CHRONIC CENTRAL SEROUS CHORIORETINOPATHY AND FUNDUS AUTOFLUORESCENCE I-Van Ho, MD, Lawrence Yannuzzi, MD
Background: Chronic presentations of central serous chorioretinopathy (CSC) may be associated with large serous retinal detachments and subretinal exudates. The exudates are believed to be fibrin in acute disease and lipid in chronic detachments. Fundus autofluorescence (FAF) imaging has been used to study lipofuscin within the retinal pigment epithelium (RPE) and the integrity of the RPE tissue layer. Methods: FAF imaging results for two patients with chronic CSC and large dependent retinal detachments were evaluated. Results: As the detachments resolved, subretinal precipitates emerged. They were initially hyperautofluorescent, indicating that they were not lipid, and evolved with normalization of the autofluorescence but no RPE atrophy. Conclusion: The exudates beneath resolving chronic detachments are not lipid in nature, and they are not exclusively from lipofuscin. They are likely from fluorophores generated by detached photoreceptors and liberated into the subretinal space. These observations contribute to the knowledge of chronic CSC and the related visual prognosis. RETINAL CASES & BRIEF REPORTS 2:1–5, 2008
capillaris and RPE.3 Recently, fundus autofluorescence (FAF) imaging has been used to study the pathobiological effects of CSC 4 –7 by documenting variable stages of RPE atrophy, retinal and RPE detachments, and the presence of lipofuscin within the RPE, providing indirect information on the metabolic activity of the RPE tissue layer.8 It is believed that the integrity of the RPE cells may correlate with the degree of FAF, predictive of progression to atrophy in CSC,6 as is known to occur in age-related macular degeneration.9 We report on the FAF characteristics in two cases of CSC, one with a chronic, global serous neurosensory retinal detachment. Our purpose was to evaluate the significance of FAF in chronic disease and to propose an alternative explanation for the material of so-called “lipid deposition” seen after resolution of chronic neurosensory retinal detachment.
From Vitreous, Retina, Macula Consultants of New York and LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear, and Throat Hospital, New York, New York.
C
entral serous chorioretinopathy (CSC), a disease that typically affects young and middle-aged adults, characteristically demonstrates retinal pigment epithelium (RPE) detachment and leaks at the level of the RPE leading to serous retinal detachments.1 Although many acute cases of CSC resolve spontaneously, a more chronic and severe form of the disease may be associated with larger, dependent serous detachments and subretinal exudates. The exudates are believed to be fibrin in acute disease and lipid in association with chronic detachments.2 Despite the lack of histopathological studies, it is evident from fluorescein and indocyanine green angiography that the dysfunction occurs in the chorio-
Case Reports Supported by the Macula Foundation, Inc. (New York, NY). The authors have no financial interests in this report. Reprint requests: Lawrence Yannuzzi, MD, Vitreous, Retina, Macula Consultants of New York, 460 Park Avenue, 5th Floor, New York, NY 10022; e-mail: [email protected]
Case 1 A 37-year-old woman with a history of systemic lupus erythematosus and immune thrombocytopenic purpura presented to an
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Fig. 1. High-resolution color images showing global serous neurosensory detachment extending inferiorly with subretinal fibrin in both eyes (A and B). Fundus fluorescein angiography shows multifocal leaks at the retinal pigment epithelium level with an inferior dependent neurosensory retinal detachment in the right (C) and left (D) eyes.
ophthalmologist with a 3-month history of blurred vision in the right eye. She had global serous retinal detachment bilaterally with minimal associated ocular inflammation and normal blood pressure. The diagnosis of lupus choroidopathy was made, and treatment with systemic steroids and mycophenolate mofetil was instituted. Vision continued to decline, resulting in hospital admission for treatment with intravenous pulsed steroids and weekly intravenous cyclophosphamide. We examined the patient in our office 9 months after her presenting symptoms; visual acuity was 20/50 in the right eye and 20/200 in the left eye. Intraocular pressures were normal, and the anterior chamber and vitreous were quiet. Examination of the fundus showed bilateral chronic multifocal serous neurosensory detachments that extended inferiorly with subretinal fibrin, and the diagnosis of multifocal CSC was confirmed by fundus fluorescein angiography (Fig. 1, A–D). Corresponding optical coherence tomography showed neurosensory retinal detachment and multifocal RPE detachment bilaterally. She underwent focal thermal laser to all the angiographic leakage sites, and treatment with systemic steroids was tapered. The chronic serous inferior retinal detachment resolved over the next 4 months, and vision improved to 20/25 in the right eye and 20/20 in the left eye. FAF imaging demonstrated a residual inferior subretinal yellow deposit with corresponding hyperfluorescence (Fig. 2, A and B). At her last follow-up, 18 months after initial presentation, vision was 20/20 in both eyes with complete resolution of serous neurosensory retinal detachment and resolution of the hyperautofluorescence spots with no underlying atrophic changes (Fig. 2, C and D).
