Purpose
To describe a series of patients exhibiting annular retinal pigment epithelial (RPE) lesions in the context of chronic central serous chorioretinopathy.
Design
Retrospective comparative case series.
Methods
Consecutive patients with chronic central serous chorioretinopathy were identified from the clinical practices of 3 retina specialists. A subset of patients exhibiting annular RPE lesions on fundus autofluorescence was included for chart review and examination of multimodal imaging (study group). Patients with alternative etiologies for neurosensory detachment or pigment epitheliopathy were excluded. A second consecutive cohort of patients, with acute central serous chorioretinopathy, was also examined for the presence of annular lesions (comparative group).
Results
Sixty-seven patients with chronic central serous chorioretinopathy were identified. Fourteen eyes of 12 patients exhibited annular lesions (study eyes). Mean visual acuity of study eyes was 20/27 (logMAR 0.13, SD 0.11). Annular lesions were composed of hyperautofluorescent stellate lesions arranged in an open or closed ring with intervening foci of punctate hypoautofluorescence. Optical coherence tomography showed RPE hyperplasia at the perimeters of annular lesions with loss of ellipsoid reflectivity and preserved RPE at the lesion center. Annular lesions were confined to the posterior poles and appeared to have developed at the margins of chronic neurosensory detachment. Forty-three eyes of 30 patients with acute central serous chorioretinopathy comprised the comparative group and none of these eyes exhibited annular lesions.
Conclusions
Annular lesions occur in up to a fifth of patients with chronic central serous chorioretinopathy but carry a relatively good visual prognosis. Curvilinear RPE figures and demarcation lines are seen in various retinal conditions but the characteristics of annular lesions described here suggest that they are specific to chronic central serous chorioretinopathy.
Central serous chorioretinopathy gives rise to neurosensory detachment at the posterior pole in otherwise healthy adults. Chronic disease may leave a legacy of various retinal pigment epithelial (RPE) features including nonspecific changes, granular atrophy, descending tracks, and macular atrophy, most of which are best imaged with fundus autofluorescence.
The purpose of this paper is to describe a series of patients exhibiting a form of retinal pigment epitheliopathy with an annular configuration which, at its inferior border, is reminiscent of a catenary form and which appears to be specific to chronic central serous chorioretinopathy.
Methods
This study was approved by the Western Institutional Review Board (Olympia, Washington, USA). It complies with the Health Insurance Portability and Accountability Act of 1996 and follows the tenets of the Declaration of Helsinki.
Clinical charts and multimodal imaging were reviewed for all patients diagnosed with chronic central serous chorioretinopathy by 3 retina specialists (L.A.Y., K.B.F., and J.M.K.), in a tertiary referral setting at the private offices of the Vitreous Retina Macula Consultants of New York, between January 1, 1977 and March 31, 2014. Chronic central serous chorioretinopathy was diagnosed on the basis of neurosensory detachment due to leakage at the level of the RPE at the first visit, and if documentation of persistent subretinal fluid spanned 6 months or more. From among these patients, a subset were included for this study if they manifested, on fundus autofluorescence, RPE changes in a curvilinear arrangement resembling a demarcation line and forming a closed, or almost closed, circle (“annular” lesions). Previous color and red-free photographs and near-infrared reflectance images of these patients were also examined for any features that might be related to annular lesions.
A second cohort of patients was studied as a comparative group, consisting of consecutive patients with acute central serous chorioretinopathy presenting between June 2013 and June 2015. Acute disease was diagnosed if only a single episode of central serous chorioretinopathy had been recorded, with complete resolution of subretinal fluid on optical coherence tomography (OCT) within 6 months of onset.
Patients with any history of alternative etiologies potentially responsible for subretinal fluid or retinal pigment epitheliopathy, such as inflammation, neovascular age-related macular degeneration, rhegmatogenous retinal detachment, angioid streaks, pathologic myopia, tilted disc, optic disc pit, uveal effusion syndrome, or nanophthalmos, were excluded.
Mean visual acuities were converted to the logarithm of minimal angle of resolution (logMAR) for statistical comparisons. All patients had undergone color photography, fundus autofluorescence imaging (TRC-50IX; Topcon Corporation, Tokyo, Japan), time-domain OCT (Stratus; Carl Zeiss Meditec Inc, Dublin, California, USA), and/or spectral-domain OCT (3D OCT-2000; Topcon Corporation; Spectralis HRT+OCT; Heldelberg Engineering, Heidelberg, Germany) during the course of their disease. Most had also undergone fluorescein and/or indocyanine green angiography.
