Clinical appearance of pigmented extrafoveolar (A) vs pigmented subfoveolar (B) nevus in eyes without overlying retinal or retinal pigment epithelial changes (the visual acuity was 20/20 in both eyes).
Clinical appearance of subfoveolar nevus with overlying retinal and retinal pigment epithelial (RPE) changes and good long-term visual acuity. Subfoveolar choroidal nevus with overlying RPE detachment and visual acuity of 20/20 at 18 months' follow-up (A). Optical coherence tomographic (OCT) scan of the nevus in part A showing the foveola draped on the nasal margin of the RPE detachment (B). Subfoveolar choroidal nevus with overlying orange pigment and a visual acuity of 20/20 at 22 months' follow-up (C). The OCT scan of the nevus in part C showing the slight elevation of the foveola with optically dense material on the posterior retinal surface correlating with the orange pigment (D).
Clinical appearance of subfoveolar nevus with overlying retinal and retinal pigment epithelial (RPE) changes and reduced long-term visual acuity. Subfoveolar choroidal nevus with overlying fibrous metaplasia of the RPE and a visual acuity of 20/40 at 18 months' follow-up (A). Optical coherence tomographic (OCT) scan of the nevus in part A showing thickening of the RPE layer, particularly under the foveola, slight subretinal fluid, and diffuse optical density in the photoreceptor layer overlying the nevus, suggesting photoreceptor disruption (B). Subfoveolar choroidal nevus with overlying subtle orange pigment and a visual acuity of 20/25 at 51 months (C). The OCT scan of the nevus in part C showing overlying subretinal fluid and debris on the posterior retinal surface consistent with orange pigment (D). Circumpapillary subfoveolar choroidal nevus with subtle overlying orange pigment, mild fibrous metaplasia of the RPE, and a visual acuity of counting fingers at 21 months' follow-up (E). The OCT scan of the nevus in part E showing extensive confluent cystoid macular edema (F). Subfoveolar choroidal nevus with overlying fibrous metaplasia of the RPE and a visual acuity of 20/100 at 52 months' follow-up (G). The OCT of the nevus in part G showing dramatic cystoid macular edema (H).
Shields CL, Furuta M, Mashayekhi A, Berman EL, Zahler JD, Hoberman DM, Dinh DH, Shields JA. Visual Acuity in 3422 Consecutive Eyes With Choroidal Nevus. Arch Ophthalmol. 2007;125(11):1501-1507. doi:10.1001/archopht.125.11.1501
To evaluate visual acuity in eyes with choroidal nevus.
This was an observational case series. Of 3422 consecutive eyes with choroidal nevus, vision loss at 15 years occurred in 2% of eyes with extrafoveolar nevus and in 26% of eyes with subfoveolar nevus, particularly those with overlying retinal pigment epithelial detachment and foveal edema. A retrospective medical record review was conducted, with evaluation of visual acuity at presentation and at final examination. The main outcome measure was visual acuity.
The median visual acuity at presentation was 20/20 for eyes with either extrafoveolar or subfoveolar choroidal nevus. Using Kaplan-Meier estimates, vision loss of 3 or more logarithm of the minimum angle of resolution (logMAR) lines at 5, 10, and 15 years occurred in less than 1%, 1%, and 2% of eyes with extrafoveolar nevus compared with 15%, 20%, and 26% of eyes with subfoveolar choroidal nevus, respectively. By multivariate analysis, factors predictive of visual loss of 3 or more logMAR lines included subfoveolar nevus location (relative risk [RR], 15.52), juxtapapillary nevus location (RR, 4.52), initial visual acuity of 20/50 or worse (RR, 15.40), overlying retinal pigment epithelial detachment (RR, 22.16), and foveal edema (RR, 9.02). Factors predictive of poor final visual acuity of 20/200 or worse included subfoveolar nevus location (RR, 11.32), overlying orange pigment (RR, 3.68), overlying retinal pigment epithelial detachment (RR, 12.80), and foveal edema (RR, 18.72).
Mild vision loss over many years should be anticipated in patients with subfoveolar choroidal nevus, particularly those with overlying retinal pigment epithelial detachment, orange pigment, and foveal edema.