Case 2 A 49-year-old man presented with distortion in the left eye with best-corrected visual acuity of 20/25 in both eyes. A diagnosis of
CSC was made, and it resolved with no active treatment after 7 months. Vision remained unchanged at 20/25 in both eyes. Three years after his initial presentation, optical coherence tomography of the left retina showed no neurosensory detachment but a mildly thickened outer neurosensory retinal layer. FAF imaging also demonstrated a corresponding area of hyperautofluorescence (Fig. 3, A and B). One year later, he remained asymptomatic, and vision remained unchanged at 20/25 in both eyes. Optical coherence tomography of the retina showed smaller but persistent thickening of the outer neurosensory retina, but, interestingly, FAF imaging showed normalization of autofluorescence with no resulting RPE atrophy (Fig. 3, C and D).
Discussion FAF is generally thought to arise predominately from lipofuscin, a mixture of proteins, lipids, and chromophores generated as by-products of the retinoid cycle, located within the RPE cells.8 N-Retinyl-Nretinyldene ethanolamine (A2E) is an important component of lipofuscin with autofluorescence properties and is found within the RPE only after photoreceptor outer segment phagocytosis of A2E precursors.10 Because the RPE is intimately involved in continuous phagocytosis and degradation of shedding photoreceptor outer segments, the accumulation of lipofuscin in human postmitotic RPE is believed to be a result of impaired or overwhelmed RPE lysosomal activity.11 Hence, FAF is commonly thought to be a surrogate marker for RPE integrity as isoautofluorescence
CSC AND FAF
3
Fig. 2. High-resolution color image showing residual yellow subretinal “lipid” deposits inferiorly (A) with corresponding intense hyperautofluorescence (B) after resolution of the chronic serous neurosensory retinal detachment in the right eye. High-resolution color image showing disappearance of the subretinal “lipid” deposits (C) and normalization of fundus autofluorescence with no residual underlying retinal pigment epithelium atrophy (D).
evolves sequentially to hyperautofluorescence and eventually a hypoautofluorescent state. However, autofluorescence has also been described in A2E precursors within the photoreceptor outer seg-
Fig. 3. Fundus autofluorescence image showing an area of hyperfluorescence involving and extending below the fovea corresponding to the previously resolved neurosensory retinal detachment (A) in the left eye. There is subsequent normalization of fundus autofluorescence 1 year later, with no residual underlying retinal pigment epithelium atrophy (C). Optical coherence tomography shows no neurosensory retinal detachment but thickening of the outer neurosensory retina that corresponds to the area of hyperautofluorescence (B). This area of thickening is less but persists 1 year later (D).
