CENTRAL SEROUS CHORIORETINOPATHY AND PERIPHERAL RETINAL NEOVASCULARIZATION Irene A. Barbazetto, MD,* Marco Cinquini, MD,* Beatriz S. Takahashi, MD,* Lawrence A. Yannuzzi, MD*†
Purpose: To report a case of bullous retinal detachment complicated by peripheral retinal ischemia and neovascularization in a patient with chronic central serous chorioretinopathy (CSC). Results: Focal laser photocoagulation to the active retinal pigment epithelial leaks in the posterior pole resulted in resolution of the bullous detachment and regression of the retinal neovascularization. Conclusions: Patients with chronic CSC and a large, dependent serous detachment with peripheral retinal ischemia and neovascularization may be managed by focal treatment to active pigment epithelial leaks alone. This could spare patients of the adverse effects associated with widespread laser treatment and reduce the risk of visual loss due to vitreous hemorrhage and/or more severe complications resulting from progressive retinal ischemia. RETINAL CASES & BRIEF REPORTS 3:224 –227, 2009
From *Vitreous-Retina-Macula Consultants of New York and †The LuEsther T. Mertz Retinal Research Center of the Manhattan Eye, Ear & Throat Hospital, New York, New York.
changes were present in both eyes, along with a few serous pigment epithelial detachments (PED) which were evident clinically. One conspicuous PED with pigment epithelial hyperplastic figures was present in the superior perimacular region of the right eye. That eye also had a ridge of lipid deposition in the inferior peripheral fundus (Figure 1). The fluorescein angiography demonstrated a focal leak at the margin of the large serous pigment epithelial PED in the right eye with pooling into the subneurosensory retinal space, descending inferiorly to conform to the dependent detachment. There was also some decompensation of the pigment epithelium with slow and rather diffuse leakage in both eyes. In the inferior peripheral fundus of the right eye, there was peripheral ischemia, dilated telangiectatic changes at the junction between perfused and nonperfused retina, and peripheral neovascularization at that site as well (Figure 2). Given the active pigment epithelial leak identified angiographically, laser photocoagulation was carried out to that site. No additional treatment was attempted at the site of the peripheral ischemia in order to induce a regression of the associated neovascularization. The left eye was treated in a similar fashion to active focal leaks. Two months later, there was complete resolution of the detachments in each eye, unveiling the atrophic and pigmentary pigment epithelial degenerative changes. There was also a transient increase in the lipid deposition, but eventually clearing by 9 months after treatment (Figure 3). At that time, the visual acuity was slightly but meaningfully improved in each eye to the level of 20/70 in the right and 20/40 in the left.
Case Report A 46-year-old white woman presented with complaints of progressive decline in the vision of her right eye more than her left over a period of several months. At baseline, her bestcorrected visual acuity was 20/200 in the right and 20/50 in the left. There was no significant medical abnormality systemically, specifically the use of corticosteroids, pregnancy, or organ transplant. On clinical examination, there was a bilateral neurosensory retina detachment in the macula and a bullous dependent neurosensory detachment, again, in the right eye more than the left. Widespread pigment epithelial atrophic and pigmentary
Supported by The Macula Foundation, Inc. The authors do not have any financial interest in the subject matter. Reprint requests: Lawrence A. Yannuzzi, MD, The LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear & Throat Hospital, 210 East 64th Street, New York, NY 10065; e-mail: [email protected]
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Fig. 3. Following laser treatment increased precipitation of intraretinal and subretinal yellow exudates typical of a resolving retinal detachment were observed. Fig. 1. Left eye demonstrates yellow deposition in the macular area and peripheral lipid-like deposits demarcating an inferior dependent detachment.
While there was some remottling of the capillary circulation in the inferior peripheral fundus of the right eye, normal, complete perfusion of the peripheral retina did not occur (Figure 4). By 1 year later, there was no recurrence of the detachment in either eye.
Fig. 2. Fluorescein angiogram of the left eye shows obliteration of retinal capillaries and neovascularization at the junction between perfused and non-perfused retina (small arrow). The responsible retinal pigment epithelial leak is located superior-nasal to the disc in the paramacular area (large arrow).