Annular lesions encroaching within 200 μm of the foveal center were regarded as fovea-involving and lesions of which no part encroached within 200 μm were regarded as extrafoveal. Choroidal thickness was measured using the caliper tools of the Heidelberg viewing software, on enhanced depth imaging spectral-domain OCT scans.
A search was performed of textbooks, retinal atlases, and MedLine ( PubMed.gov ) to examine the literature on the imaging and differential diagnosis of RPE demarcation lines and other features with similar morphologies. Search terms included combinations of the following: retinal pigment epithelium; demarcation; stellate; radial; tide mark; central serous; pachychoroid; dystrophy.
Results
A total of 67 patients met the diagnostic criteria for chronic central serous chorioretinopathy in at least one eye. Dates of first presentation to our practice ranged from 1977 to 2014 and dates of final follow-up ranged from 2008 to 2014. Of the 67 patients with chronic central serous chorioretinopathy, 14 eyes of 12 patients exhibited annular lesions (study eyes, see Table ). The mean age of this cohort was 62 (standard deviation [SD]: 10) years and 6 of the 12 patients were male. Mean visual acuity for study eyes was 20/27 (logMAR 0.13, SD 0.11) and mean choroidal thickness was 436 μm (SD 99 μm).
| Patient # | Age | Sex | Eye | Annular Lesion | SRF | Ellipsoid band | VA | SFCT (μm) |
|---|---|---|---|---|---|---|---|---|
| 1 | 57 | M | OD | Foveal | No | Lost volume | 20/25 | 443 |
| 2 | 56 | M | OS | Foveal | Yes | Lost volume | 20/40 | 302 |
| 3 | 64 | F | OD | Foveal | No | Normal | 20/25 | 334 |
| OS | Foveal | No | Normal | 20/25 | 348 | |||
| 4 | 43 | M | OD | Foveal | No | Lost volume | 20/25 | 516 |
| OS | Extrafoveal | No | Lost volume | 20/20 | 510 | |||
| 5 | 83 | F | OS | Extrafoveal | (No OCT of lesion) | 20/20 | 444 | |
| 6 | 60 | M | OD | Extrafoveal | No | Lost volume | 20/30 | 608 |
| 7 | 61 | F | OS | Extrafoveal | No | Disrupted reflectivity | 20/25 | 511 |
| 8 | 52 | M | OD | Foveal | No | Normal | 20/30 | 412 |
| 9 | 67 | F | OS | Extrafoveal | Yes | Normal | 20/40 | 330 |
| 10 | 56 | F | OD | Foveal | Yes | Disrupted reflectivity | 20/20 | 442 |
| 11 | 70 | F | OD | Foveal | No | Lost volume | 20/25 | 310 |
| 12 | 73 | M | OS | Extrafoveal | No | Normal | 20/50 | 599 |
A total of 43 eyes of 30 patients met the diagnostic criteria for acute central serous chorioretinopathy (control eyes). The mean age of this cohort was 53 years and 17 of the 30 patients were male.
Figures 1 and 2 illustrate the multimodal imaging characterictics of annular lesions in 2 patients. Annular lesions were visualized best by fundus autofluorescence, which showed alternating hyper- and hypoautofluorescence along the perimeter of each lesion. The perimeter itself was composed of multiple small stellate hyperautofluorescent figures with intervening foci of hypoautofluorescence due to multifocal focal RPE atrophy as seen on OCT. Corresponding masking and transmission defects were seen on fluorescein and indocyanine green angiography. The stellate lesions themselves were composed of a central hyperautofluorescent core, with corresponding RPE thickening on cross-sectional OCT, and a number of radial hyperautofluorescent spokes along which the degree of autofluorescence decreased gradually into the background with increasing distance from the center of the stellate lesion. In 7 of the 14 eyes the superior border of the annular lesion was contiguous with granular autofluorescence changes typical of central serous chorioretinopathy ( Figure 2 , Top row, second image).
Cross-sectional OCT of the annular perimeter showed focal RPE thickening ( Figures 1 and 2 ). Within the annular lesions in 8 eyes, the RPE appeared to be preserved. However, 2 eyes showed reduced or disrupted reflectivity of the ellipsoid band and 6 eyes showed apparent loss of tissue volume in the ellipsoid band, suggesting that the relative hyperautofluorescence of the annular centers was attributable to an unmasking of RPE autofluorescence. Subretinal fluid was seen within the annular lesions in 3 eyes.
Figure 3 exhibits the variations in morphology and location of annular lesions, which, in all cases, were confined to the posterior pole. Peripheral and/or ultra-wide-field autofluorescence did not reveal annular lesions in the peripheral retina of any of the included patients. Annular lesions involved the fovea in 9 eyes and were extrafoveal in 5 eyes.