ments,12 in devitalized subretinal blood–forming autofluorescent compounds from free-radical attack on photoreceptor outer segment lipids,13 in subretinal macrophages laden with phagocytized outer segments in acute CSC,6 and in shed photoreceptor outer segments in the subretinal space of vitelliform and pseudovitelliform macular dystrophy.14 The findings of these studies help to show that hyper-FAF may not be exclusively from lipofuscin within the RPE and can be a result of other fluorophores within the neurosensory retina or in the subretinal space. After resolution of chronic serous detachment (as in Case 1), clinically evident subretinal precipitates are commonly ascribed to subretinal lipid.15 However, we found that these deposits were intensely hyperautofluorescent in contrast to lipid precipitates seen in CSC, age-related macular degeneration, diabetes, and other retinovascular diseases or in subretinal fibrin deposits, which do not demonstrate hyperautofluorescence (Fig. 4, A–D). Interestingly, 18 months after initial presentation in Case 1, the subretinal precipitates had largely resolved with normalization of FAF and no residual RPE atrophy or degeneration (Fig. 2). In Case 2, with a longer longitudinal follow-up, normalization of the FAF pattern with no residual RPE atrophy was demonstrated (Fig. 3). The explanation for these peculiar observations is not clearly understood. However, we hypothesize that
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Fig. 4. High-resolution color fundus image (A) demonstrates a lipid deposit in the temporal macula of a patient with resolved chronic serous chorioretinopathy (CSC). The corresponding fundus autofluorescence image shows a mottled autofluorescence pattern representing the area of previous CSC involvement, but lipid deposits were isoautofluorescent (B). High-resolution color images of subretinal fibrin in the acute phase of Case 1 (C) that was isoautofluorescent, but an area of retinal pigment epithelium degeneration superior to the subretinal fibrin deposits demonstrated hyperautofluorescence (D).
the presumed lipoprotein precipitates after chronic global serous retinal detachment, as in Case 1, are not lipid. The FAF characteristic of the deposits suggests an alternative material because lipids do not demonstrate hyperautofluorescence. We believe that these deposits may in fact be a mixture of fluorophore compounds derived from the photoreceptor outer segments. These can be in the form of one of the following or a mixture of them: shed photoreceptor outer segments along with their A2E precursor fluorophores14; macrophages filled with photoreceptor outer segments6; and/or fluorophore compounds created from oxidative stress on photoreceptor outer segment membrane lipids.13,16 An alternative hypothesis is that hyperautofluorescence does not exclusively originate from an unhealthy RPE laden with lipofuscin. This is evident in both Case 1 and Case 2, where there was resolution of the hyperautofluorescence with no residual underlying RPE atrophy or degeneration (Figs. 2 and 3)—which is expected when hyperautofluorescence represents unhealthy lipofuscin-laden RPE cells. In Case 1, we believe that the relatively healthy and robust RPE cells were responsible for removal of the subretinal fluorophores, resulting in normalization of the autofluorescence. In Case 2, the hyperfluorescence probably originated from oxidative stress–induced fluorophores within the photoreceptor outer segments that resolved with subsequent RPE phagocytosis and recycling of
the photoreceptor outer segments, essentially the normal metabolic pathway for these molecules. This study provides further evidence that FAF can originate from compounds other than lipofuscin located within the RPE layer and be located in the subretinal space or within the neurosensory retina. Hence, hyper-FAF may not necessarily be predictive of impending RPE atrophy or degeneration. Furthermore, we were previously aware of two types of clinically visible subretinal precipitates in CSC— specifically, fibrin in the acute stage and lipid in the chronic case or secondary to choroidal neovascular complications (Fig. 4). Here, we have described a third type of subretinal precipitate seen in chronic CSC that is characteristically hyperautofluorescent, located anterior to the RPE location, and probably composed of fluorophores derived from the photoreceptor outer segments. The use of FAF imaging allowed us to appreciate the different subretinal precipitates in chronic CSC, leading to better understanding of the pathogenesis and overall clinical nature of this disorder. Key words: autofluorescence, central serous chorioretinopathy, lupus choroidopathy, steroids, subretinal fibrosis, subretinal deposition. References 1. Yannuzzi LA, Shakin JL, Fisher YL, Altomonte M. Peripheral retinal detachments and retinal pigment epithelial tracts
5
CSC AND FAF
2.
3.
4.
5.
6. 7.
8. 9.
secondary to central serous pigment epitheliopathy. Ophthalmol 1984;91:1554 –1572. Spaide RF, Campeas L, Haas A, et al. Central serous chorioretinopathy in younger and older adults. Ophthalmology 1996;103:2070–2079. Piccolino FC, Borgia L. Central serious chorioretinopathy and indocyanine green angiography. Retina 1994;14:231– 242. von Ruckmann A, Schmidt KG, Fitzke FW, et al. Serous central chorioretinopathy: acute autofluorescence of the pigment epithelium of the eye. Ophthalmologe 1999;96:6–10. von Ruckmann A, Fitzke FW, Fan J, et al. Abnormalities of fundus autofluorescence in central serous retinopathy. Am J Ophthalmol 2002;133:780–786. Spaide RF, Klancnik JM. Fundus autofluorescence and central serous chorioretinopathy. Ophthalmology 2005;112:825–833. Eandi CH, Ober M, Iranmanesh R, et al. Acute central serous chorioretinopathy and fundus autofluorescence. Retina 2005; 25:989–993. Delori FC, Dorey CK, Staurenghi G, et al. Invest Ophthalmol Vis Sci 1995;36:718–729. Schmitz-Valckenberg S, Bindewald-Wittch A, Dolar-Szczasny J, et al. Correlation between the area of increased autofluorescence surrounding geographic atrophy and disease
10.
