1. In the name of God Central serous chorioretinopathy

2. Hamid Fesharaki MD, Eye Dpt Isfahan University of medical sciences

3. CSC (CSR) Development of a well cicumscribed serous detachment of the sensory retina resulting from altered barrier and deficient pumping functions of the RPE. Although the primary pathology may involve the choriocapillaris. Small whitish sub-retinal precipitates, or gray- white subretinal sheets (fibrin) may be noted.

4. The underlying cause of the problem is an unexplained defect in the vascular layer supporting the retinal pigment epithelium (the choroicapillaris) which becomes more leaky and creates a small blister in the retinal pigment epithelium. A defect in the RPE lets fluid escape under the retina and this is usually associated with clinical symptoms. Some cases may also show serous detachments of the RPE or PED usually under the superior half of the serous detachment.

6. Symptoms of CSR This condition usually occurs in patients between the ages of 30 and 50 and presents with mild blurring of vision in one eye. Another common symptom is feeling that objects appear smaller in the affected eye (micropsia).

7. Examination may reveal that both eyes are affected even if symptoms are confined to one eye only. CSC is often bilateral with asymmetric findings. The condition is usually diagnosed by clinical examination alone. although for more difficult cases further investigations using F A, and O C T, may be employed. CSC

8. CSC is common in Caucasians, Asians, and Hispanics, and rare in African Americans. Symptomatic patients describe the sudden onset of blurred and dim vision, micropsia (objects appear smaller than they are), metamorphopsia (objects appear distorted), paracentral scotomata, or decreased color vision. vision ranges from 20/20 to 20/200, but in most patients vision is better than 20/30. The decreased vision can often be corrected with a hyperopic correction. In rare cases, these symptoms are accompanied by a migraine-like headache. CSC

9. Certain personality types, including type A personality, hypochondria, hysteria, conversional neurosis, and psychiatric medication use have been associated with CSC, although no proven association with any personality has been made. Patients with elevated levels of corticosteroids due to either corticosteroid administration (inhaled, topical, or systemic) or Cushing syndromeare at an increased risk of developing esc. Finally, stress has also been implicatedas an etiologic factor, but no conclusive proof has been presented. CSC

10. Fluorescein Angiography of CSC Three characteristic fluorescein angiographic patterns are seen in esc: 1.expansile dot pattern 2.smokestack pattern 3. diffuse pattern

11. An expansile dot of hyperfluorescence is the most common presentation. The dot representsa small, focal hyperfluorescent leak from the choroid through the RPE that appears in the early phase of the angiogram and increases in size and intensity as the angiogram progresses. FA in CSR Expansile dot pattern

12. Smokestack & subretinal pooling

13. FA in CSC Smokestack pattern

14. Fluorescein dye also slowly pools into the sub retinal detachment as the angiogram progresses Late-phase frames of the angiogram at 10 or 15 minutes are often required to detect very slow leaks or to discern the extent of fluorescein pooling in the subsensory retinal space. Fluorescein Angiography of CSC

15. Angiogram 10 minutes post dye injection showing l eakage Angiogram 20 minutes post dye injection showing leakage

16. Diffuse leakage

17. Fluorescein Angiography of CSC In rare cases, an extensive, often gravity-dependent, serous detachment of the retina may develop from one or more leak points outside the posterior pole. This situation producesa diffuse pattern of fluorescein leakage, often without any obvious leakage point. Patient s with this condition often have large areas of serous detachment and extensive RPE changes. Thus, CSC must be considered in the differential diagnosis of nonrhegmatogenous serous retinal detachment. Indolent cases also occur, in which fluid moves chronically from the choroid to the subretinal space and causes areas of abnormal RPE to expand.

18. Central Serous Retinopathy (CSR) 1. a large, elevated area of serous retinal detachment can be seen occupying much of the temporal macula. Within the central area of this detachment, there appears to be a second, smaller, ring of elevation. FA and OCT confirmed that this smaller elevated ring is a PED within the larger area of serous retinal detachment.

19. 2. Early views of the FA demonstrate a hot spot of hyperflourescence that spreads to fill the PED.

20. 3. Late views with flourescein reveal the pooling of fluid within the serous detachement, correlating with the clinical appearance of the fundus.

21. ّFA Multispot leakage in CSC In some patients, several leaking expansile dots may be present. If no expansile dot is seen in the macula, the extramacular space, especially superiorly, should be evaluated.

22. Figure 10-3: Fluorescein angiogram of central serous chorioretinopathy shows active disease with both a RPE detachment (small arrows) and a sensory retinal detachment (large arrows). Two foci of inactive disease (open arrows) are also present. Figure 10-2: Central serous chorioretinopathy with sensory retinal detachment (arrows) extending into the fovea.

23. Fluorescein Angiography of CSC Leakage should not be interpreted as the only reason for the accumulation of subretinalfluid. Although a leak is necessary for fluid to enter the subretinal space, this fluid would normally be removed promptly by the RPE/ choroid. However, the fluid continues to accumulate because the primary disease is probably a diffuse abnormality of the RPE/choroid that impairs fluid removal. Thus, a localized serous detachment of the RPE without overlying neurosensory elevation can be seen.

