Central Serous Chorioretinopathy (CSCR)

Overview

Epidemiology & Risk Factors

• Corticosteroid use (any route — oral, inhaled, nasal spray, topical skin creams, endogenous Cushing syndrome) — strongly linked
• Type A personality / psychological stress
• Systemic hypertension
• Pregnancy (women)
• Lupus (SLE)
• Helicobacter pylori infection
• Renal dialysis
• Sleep apnoea syndrome
• A SNP in the complement factor H gene (chromosome 1) predisposes to the chronic form in White individuals

Presentation

• Unilateral blurred or dim vision
• Metamorphopsia (distortion) and micropsia (objects appear smaller)
• Central scotoma
• Mild dyschromatopsia (faded colors)
• May be asymptomatic

• VA typically 6/9–6/18 (20/30–20/60); improves with a low-strength convex/plus lens (acquired hypermetropia from retinal elevation)
• Round or oval serous detachment of the neurosensory retina at the macula — sloping margins that merge gradually into the attached retina
• Subretinal fluid (clear or turbid); precipitates may be on the posterior retinal surface
• One or more depigmented RPE foci or small PEDs within the detachment
• FAF imaging reveals RPE atrophy/hyperplasia from prior episodes; gravitational tract visible in chronic cases

Fundus Appearance

Fundus photo: localized serous detachment of the neurosensory retina in the macula — Wills Eye Manual, Fig. 11.15.1

Investigations

1. Amsler grid — documents area of field distortion
2. Slit-lamp biomicroscopy — rule out concurrent CNV; examine optic disc for pit
3. OCT (enhanced-depth imaging) — gold standard for demonstrating subretinal fluid, PEDs, and choroidal thickening (pachychoroid)
4. IVFA (fluorescein angiography) — nearly pathognomonic "smoke-stack" pattern of dye leakage in 10–20% of cases; "ink blot" pattern in others
5. ICGA (indocyanine green angiography) — choroidal artery/choriocapillaris filling delays; multifocal hyperfluorescent patches in early phase
6. For chronic CSCR: systemic workup including cortisol levels and renal function

IVFA: classic "smoke-stack" pattern of dye leakage — Wills Eye Manual, Fig. 11.15.2

EDI-OCT: subretinal fluid (black cavity) and underlying choroidal thickening — Wills Eye Manual, Fig. 11.15.3

Course & Prognosis

Differential Diagnosis

• AMD — patient usually ≥50 years, drusen, often bilateral, may have CNV
• Optic pit — contiguous serous RD with the optic disc
• Rhegmatogenous retinal detachment / macular hole
• Choroidal tumor (hemangioma, melanoma)
• Hypertensive retinopathy
• PED — more distinct, elevated margins (may accompany CSCR or AMD)
• Uveal effusion syndrome, inflammatory choroidal disorders, chronic renal failure

Management

1. Observation

• First-line for acute CSCR — usually self-limited with good visual prognosis.

2. Stop Corticosteroids

• Discontinue all forms if possible (oral, topical, nasal, inhaled).

Key tip (Kanski): All forms of corticosteroid treatment should be discontinued in patients with chronic or recurrent CSCR.

3. Laser Photocoagulation

• May accelerate visual recovery but long-term benefit is unclear and may increase CNV risk.
• Consider when:
  • Detachment persists for several months
  • Recurrence with residual visual deficit
  • Contralateral eye involvement after a prior episode
  • Occupational necessity for prompt visual recovery

4. Photodynamic Therapy (PDT)

• Useful for chronic CSCR.
• Half-dose PDT may be considered for rapid SRF resolution in acute CSCR.

5. Mineralocorticoid Receptor Antagonists

• Eplerenone and spironolactone — associated with improved anatomic and visual outcomes in chronic CSCR.
• A 2025 meta-analysis (PMID: 40513762) specifically evaluated both agents for chronic CSCR.

6. Anti-VEGF Therapy

• If choroidal neovascularization (CNV) develops as a complication.