11.
12.
13.
14. 15.
16.
progression in patients with AMD. Invest Ophthalmol Vis Sci 2006;47:2648–2654. Reinboth JJ, Gautschi K, Munz K, et al. Lipofuscin in retina: quantitative assay for an unprecedented autofluorescent compound (pyridinium bis-retinoid, A2-E) of ocular age pigment. Exp Eye Res 1997;65:639–643. Holz FG, Schuett F, Kopitz J, et al. Inhibition of lysosomal degradative functions in RPE cells by a retinoid component of lipofuscin. Invest Ophthalmol Vis Sci 1999;40:737–743. Liu J, Itagaki Y, Ben-Shabat S, et al. The biosynthesis of A2E, a fluorophore of aging retina, involves the formation of precursor, A2-PE, in the photoreceptor outer segment membrane. J Biol Chem 2000;275:29354–29360. Sawa M, Ober M, Spaide RF. Autofluorescence and retinal pigment epithelial atrophy after subretinal hemorrhage. Retina 2006;26:119–120. Spaide RF, Noble K, Morgan A, et al. Vitelliform macular dystrophy. Ophthalmology 2006;113:1392–1400. Yannuzzi LA, Shakin JL, Fisher YL, et al. Peripheral retinal detachments and retinal epithelial atrophic tracts secondary to central serous pigment epitheliopathy. Ophthalmology 1984; 91:1554–1572. Katz ML, Christianson JS, Gao CL, et al. Iron-induced fluorescence in the retina: dependence on vitamin A. Invest Ophthalmol Vis Sci 1994;35:3613–3624.
Background: Chronic presentations of central serous chorioretinopathy (CSC) may be associated with large serous retinal detachments and subretinal exudates. The exudates are believed to be fibrin in acute disease and lipid in chronic detachments. Fundus autofluorescence (FAF) imaging has been used to study lipofuscin within the retinal pigment epithelium (RPE) and the integrity of the RPE tissue layer. Methods: FAF imaging results for two patients with chronic CSC and large dependent retinal detachments were evaluated. Results: As the detachments resolved, subretinal precipitates emerged. They were initially hyperautofluorescent, indicating that they were not lipid, and evolved with normalization of the autofluorescence but no RPE atrophy. Conclusion: The exudates beneath resolving chronic detachments are not lipid in nature, and they are not exclusively from lipofuscin. They are likely from fluorophores generated by detached photoreceptors and liberated into the subretinal space. These observations contribute to the knowledge of chronic CSC and the related visual prognosis. RETINAL CASES & BRIEF REPORTS 2:1–5, 2008
capillaris and RPE.3 Recently, fundus autofluorescence (FAF) imaging has been used to study the pathobiological effects of CSC 4 –7 by documenting variable stages of RPE atrophy, retinal and RPE detachments, and the presence of lipofuscin within the RPE, providing indirect information on the metabolic activity of the RPE tissue layer.8 It is believed that the integrity of the RPE cells may correlate with the degree of FAF, predictive of progression to atrophy in CSC,6 as is known to occur in age-related macular degeneration.9 We report on the FAF characteristics in two cases of CSC, one with a chronic, global serous neurosensory retinal detachment. Our purpose was to evaluate the significance of FAF in chronic disease and to propose an alternative explanation for the material of so-called “lipid deposition” seen after resolution of chronic neurosensory retinal detachment.
From Vitreous, Retina, Macula Consultants of New York and LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear, and Throat Hospital, New York, New York.