Discussion Choroidal central serous chorioretinopathy (CSC) is a disorder that can mimic several other macular and retinal diseases, like age-related macular degeneration (AMD), polypoidal choroidal vasculopathy (PCV), or in rare instances, even Harada disease or the uveal effusion syndrome. While CSC is usually associated with small to moderated sized shallow, serous, retinal detachments and PEDs in association with focal pigment epithelial leaks, a small subset of patients have been reported to present with large, bullous, serous retinal detachments.1– 6 Peripheral neovascularization, an uncommon finding in chronic CSC, is believed to be the result of retinal ischemia, which can develop in these dependent detachments once they become chronic.3 These cases can be of diagnostic and therapeutic challenge, especially when occurring in the absence of contributing systemic conditions like corticosteroid therapy, hemodialysis, pregnancy, or organ transplantation. Advanced imaging technologies including fluorescein and indocyanine green (ICG) angiography, as well as optical coherence tomography (OCT), have proven to be of value in establishing the correct diagnosis and in guiding various forms of therapy.7–11 The purpose of this report is to describe a patient with this severe, chronic form of CSC with a dependent retinal detachment complicated by peripheral retinal ischemia and neovascularization, managed successfully with focal laser photocoagulation to a retinal pigment epithelial leak in the perimacular area. The natural course of CSC is usually very good with spontaneous resolution of neurosensory detachments. Recurrences are known to occur in some peo-
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Fig. 4. Color photography (A) and fluorescein angiogram (B) of the left eye show the absence of active leakages and peripheral ischemia with resolution of the dependent detachment 6 months after focal laser treatment.
ple, particularly those at high risk from abnormal behavioral patterns,12 the use of corticosteroids, organ transplants, or both. None of these risk factors were evident in our patient. Chronic-recurrent exudative disease or merely persistent detachment may lead to gravitating, dependent detachments or even bullous elevations in the retina. It is also known that longstanding retinal detachments from any cause may result in secondary retinal vascular changes, including capillary telangiectasia, retinal ischemia, and in rare cases, neovascularization, exactly as seen in our patient. Furthermore, in chronic CSC, even a singular focal RPE leak could be the causative factor for a dependent detachment. These active focal leaks are frequently located at the junction between attached and detached retinal pigment epithelium and can present with visible areas of retinal pigment epithelial “blow-out” or so-called micropigment epithelial rips.13 Our patient had such an abnormal PED, which was the source of active leakage and chronic, inferior detachment (Figure 1). In this case, the chronic detachment led to overlaying telangiectatic vascular changes, an extensive area of ischemia, and ultimately retinal neovascularization. Treatment of chronic CSC with persistent dependent detachments is a matter of controversy. All studies have been retrospective and uncontrolled in nature. Most have demonstrated a rapid resolution of the neurosensory detachments, but without indisputable evidence of visual acuity benefit compared to untreated patients.4,6,11,14 Chronic detachment of the macula is known to be a risk for photoreceptor as well as pigment epithelial degenerative change and cystic degeneration within the retina itself. In our patient, there were double risk factors, the pres-
ence of peripheral ischemia and neovascularization, which may result in severe vision loss due to other mechanisms, such as vitreous hemorrhage, and/or tractional detachments. Generally, laser photocoagulation treatment for peripheral neovascularization is carried out with scattered treatment to the associated ischemic zones. However, given the technical difficulty inherent in laser treatment of detached retina, it was elected to selectively photocoagulate the active RPE leak without additional laser treatment to the peripheral ischemic retina. The result was typical of resolving retinal detachment with precipitation of intraretinal and subretinal yellow exudates. The progressive resolution of the dependent detachment was associated with an increase in the lipid-like precipitation, which was not unexpected. The serous component of the detachment is presumed to diffuse into the choroidal circulation leaving a legacy of precipitating larger lipophilic molecules. The sequence is also known to occur following resolution of other retinal exudative abnormalities such as diabetic retinopathy or Coats disease. In our patient the detachment resolved, the neovascularization regressed, and the lipid-like exudation initially increased slowly but clearly over several months. The nature or composition of these yellow exudates has recently been described to contain a chromophore, evident with fundus autofluorescence and presumed to be the result of disseminated photoreceptor outer segments into the subretinal space.15,16 In summary, we present a rare case of retinal neovascularization overlying a chronic dependent detachment in a patient with CSC. Of particular importance in the management of this patient was the evolution of intraretinal abnormalities following the selective treatment of the RPE leak. The minimally interventional
227
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therapeutic approach was effective in resolving the detachment, inducing a regression of the neovascularization and eliminating the need for widespread laser treatment.
8. 9.
References 1.
2.
3.
4.
5.
6.
7.