24. persistent CSR Vision usually improved to 95% of its original level by 3 months without specific treatment. Recurrences occur in 30% of patients and in a very small number, the condition may become chronic. In these patients the treatment options include Diamox tablets, betablocker tablets and photodynamic laser therapy. This is a picture of a fundus in a patient with persistent CSR.

25. This is an early FA picture of this patient

26. late FA picture

27. RPE atrophic areas indicate previous episodes of CSC

28. 47-year-old man had CSC persisting for 6 months in the right eye. Note the subretinal precipitates and RPE defects. (b) FFA showed a subfoveal inkblot leak, and an extrafoveal smokestack. Faint hyperfluorescence of RPE defects was also evident. (c) The CSC resolved spontaneously over 6 months with more precipitates and RPE mottling; vision improved marginally (20/200–20/120). (d) Angiogram confirmed the absence of leakage, and showed RPE window defects in and around fovea.

29. Dry AMD

30. Other Imaging Modalities for CSC: Optical coherence tomography (OCT) is an excellent, noninvasive method to use for diagnosing and following the resolution of the subretinal fluid in cSc. Subtle fluid accumulation beneath the sensory retina and the RPE not evident on fluorescein angiography (FA) and clinical examination can often be picked up by OCT. Once the diagnosis is established, OCT can be used to follow and document the resolution of the subretinal fluid.

31. Other Imaging Modalities for CSC: Optical coherence tomography (OCT) is an excellent, noninvasive method to use for diagnosing and following the resolution of the subretinal fluid in CSC. Subtle fluid accumulation beneath the sensory retina and the RPE not evident on fluorescein angiography (FA) and clinical examination can often be picked up by OCT. Once the diagnosis is established, OCT can be used to follow and document the resolution of the subretinal fluid.

32. CSR

35. (Patient #1). OCT fundus photograph of the left eye showing an area of PED (thick arrow) and a possible leakage site (thin arrow) (top left) in a patient with CSC. The corresponding fluorescein angiogram shows hyperfluorescence corresponding to PED (thick arrow) and smokestack pattern of leak (thin arrow) (top right). Raster line scan shows dome-like elevation of PED (thick arrow) and irregular undulations of RPE at the leakage site (thin arrow). The RPE breach is not seen in this scan (bottom).

36. ( Patient #2) Fundus fluorescein angiogram of the left eye of a patient with acute CSC. The arrow indicates the leakage site to be studied (top left). OCT fundus photograph shows the placement of the slice navigator at the point of interest to be studied further (top right). The Raster line scan through the leakage site shows irregular retinal pigment epithelium (RPE) with a microrip (arrow) (bottom left).

37. OCT images demonstrate a discrete blister of fluid underneath the RPE just temporal to the foveal depression. This defines the PED. The overlying serous retinal detachement is also evident.

38. CSR

39. Fundus autofluorescence demonstrates hypoautofluorescence corresponding precisely to the site of the focal RPE leak seen on FA, as well as pigment mottling in the area of the RPE disturbance. In addition, central macular autofluorescence correlates with the level of central geographic retinal pigment epithelial atrophy, and lower levels are associated with poorer vision. Other Imaging Modalities for CSC:

41. Central Serous Retinopathy Acute - Fundus Autofluorescence 42-year-old man was seen in the office on October 5, 2011. He had noticed starting in August after a course of antibiotic and steroids, that he developed new spots in his vision in the right eye. He may have had an episode like this sometime in the past. He did take steroids a few years ago and his vision did change at that time, but then returned.

42. Central Serous Retinopathy Acute - Fluorescein Angiogram 42-year-old man was seen in the office on October 5, 2011. He had noticed starting in August after a course of antibiotic and steroids, that he developed new spots in his vision in the right eye. He may have had an episode like this sometime in the past. He did take steroids a few years ago and his vision did change at that time, but then returned.

43. Chorioretinal atrophy - areas of cell death within the RPE and adjacent tissue layers - is a key feature of AMD.

44. Other Imaging Modalities for CSC: Indocyanine green (ICG) angiography can be used to show choroidal vascular abnormalities, including filling delays in the choroidal arteries and choriocapillaris, venous dilation, hyperpermeability of the choroidal vessels, and characteristic multifocal choroidal hyperfluorescent patches that appear early in the angiogram.These areas slowly enlargeduring the angiogram but are less prominent in late views. In addition, a characteristic washout pattern is often evident that remains unchanged during clinicallyinactive phases. ICG can be useful in helping to distinguish atypical diffuse CSC in older patients from occult CNV in exudative AMD, idiopathic polypoidal choroidal vasculopathy.