C
entral serous chorioretinopathy (CSC), a disease that typically affects young and middle-aged adults, characteristically demonstrates retinal pigment epithelium (RPE) detachment and leaks at the level of the RPE leading to serous retinal detachments.1 Although many acute cases of CSC resolve spontaneously, a more chronic and severe form of the disease may be associated with larger, dependent serous detachments and subretinal exudates. The exudates are believed to be fibrin in acute disease and lipid in association with chronic detachments.2 Despite the lack of histopathological studies, it is evident from fluorescein and indocyanine green angiography that the dysfunction occurs in the chorio-
Case Reports Supported by the Macula Foundation, Inc. (New York, NY). The authors have no financial interests in this report. Reprint requests: Lawrence Yannuzzi, MD, Vitreous, Retina, Macula Consultants of New York, 460 Park Avenue, 5th Floor, New York, NY 10022; e-mail: [email protected]
Case 1 A 37-year-old woman with a history of systemic lupus erythematosus and immune thrombocytopenic purpura presented to an
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2008
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Fig. 1. High-resolution color images showing global serous neurosensory detachment extending inferiorly with subretinal fibrin in both eyes (A and B). Fundus fluorescein angiography shows multifocal leaks at the retinal pigment epithelium level with an inferior dependent neurosensory retinal detachment in the right (C) and left (D) eyes.
ophthalmologist with a 3-month history of blurred vision in the right eye. She had global serous retinal detachment bilaterally with minimal associated ocular inflammation and normal blood pressure. The diagnosis of lupus choroidopathy was made, and treatment with systemic steroids and mycophenolate mofetil was instituted. Vision continued to decline, resulting in hospital admission for treatment with intravenous pulsed steroids and weekly intravenous cyclophosphamide. We examined the patient in our office 9 months after her presenting symptoms; visual acuity was 20/50 in the right eye and 20/200 in the left eye. Intraocular pressures were normal, and the anterior chamber and vitreous were quiet. Examination of the fundus showed bilateral chronic multifocal serous neurosensory detachments that extended inferiorly with subretinal fibrin, and the diagnosis of multifocal CSC was confirmed by fundus fluorescein angiography (Fig. 1, A–D). Corresponding optical coherence tomography showed neurosensory retinal detachment and multifocal RPE detachment bilaterally. She underwent focal thermal laser to all the angiographic leakage sites, and treatment with systemic steroids was tapered. The chronic serous inferior retinal detachment resolved over the next 4 months, and vision improved to 20/25 in the right eye and 20/20 in the left eye. FAF imaging demonstrated a residual inferior subretinal yellow deposit with corresponding hyperfluorescence (Fig. 2, A and B). At her last follow-up, 18 months after initial presentation, vision was 20/20 in both eyes with complete resolution of serous neurosensory retinal detachment and resolution of the hyperautofluorescence spots with no underlying atrophic changes (Fig. 2, C and D).
Case 2 A 49-year-old man presented with distortion in the left eye with best-corrected visual acuity of 20/25 in both eyes. A diagnosis of
CSC was made, and it resolved with no active treatment after 7 months. Vision remained unchanged at 20/25 in both eyes. Three years after his initial presentation, optical coherence tomography of the left retina showed no neurosensory detachment but a mildly thickened outer neurosensory retinal layer. FAF imaging also demonstrated a corresponding area of hyperautofluorescence (Fig. 3, A and B). One year later, he remained asymptomatic, and vision remained unchanged at 20/25 in both eyes. Optical coherence tomography of the retina showed smaller but persistent thickening of the outer neurosensory retina, but, interestingly, FAF imaging showed normalization of autofluorescence with no resulting RPE atrophy (Fig. 3, C and D).
Discussion FAF is generally thought to arise predominately from lipofuscin, a mixture of proteins, lipids, and chromophores generated as by-products of the retinoid cycle, located within the RPE cells.8 N-Retinyl-Nretinyldene ethanolamine (A2E) is an important component of lipofuscin with autofluorescence properties and is found within the RPE only after photoreceptor outer segment phagocytosis of A2E precursors.10 Because the RPE is intimately involved in continuous phagocytosis and degradation of shedding photoreceptor outer segments, the accumulation of lipofuscin in human postmitotic RPE is believed to be a result of impaired or overwhelmed RPE lysosomal activity.11 Hence, FAF is commonly thought to be a surrogate marker for RPE integrity as isoautofluorescence
CSC AND FAF
3
Fig. 2. High-resolution color image showing residual yellow subretinal “lipid” deposits inferiorly (A) with corresponding intense hyperautofluorescence (B) after resolution of the chronic serous neurosensory retinal detachment in the right eye. High-resolution color image showing disappearance of the subretinal “lipid” deposits (C) and normalization of fundus autofluorescence with no residual underlying retinal pigment epithelium atrophy (D).