Akiyama K, Kawamura M, Ogata T, Tanaka E. Retinal vascular loss in idiopathic central serous chorioretinopathy with bullous retinal detachment. Ophthalmology 1987;94:1605–1609. Gass JD. Bullous retinal detachment. An unusual manifestation of idiopathic central serous choroidopathy. Am J Ophthalmol 1973;75:810–821. Gass JD, Little H. Bilateral bullous exudative retinal detachment complicating idiopathic central serous chorioretinopathy during systemic corticosteroid therapy. Ophthalmology 1995;102:737–747. Otsuka S, Ohba N, Nakao K. A long-term follow-up study of severe variant of central serous chorioretinopathy. Retina 2002;22:25–32. Paulmann H, Heimann K. [Contribution to the problem of central serous retinal detachment (authors’ transl)]. Klin Monatsbl Augenheilkd 1975;167:324–332. Sahu DK, Namperumalsamy P, Hilton GF, de Sousa NF. Bullous variant of idiopathic central serous chorioretinopathy. Br J Ophthalmol 2000;84:485–492. Iida T, Yannuzzi LA, Spaide RF, et al. Cystoid macular
10.
11.
12. 13. 14.
15. 16.
degeneration in chronic central serous chorioretinopathy. Retina 2003;23:1–7. Slakter JS, Yannuzzi LA, Guyer DR, et al. Indocyanine-green angiography. Curr Opin Ophthalmol 1995;6:25–32. Spaide RF, Hall L, Haas A, et al. Indocyanine green videoangiography of older patients with central serous chorioretinopathy. Retina 1996;16:203–213. Yannuzzi LA, Freund KB, Goldbaum M, et al. Polypoidal choroidal vasculopathy masquerading as central serous chorioretinopathy. Ophthalmology 2000;107:767–777. Yannuzzi LA, Slakter JS, Gross NE, et al. Indocyanine green angiography-guided photodynamic therapy for treatment of chronic central serous chorioretinopathy: a pilot study. Retina 2003;23:288–298. Yannuzzi LA. Type A behavior and central serous chorioretinopathy. Trans Am Ophthalmol Soc 1986;84:799–845. Goldstein BG, Pavan PR. ‘Blow-outs’ in the retinal pigment epithelium. Br J Ophthalmol 1987;71:676–681. Yannuzzi LA, Shakin JL, Fisher YL, Altomonte MA. Peripheral retinal detachments and retinal pigment epithelial atrophic tracts secondary to central serous pigment epitheliopathy. Ophthalmology 1984;91:1554–1572. Spaide RF, Klancnik JM Fundus autofluorescence and central serous chorioretinopathy. Ophthalmology 2005;112:825–833. Ho IV, Yannuzzi LA. Chronic central serous chorioretinopathy and fundus autofluorescence. Retinal Cases & Brief Reports 2008;2:1–5.
Purpose: To report a case of bullous retinal detachment complicated by peripheral retinal ischemia and neovascularization in a patient with chronic central serous chorioretinopathy (CSC). Results: Focal laser photocoagulation to the active retinal pigment epithelial leaks in the posterior pole resulted in resolution of the bullous detachment and regression of the retinal neovascularization. Conclusions: Patients with chronic CSC and a large, dependent serous detachment with peripheral retinal ischemia and neovascularization may be managed by focal treatment to active pigment epithelial leaks alone. This could spare patients of the adverse effects associated with widespread laser treatment and reduce the risk of visual loss due to vitreous hemorrhage and/or more severe complications resulting from progressive retinal ischemia. RETINAL CASES & BRIEF REPORTS 3:224 –227, 2009
From *Vitreous-Retina-Macula Consultants of New York and †The LuEsther T. Mertz Retinal Research Center of the Manhattan Eye, Ear & Throat Hospital, New York, New York.
changes were present in both eyes, along with a few serous pigment epithelial detachments (PED) which were evident clinically. One conspicuous PED with pigment epithelial hyperplastic figures was present in the superior perimacular region of the right eye. That eye also had a ridge of lipid deposition in the inferior peripheral fundus (Figure 1). The fluorescein angiography demonstrated a focal leak at the margin of the large serous pigment epithelial PED in the right eye with pooling into the subneurosensory retinal space, descending inferiorly to conform to the dependent detachment. There was also some decompensation of the pigment epithelium with slow and rather diffuse leakage in both eyes. In the inferior peripheral fundus of the right eye, there was peripheral ischemia, dilated telangiectatic changes at the junction between perfused and nonperfused retina, and peripheral neovascularization at that site as well (Figure 2). Given the active pigment epithelial leak identified angiographically, laser photocoagulation was carried out to that site. No additional treatment was attempted at the site of the peripheral ischemia in order to induce a regression of the associated neovascularization. The left eye was treated in a similar fashion to active focal leaks. Two months later, there was complete resolution of the detachments in each eye, unveiling the atrophic and pigmentary pigment epithelial degenerative changes. There was also a transient increase in the lipid deposition, but eventually clearing by 9 months after treatment (Figure 3). At that time, the visual acuity was slightly but meaningfully improved in each eye to the level of 20/70 in the right and 20/40 in the left.