Choroidal nevus is the most common clinically detected intraocular tumor.1,2 In the Blue Mountains Eye Study,3 choroidal nevi were found in 7% of the white population. This benign tumor manifests as a pigmented or nonpigmented mass deep to the retina, often with overlying drusen and retinal pigment epithelial (RPE) alterations.1- 7 Choroidal nevus can produce central vision loss and peripheral visual field loss.4,8- 11 Rarely, choroidal nevus can evolve into malignant melanoma.7,12- 18
Visual field defects were documented in 38% of 42 eyes with choroidal nevus evaluated by Tamler and Maumenee4 and in 85% of 21 eyes analyzed by Flindall and Drance10 using static and kinetic techniques. In 1971, Naumann and associates6 found central visual acuity loss in 13 of 124 eyes (10%) with choroidal nevus. Gonder and coworkers8 later described 206 patients with choroidal nevi posterior to the equator of the eye and found 22 (11%) with visual acuity loss. The vision loss was because of subfoveal fluid (50%), presumed photoreceptor degeneration (42%), and choroidal neovascularization (8%).8 In 2005, Shields and associates11 evaluated optical coherence tomography (OCT) of the retina overlying 120 consecutive patients with choroidal nevus to better ascertain the reasons for visual loss and found overlying retinal edema (15%), photoreceptor attenuation (51%), retinal thinning (22%), subretinal fluid (26%), and RPE detachment (12%). In this report, we analyze a large cohort of 3422 eyes with stable choroidal nevus to ascertain initial and final visual acuity, loss of visual acuity over time, and factors related to visual acuity outcomes.
A retrospective medical record review was performed on all patients with the clinical diagnosis of choroidal nevus evaluated at the Ocular Oncology Service at Wills Eye Institute between April 1, 1970, and June 1, 2006. Institutional review board approval was obtained for this retrospective study. Patients with evidence of tumor transformation into melanoma were not included in the analysis. Clinical data were collected at initial examination regarding patient age, race, sex, medical history (dysplastic nevus syndrome; cutaneous, choroidal, or conjunctival melanoma; or neurofibromatosis), ocular melanocytosis, symptoms, and best-corrected visual acuity by Snellen charts. Vision data were evaluated using logarithm of the minimum angle of resolution (logMAR) conversion. Data regarding specific features of the tumor were collected from large detailed fundus drawings that were made of each patient at first examination and from fundus photographs. These data included tumor epicenter quadrantic location (inferior, temporal, superior, nasal, or macular), tumor epicenter anteroposterior location (macular, macular to equator, or equator to ora serrata), distance of the tumor margin to the optic disc margin and foveola (in millimeters), largest tumor basal dimension and thickness (in millimeters), tumor color (pigmented, mixed, or nonpigmented), and presence of amelanotic halo around the nevus (halo nevus). Other related data included subretinal fluid, orange pigment, drusen, RPE alterations (hyperplasia, detachment, fibrosis, and atrophy), and choroidal neovascular membrane. The status of the foveola (involvement with underlying choroidal nevus, subretinal fluid, and retinal edema) was recorded. The final best-corrected visual acuity at date last seen was recorded.
A series of univariate Cox proportional hazards regressions assessed the degree of relationship of all of the variables previously listed to 3 outcomes, including the initial visual acuity, final visual acuity, and visual acuity loss of 3 or more logMAR lines. All of the variables were analyzed as discrete variables except for patient age at presentation, tumor basal dimension, tumor thickness, and distance of the tumor to the optic disc margin and foveola, which were evaluated as continuous variables. Subsequent multivariate models included variables that were significant on a univariate level (P < .05) to identify the combination of factors best related to the 3 outcomes. Kaplan-Meier survival estimates were calculated on time to loss of 3 or more lines of logMAR visual acuity.
There were 3422 eyes of 3187 patients with choroidal nevus. The mean patient age at presentation was 60 years (median, 62 years; range, 4-97 years). The patient and tumor features are listed in Table 1. The tumor was subfoveolar in 205 (6.0%) eyes and extrafoveolar in 3217 (94.0%) eyes. The mean tumor base and thickness was 5.7 and 1.7 mm for the subfoveolar nevi, respectively, and 5.1 and 1.6 mm for the extrafoveolar nevi, respectively. Eyes with subfoveolar nevus displayed subretinal fluid in 60 cases and foveal retinal edema in 14 cases, compared with 285 cases and 11 cases, respectively, in eyes with extrafoveolar nevus (Table 1).
