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Central Serous Choroidopathy (CSC)



Correspondance:Asstt. Professor, Department of Ophthalmology, LLRM Medical College,Meerut

Introduction


Central serous chorioretinopathy (CSC) is one of several chorioretinal disorders characterized by serous detachment of the neurosensory retina and ⁄ or the retinal pigment epithelium (RPE). CSC is one of the 10 most common diseases of the posterior segment of the eye and a frequent cause of mild to moderate visual impairment.1

History of the disease2,3,4,5,6


1866               – Von graefe – first described the disease as recurrent serous retinitis.
1916               – Fuch’s work on the disease was appreciated.
1927               – Horniker – named disease as “Central Angiospastic Retinitis”
1930               – Walsh & Sloane – “idiopathic flat detachment of macula”
1930               – Gifford and Marquardt -  Theory on Angioneurotic diathesis.
1953               – Klien – theory on autonomic nervous system dysfunction.
1950’s            – Bennett & Maumenee – spectrum of macular disciform degeneration.
1955               – Bennett – “central serous retinopathy”
1960’s            – Maumenee and Gass – FA appearance of CSC.
1967               – Gass – “central serous choroidopathy”

Definition


Active CSC is characterized by detachment of the neurosensory retina caused by accumulation of serous fluid between the photoreceptor outer segments and the RPE in combination with monofocal or multifocal changes in the RPE

Pathogenesis


The patho-physiology of CSC is still not completely under­stood. It is important to be aware of the anatomy of the choriocapillaris-Bruch's membrane-RPE layer. The widely fenestrated endothelium of the choriocapillaris allows leakage of small protein molecules and fluid into the intercellular space. But the RPE represents an impermeable barrier to the diffusion of fluid into the subretinal space. The RPE pump acts in a vitreous choriocapillaries direction to keep the subretinal space dry. It is difficult to accept that a single, isolated disturbance of a few RPE cells may overwhelm the RPE pump of the neighboring normal RPE. It is plausible to assume that at the basis of the disease there is a more diffuse dysfunction of the RPE cells, the choroid, or both.

RPE dysfunction theory 7,8


When evaluating possible patho-physiologic processes at the RPE level, one needs to consider five points:

RPE damaged via immunologic infections circulatory and neuronal  mechanism

RPE secretes ions in chorioretinal direction (towards retina)

Choroidal fluid gets attracted into this area.

Strong flow disrupts the diffusion barrier in this area.

Since the defective area is so small (in the RPE), only a tiny leakage point is visible during the earliest phase of FA. Subsequently, there is rapid increase in fluorescein stained liquid in the subretinal blister during the following stages of angiography. This demonstrates the high speed and large amount of the fluid passing through the diseased area of the RPE.

Choroid dysfunction theory 9,10,11


Psychogenic, pregnancy, transplantation, type A, raised cortisol levels

Adrenergic reaction causes damage to the choriocapillaries

Hyperpermeability of choriocapillaries

RPE cell degeneration

Secondary changes in RPE causes leaks

Serous retinal detachment

The hydrostatic pressure of the fluid pooling under the detached RPE will then mechanically cause a solution of continuity in the RPE layer with the subsequent leakage of fluid in the sub retinal space and neurosensory detachment of retina.
Three reports from France have described elevated prevalence of Helicobacter pylori infection in patients with CSC compared to the background population (Mauget-Faysse et al. 2002; Ahnoux-Zabsonre et al. 2004; Cotticelli et al. 2006).12,13,14
Recent evidence suggests that the outer segment of photoreceptors (OS) may elongate and the ONL may become thinner close to a serous retinal detachment. Because the OS is enveloped by RPE microvilli that enable mutual metabolism, the first retinal insult resulting from a RD may be in the photoreceptor OS, which ensures apoptosis of the photoreceptor cell bodies. In its physiologic cycle, the outer part of photoreceptor OS is phagocytized continuously by RPE cell, whereas its inner part is regenerated at the junction with IS. If the neurosensory retina is detached from the RPE, the tip of OS fails to be phagocytized by the RPE, which may develop elongation of the OS. Chronic disturbed renewal of the OS may lead apoptosis of cone cells and thinning of the ONL.

Types :

Clinically, it is of 2 types :-

  1. Typical or Classic CSC – seen in younger patients & causes an acute localized detachment of retina with mild to moderate loss of visual acuity associated with one or few focal leaks seen during FFA.
  2. Atypical CSC – Further of 2 types: -
    1. Chronic CSC or Diffuse retinal pigment epitheliopathy – widespread alteration of pigmentation of the RPE related to the chronic presence of shallow subretinal fluid.
    2. bullous retinal detachments usually located inferiorly.

Histologically, (Spitznas) it is of 3 types: -

  1. NSR Detachment.
  2. RPE Detachment.
  3. Both NSR & RPE Detachment.

Demography 15,16


Age – It affects young to middle aged individuals 20 – 45 years of age. In women age tends to be higher. If age is >50 years – diagnosis is seriously questioned as mostly later it turns out to be “age related macular degeneration” and “choroidal neovascularization”.
Sex – Male predominance – 8 to 10:1
Race –Commonly affects Whites, Hispanics, Asians – Japanese mostly. African-Americans are affected very less. Severe form occurs with - south east Asian and Latin origins.

Systemic associations of the disease17,18,19,20,21,22

  1. Migraine like headache
  2. Type A personality
  3. Hypochondrial behavior
  4. Hysteria
  5. Conversional neurosis
  6. Increased Cortisol levels in patients with Cushing’s disease.
  7. Long term corticosteroid treatment in organ transplants & Respiratory allergies.