Central Serous Chorioretinopathy (CSCR)

Definition

Epidemiology

• Typically affects young to middle-aged males (20–45 years)
• Male : Female = 3:1 (females tend to present at an older age)
• Usually unilateral, though bilateral cases occur (more common in older patients)
• In unilateral CSCR, ~two-thirds of fellow eyes show signs of pachychoroid pigment epitheliopathy

Pathogenesis

1. Dilated outer choroidal (Haller layer) vessels → increased hydrostatic pressure in choriocapillaris
2. Impaired RPE pump function → breakdown of the outer blood-retinal barrier
3. Fluid accumulates in the subretinal space → neurosensory detachment

Risk Factors / Associations

Clinical Features

Symptoms

• Unilateral blurred or dim vision
• Metamorphopsia (distortion of straight lines)
• Micropsia (objects appear smaller)
• Central scotoma
• Mild dyschromatopsia (faded colours)
• Occasional acquired hypermetropia (retinal elevation shifts focal point forward)

Signs

Fundus Photograph

Fundus photo: localized serous detachment of neurosensory retina at macula — Wills Eye Manual Fig. 11.15.1

Investigations

1. Amsler Grid

2. Slit-Lamp Biomicroscopy (with 90D/contact lens)

• Identifies the macular serous detachment
• Rules out CNV or optic disc pit

3. Optical Coherence Tomography (OCT) — Investigation of Choice

• Optically empty (black) space beneath elevated neurosensory retina = subretinal fluid
• May show associated RPE detachments (PEDs)
• Enhanced depth imaging (EDI-OCT): demonstrates choroidal thickening (pachychoroid) — key diagnostic feature

OCT (Fig. 14.42E, Kanski): neurosensory detachment with subretinal fluid in acute CSCR

4. Fundus Fluorescein Angiography (FFA/IVFA)

FA Fig. 14.43 (Kanski): (A)(B) ink blot; (C)(D) smoke-stack patterns

5. Indocyanine Green Angiography (ICGA)

• Early phase: dilated/compromised choroidal vessels; choriocapillaris filling delay
• Mid-phase: multifocal hyperfluorescent patches from choroidal hyperpermeability
• Subclinical foci commonly visible; essential for guiding PDT

6. Fundus Autofluorescence (FAF)

• Hypoautofluorescence at leakage site and old lesions (RPE loss)
• Hyperautofluorescence = RPE lipofuscin accumulation
• Reveals gravitational tracts in chronic cases

7. Systemic Workup (chronic CSCR)

• Serum/24-hour urinary cortisol (exclude Cushing syndrome)
• Renal function tests

Course & Prognosis

Differential Diagnosis

Management

1. Observation (First-line for Acute CSCR)

• Most acute cases resolve spontaneously within 3–6 months
• Monitor with OCT; reassure patient

2. Eliminate Risk Factors

• Stop all corticosteroids (systemic, topical, nasal, inhaled) — mandatory in chronic/recurrent cases

Key tip: "All forms of corticosteroid treatment should be discontinued if possible in patients with chronic or recurrent CSCR." — Kanski

3. Laser Photocoagulation

• Conventional laser: May accelerate visual recovery but long-term benefit uncertain; carries risk of CNV formation
• Subthreshold micropulse diode laser to the RPE leakage site: Better safety profile — good results in several studies; significantly less retinal damage on OCT
• Indications:
  • Persistence >3–4 months
  • Recurrence with residual visual deficit
  • Occupational necessity for prompt visual recovery
  • Contralateral eye involvement after prior vision loss

4. Photodynamic Therapy (PDT) with Verteporfin

• Treatment of choice for chronic CSCR
• Half/reduced dose PDT (30–50% standard dose + 50% light intensity): Complete SRF resolution including severe chronic cases; significantly lower risk of choroidal ischaemia than full-dose regimens
• Half-dose PDT may also accelerate resolution in acute CSCR

5. Mineralocorticoid Receptor Antagonists

• Spironolactone (40 mg BD) and Eplerenone — associated with faster SRF resorption and improved visual outcomes in both acute and chronic CSCR
• Mechanism: block mineralocorticoid receptors in choroidal vasculature → reduce hyperpermeability
• Supported by a 2025 systematic review and meta-analysis (PMID: 40513762)

6. Anti-VEGF Therapy

• Bevacizumab alone is not effective in pure CSCR (no CNV)
• Indicated if macular neovascularization (MNV/CNV) develops as a complication
• Often used in combination with PDT

7. Others (Limited Evidence)

• Aspirin, beta-blockers, mifepristone — case reports suggest benefit; controlled data lacking

Summary Table

Eales Disease

Definition

Named after British physician Henry Eales (1852–1913), who described recurrent vitreous haemorrhage in young men.

