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Ocular System

Central serous chorioretinopathy (CSC)

Nonsurgical retinopathy characterized by serous retinal detachment and/or retinal pigment epithelial (RPE) detachment, changes most often confined to the macula, and associated with leakage of fluid through the RPE into the subretinal space.

Nonsurgical retinopathy characterized by serous retinal detachment and/or retinal pigment epithelial (RPE) detachment, changes most often confined to the macula, and associated with leakage of fluid through the RPE into the subretinal space.

  • #4 M/C nonsurgical retinopathy

History:

CSC has been known by many names since the original description by von Graefe in 1866,and these names reflect the course of progress in our understanding of the pathogenesis of the disease. von Graefe described the disease as a recurrent central retinitis, whereas Horniker in 1922 called it capillarospastic central retinitis to reflect his belief that vasospasm was the mechanism. Other names of the 20th century include central angiospastic retinopathy and central serous retinopathy. Maumenee described fluorescein angiographic (FA) characteristics; fluorescein leakage at the level of the RPE revealed that the choroid and RPE were the primary tissues involved. Gass further characterized the angiographic findings and coined the term central serous chorioretinopathy. Since we now understand that hyperpermeability of the choroid causes leakage through the RPE, resulting in a neurosensory retinal detachment, CSC is the preferred term.


Etiopathogenesis

Multifactorial pathophysiology, which causes retinal pigment epithelial (RPE) disturbances and circulatory changes in the choroid. There is choroidal inflammation, which causes stasis and ischemia. The choroidal vessels become hyperpermeable, and the choroid becomes thickened. This leads to increased tissue pressure, which disturbs the anatomic integrity of RPE and causes PED. The micro-defects in the RPE hamper its barrier functions. The choroidal fluid crosses the RPE and cause neurosensory detachment. It gets collected in the subretinal space. Another hypothesis is the loss of polarity of RPE.


Clinical features

Commonly presents with metamorphopsia, central scotoma associated with a modest reduction in visual acuity.

  • Sudden painless loss of vision
  • Micropsia
  • Metamorphopsia
  • Relative scotoma
  • Color vision disturbances
  • Temporary hyperopia

Diagnosis

OCT (optical coherence tomography):

First-line investigation
  • Presence of subretinal fluid (SRF) (characteristic)
(a) OCT image of a CSC patient prior to photodynamic treatment showing a large amount of subretinal fluid involving the fovea. (b) OCT image of the same patient 3 months after half-dose photodynamic treatment showing complete resolution of subretinal fluid. | Gemenetzi, M., De Salvo, G. & Lotery, A. Central serous chorioretinopathy: an update on pathogenesis and treatment. Eye 24, 1743–1756 (2010). https://doi.org/10.1038/eye.2010.130

Fluorescein angiography:

  • Enlarging ink-blot/smoke-stack pattern (outer blood-retinal barrier disrupted)
Early and late fluorescein angiograms of a 29-year-old patient affected by chronic central serous chorio-retinopathy revealing an irregularity of the retinal pigment epithelium, resembling a “honeycomb” pattern, near the point of fluorescein leakage. Window defects and late-phase leakage from the expanding point in the superior macula are evident, toward the fovea in a “steam” configuration. | Semeraro, F., Morescalchi, F., Russo, A., Gambicorti, E., Pilotto, A., Parmeggiani, F., Bartollino, S., & Costagliola, C. (2019). Central Serous Chorioretinopathy: Pathogenesis and Management. Clinical ophthalmology (Auckland, N.Z.), 13, 2341–2352. https://doi.org/10.2147/OPTH.S220845

Management

Focal laser photocoagulation:

Enabling sealing of any focal RPE defect that causes leakage as demonstrated on fluorescein angiography

Photodynamic therapy (PDT) with verteporfin:

PDT alters choroidal vasculature structure and perfusion, reducing choroidal permeability which reduces the subretinal fluid associated with the macular NSD associated with CSC

Mineralocorticoid antagonism:

Alters choroidal vascular permeability preventing generation of SRF
  • Spironolactone
  • Eplerenone

Anti-VEGF treatment:

Treatment of CSC with secondary choroidal neovascular membranes
  • Intravitreal bevacizumab

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