Central serous chorioretinopathy: updates in the pathogenesis, diagnosis and therapeutic strategies

Abstract

Central serous chorioretinopathy (CSCR), first described by Albrecht von Graefe in 1866, is characterized by focal serous detachment of the neural retina and/or retinal pigment epithelium (RPE) in the posterior pole. CSCR is the first ever described pachychoroid disease. Most recently, hypothetical venous overload choroidopathy is also proposed due to its distinguished morphological and pathological characteristics, including choroidal thickening, choriocapillaris hyperpermeability, remodelling, and intervortex venous anastomoses. Identification of genetic variants is necessary to comprehend the pathophysiology of CSCR. The novel multimodality imaging platforms, including the ultra-widefield imaging system, flavoprotein fluorescence, fluorescence lifetime imaging ophthalmoscopy, and multispectral imaging system, have been used for diagnosing and managing CSCR. Half-dose photodynamic therapy (PDT) remains the mainstay of clinical practice, with about 95% of patients with chronic CSCR improving to visual acuity (VA) of 20/30 or better. The use of oral eplerenone for routine clinical care remains controversial, and long-term randomized clinical trials are warranted to investigate its efficacy in acute and chronic CSCR. While CSCR has generally been recognized as a self-limiting disease with good prognosis, the underlying pathogenesis is still not fully understood, and treatments are often not fully effective. With new evidence emerging about pachydrusen being a disease precursor in both CSCR and polypoidal choroidal vasculopathy (PCV), it would be interesting to investigate whether CSCR can be a precursor to PCV. In this review, we highlighted the currently available evidence on the pathogenesis, diagnosis, multimodality imaging features, and management strategies, including recent findings related to CSCR.

Keywords: Central serous chorioretinopathy; Diagnosis; Epidemiology; Multimodality imaging; Pathogenesis; Treatment.

Fig. 1

A 54-year-old female complained of blurred vision and metamorphopsia for two weeks. A colour fundus photo of the patient with central serous chorioretinopathy (CSCR) reveals several yellow pinpoint deposits (white arrows) on the temporal side of the foveal with subretinal shallow detachment in the macular (a, right eye). The fundus of the left eye is normal (b)

Fig. 2

Clinical characteristics of central serous chorioretinopathy detected by fundus autofluorescence (FAF) and fundus fluorescence angiography (FFA). FAF shows pinpoint hypercyanescence in the macular area (a). FFA shows hyperfluorescent leakage points (orange arrows) at the lower part of the fovea in the early phase, with a progressive increase in intensity and size over time (b–e). Spot-like transparent fluorescence is visible at the posterior pole in the left eye (f–i). The hyperfluorescent leakage points (orange arrows) at the low part of the macular were also detected in the early phase in the left eye (g, h)

Fig. 3

Clinical characteristics of central serous chorioretinopathy detected by indocyanine green angiography (ICGA). ICGA showing massive choroidal dye filling delay (a) and hyperfluorescent lesions in correspondence with the FFA findings (red arrows) in the left eye. Abnormal dilation of choroidal vessels and related choroidal hyperpermeability is found in the posterior pole (orange arrows) in the right eye (a–d). Dilated choroidal vessels are also visible at the posterior pole (red arrows) in the left eye (e–h)

Fig. 4

Clinical characteristics of central serous chorioretinopathy detected by Optical coherence tomography (OCT). OCT B-scan of the right eye showing visible subretinal fluid (low-density area) indicates the area of serous retinal detachment (a). OCT B-scan showing thickness map of the right eye (b). OCT B-scan of the left eye is normal (c, d). The choroidal thickness of both eyes was obviously increased, with 488 nm in the right eye and 450 nm in the left eye

Fig. 5

Clinical characteristics of central serous chorioretinopathy detected by structural en-face optical coherence tomography angiography (OCTA). Automated segmentation of the superficial retina (3 × 3 mm) showing dark spots under the fovea indicates pigmented epithelial detachment (PED) at the superficial, deep retinal capillary, and retina plexuses (a–c) of the left eye. Thickness map of automated segmentation images showing high-density area corresponding to the subretinal fluid (black arrows, d–f)

Fig. 6

Clinical characteristics of central serous chorioretinopathy detected by optical coherence tomography angiography (OCTA). Automated segmentation of the superficial retina (at a range of 3 × 3 mm) showing foveal lesions on the topographic map. Focal protrusion of retinal pigment epithelium and high-density area of the choriocapillaris layer that corresponded to serous retinal detachment (black arrows) has also been noted (a, b). c Automated segmentation of the deep retina of the right eye. d Thickness map of automated segmentation of the deep retina of the right eye. The lesion which was showing by the white arrows in (c) and black arrows in (d) corresponded to serous retinal detachment which was indicated by black arrows in (b)

Fig. 7

Clinical characteristics of central serous chorioretinopathy detected by multispectral imaging (MSI, C2000) in the right eye. MSI showing leakages of retinal pigment epithelium (RPE) in the right eye using different wavelengths (a–g). RPE leakages are characteristically observed as pinpoints of hypercyanescence at longer wavelengths of 680–850 nm (d–g); the focal hyperreflective areas (white arrows) and multifocal hyperfluorescent dots (orange arrows) shown in MSI corresponded well to the RPE leakage as detected by fundus fluorescence angiography

Fig. 8

Clinical characteristics of central serous chorioretinopathy detected by multispectral imaging (MSI, C2000) in the left eye of the same patient using different wavelengths (a–g). MSI of the left eye shows the exact pinpoints (orange arrows, d–g) of hypercyanescence as the right eye at the wavelengths of 680 to 850 nm