45. Central Serous Retinopathy Acute - Indocyanine Green Angiogram - Leaky Choriocapillaris 42-year-old man was seen in the office on October 5, 2011. He had noticed starting in August after a course of antibiotic and steroids, that he developed new spots in his vision in the right eye. He may have had an episode like this sometime in the past. He did take steroids a few years ago and his vision did change at that time, but then returned. ICG in CSC

46. Differential Diagnosis The presence of subretinal fluid in older patients with CSC requires the physician to also considera diagnosis of CNV associated with age-related macular degeneration optic nerve pits idiopathic polypoidal choroidal vasculopathy idiopathic uveal effusion syndrome (lUES). Features that help to differentiate CSC from these other entities include the following: A pinpoint leak relative to a large area of subretinal fluid most likely representsCSC. whereas the area of subretinal fluid associated with CNV and idiopathic polypoidal choroidal vasculopathy usually corresponds closely to the area of leakageon angiography. The leakage in lUES is usually diffuse.

47. Dry AMD

48. Wet AMD

52. Occult CNV

53. Multilobulated PED

54. Dlsciform scar

55. Differential diagnosis: Optic nerve pits are often visible on the nerve and are contiguous with the schisiscavity and/or subretinal fluid accumulation. No pinpoint leakage is seen..Multifocal RPE abnormalities, including small serous pigment epithelial detachments(PED) in 1 or both eyes, more likely represent CSC whereas the presence of large drusen more likely represents age-related macular degeneration The yellow-white exudates of Vogt-Koyanagi-Harada syndrome can appear similar to CSC; however, the granulomatous uveitis seen in the former helps differentiate the diseases. Saccular outpouchings are characteristic of idiopathic polypoidal choroidal vasculopathy; these can be differentiated from the multifocal hyperfluorescent patches of CSC through ICG angiography.

56. Optic disc Pit

57. The patient underwent a standard vitrectomy, removal of the posterior hyaloid, with perfluorocarbon, laser photocoagulation, fluid-gas exchange and 12% perfluoropropane (C 3 F 8 ) injection. The whole procedure was explained to the patient beforehand. The drainage of subretinal fluid was performed under direct visualization (Landers flat lens) through a 39- gauge cannula. In addition, a sample of the vitreous humor was taken for comparative analysis. There were no intercurrent events during the surgical procedure

58. Polypoidal lesions in AMDPolypoidal lesions in AMD Patients with typical wet age-related macular degeneration (AMD) may also have signs of polypoidal choroidal lesions using indocyanine gree n angiography (ICGA), according to a new study. Polypoidal choroidal vasculopathy (PCV) is a condition characterized by multiple, recurrent, serosanguinous PED

59. late geographic hyperfluorescence (LGH) on indocyanine green angiography (ICGA) in cases of polypoidal choroidal vasculopathy (PCV).

60. ICG1 FA1 FA2 ICG2 Polypoidal choroidal vasculopathy

61. VKH

63. VMT

64. Differential diagnosis: In elderly patients, lymphoma can appear similar to CSC, but lymphoma is usually multifocal and bilateral..Absence of blood or significant lipid is more likely to represent CSC, whereas thepresence of these signs is more likely to represent CNV or idiopathic polypoidal choroidal vasculopathy. Older patients presenting with CNV occasionally may show evidence suggestive of previous CSC.

65. lymphoma

66. Preoperative fundus photography (a) revealed almost total exudative retinal detachment and bilateral retinal vascular dilation and tortuosity. Fundus photographic (b), FA (c), ICGA (d), FAF (e), and SD-OCT (f) images at 2 months after the final surgery. Red circles (c, d, e and f) indicate the same location in the fundus. Green lines indicate the scan positions on SD-OCT image (f). Scattered pigmentary spots appeared in the fundus photograph (b). FA showed the leopard-spot pattern of hypofluorescence (c) and ICGA demonstrated hypofluorescent spots on a background of diffuse hyperfluorescence of the choroid (d). Scattered hyperfluorescent spots were observed on FAF imaging (e). SD-OCT revealed multiple focal thickening of the RPE layer. (f). Comparing the images in the red circles, the RPE lesion appeared hypofluorescent on FA (c) and ICGA (d), and hyperfluorescent on FAF imaging (e) nanophthalmic uveal effusion syndrome

67. I U E S

68. Inferior RD

69. C M E

70. Swelling in the macular edema results from fluid build up and thickening within the layers of retinal tissue. Tiny blood vessels which surround the macula are usually responsible for the leakage. Many disorders including diabetes, vein occlusions, uveitis (inflammation), and cataract surgery can cause macular edema

71. Cellophane maculopathy

72. Macular schisis in a case of juvenile retinoschisis is shown in the fundus image above X-LINKED HEREDITARY RETINOSCHISIS FAMILIAL EXUDATIVE VITREORETINOPATHY

73. Commotio Retinae

74. Macular hole C R A O

75. Natural Course and Management. The visual prognosis of CSC is usually good except in chronic, recurrent cases and in casesof bullous CSC Most eyes with CSC (80%-90%) undergo spontaneous resorption of subretinalfluid within 3-4 months; recovery of visual acuity usually follows, but can take upto a year. Mild metamorphopsia, faint scotomata, abnormalities in contrast sensitivity, andmild color vision deficits frequently persist. Some eyes suffer permanently diminished visual acuity, and many (40%-50%) experience one or more recurrences. A small subset of patients have poor visual outcomes.

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