Clinical characteristics and long-term visual outcome of severe phenotypes of chronic central serous chorioretinopathy

Figures & data

Figure 1 Illustration of the 4 criteria of severity on FA and OCT.

Notes: The 4 criteria are as follows: 1) cumulative areas of >5 DD of diffuse atrophic retinal pigment epithelium alterations as visualized on mid-phase FA (A). 2) Multiple (at least 2) “hot spots” of leakage separated by at least 1 DD of non-hyperfluorescent healthy-appearing retina on mid-phase FA (B). 3) An area of diffuse fluorescein leakage with a surface of >1 DD on mid-phase FA, without an evident leaking focus (C). 4) Presence of posterior cystoid retinal degeneration on OCT (D and E).
Abbreviations: DD, optic disc diameters; FA, fluorescein angiography; OCT, optical coherence tomography.

Table 1 Patient demographics

Characteristics	Cases
Patients (eyes)	143 (199)
Male gender, n (%)	121 (87)
Caucasian ethnicity, n (%)	119 (83)
Mean age at diagnosis of CSC (range)	46 (26–78)
Mean available follow-upa in years (range)	7.5 (0.3–30.6)
Patients with recentb use of steroids, n (%)	25c (18)
BCVA at first presentation (ETDRS letters)	70 ± 18

Notes:

a The mean time from CSC diagnosis to final visit.

b Within 3 months prior to diagnosis.

c One patient was diagnosed with Cushing syndrome. Data presented as mean ± SD unless otherwise indicated.

Abbreviations: BCVA, best-corrected visual acuity; CSC, central serous chorioretinopathy; ETDRS, Early Treatment of Diabetic Retinopathy Study.

Figure 2 Clinical features on multimodal imaging of the right eye of a 44-year-old male patient with severe chronic central serous chorioretinopathy and a bullous inferior retinal detachment.

Notes: Color fundus photograph showed extensive retinal abnormalities in the macula, with multifocal areas of whitish fibrinous subretinal material (A). The white arrow shows the scanning plane which is depicted on the SD-OCT scans (D and E). FA imaging (B) revealed multiple foci of leakage and widespread RPE alterations. The areas of hyperfluorescence on mid-phase ICGA (C) depicted diffuse choroidal hyperpermeability which is larger as compared to the abnormalities on FA. An SD-OCT scan (D) at first presentation and prior to treatment revealed a subretinal SRF accumulation, a subfoveal RPE detachment, and PCRD in the outer nuclear layer of the retina. At ~4 months after half-dose PDT using a large spot size of 1,200 µm centered on the hyperfluorescent zones on ICGA, both SRF and intraretinal fluid on OCT had resolved completely (E). The choroid before treatment was markedly thickened (D). This choroidal thickness reduced after PDT but showed large cavities in the deep choroidal (Haller) layers, with limited or no RPE damage and spontaneous resolution of SRF layers (E).
Abbreviations: FA, fluorescein angiography; ICGA, indocyanine green angiography; PCRD, posterior cystoid retinal degeneration; PDT, photodynamic therapy; RPE, retinal pigment epithelium; SD-OCT, spectral-domain optical coherence tomography; SRF, subretinal fluid.

Table 2 Distribution of each criterion of severity among the severe cases of chronic central serous chorioretinopathy

Criteria	Frequency and specifications
Eyes with DARA, n (%)	145 (73)
Mean DARA surface (DD)	8.6 ± 4
Location of DARA, n (%)	75 (52) covering the fovea
	47 (32) within 1 DD of the foveaa
	23 (16) outside 1 DD of the fovea
Eyes with PCRD, n (%)	69 (35)
Eyes with multiple hot spots, n (%)	115 (58)
Mean number of hot spots (range)	3 (2–7)
Location of hot spots, n (%)	88 (75) inside the temporal vascular arcade
	3 (3) outside the temporal vascular arcade
	26 (22) both inside and outside
Eyes with diffuse leakage, n (%)	60 (30)

Notes:

a DARA surface overlaps with the area as wide as 1 DD around the fovea, but does not cover the foveal depression. Data presented as mean ± SD unless otherwise indicated.

Abbreviations: DARA, diffuse atrophic retinal pigment epithelium (RPE) alterations; DD, optic disc diameters; PCRD, posterior cystoid retinal degeneration.

