Central Serous Retinopathy (CSR) or Central Serous Chorioretinopathy (CSCR)

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Central Serous Retinopathy
Overview

Central Serous Chorioretinopathy (CSCR or CSR) is a relatively common cause of visual impairment and is characterized by the accumulation of sub-retinal fluid in the macula and/or mid-peripheral retina.

Incidence

CSR is predominantly a disease of men 20 to 45 years of age (however 10% of patients with CSR are women and patients > 50 years.

CSR patients often transition to have Age-Related Macular Degeneration in later life. Patients diagnosed with CSR-like disease after age 50 should raise a high suspicion of neovascular age-related macular degeneration (ARMD).

CSR is more common in Caucasians, Hispanics and Asians, and less common in those of African descent. Persons with CSCR have a reported higher prevalence of type-A personality (high stress personality type), migraine-like headaches or psychiatric conditions, including hypochondria, hysteria and conversion disorder. High-stress occupations or lifestyles also have been associated with CSCR.

The disease has been associated with autonomic nervous system dysfunction and is often seen or exacerbated by:

  • Corticosteroid exposure such as intravenous, oral, nasal or topical use
  • Liver disease
  • Phosphodiesterase-5 inhibitor use (Viagra, Cialis)
  • Obstructive sleep apnea
  • Pregnancy
  • Cushing syndrome (an endocrine disorder with elevated cortisol levels)
  • High blood pressure
  • Organ transplantation
  • Systemic Lupus Erythematosis

Symptoms

  • Decreased Vision – Images appear blurry in the affected eye
  • Micropsia – Images appear smaller in the affected eye
  • Metamorphopsia – Images appear distorted in the affected eye

Clinical Findings

  • Pigment Epithelial Detachment (PED) – Elevation of the retinal pigment epithelium (layer of cells below the retina)
  • Sub-Retinal Fluid (SRF) – Leakage and accumulation of fluid above the retinal pigment epithelium and below the retina

Disease Progression

The typical natural history of CSCR is complete spontaneous resolution of sub-retinal fluid with restoration of visual acuity within three months of initial onset of symptoms.

However, up to 20 percent of patients may have persistent serous macular detachment and vision loss past three months, and may require treatment

Some patients (even when the sub-retinal fluid completely resolves) may be left with some degree of subjective visual impairment such as micropsia, metamorphopsia or reduced vision or color perception.

If subretinal fluid has not resolved by three months, the patient is defined as having Chronic CSR, and treatment is often considered, however treatment timing is determined on an individual basis.

A small percentage of CSR patients experience multiple relapses and are categorized as having Chronic Recurrent CSR which can often lead to significant damage to the retinal photoreceptors and retinal pigment epithelium leading to permanent visual loss.

Diagnosis

The following are used for the diagnosis of patients with CSR:

  • History
  • Visual Acuity – to access visual loss
  • Amsler Grid Test - may demonstrate metamorphopsia (distorted vision
  • Dilated Fundoscopic Examination - essential to appreciate the characteristic findings of CSCR.
  • Fluorescein Angiography (FA) - often demonstrates single or multiple discrete leakage points where subretinal fluid originates or that may reveal evidence of a pigment epithelial detachment (PED). The classic “smokestack” appearance occurs in less than 20 percent of cases.
  • Ocular coherence tomography (OCT) -demonstrates the neurosensory detachment of the retina in addition to any PED associated with the CSR. OCT is the best way to follow changes in the amount of sub-retinal fluid to determine if the disease is improving or worsening.
  • Enhanced-Depth Spectral Domain OCT - has shown increased sub-foveal choroidal thickness in some patients with CSCR as compared to normal eyes
  • Indocyanine Green angiography (ICG) - may be required to differentiate between CSCR and AMD or Polyploidal Choroidal Vasculopathy (PCV).

Treatment

The following are current treatments for CSR:

  • Observation – 80% of CSR patients will improve without treatment
  • Photodynamic Therapy – to treat leaks close to or under the center of the fovea (center of the retina)
  • Focal Laser Photocoagulation – to treat small leaks away from the fovea (center of the retina)
  • Anti-VEGF Therapy - Case studies and anecdotal reports of intravitreal anti-VEGF medications in patients with persistent or chronic CSR have shown improvements in visual acuity, resolution of neurosensory detachments and decreased RPE leakage on FA. Prospective studies using anti-VEGF medications have shown inconsistent results. Controlled clinical trials are necessary to determine the tolerability and efficacy of anti-VEGF therapies in CSR.

Several small studies have shown mixed results from a variety of systemic medications for CSCR, including

  • Carbonic Anhydrase Inhibitors (acetazolamide),
  • Adrenergic Receptor Antagonists (metoprolol, propranolol),
  • Steroid Hormone Antagonists (ketoconazole, mifeprestone, finasteride, eplerenone.

Central serous chorioretinopathy is a disease of working-aged patients, many of whom have occupations that are associated with high levels of stress and lack of sleep. Treatment also includes:

  • Plenty of rest
  • Reduction of stress