Case Report A 46-year-old white woman presented with complaints of progressive decline in the vision of her right eye more than her left over a period of several months. At baseline, her bestcorrected visual acuity was 20/200 in the right and 20/50 in the left. There was no significant medical abnormality systemically, specifically the use of corticosteroids, pregnancy, or organ transplant. On clinical examination, there was a bilateral neurosensory retina detachment in the macula and a bullous dependent neurosensory detachment, again, in the right eye more than the left. Widespread pigment epithelial atrophic and pigmentary
Supported by The Macula Foundation, Inc. The authors do not have any financial interest in the subject matter. Reprint requests: Lawrence A. Yannuzzi, MD, The LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear & Throat Hospital, 210 East 64th Street, New York, NY 10065; e-mail: [email protected]
224
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Fig. 3. Following laser treatment increased precipitation of intraretinal and subretinal yellow exudates typical of a resolving retinal detachment were observed. Fig. 1. Left eye demonstrates yellow deposition in the macular area and peripheral lipid-like deposits demarcating an inferior dependent detachment.
While there was some remottling of the capillary circulation in the inferior peripheral fundus of the right eye, normal, complete perfusion of the peripheral retina did not occur (Figure 4). By 1 year later, there was no recurrence of the detachment in either eye.
Fig. 2. Fluorescein angiogram of the left eye shows obliteration of retinal capillaries and neovascularization at the junction between perfused and non-perfused retina (small arrow). The responsible retinal pigment epithelial leak is located superior-nasal to the disc in the paramacular area (large arrow).
Discussion Choroidal central serous chorioretinopathy (CSC) is a disorder that can mimic several other macular and retinal diseases, like age-related macular degeneration (AMD), polypoidal choroidal vasculopathy (PCV), or in rare instances, even Harada disease or the uveal effusion syndrome. While CSC is usually associated with small to moderated sized shallow, serous, retinal detachments and PEDs in association with focal pigment epithelial leaks, a small subset of patients have been reported to present with large, bullous, serous retinal detachments.1– 6 Peripheral neovascularization, an uncommon finding in chronic CSC, is believed to be the result of retinal ischemia, which can develop in these dependent detachments once they become chronic.3 These cases can be of diagnostic and therapeutic challenge, especially when occurring in the absence of contributing systemic conditions like corticosteroid therapy, hemodialysis, pregnancy, or organ transplantation. Advanced imaging technologies including fluorescein and indocyanine green (ICG) angiography, as well as optical coherence tomography (OCT), have proven to be of value in establishing the correct diagnosis and in guiding various forms of therapy.7–11 The purpose of this report is to describe a patient with this severe, chronic form of CSC with a dependent retinal detachment complicated by peripheral retinal ischemia and neovascularization, managed successfully with focal laser photocoagulation to a retinal pigment epithelial leak in the perimacular area. The natural course of CSC is usually very good with spontaneous resolution of neurosensory detachments. Recurrences are known to occur in some peo-
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2009
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NUMBER 2
Fig. 4. Color photography (A) and fluorescein angiogram (B) of the left eye show the absence of active leakages and peripheral ischemia with resolution of the dependent detachment 6 months after focal laser treatment.