The mean initial logMAR visual acuity for extrafoveolar and subfoveolar choroidal nevi was 0 (Snellen equivalent, 20/20). The initial median ± SD logMAR visual acuity for extrafoveolar nevi was 0.09 ± 0.22 (range, 0-3.00); and for subfoveolar nevi, 0.20 ± 0.35 (range, 0-2.00). The final median ± SD logMAR visual acuity for extrafoveolar nevi was 0.14 ± 0.31 (range, 0-4.00); and for subfoveolar nevi, 0.34 ± 0.53 (range, 0-3.00). Of the 2334 patients with stable choroidal nevi who returned for a follow-up examination, the mean final logMAR visual acuity for extrafoveolar choroidal nevi was 0.10 (Snellen equivalent, 20/25); and for subfoveolar choroidal nevi, 0.18 (Snellen equivalent, 20/30) (Figure 1). The mean follow-up was 5 years (range, 3 months to 36 years). Kaplan-Meier estimates of 3 lines of logMAR visual acuity loss at 2, 5, 10, and 15 years are given in Table 2 and in Figure 2 and Figure 3.
The multivariate analyses for initial and final visual acuity of 20/50 to 20/100 and 20/200 or worse in the entire group of 3422 eyes is listed in Table 3. The most important factors for poor final visual acuity of 20/200 or worse included macular location of the nevus and overlying orange pigment, RPE detachment, and foveal edema (Table 3). Factors predictive of loss of 3 or more logMAR lines included reduced initial visual acuity of 20/50 (logMAR, 0.4) or worse, subfoveolar location, juxtapapillary location, nevus thickness greater than 2 mm, related RPE detachment, and foveal edema (Table 4). Factors predictive of intermediate (20/50-20/100 [logMAR, 0.4-0.7]) or poor (20/200 or worse [logMAR, 1.0 or worse]) visual acuity at initial and final examination in eyes with subfoveolar choroidal nevus using multivariate analysis are listed in Table 5. In the 127 eyes with subfoveolar choroidal nevus for which the patient returned for a follow-up examination, factors predictive of loss of 3 or more logMAR lines included Hispanic race, intermediate (20/50 or worse) initial visual acuity, and overlying orange pigment (Table 6).
Choroidal nevus can lead to reduced central and peripheral visual acuity, depending on its location. Tamler and Maumenee4 demonstrated visual field defects in 38% of 42 choroidal nevi, but remarked that it was not clear at that time how a choroidal nevus could affect the retinal function if the retina was not involved with tumor. Naumann and associates9 later described a patient with paracentral scotoma from a choroidal nevus, found on histopathological examination to have loss of the outer retinal layers with complete loss of the rods and cones overlying the tumor. These findings sufficiently explained the symptomatic scotoma.
Optical coherence tomography has been useful in further delineating the extent of retinal damage overlying choroidal nevus. Shields and associates11 used OCT to analyze the retina overlying 120 consecutive choroidal nevi and found overlying retinal edema in 15%, photoreceptor thinning or complete absence in 51%, and general retinal thinning in 22%.11 These in vivo OCT findings corroborated previous histopathological findings.5,6,9
In the current analysis, we specifically analyzed subfoveolar vs extrafoveolar choroidal nevi to appreciate the comparative effects of tumor location on central visual acuity. Eyes with subfoveolar nevi composed 205 of the 3422 eyes (6.0%). A previous study8 found subfoveal location of choroidal nevus in 28 of 375 patients (7.5%). At initial examination, eyes with subfoveolar choroidal nevus displayed a mean visual acuity of 20/20, similar to those with an extrafoveolar nevus. However, on final examination, eyes with a subfoveolar choroidal nevus had a slightly reduced mean visual acuity of 20/30 compared with 20/25 in those with an extrafoveolar nevus. The difference between these 2 groups was more remarkable when assessing loss of visual acuity over time, because 26% of the subfoveolar nevus group showed loss of 3 logMAR lines of visual acuity by 15 years, whereas only 2% of the extrafoveolar group manifested a similar loss. In fact, eyes with a subfoveolar nevus had a 16 times higher RR for vision loss compared with eyes with an extrafoveolar nevus (Table 4).
