Central Serous Choroidopathy (CSC)

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


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”


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


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.




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


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

After resolution,

FFA 23
Three types of leakages are seen-




Figure – FFA showing inkblot pattern of leakage

Ink blot pattern



Figure – FFA showing smoke stack pattern of leakage

Smoke stack pattern



Autofluorescence photography 24,25


Figure 2 – Fundus Autofluorescence Photograph showing damaged RPE



OCT 27-31


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


Laser Photocoagulation


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



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




Stress, Hypercortisolism



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


Paucifocal (1-5)

Moderate 51-100 µ


Photocoagulation if safe, otherwise PDT


Multiple semi confluent hypopigmented RPE lesions; confluent subretinal material

Shallow, often < 50 µ

Past, current, inconclusive or none



RPE depigmentation without RPE atrophy


Past, inconclusive or none



CNV plus CSC Sequelae; subretinal fibrosis

Variable mainly around CNV

History of CSC, associated RPE changes

PDT &/or intravitreal Anti-VEGF


  1. Wang M, Munch CI, Hasler PW et al. Central serous chorioretinopathy - Acta Ophthalmol. 2008: 86: 126–145.
  2. Van Graefe A. Veber Central receidivirende retinitis. Albert Van Graefes Arch Ophthalmol 1866; 12:211-215.
  3. Klien BA. Macular lesion of vascular orifices: II Functional vascular conditions leading to damage of the macula lutea. Am J Ophthalmol 1953; 36:1-13.
  4. Maumenee AE. Discussion of Gass FD: Pathogenesis of hemorrhagic disciform lesion of posterior ocular fundus. A histopathologic study. Presented to Wilmer annual meeting, Baltimore, MD, May 26, 1964.
  5. Gass JDM. Pathogenesis of disciform detachment of the neuroepithelium. II Idiopathic central serous chorioretinopathy. Am J Ophthalmol 1967; 63:587-615.
  6. Bennet G. Central serous retinopathy. Br J Ophthalmol 1995; 39:605-618.
  7. Marmor MF. New hypotheses on the pathogenesis & treatment of serous retinal detachment. Graefes Arch Clin Exp Ophthalmol 1988; 226:548-552.
  8. Spitznas M. Pathogenesis of central serous retinopathy: anew working hypothesis. Graefes Arch Clin Exp Ophthalmol 1986; 224: 321-324.
  9. Spaide RF, Goldbaum M, Wong DWK et al. Serous detachment of the retina. Retina 2003; 23:820-846.
  10. Ciardella AP, Borodoker N, Costa DLL et al. The expanding clinical spectrum of central serous chorioretinopathy. Comp Ophthalmol Update 2003; 4:71-84.
  11. Guyer DR, Yannuzi LA, Slakter JS et al. Digital indocyanine green videoangiography of central serous chorioretinopathy. Arch Ophthalmol 1994; 112:1057-1062.
  12. Ahnoux-Zabsonre A, Quaranta M & Mauget-Faysse M (2004): Prevalence de l’Helicobacter pylori dans la chorioretinopathie sereuse centrale et l’epitheliopathie retinienne diffuse. J Fr ophtalmol 27: 1129–1133.
  13. Cotticelli L, Borrelli M, D’Alessio AC et al. (2006): Central serous chorioretinopathy and Helicobacter pylori. Eur J Ophthalmol 16: 274–278.
  14. Mauget-Faysse M, Kodjikian L, Quaranta M, Ben Ezra D, Trepsat C, Mion F & Megraud F (2002): Ro ˆle de l’Helicobacter pylori dans la choriore ´tinopathie se ´reuse centrale et l’e ´ pithe ´ liiopathie re ´ tinienne diffuse. J Fr Ophtalmol 25: 1021–1025
  15. Castro-Correia J, Coutinho MF, Rosas V & Maia J (1992): Long-term follow-up of central serous retinopathy in 150 patients. Doc Ophthalmol 81: 379–386.
