Central serous chorioretinopathy (CSCR) was first described by von Graefe in 1866 as “relapsing central luetic retinitis” and by Bennett in 1955 as “central serous retinopathy” associated with a primary choroidal pathology. A history is often elicited in these patients of a psychological stressor occurring at peak symptom onset, sleep deprivation, and/or a history of exogenous corticosteroid exposure. In 2013, the term pachychoroid was introduced as a descriptive term for choroidal morphologic and pachymetric features of CSCR, which were recognized as residing within a broader range of conditions referred to as the pachychoroid disease spectrum. These include the following:
Pachychoroid pigment epitheliopathy (PPE) or form fruste CSCR.
Pachychoroid neovasculopathy (a late complication of PPE) (▶ Fig. 45.5).
Focal choroidal excavation.
Bullous exudative retinal detachment: a rare manifestation due to the breakdown of physiological mechanisms governing outer retinal hydration resulting in neurosensory detachment of over 10 disc diameters.
Aneurysmal type 1 neovascularization (NV; previously known as polypoidal choroidal vasculopathy).
Peripapillary pachychoroid syndrome: a newly defined condition with pachychoroid features located around the optic nerve.
Fig. 45.1 Acute central serous chorioretinopathy. (a) Spectral domain optical coherence tomography (b) with enhanced depth imaging demonstrates serous macular detachment associated with a nasal serous pigment epithelial detachment. Prominent Haller’s layer vessels (pachyvessels) within a thickened choroid are evident. The inner choroidal layers are attenuated beneath the area of detachment.
Fig. 45.2 (a,b) Acute central serous chorioretinopathy demonstrating acute focal leakage point emanating from the pigment epithelial detachment peak as a central lucency among the fibrinous subretinal fluid. (c) This was confirmed on fluorescein angiography. A pachyvessel underlies this area.
Fig. 45.3 (a,b) Chronic central serous chorioretinopathy. Enhanced depth imaging optical coherence tomography demonstrates neurosensory detachment and a shallow irregular pigment epithelial detachment over a thickened choroid with attenuated inner choroidal layers and pachyvessels. The overlying foveal macular thickness is reduced due to loss of outer retinal tissue.
Fig. 45.4 Chronic central serous chorioretinopathy with recurrent serous detachment. (a) Color fundus photograph demonstrates reduced choroidal tessellation at the posterior pole. (b) Arteriovenous-phase fluorescein angiography shows two focal leakage points overlying a staining pigment epithelial detachment. (c) Midphase indocyanine green angiography shows central choroidal vascular hyperpermeability. (d) Fundus autofluorescence demonstrates mottled hypoautofluorescence centrally and inferior hyperfluorescence due to the chronic descending subretinal fluid.
Fig. 45.5 (a,b) Treatment-naive pachychoroid neovasculopathy. A thickened choroid is present with attenuated inner choroidal layers and pachyvessels. There is an irregular pigment epithelial detachment with hyperreflective sub-retinal pigment epithelium contents. A shallow serous detachment with an area of subretinal hyperreflective material overlies the type 1 neovascular lesion.
45.1.1 Common Symptoms
Common symptoms and examination findings are outlined in ▶ Table 45.1.
Painless acute/subacute decrease in central vision with metamorphopsia, micropsia, and a hypermetropic shift. Spontaneous resolution
Serous RD with or without serous PED. Reduced fundus tessellation
Prolonged and/or relapsing and remitting decrease in central vision with metamorphopsia, micropsia, and a hypermetropic shift for 6 mo or longer
Subfoveal/parafoveal subretinal fluid that appears more fibrinous in nature. RPE and outer retinal disruption, including both atrophy and pigment aggregation. Reduced fundus tessellation
RPE changes reminiscent of CSCR occurring without evidence of subretinal fluid. Changes may be central or peripapillary. Reduced fundus tessellation
Central or paracentral decrease in vision and/or distortion when exudation occurs centrally. May be asymptomatic when fluid is eccentric to the fovea
RPE changes reminiscent of CSCR. Subretinal, intraretinal, and/or sub-RPE fluid in the absence of soft drusen. Hemorrhage is rare unless aneurysms occur. Reduced fundus tessellation
Aneurysmal type 1 NV 2° to pachychoroid disease
As per pachychoroid NV, but symptomatic exudation is more common
As per pachychoroid NV, but lipid and hemorrhage are more common
Bullous exudative RD
Similar to chronic CSCR with visual symptoms over a greater proportion of the visual field
Gravitating bullous RD with turbid subretinal fluid. Multiple PEDs of varying size. Polygonal or crescent-shaped RPE tears may be present
Similar to chronic CSCR with symptoms preferentially prevalent adjacent to the blind spot (optic nerve) extending to fixation
Single or multiple PEDs. Reduced fundus tessellation. Peripapillary RPE changes. Choroidal folds may be present
Abbreviations: CSCR, central serous chorioretinopathy; NV, neovasculopathy; PED, pigment epithelial detachment; PPE, pachychoroid pigment epitheliopathy; PPS, peripapillary pachychoroid syndrome; RD, retinal detachment; RPE, retinal pigment epithelium.
45.2 Key Diagnostic Tests and Findings
Relevant investigations and associated findings are summarized in ▶ Table 45.2. Optical coherence tomography (OCT), fluorescein angiography (FA), fundus autofluorescence (FAF), indocyanine green angiography (ICGA), and OCT angiography (OCTA) all have potentially important roles in diagnosis.