PurposeThe aim of this study was to investigate disease onset and disease progression in patients with severe chronic central serous chorioretinopathy (cCSC).Patients and methodsThe medical records of 143 cCSC patients (199 eyes) were reviewed. All cases had visual complaints for >6 months and showed signs of a severe disease phenotype on optical coherence tomography (OCT) and fluorescein angiography (FA). Clinical presentation at onset was evaluated, together with disease progression on multimodal imaging and final treatment outcome.ResultsTwenty-eight cases (14%) had a documented history of an acute episode of CSC, whereas 145 cases (73%) showed pre-existing features of chronicity already at first presentation. The first clinical presentation could not be evaluated in 13% of cases. Best-corrected visual acuity (BCVA) was 70 ± 18 Early Treatment of Diabetic Retinopathy Study (ETDRS) letters at onset and 70 ± 22 ETDRS letters at final visit (p = 0.770). Among all studied cases, 173 eyes (87%) were treated, which resulted in complete resolution of subretinal fluid (SRF) in 76% of eyes at final visit. In eyes with fluorescein angiographic follow-up, the area of diffuse atrophic retinal pigment epithelium (RPE) abnormalities (diffuse atrophic RPE alterations [DARA]) had increased significantly in 43 eyes (68%) at final visit.ConclusionCSC encompasses a clinical spectrum that includes a range of severe phenotypes, in which retinal abnormalities tend to be progressive. Nevertheless, the long-term visual acuity may remain fairly stable with treatment. Few patients with severe chronic CSC have a history of acute CSC, which could indicate that there may be pathogenetic differences between these 2 CSC variants.

Purpose: To investigate genetic associations in white patients with acute central serous chorioretinopathy (aCSC) and to assess genetic differences between aCSC and chronic CSC (cCSC). Methods: A total of 135 aCSC patients, 272 cCSC patients, and 1,385 control individuals were included. Eight single nucleotide polymorphisms were genotyped for ARMS2 (rs10490924), CFH (rs800292, rs1061170, rs1065489, rs1329428, rs2284664, rs3753394), and NR3C2 (rs2070951). Also, C4B gene copy numbers were analyzed. Results: Three single nucleotide polymorphisms in the CFH gene were significantly associated with aCSC: rs800292 (P = 0.003, odds ratio = 1.53 [95% confidence interval = 1.15–2.03]), rs1061170 (P = 0.002, odds ratio = 0.64 [95% confidence interval = 0.48–0.86]), and rs1329428 (P = 5.87 × 10−6, odds ratio = 1.83 [95% confidence interval = 1.40–2.38]). A significant difference was found in the distribution of C4B gene copy numbers in aCSC patients compared with controls (P = 0.0042). No differences could be found among the selected variants between aCSC and cCSC patients. Conclusion: Three variants in the CFH gene and copy number variations in C4B were found to be significantly associated with the risk of aCSC development. Despite the differences in clinical presentation, acute and chronic CSC may share a similar genetic predisposition based on our present analysis. Other genetic and/or nongenetic risk factors may be more influential in the differentiation toward an acute or a chronic phenotype of CSC.

PurposeTo describe a spectrum of severe chronic central serous chorioretinopathy (cCSC) cases and their response to photodynamic therapy (PDT).Patients and methodsA total of 66 patients (81 eyes) with active severe cCSC were studied, and their response to PDT was compared with a control group consisting of 35 active cCSCs (37 eyes) that did not display characteristics of severity. Best-corrected visual acuity (BCVA) and complete resolution of subretinal fluid (SRF) were considered as main outcome measures.ResultsIn severe cCSC cases, we found cumulative areas of diffuse atrophic retinal pigment epithelium alterations in 48 eyes (59%), multiple “hot spots” of leakage in 36 eyes (44%), posterior cystoid retinal degeneration in 25 eyes (31%), and 13 eyes (16%) had a diffuse leakage on fluorescein angiography. After PDT treatment, BCVA increased in both groups, from 66 to 72 Early Treatment of Diabetic Retinopathy Study (ETDRS) letters in the case group (P<0.001), and from 78 to 82 ETDRS letters in the control group (P<0.001). SRF had resolved completely in 87% of severe cCSC cases and 95% of controls at final follow-up visit.ConclusionA spectrum of severe cCSC exists, and PDT seems to be an effective treatment in both severe cCSC and nonsevere cCSC in terms of resolution of SRF. Final BCVA shows a significant improvement in both groups after PDT treatment.

PurposeTo assess parameters on optical coherence tomography (OCT), and their correlation with best-corrected visual acuity (BCVA) in patients with non-resolving central serous chorioretinopathy (CSC).MethodsFor 25 non-resolving CSC patients treated with photodynamic therapy (PDT), the thickness of retinal layers was assessed on the foveal spectral-domain (SD) OCT scan. Evaluated OCT parameters included the central retinal thickness (CRT), defined as the internal limiting membrane (ILM) to ellipsoid zone (EZ) distance, and the second band thickness (SBT), defined as the EZ to hyperreflective subretinal accumulation distance. Integrity of the external limiting membrane (ELM) and the EZ bands was also determined. These parameters, along with BCVA and CRT measured automatically by SD-OCT device software were obtained before PDT, after PDT, and at final visit. After Bonferroni correction, a p-value <0.007 was considered statistically significant.ResultsTwenty-five patients could be included at last visit before PDT and first visit after PDT. At final visit, 24 patients could be included, since 1 patients was lost to follow-up. Mean CRT was 112 μm at last visit before PDT, 118 μm at first visit after PDT (p = 0.030), and 127 μm at final visit (p<0.001compared to baseline). Mean SBT was 74 μm, 26 μm (p<0.001 compared to baseline), and 21 μm (p<0.001 compared to baseline), respectively. Mean BCVA in Early Treatment of Diabetic Retinopathy Study letters was 79 at baseline, 85 at first visit after PDT (p = 0.005 compared to baseline), and 87 at final visit (p = 0.001 compared to baseline). BCVA had an estimated correlation of β = 0.103 (p = 0.114) with CRT, β = -0.051 (p = 0.014) with SBT, β = 0.615 (p = 0.600) with the integrity of the ELM, and β = 4.917 with the integrity of the EZ (p = 0.001).ConclusionsIn non-resolving CSC patients treated with half-dose PDT, the CRT increased at final visit in comparison to the last visit before PDT. The continuity of the EZ on SD-OCT was positively correlated with BCVA. We propose that the distance between ILM and EZ should be used as a reliable CRT measurement in non-resolving CSC patients treated with half-dose PDT.