Table 3 Characteristics at first presentation and disease progression in severe cases of chronic central serous chorioretinopathy

Characteristics	Severe manifestation of CSC	Final follow-up visit	p-value
BCVA (ETDRS letters)	68 ± 19	70 ± 22	0.061
Eyes with SRF, n (%)	173 (87)	47 (24)	<0.001
Eyes with PCRD, n (%)	69 (35)	35 (18)	<0.001
CFTa (µm)	105 ± 31	106 ± 30	0.930
DARA surfaceb (DD)	6.3 ± 3	9.0 ± 5	<0.001
Mean number of treatmentsc (range)	n/a	2.5 (1–20)	n/a
Mean number of recurrencesd (range)	n/a	1.2 (0–9)	n/a

Notes:

a CFT could be measured manually in 63 eyes (32%) with a spectral-domain optical coherence tomography.

b In 68 cases (34%), a follow-up FA (at least 1 year later) was available to be compared with baseline FA.

c Twenty-six cases (13%) were never treated and were not included in this analysis.

d Twenty-one cases (11%) never showed a complete resolution of SRF, and were not included in this analysis. Data presented as mean ± SD unless otherwise indicated. Bold font indicates data were considered statistically significant.

Abbreviations: BCVA, best-corrected visual acuity; CSC, central serous chorioretinopathy; CFT, central foveal thickness; DARA, diffuse atrophic retinal pigment epithelium (RPE) alterations; DD, optic disc diameters; ETDRS, Early Treatment of Diabetic Retinopathy Study; FA, fluorescein angiography; n/a, not applicable; PCRD, posterior cystoid retinal degeneration; SRF, subretinal fluid.

Table 4 Pearson correlation coefficients between patient characteristics and final visual outcome

Characteristics	Correlation with BCVA outcome
	r	Evaluated cases	Multivariate p-value
Male gender	0.161	197	0.024
Age at disease onset	−0.086	197	0.225
Caucasian ethnicity	−0.045	197	0.526
Recent steroid use	0.042	197	0.551
Disease duration	−0.105	197	0.143
Baseline BCVA	0.543	197	<0.001
Presence of PCRD	−0.297	195	<0.001
Presence of multifocal hot spots	0.023	195	0.023
Mean number of hot spots	−0.036	152	0.656
Location of multifocal hot spots	−0.036	154	0.657
Presence of diffuse leakage	−0.163	195	0.023
Presence of DARA	−0.265	197	<0.001
Surface of DARA	−0.343	197	<0.001
Location of DARA	0.400	197	<0.001
DARA involving the fovea	−0.386	197	<0.001
DARA within 1 DD of the fovea, but sparing the fovea	0.128	197	0.072
DARA more than 1 DD outside the fovea	0.309	197	<0.001
Mean number of recurrences	−0.063	176	0.409

Notes: r, correlation coefficient. Bold font indicates data were considered statistically significant.

Abbreviations: BCVA, best-corrected visual acuity; DARA, diffuse atrophic retinal pigment epithelium (RPE) alterations; DD, optic disc diameter; PCRD, posterior cystoid retinal degeneration.

Table 5 Frequency of all applied treatments in eyes with severe chronic central serous chorioretinopathy

Treatment modality	Frequency and specifications
Mean number of treatments per case (range)	2.4 (1–20)
Number of untreated cases, n (%)	25 (14)
Total number of PDTs, n (%)	246 (60)
Successfula PDT, n (%)	153 (62)
PDT modality, n (%)	6 (2) full settings
	160 (66) half-dose
	39 (16) half-time
	6 (2) half-fluency
	35 (14) unknown
PDT spot size in µm (range)	4,457 (1,000–8,800)b
Total number of SMLs, n (%)	81 (20)
Successfula SMLs, n (%)	7 (9)
Total number of conventional thermal laser treatments	84 (20)
Successfula conventional thermal laser treatments, n (%)	54 (64)

Notes:

a Successful treatment was defined by a complete resolution of subretinal fluid and/or disappearance of posterior cystoid retinal degeneration.

b An average size is reported in case multiple PDT spots were used.

Abbreviations: PDT, photodynamic therapy; SML, subthreshold micropulse diode laser.