  16. Spaide RF, Campeas L, Haas A et al. (1996): Central serous chorioretinopathy in younger and older adults. Ophthalmology 103: 2070–2079.
  17. Bouzas EA, Scott MH, Mastorakos G, Chrousos GP & Kaiser-Kupfer MI (1993): Central serous chorioretinopathy in endogenous hypercortisolism. Arch Ophthalmol 111: 1229–1233.
  18. Carvalho-Recchia CA, Yannuzzi LA, Negrao S, Spaide RF, Freund KB, Rodriguez-Coleman H, Lenharo M & Iida T (2002): Corticosteroids and central serous chorioretinopathy. phthalmology 109: 1834–1837.
  19. Haimovici R, Gragoudas ES, Duker JS, Sjaarda RN & Eliott D (1997): Central serous chorioretinopathy associated with inhaled or intranasal corticosteroids. Ophthalmology 104: 1653–1660.
  20. Haimovici R, Koh S, Gagnon DR, Lehrfeld T & Wellik S (2004): Risk factors for central serous chorioretinopathy: a case–control study. Ophthalmology 111: 244–249. Haimovici R, Rumelt S & Melby J (2003):
  21. Endocrine abnormalities in patients with central serous chorioretinopathy. Ophthalmology 110: 698–703.
  22. Iwami S (1995): A new method to elicit pathological entoptic phenomenon from the retina–stenopeic flicker test. Nippon Ganka Gakkai Zasshi 99: 595–600.
  23. Gackle HC, Lang GE, Freissler KA & Lang GK (1998): Central serous chorioretinopathy. Clinical, fluorescein angiography and demographic aspects. Ophthalmologe 95:529–533.
  24. Eandi CM, Ober M, Iranmanesh R, Peiretti E & Yannuzzi LA (2005): Acute central serous chorioretinopathy and fundus autofluorescence. Retina 25: 989–993.
  25. Framme C, Walter A, Gabler B, Roider J, Sachs HG & Gabel VP (2005): Fundus autofluorescence in acute and chronic recurrent central serous chorioretinopathy. Acta Ophthalmol Scand 83: 161–167.
  26. Guyer DR, Yannuzzi LA, Slakter JS, Sorenson JA, Ho A & Orlock D (1994): Digital indocyanine green videoangiography of central serous chorioretinopathy. Arch Ophthalmol 112: 1057–1062.
  27. Royce W. S. Chen et al. Speed and Resolution Improve in Newest OCT – Review of Ophthalmology 2007 Pages 84-88.
  1. Srinivasan V, Wojtkowski M, Witkin A, Duker J, et al. High-definition and 3-dimensional imaging of macular pathologies with high-speed ultra high resolution optical coherence tomography. Ophthalmology 2006;113:2054.
  2. Wojtkowski M, Srinivasan V, Fujimoto J, Ko T, et al. Three-dimensional retinal imaging with high-speed ultrahigh-resolution optical coherence tomography. Ophthalmology 2005;112:1734-46.
  3. Puliafito C, Hee M, Lin C, Reichel E, et al. Imaging of macular diseases with optical coherence tomography. Ophthalmology 1995;102:217- 229.
  4. Montero JA, Ruiz-Moreno JM. Optical coherence tomography characterisation of idiopathic central serous chorioretinopathy. Br J Ophthalmol 2005 89: 562-564.
  5. Timothy Y.Y. Lai, et al. The Clinical Applications of Multifocal Electroretinography: A Systematic Review - Surv. Ophthalmol 52:61--96, 200
  1. T. Vajaranant et al. Localized retinal dysfunction in central serous chorioretinopathy as measured using the multifocal Electroretinogram - Ophthalmology, Volume 109, Issue 7, Pages 1243-1250.
  2. Pascal W. Hasler Microperimetry - a method which combines Perimetry and macular topography - Oftalmolog December 2007.
  3. Chen SN, Hwang JF, Tseng LF et al. Subthreshold Diode Micropulse Photocoagulation for the Treatment of Chronic Central Serous Chorioretinopathy with Juxtafoveal Leakage Ophthalmology 2008;115:2229–2234.
  4. Lai TY, Chan WM, Li H, Lai RY, Liu DT & Lam DS (2006): Safety enhanced photodynamic therapy with half dose verteporfin for chronic central serous chorioretinopathy: a short term pilot study. Br J Ophthalmol 90: 869–874.
  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.