- Email: [email protected]
Idiopathic polypoidal choroidal vasculopathy of the macula
Idiopathic Polypoidal Choroidal Vasculopathy of the Macula Ramana S. Moorthy, MD,1 Alice T. Lyon, MD,2 Maurice F. Rabb, MD,3 Richard F. Spaide, MD,4 Lawrence A. Yannuzzi, MD,4 Lee M. Jampol, MD2 Objective: The authors evaluated the clinical, fluorescein, and indocyanine green (ICG) angiographic characteristics of the macular variant of idiopathic polypoidal choroidal vasculopathy (IPCV). Design: Observational case series. Participants: The records, photographs, and fluorescein and ICG angiograms of eight eyes of seven patients with IPCV lesions confined to the macula were reviewed. Main Outcome Measures: The visual acuity, fundus examination, fluorescein and ICG angiographic characteristics, and clinical course were compared. Results: All patients demonstrated polypoidal lesions arising from macular choroidal vessels on ICG angiography. One patient had bilateral lesions. These lesions appeared hyperfluorescent in the early phases of both fluorescein and ICG angiography. Late-phase leakage was seen in cases associated with subretinal fluid or exudate. None of these patients demonstrated polypoidal lesions arising from the peripapillary choroidal circulation or peripapillary choroidal neovascularization. Three eyes with polypoidal lesions that were associated with subretinal fluid and exudates were treated with photocoagulation. Five eyes were not treated. Final visual acuity ranged from 20/20 to hand motions. Severe visual loss was associated with vitreous and subretinal hemorrhage, but this resolved without permanent severe visual loss in several cases. Conclusions: In the macular variant of IPCV, ICG and fluorescein angiography demonstrate characteristic macular polypoidal lesions without evidence of peripapillary lesions. The vascular origin of these polypoidal lesions appears to be the macular choroidal circulation. This is distinguished from classic IPCV, in which lesions appear to arise from the peripapillary choroidal circulation. Visual prognosis appears to be good, with most patients retaining visual acuity of 20/80 or better. If subretinal fluid or exudates reduce visual acuity, photocoagulation should be considered. Ophthalmology 1998;105:1380 –1385 Idiopathic polypoidal choroidal vasculopathy (IPCV),1 also called posterior uveal bleeding syndrome2 or multiple recurrent serosanguineous retinal pigment epithelial detachment syndrome,3,4 is a recently described entity. It has been recognized as a distinct cause of recurrent hemorrhage and exudation in the macula and may cause severe permanent visual loss. Previous reports, including one describing the histopathologic
Originally received: April 23, 1997. Revision accepted: January 12, 1998. Manuscript no. 97221 1 Associated Vitreoretinal and Uveitis Consultants, Indianapolis, Indiana. 2 Northwestern University Medical School, Chicago, Illinois. 3 The University of Illinois at Chicago, Chicago, Illinois. 4 Vitreous-Retina-Macula Consultants of New York, PC, New York, New York. Presented in part as a poster at the Association for Research in Vision and Ophthalmology, Fort Lauderdale, Florida, April 1996, and the Annual Meeting of the American Academy of Ophthalmology, Chicago, Illinois, October 1996. Supported in part by an unrestricted grant from Research to Prevent Blindness, Inc., New York, New York (ATL, LMJ), The Illinois Retina Fund, Chicago, Illinois (MFR), and The Macula Foundation of the LuEsther T. Mertz Retinal Research Laboratory, New York, New York (RFS, LAY). Reprint requests to Alice T. Lyon, MD, Department of Ophthalmology, Northwestern University Medical School, Suite 440, 645 N. Michigan Ave, Chicago, IL 60611.
1380
changes in this syndrome,5 have emphasized the peripapillary location of these lesions and propose a peripapillary choroidal origin of the vascular abnormalities. There has been one study of the indocyanine green (ICG) angiographic findings of IPCV in 12 patients.6 This revealed a temporal peripapillary choroidal origin of a vascular network with polypoidal structures at the border. We describe the clinical and ICG angiographic characteristics of seven patients with IPCV in whom the primary lesions were located solely within the macula. There was no clinical or angiographic evidence of peripapillary choroidal origin of these vascular lesions.