Although the role played by corticosteroids in CSC is not well understood, it is probable that among other mechanisms, the anti- inflammatory properties of steroids may cause delayed healing of the RPE defect. Cortisol, by suppressing synthesis of extracellular matrix components and inhibiting fibroblastic activity, also may damage directly the RPE cells or their tight junctions and may delay any reparative process in damaged RPE cells.

 

 

Symptoms

One of the most frequent complaint is transiently seeing a dark spot in the centre of visual field. The dark spot, which is the subjective representation of a relative scotoma in the centre of the visual field is usually most prominent in the morning immediately after awakening. Patients often report seeing it most clearly when opening their eyes and looking at the ceiling of their bedroom, presumably because the typical ceiling is bright white and unstructured. These characteristics are typical of a relative scotoma, and like the relative scotoma produced by light (i.e. An after-image) it fades within a few seconds, presumably because of the Troxler effect, a retinal function that subtracts any stationary background stimulus.22

Signs

Functional testing of eyes with serous retinal detachment has demonstrated that: -

After resolution,

FFA 23
Three types of leakages are seen-

 

12

11

Figure – FFA showing inkblot pattern of leakage

Ink blot pattern

1

2

Figure – FFA showing smoke stack pattern of leakage

Smoke stack pattern

 

 

Autofluorescence photography 24,25

Autofluorescence

Figure 2 – Fundus Autofluorescence Photograph showing damaged RPE

 

 
ICGA 26

OCT 27-31

oct

Figure 1 – Spectral Domain OCT showing typical NSR elevation with sub retinal fluid

Multifocal Electroretinogram (mfERG) 32,33


mfERG has been used to identify focal regions of decreased retinal function, even in asymptomatic or clinically inactive eyes. Furthermore, investigators, including Lai et al, are using mfERG as a means of assessing the efficacy and safety of new treatment modalities for CSC. During acute CSC, retinal dysfunction is reflected by reduction in mfERG response amplitudes and delay in implicit times. With the use of mfERG, it has also been demonstrated that the felloweye of patients with CSC may have abnormal mfERG responses. It has been shown that mfERG abnormalities may persist even after resolution of the subretinal fluid clinically. Thus, mfERG may therefore have a useful role in providing an objective measure of retinal function in research on the treatment for CSC.

Microperimetry 34


Microperimetry-1 (MP1, Nidek technologies) is an instrument for fundus-related perimetry. It captures fundus images of the patient’s retina and at the same time projects light stimuli onto the retina. The light stimuli size have been correlated to Goldmann stimuli sizes (Goldmann I-V) and the pattern are chosen by the operator and can therefore be adapted to different diseases of the macula. The patient’s subjective response to each stimulus (seen/not seen) is recorded (functional information) together with the retinal location of the stimulus (anatomical information). Retinal Microperimetry (MP1) allows an accurate analysis of the central retinal function, combining a digital retinography, a computerized perimetry and a fixation assessment in one exam. In combination with other retinal investigation devices, MP1 has already helped us and will in the future help us to follow and to understand retinal diseases. It has also shown that, despite clinical resolution of CSC, there is lower retinal sensitivity in the macula even once visual acuity returned to 20/20. Fixation studies showed stability of central fixation. Springer et al. investigated patients with Central Serous Choroidopathy (CSC). The MP1 enables quantification of functional defects in patients with CSCR.

Natural History of CSC

Treatment

Laser Photocoagulation

Indications

If leakage point is within 500 microns from the center of fovea, wait for 6 months before treating.

Technique

Complications

Recently, Subthreshold diode laser has been tried in the treatment of ICSC with point source leakage.35

Photodynamic Therpy
Indications: -

The use of half-dose verteporfin36 (3 mg/m2) or low fluence PDT (50% reduced light fluence) is done as a precaution against permanent RPE or choriocapillaries damage.

Prognostic Indicator37
Recently Matsumoto H et al has suggested a positive correlation between the ONL thickness with the BCVA in resolved CSC. Discontinuity of the IS/OS line was prevalent in eyes with a thinner ONL and lower BCVA. Despite the good visual outcomes and normal appearance of the IS/OS line, micropsia, and central darkness of the vision often persist in patients with resolved CSC. This phenomenon may be attributed to the decreased cone cells at the fovea.

Table 1. Summary of treatment regimens for CSC

Duration

Examination

Detachment

Stress, Hypercortisolism

Treatment

Acute

Fine granular subretinal deposits if duration more than a few weeks; monofocal leakage

High bullous OCT > 100 µ subretinal fluid

Common, recurrent

Conservative, counseling; if no resolution within 3 months of onset, consider focal photocoagulation if safe, otherwise PDT

Recurrent

Paucifocal (1-5)

Moderate 51-100 µ

Common

Photocoagulation if safe, otherwise PDT

Chronic

Multiple semi confluent hypopigmented RPE lesions; confluent subretinal material

Shallow, often < 50 µ

Past, current, inconclusive or none

PDT

Sequelae

RPE depigmentation without RPE atrophy

None

Past, inconclusive or none

None

Neovascularization

CNV plus CSC Sequelae; subretinal fibrosis

Variable mainly around CNV

History of CSC, associated RPE changes

PDT &/or intravitreal Anti-VEGF

References

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  2. Pascal W. Hasler Microperimetry - a method which combines Perimetry and macular topography - Oftalmolog December 2007.
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  5. Matsumoto H, Taku S, Kishi S. Outer Nuclear Layer Thickness at the Fovea Determines Visual Outcomes in Resolved Central Serous Chorioretinopathy. Am J Ophthalmol 2009;148: 105–110.