evolves sequentially to hyperautofluorescence and eventually a hypoautofluorescent state. However, autofluorescence has also been described in A2E precursors within the photoreceptor outer seg-
Fig. 3. Fundus autofluorescence image showing an area of hyperfluorescence involving and extending below the fovea corresponding to the previously resolved neurosensory retinal detachment (A) in the left eye. There is subsequent normalization of fundus autofluorescence 1 year later, with no residual underlying retinal pigment epithelium atrophy (C). Optical coherence tomography shows no neurosensory retinal detachment but thickening of the outer neurosensory retina that corresponds to the area of hyperautofluorescence (B). This area of thickening is less but persists 1 year later (D).
ments,12 in devitalized subretinal blood–forming autofluorescent compounds from free-radical attack on photoreceptor outer segment lipids,13 in subretinal macrophages laden with phagocytized outer segments in acute CSC,6 and in shed photoreceptor outer segments in the subretinal space of vitelliform and pseudovitelliform macular dystrophy.14 The findings of these studies help to show that hyper-FAF may not be exclusively from lipofuscin within the RPE and can be a result of other fluorophores within the neurosensory retina or in the subretinal space. After resolution of chronic serous detachment (as in Case 1), clinically evident subretinal precipitates are commonly ascribed to subretinal lipid.15 However, we found that these deposits were intensely hyperautofluorescent in contrast to lipid precipitates seen in CSC, age-related macular degeneration, diabetes, and other retinovascular diseases or in subretinal fibrin deposits, which do not demonstrate hyperautofluorescence (Fig. 4, A–D). Interestingly, 18 months after initial presentation in Case 1, the subretinal precipitates had largely resolved with normalization of FAF and no residual RPE atrophy or degeneration (Fig. 2). In Case 2, with a longer longitudinal follow-up, normalization of the FAF pattern with no residual RPE atrophy was demonstrated (Fig. 3). The explanation for these peculiar observations is not clearly understood. However, we hypothesize that
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Fig. 4. High-resolution color fundus image (A) demonstrates a lipid deposit in the temporal macula of a patient with resolved chronic serous chorioretinopathy (CSC). The corresponding fundus autofluorescence image shows a mottled autofluorescence pattern representing the area of previous CSC involvement, but lipid deposits were isoautofluorescent (B). High-resolution color images of subretinal fibrin in the acute phase of Case 1 (C) that was isoautofluorescent, but an area of retinal pigment epithelium degeneration superior to the subretinal fibrin deposits demonstrated hyperautofluorescence (D).
the presumed lipoprotein precipitates after chronic global serous retinal detachment, as in Case 1, are not lipid. The FAF characteristic of the deposits suggests an alternative material because lipids do not demonstrate hyperautofluorescence. We believe that these deposits may in fact be a mixture of fluorophore compounds derived from the photoreceptor outer segments. These can be in the form of one of the following or a mixture of them: shed photoreceptor outer segments along with their A2E precursor fluorophores14; macrophages filled with photoreceptor outer segments6; and/or fluorophore compounds created from oxidative stress on photoreceptor outer segment membrane lipids.13,16 An alternative hypothesis is that hyperautofluorescence does not exclusively originate from an unhealthy RPE laden with lipofuscin. This is evident in both Case 1 and Case 2, where there was resolution of the hyperautofluorescence with no residual underlying RPE atrophy or degeneration (Figs. 2 and 3)—which is expected when hyperautofluorescence represents unhealthy lipofuscin-laden RPE cells. In Case 1, we believe that the relatively healthy and robust RPE cells were responsible for removal of the subretinal fluorophores, resulting in normalization of the autofluorescence. In Case 2, the hyperfluorescence probably originated from oxidative stress–induced fluorophores within the photoreceptor outer segments that resolved with subsequent RPE phagocytosis and recycling of
the photoreceptor outer segments, essentially the normal metabolic pathway for these molecules. This study provides further evidence that FAF can originate from compounds other than lipofuscin located within the RPE layer and be located in the subretinal space or within the neurosensory retina. Hence, hyper-FAF may not necessarily be predictive of impending RPE atrophy or degeneration. Furthermore, we were previously aware of two types of clinically visible subretinal precipitates in CSC— specifically, fibrin in the acute stage and lipid in the chronic case or secondary to choroidal neovascular complications (Fig. 4). Here, we have described a third type of subretinal precipitate seen in chronic CSC that is characteristically hyperautofluorescent, located anterior to the RPE location, and probably composed of fluorophores derived from the photoreceptor outer segments. The use of FAF imaging allowed us to appreciate the different subretinal precipitates in chronic CSC, leading to better understanding of the pathogenesis and overall clinical nature of this disorder. Key words: autofluorescence, central serous chorioretinopathy, lupus choroidopathy, steroids, subretinal fibrosis, subretinal deposition. References 1. Yannuzzi LA, Shakin JL, Fisher YL, Altomonte M. Peripheral retinal detachments and retinal pigment epithelial tracts
5
CSC AND FAF
2.