Case Reports Case 1. A 49-year-old black man presented with loss of vision in the right eye due to trauma. His visual acuity was light perception in the right eye and 20/30 in the left eye. In the left eye, polypoidal lesions with a branching vascular network were present on clinical examination (Fig 1). Eleven years later, the patient complained of blurry vision in the left eye for 4 weeks. He had a history of hypertension and diabetes mellitus, which were under good control. His visual acuity was light perception in the right eye and 20/60 in the left eye. The right eye had iris atrophy, 360 degrees of posterior synechiae, and a dense cataract that prevented fundus examination. The left eye had a
Moorthy et al 䡠 Idiopathic Polypoidal Choroidal Vasculopathy
Figure 1. Case 1. Fundus photograph, left eye, with polypoidal lesions at ends of the choroidal vascular network.
1
2
Figure 2. Case 1. Fundus photograph, left eye, 13 years after Figure 1, with resolution of initial lesion leaving a scar and presence of new polypoidal lesions. These are interconnected by a large choroidal vascular network, as can be seen on the fluorescein (Fig 3) and ICG (Fig 4) angiograms. Figure 3. Case 1. Fluorescein angiogram, left eye, revealing a large branching choroidal vascular network originating from macular choroidal vessels.
3
4
Figure 4. Case 1. ICG angiogram, left eye, revealing a large branching choroidal vascular network originating from macular choroidal vessels. Figure 5. Case 2. Initial fundus photograph, left eye, with neurosensory fluid and exudates.
5
6
Figure 6. Case 2. ICG angiogram with hypofluorescent laser scars following laser of polypoidal lesions temporally, with additional polypoidal excrescences at the edge of the choroidal vessels nasally. Figure 7. Case 2. Final fundus photograph, left eye, showing photocoagulation scars and resolution of fluid and exudates. Figure 8. Case 4. Fundus photograph, right eye, of two hemorrhagic retinal pigment epithelial detachments and one chorioretinal scar.
7
8
normal anterior segment, except for mild nuclear sclerotic changes in the lens. Intraocular pressures were normal. Fundus examination of the left eye revealed two discrete, elevated, orange subretinal lesions with overlying subretinal fluid, which extended into the fovea. One lesion was located inferonasal and the other slightly temporal to the fovea. Subretinal hard exudates were present. Fluorescein angiography results revealed three well-defined hyperfluorescent lesions inferonasal, tempo-
ral, and superotemporal to the fovea, two of which leaked. Because of the presence of subretinal fluid in the fovea and the decreased vision, the patient was offered laser photocoagulation. However, on his return approximately 5 weeks later, the fluid had resolved spontaneously. Two polypoidal lesions were still visible. The vision improved to 20/50. Results of ICG angiography at this time revealed evidence of two macular dilated polypoidal lesions in the choroidal vasculature. These
1381
Ophthalmology Volume 105, Number 8, August 1998 lesions appeared to arise from discrete choroidal vessels and did not arise from the peripapillary choroidal circulation. One year later, the two polypoidal lesions were unchanged, but the neurosensory detachment of the fovea had resolved. The visual acuity was 20/25 in the left eye. Thirteen years after initial presentation, the vision declined to 20/50, the temporal lesion was larger, and a new polyp was present inferior to the fovea (Fig 2). Fluorescein angiography revealed leakage from the two polyps inferior to the fovea and the larger polyp temporal to the fovea (Fig 3). ICG angiography clearly demonstrated branching choroidal vessels in the macula that feed the three polypoidal lesions (Fig 4). There was fluid in the fovea, which lessened, and the vision returned to 20/25 within 3 months. Case 2. A 50-year-old white woman presented with a complaint of decreased vision in both eyes for 6 months. Visual acuity was 20/25 in the right eye and 20/60 in the left eye. The right fundus was normal. In the left eye, there was a shallow neurosensory macular detachment surrounded by a rim of subretinal lipid exudate (Fig 5). ICG angiography revealed a network of choroidal vessels with polypoidal extensions at its edge. Several of the small polypoidal lesions were photocoagulated in an effort to reduce the chronic exudation. Six weeks after laser photocoagulation, her vision was stable at 20/50. Eighteen months after presentation, an area of thickening with more lipid exudation was present inferior to the center of the macula of the left eye. This appeared to arise