In the entire group of 2334 eyes with choroidal nevus for which the patient returned for follow-up, the most important factors for intermediate (20/50-20/100) or poor (20/200 or worse) final visual acuity included subfoveolar nevus location and retinal or RPE changes overlying the nevus, such as foveal edema, subretinal fluid, orange pigment, and RPE detachment (Table 5). Similar to the previous results, subfoveolar location imparted a 21 times higher RR for intermediate final visual acuity and an 11 times higher RR for poor final visual acuity compared with extrafoveolar nevus (Table 3). Some of the related retinal and RPE findings, such as subretinal fluid and orange pigment, that were associated with the visual outcome have also been shown in previous reports13,14,16,18 to predict tumor growth into melanoma. In this analysis, only stable choroidal nevi without growth were included.
Based on published OCT data, retinal and RPE alterations are fairly common overlying choroidal nevi and these features could translate to visual field loss if the nevus is extrafoveal and central vision loss if the nevus is subfoveal. In the current analysis of all 2334 eyes with choroidal nevus, the most important factor for poor final visual acuity was foveal edema, which imparted a 19 times RR (Table 3), and the most important factor for visual acuity loss was overlying RPE detachment, which imparted a 22 times RR (Table 4). Most of the information on the foveal features in our study, which extends over 4 decades, was gathered by clinical examination; and only a few patients underwent OCT imaging of the fovea. Related clinical features, such as RPE detachment, can be difficult to visualize clinically. In an analysis of OCT findings of 120 patients with choroidal nevi, RPE detachment overlying the nevus was found on OCT in 12% of cases, whereas it was visualized clinically in only 2%.11 Optical coherence tomography could be beneficial in estimating risks for poor visual outcome.
This analysis focused on visual results in a large cohort of 3422 consecutive patients with choroidal nevus. The results, however, could be biased toward larger or thicker nevi because many patients were referred to this ocular oncology center with the diagnosis of possible small choroidal melanoma vs choroidal nevus. In this cohort, the mean nevus diameter was 5.1 mm and the mean nevus thickness was 1.6 mm. In comparison, Sumich and associates3 analyzed 264 choroidal nevi and found a mean nevus diameter of 1.25 mm (thickness not recorded).
In summary, central visual acuity can be diminished in eyes with choroidal nevus, particularly if the nevus is subfoveolar and there are related retinal and RPE alterations affecting the foveola. Patients with subfoveolar choroidal nevi should be forewarned that visual acuity could decrease slowly over time.
Correspondence: Carol L. Shields, MD, Ocular Oncology Service, Wills Eye Institute, 840 Walnut St, Ste 1440, Philadelphia, PA 19107 (firstname.lastname@example.org).
Submitted for Publication: March 1, 2007; final revision received April 17, 2007; accepted April 19, 2007.
Author Contributions: Dr C. L. Shields had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.
Financial Disclosure: None reported.
Funding/Support: This study was supported by the Retina Research Foundation, Charles L. Schepens Lecture of the Retina Society, Capetown, South Africa (Dr C. L. Shields); Mellon Charitable Giving from the Martha W. Rogers Charitable Trust, Philadelphia, Pennsylvania (Dr C. L. Shields); the LuEsther Mertz Retina Research Foundation, New York, New York (Dr C. L. Shields); a donation from Michael, Bruce, and Ellen Ratner, New York (Drs C. L. Shields and J. A. Shields); the Eye Tumor Research Foundation, Philadelphia (Drs C. L. Shields and J. A. Shields); and the Paul Kayser International Award of Merit in Retina Research, Houston, Texas (Dr J. A. Shields).
Previous Presentation: This study was presented at the International Congress of Ocular Oncology; June 29, 2007; Siena, Italy.
Additional Contributions: Rishita Nutheti, International Centre for Advancement of Rural Eye Care, L. V. Prasad Institute, Hyderabad, India, performed the statistical analysis.