Epidemiology

• Most common in young males aged 20–35 years
• Particularly prevalent in the Indian subcontinent (South Asia) — important cause of visual morbidity there
• Rare in White populations
• Usually bilateral, though often asymmetrical

Aetiology & Pathogenesis

1. Tubercular Hypersensitivity (Most favoured)

• Mycobacterium tuberculosis protein hypersensitivity → immune-mediated retinal periphlebitis
• Evidence: tuberculin skin test positivity, positive QuantiFERON-TB, PCR for M. tuberculosis DNA in vitreous samples in some cases
• However, evidence is conflicting — some patients may have active tubercular vasculitis, others only hypersensitivity

2. Oxidative Stress

• Increased free radicals and decreased antioxidant enzymes demonstrated in patients

3. Autoimmune Mechanism

• Molecular mimicry: retinal S-antigen may trigger autoimmune response

4. Other associations

• Helicobacter pylori, focal sepsis, metabolic disorders — reported but unconfirmed

Perivenous inflammatory infiltrate → endothelial damage → venous occlusion → retinal ischaemia → VEGF upregulation → neovascularization → vitreous haemorrhage

Stages of Eales Disease

Clinical Features

Symptoms

• Floaters — due to vitreous haemorrhage
• Sudden painless visual loss — vitreous haemorrhage (most common presentation)
• Positive family history may be present
• Systemic neurological features have been reported (rare)

Signs

Anterior Segment

• Mild anterior uveitis often present
• Keratic precipitates (fine)
• Flare and cells in anterior chamber

Posterior Segment (Fundus)

• Peripheral periphlebitis — venous sheathing (white cuffing around veins), superficial retinal haemorrhages, cotton-wool spots
• Pigmented chorioretinal scars at sites of resolved lesions

• Branch retinal vein occlusion (BRVO) of peripheral veins
• Peripheral capillary non-perfusion
• Microaneurysms, venous tortuosity, vascular shunts

• Peripheral retinal neovascularization at the junction of perfused and non-perfused retina
• Disc new vessels (neovascularization of disc — NVD) can sometimes develop
• Recurrent vitreous haemorrhage — occurs in ~one-third of eyes; typically limited, absorbs over weeks but can persist

Fundus Photographs

Fig. 13.59C (Kanski): Vascular occlusion, haemorrhage and localized laser scars in Eales disease

Fig. 13.59B (Kanski): Peripheral retinal neovascularization in Eales disease

Fig. 13.59D (Kanski): FA showing extensive areas of capillary non-perfusion and active neovascularization

Complications

1. Tractional retinal detachment (from fibrovascular proliferation)
2. Macular epiretinal membrane
3. Neovascular glaucoma (NVG) — from anterior segment neovascularization
4. Cataract
5. Persistent vitreous haemorrhage
6. Permanent visual loss

Investigations

Ocular

Systemic (to rule out other causes)

Key point: Eales disease is a diagnosis of exclusion — all secondary causes of retinal vasculitis and peripheral neovascularization must be ruled out.

Differential Diagnosis

Management

1. Corticosteroids (Inflammatory Stage)

• Periocular, systemic, topical and intravitreal steroids are helpful in the inflammatory/active periphlebitis stage
• Reduce venous inflammation and prevent progression to occlusion

2. Anti-Tubercular Treatment (ATT)

• Controversial but strongly advocated by many authorities, especially in endemic regions
• Indications to consider ATT (combined with steroids):
  • Strongly positive tuberculin skin test or QuantiFERON
  • Severe ocular inflammatory signs
  • To prevent reactivation of prior TB infection
• Standard 4-drug ATT regimen: Isoniazid + Rifampicin + Pyrazinamide + Ethambutol

3. Scatter (Panretinal) Photocoagulation (PRP)

• Treatment of choice for non-perfused retina — reduces neovascular stimulus (VEGF)
• Applied to areas of capillary non-perfusion to induce regression of new vessels
• Cryotherapy is an alternative if laser is not accessible

4. Intravitreal Anti-VEGF Agents

• Commonly used in the neovascular/proliferative stage
• Induces regression of retinal new vessels
• May be used as adjunct before vitrectomy to reduce intraoperative bleeding

5. Vitrectomy

• Persistent non-clearing vitreous haemorrhage
• Tractional retinal detachment
• Macular epiretinal membrane

Prognosis

• Generally good in the majority of cases — most patients retain useful vision
• Worse prognosis with:
  • Macular involvement
  • Dense persistent vitreous haemorrhage
  • Tractional retinal detachment
  • Neovascular glaucoma

Summary