from polypoidal lesions along the inferonasal border of the original lesion (Fig 6). These polypoidal lesions were photocoagulated. The patient’s visual acuity improved to 20/20 in the left eye 2 years after initial presentation. No other polypoidal lesions were evident on fundus examination (Fig 7) or ICG angiography. The right eye remained asymptomatic. Case 3. A 72-year-old white woman presented with complaints of decreased vision in the left eye. Visual acuity was 20/30 in the right eye and 20/70 in the left eye. There was an exudative detachment of the macula of the left eye, with an orange-red nodule along the nasal portion of the detachment. This red nodule was treated with krypton laser photocoagulation, with resolution of subretinal fluid. Her visual acuity was stable for several months. An untreatable area of typical discrete choroidal neovascularization with massive subretinal hemorrhage developed in the right eye, which evolved into a disciform scar. Seven years after presentation, the patient noted a gradual decline of vision in her left eye to 20/200. She received additional laser photocoagulation directed toward polypoidal structures at the border of a larger plaque-like lesion evident with ICG angiography. Several ICG angiograms revealed no evidence of peripapillary choroidal vascular involvement. She had fluctuating vision for several months after the laser treatment, and her visual acuity was 20/400 in the left eye 8 years after presentation. Fundus examination revealed additional polypoidal lesions along the inferior margin of an area of central subretinal fluid. ICG angiography confirmed a recurrence of polypoidal lesions along the inferior border of the central plaque-like lesion. There was again no evidence of peripapillary involvement. Laser photocoagulation was directed toward these polypoidal excrescences. Two and one-half years later (10 years after initial presentation), her visual acuity was 20/80 in the left eye. Case 4. A 45-year-old white woman presented with a visual acuity of 20/20 in both eyes. There was an atrophic extramacular scar in each eye approximately 1⁄2 disc diameter in size. On examination 2 years later, she had what appeared to be two hemorrhagic retinal pigment epithelial detachments in the right eye (Fig 8). These blocked fluorescence. Over the next few months these detachments disappeared without symptoms. Three years after initial presentation, the two lesions were not clinically evident (Fig 9). Fluorescein angiography revealed three window defects (Fig 10). On ICG angiography, there was a network of vessels with small polypoidal lesions in the region of the originally hemorrhagic lesions (Fig 11). The visual acuity was 20/20.
1382
Case 5. A 54-year-old man from Thailand presented with distorted vision in the right eye. The visual acuity was 20/25 in the right eye and counting fingers in the left eye. There was an old macular scar from prior inflammation in the left eye. In the right eye, a small polypoidal lesion was noted at the edge of a hemorrhagic pigment epithelial detachment (Fig 12). There was leakage from the polypoidal lesion with fluorescein angiography. One month later, there was a large subretinal hemorrhage (Fig 13). Fluorescein angiography revealed window defects (Fig 14). ICG angiography revealed a network of vessels with a small polypoidal lesion (Fig 15). Six months after initial presentation, a new larger polypoidal lesion was present in the right eye; the hemorrhage was resolving; and the visual acuity was 20/20 (Fig 16) without treatment. Case 6. A 50-year-old black woman noted decreased vision in the right eye. Her visual acuity was 20/20 in each eye. On examination there was a polyp temporal to the macula, with neurosensory fluid extending into the macula (Fig 17). The left eye had multiple polyps with a connecting vascular network in the superotemporal macula (Fig 18). Laser photocoagulation was performed in the right eye. Subsequently, a classic choroidal neovascular membrane developed, and the vision dropped to 20/200. The left eye developed a dense vitreous hemorrhage, with hand motions vision. Ultrasound examination revealed that the retina was attached and the hemorrhage appeared to arise from the polypoidal lesions. Case 7. A 52-year-old white woman presented with decreased vision in the right eye. Her visual acuity was 20/300 in the right eye and 20/30 in the left eye. The left eye was normal. There was a macular hemorrhage in the right eye. Fluorescein and ICG angiography revealed a network of vessels with polypoidal excrescences in the right eye (Fig 19). No subsequent evaluation was available.