3.
4.
5.
6. 7.
8. 9.
secondary to central serous pigment epitheliopathy. Ophthalmol 1984;91:1554 –1572. Spaide RF, Campeas L, Haas A, et al. Central serous chorioretinopathy in younger and older adults. Ophthalmology 1996;103:2070–2079. Piccolino FC, Borgia L. Central serious chorioretinopathy and indocyanine green angiography. Retina 1994;14:231– 242. von Ruckmann A, Schmidt KG, Fitzke FW, et al. Serous central chorioretinopathy: acute autofluorescence of the pigment epithelium of the eye. Ophthalmologe 1999;96:6–10. von Ruckmann A, Fitzke FW, Fan J, et al. Abnormalities of fundus autofluorescence in central serous retinopathy. Am J Ophthalmol 2002;133:780–786. Spaide RF, Klancnik JM. Fundus autofluorescence and central serous chorioretinopathy. Ophthalmology 2005;112:825–833. Eandi CH, Ober M, Iranmanesh R, et al. Acute central serous chorioretinopathy and fundus autofluorescence. Retina 2005; 25:989–993. Delori FC, Dorey CK, Staurenghi G, et al. Invest Ophthalmol Vis Sci 1995;36:718–729. Schmitz-Valckenberg S, Bindewald-Wittch A, Dolar-Szczasny J, et al. Correlation between the area of increased autofluorescence surrounding geographic atrophy and disease
10.
11.
12.
13.
14. 15.
16.
progression in patients with AMD. Invest Ophthalmol Vis Sci 2006;47:2648–2654. Reinboth JJ, Gautschi K, Munz K, et al. Lipofuscin in retina: quantitative assay for an unprecedented autofluorescent compound (pyridinium bis-retinoid, A2-E) of ocular age pigment. Exp Eye Res 1997;65:639–643. Holz FG, Schuett F, Kopitz J, et al. Inhibition of lysosomal degradative functions in RPE cells by a retinoid component of lipofuscin. Invest Ophthalmol Vis Sci 1999;40:737–743. Liu J, Itagaki Y, Ben-Shabat S, et al. The biosynthesis of A2E, a fluorophore of aging retina, involves the formation of precursor, A2-PE, in the photoreceptor outer segment membrane. J Biol Chem 2000;275:29354–29360. Sawa M, Ober M, Spaide RF. Autofluorescence and retinal pigment epithelial atrophy after subretinal hemorrhage. Retina 2006;26:119–120. Spaide RF, Noble K, Morgan A, et al. Vitelliform macular dystrophy. Ophthalmology 2006;113:1392–1400. Yannuzzi LA, Shakin JL, Fisher YL, et al. Peripheral retinal detachments and retinal epithelial atrophic tracts secondary to central serous pigment epitheliopathy. Ophthalmology 1984; 91:1554–1572. Katz ML, Christianson JS, Gao CL, et al. Iron-induced fluorescence in the retina: dependence on vitamin A. Invest Ophthalmol Vis Sci 1994;35:3613–3624.