ple, particularly those at high risk from abnormal behavioral patterns,12 the use of corticosteroids, organ transplants, or both. None of these risk factors were evident in our patient. Chronic-recurrent exudative disease or merely persistent detachment may lead to gravitating, dependent detachments or even bullous elevations in the retina. It is also known that longstanding retinal detachments from any cause may result in secondary retinal vascular changes, including capillary telangiectasia, retinal ischemia, and in rare cases, neovascularization, exactly as seen in our patient. Furthermore, in chronic CSC, even a singular focal RPE leak could be the causative factor for a dependent detachment. These active focal leaks are frequently located at the junction between attached and detached retinal pigment epithelium and can present with visible areas of retinal pigment epithelial “blow-out” or so-called micropigment epithelial rips.13 Our patient had such an abnormal PED, which was the source of active leakage and chronic, inferior detachment (Figure 1). In this case, the chronic detachment led to overlaying telangiectatic vascular changes, an extensive area of ischemia, and ultimately retinal neovascularization. Treatment of chronic CSC with persistent dependent detachments is a matter of controversy. All studies have been retrospective and uncontrolled in nature. Most have demonstrated a rapid resolution of the neurosensory detachments, but without indisputable evidence of visual acuity benefit compared to untreated patients.4,6,11,14 Chronic detachment of the macula is known to be a risk for photoreceptor as well as pigment epithelial degenerative change and cystic degeneration within the retina itself. In our patient, there were double risk factors, the pres-
ence of peripheral ischemia and neovascularization, which may result in severe vision loss due to other mechanisms, such as vitreous hemorrhage, and/or tractional detachments. Generally, laser photocoagulation treatment for peripheral neovascularization is carried out with scattered treatment to the associated ischemic zones. However, given the technical difficulty inherent in laser treatment of detached retina, it was elected to selectively photocoagulate the active RPE leak without additional laser treatment to the peripheral ischemic retina. The result was typical of resolving retinal detachment with precipitation of intraretinal and subretinal yellow exudates. The progressive resolution of the dependent detachment was associated with an increase in the lipid-like precipitation, which was not unexpected. The serous component of the detachment is presumed to diffuse into the choroidal circulation leaving a legacy of precipitating larger lipophilic molecules. The sequence is also known to occur following resolution of other retinal exudative abnormalities such as diabetic retinopathy or Coats disease. In our patient the detachment resolved, the neovascularization regressed, and the lipid-like exudation initially increased slowly but clearly over several months. The nature or composition of these yellow exudates has recently been described to contain a chromophore, evident with fundus autofluorescence and presumed to be the result of disseminated photoreceptor outer segments into the subretinal space.15,16 In summary, we present a rare case of retinal neovascularization overlying a chronic dependent detachment in a patient with CSC. Of particular importance in the management of this patient was the evolution of intraretinal abnormalities following the selective treatment of the RPE leak. The minimally interventional
227
CHRONIC CSC
therapeutic approach was effective in resolving the detachment, inducing a regression of the neovascularization and eliminating the need for widespread laser treatment.
8. 9.
References 1.
2.
3.
4.
5.
6.
7.
Akiyama K, Kawamura M, Ogata T, Tanaka E. Retinal vascular loss in idiopathic central serous chorioretinopathy with bullous retinal detachment. Ophthalmology 1987;94:1605–1609. Gass JD. Bullous retinal detachment. An unusual manifestation of idiopathic central serous choroidopathy. Am J Ophthalmol 1973;75:810–821. Gass JD, Little H. Bilateral bullous exudative retinal detachment complicating idiopathic central serous chorioretinopathy during systemic corticosteroid therapy. Ophthalmology 1995;102:737–747. Otsuka S, Ohba N, Nakao K. A long-term follow-up study of severe variant of central serous chorioretinopathy. Retina 2002;22:25–32. Paulmann H, Heimann K. [Contribution to the problem of central serous retinal detachment (authors’ transl)]. Klin Monatsbl Augenheilkd 1975;167:324–332. Sahu DK, Namperumalsamy P, Hilton GF, de Sousa NF. Bullous variant of idiopathic central serous chorioretinopathy. Br J Ophthalmol 2000;84:485–492. Iida T, Yannuzzi LA, Spaide RF, et al. Cystoid macular
10.
11.
12. 13. 14.
15. 16.
degeneration in chronic central serous chorioretinopathy. Retina 2003;23:1–7. Slakter JS, Yannuzzi LA, Guyer DR, et al. Indocyanine-green angiography. Curr Opin Ophthalmol 1995;6:25–32. Spaide RF, Hall L, Haas A, et al. Indocyanine green videoangiography of older patients with central serous chorioretinopathy. Retina 1996;16:203–213. Yannuzzi LA, Freund KB, Goldbaum M, et al. Polypoidal choroidal vasculopathy masquerading as central serous chorioretinopathy. Ophthalmology 2000;107:767–777. Yannuzzi LA, Slakter JS, Gross NE, et al. Indocyanine green angiography-guided photodynamic therapy for treatment of chronic central serous chorioretinopathy: a pilot study. Retina 2003;23:288–298. Yannuzzi LA. Type A behavior and central serous chorioretinopathy. Trans Am Ophthalmol Soc 1986;84:799–845. Goldstein BG, Pavan PR. ‘Blow-outs’ in the retinal pigment epithelium. Br J Ophthalmol 1987;71:676–681. Yannuzzi LA, Shakin JL, Fisher YL, Altomonte MA. Peripheral retinal detachments and retinal pigment epithelial atrophic tracts secondary to central serous pigment epitheliopathy. Ophthalmology 1984;91:1554–1572. Spaide RF, Klancnik JM Fundus autofluorescence and central serous chorioretinopathy. Ophthalmology 2005;112:825–833. Ho IV, Yannuzzi LA. Chronic central serous chorioretinopathy and fundus autofluorescence. Retinal Cases & Brief Reports 2008;2:1–5.
