%), and nodularity (8%). On EDI-OCT, the retinal findings overlying choroidal nevus include photoreceptor loss (43%) as well as irregularity in the ellipsoid layer (37%), external limiting membrane (18%), outer nuclear layer (8%), outer plexiform layer (8%), inner nuclear layer (6%), inner plexiform layer (0%), ganglion cell layer (0%), and nerve fiber layer (0%).7 The findings on EDI-OCT of RPE alterations, photoreceptor loss, and intraretinal cystoid edema are related to chronic retinal degeneration and suggest stable, chronic choroidal nevus. Conversely, the presence of SRF with photoreceptor preservation (“shaggy photoreceptors”) suggests a more active lesion with risk for growth into a melanoma.8 Based on these criteria, our case did not show risk for progression into a melanoma.
In summary, we present an interesting case of long-standing choroidal nevus with overlying outer retinal damage. Enhanced depth imaging optical coherence tomography is an important diagnostic tool for differentiation between choroidal nevus and other simulating conditions. Furthermore, EDI-OCT provides indispensable information concerning retinal and RPE alterations overlying choroidal tumors.
- Qui M, Shields CL. Choroidal nevus in the United States adult population: Racial disparities and associated factors in the National Health and Nutrition Examination Survey. Ophthalmology. 2015;122(10):2071–2083.
- Shields CL, Furuta M, Mashayekhi A, et al. Clinical spectrum of choroidal nevi based on age at presentation in 3422 consecutive eyes. Ophthalmology. 2008;115(3):546–552. doi:10.1016/j.ophtha.2007.07.009 [CrossRef]
- Shields CL, Furuta M, Mashayekhi A, et al. Visual acuity in 3422 consecutive eyes with choroidal nevus. Arch Ophthalmol. 2007;125(11):1501–1507. doi:10.1001/archopht.125.11.1501 [CrossRef]17998511
- Shields CL, Manalac J, Das C, Saktanasate J, Shields JA. Review of spectral domain enhanced depth imaging optical coherence tomography of tumors of the choroid. Indian J Ophthalmol. 2015;63(2):117–121. doi:10.4103/0301-4738.154377 [CrossRef]25827541
- Gunduz K, Adefusika JA, Link TP, Pulido JS. Uveal Tumors: Diagnostic Techniques: Angiography. In: Singh AD, Damato B, eds. Clinical Ophthalmic Oncology, Basic Principles and Diagnostic Techniques. 2nd ed. Heidelberg, Germany; Springer-Verlag; 2014:173–192. doi:10.1007/978-3-642-40489-4_18 [CrossRef]
- Vallabh NA, Sahni JN, Parkes CK, Czanner G, Heimann H, Damato B. Near-infrared reflectance and autofluorescence imaging characteristics of choroidal nevi. Eye (Lond). 2016;30(12):1593–1597. doi:10.1038/eye.2016.183 [CrossRef]
- Shah SU, Kaliki S, Shields CL, Ferenczy SR, Harmon SA, Shields JA. Enhanced depth imaging optical coherence tomography of choroidal nevus in 104 cases. Ophthalmology. 2012;119(5):1066–1072. doi:10.1016/j.ophtha.2011.11.001 [CrossRef]22297027
- Shields CL, Kaliki S, Rojanaporn D, Ferenczy SR, Shields JA. Enhanced depth imaging optical coherence tomography of small choroidal melanoma: Comparison with choroidal nevus. Arch Ophthalmol. 2012;130(7):850–856. doi:10.1001/archophthalmol.2012.1135 [CrossRef]22776921