Results Eight eyes of seven patients with macular lesions of IPCV were reviewed. The patients ranged in age from 45 to 72 years, and six were less than 55 years of age. Five were female and two were male. Four were black, two were white, and one was of Asian ancestry. In each case, subretinal, orange-red polypoidal structures were present in the macular region of at least one eye. The lesions were bilateral in one patient and unilateral in two patients. Four others were possibly bilateral cases as well. Usually more than one polypoidal lesion was evident when such lesions were present. Follow-up was available on six patients and ranged from 2 months to 13 years (average, 3.6 years). Visual acuity ranged from 20/20 to 20/300 at presentation. Final visual acuity ranged from 20/20 to hand motions. Three eyes were treated with laser photocoagulation, and five eyes received no treatment. The ICG angiogram was used to determine which areas to photocoagulate. Visual loss was associated with subretinal fluid, exudate, or hemorrhage. The lesions were variable in appearance. Neurosensory detachment with exudate was the most prominent feature in two eyes. Orange-red vascular polypoidal lesions associated with a deep network of branching choroidal vessels were striking features in three eyes. Hemorrhagic retinal pigment epithelial detachments occurred in two eyes. In two eyes, there was subretinal hemorrhage. One eye developed a dense vitreous hemorrhage. One patient had a small chorioretinal scar in each eye and later developed polypoidal lesions that spontaneously regressed. With time, there was considerable variation in the lesions: the polyps grew, regressed, bled, and leaked, and new lesions developed. In two cases, the exudative component involving the macula responded to repeated laser photocoagulation with improvement in vision. In two cases, the hemorrhage cleared spontaneously and with minimal residual visual defect.
Moorthy et al 䡠 Idiopathic Polypoidal Choroidal Vasculopathy
Figure 9. Case 4. Fundus photograph, right eye, with spontaneous resolution of hemorrhagic pigment epithelial detachments.
9
Figure 10. Case 4. Fluorescein angiogram following resolution of hemorrhagic pigment epithelial detachment, with window defects corresponding to those regions.
10
Figure 11. Case 4. ICG angiogram following resolution of hemorrhagic pigment epithelial detachments, with two areas of vascular channels corresponding to the lesions.
11
Figure 12. Case 5. Initial fundus photograph, right eye, with small orange lesion at the superior edge of the hemorrhagic pigment epithelial detachment.
12
Figure 13. Case 5. Fundus photograph 1 month after Figure 12, with a large hemorrhagic pigment epithelial detachment involving the fovea.
13
Figure 14. Case 5. Fluorescein angiogram with ill-defined leakage at the superior margin of the hemorrhage.
14
Figure 15. Case 5. ICG angiogram with leakage from polypoidal lesion at the superior edge of the hemorrhage. Figure 16. Case 5. Fundus photograph 6 months following presentation, with marked reduction of the hemorrhage and good preservation of the macula.
15
16
With fluorescein angiography, the polypoidal lesions pooled fluorescein and some leaked. Such lesions tended to have more associated subretinal fluid. Window defects were seen that corresponded to regressed lesions. In other cases, the regressed lesions left no visible defect. In six patients the deep choroidal vessels feeding the polyps were evident. The choroidal origin of these vessels was well demonstrated with ICG angiography. In all six patients who underwent ICG angiography, there was a network of choroidal vessels originating in the macular area with terminal polypoidal excrescences. In five
cases, there was a fine network of vessels with small polypoidal extensions at the border. In one case, the macular choroidal vessels were well delineated with large polypoidal lesions at the ends.
Discussion Idiopathic polypoidal choroidal vasculopathy, a term coined by Yannuzzi and colleagues1 in 1990, was originally de-
1383
Ophthalmology Volume 105, Number 8, August 1998
Figure 17. Case 6. Fundus photograph, right eye, with isolated orange polyp and surrounding neurosensory retinal detachment. Figure 18. Case 6. Fundus photograph, left eye of same patient as in Figure 17, with multiple polyps temporally.
17
18
Figure 19. Case 7. ICG angiogram, right eye, revealing network of macular choroidal vessels with polypoidal excrescences and extensive subretinal hemorrhage.
19 scribed in 1985.3 Investigators have reported that the lesions arise from the peripapillary region.1–5 In the macular variant of IPCV in this report, the origin of these polypoidal lesions was solely from the macular choroidal circulation. This macular variant may be mistaken for age-related macular degeneration more readily than the peripapillary variant. Macular IPCV is important to identify because the course, management, and visual prognosis are distinctly different from age-related macular degeneration. Stern and colleagues3 first described multiple recurrent serosanguineous retinal pigment epithelial detachments in black women. In a follow-up report, Perkovich and colleagues4 described nine other female patients with a similar presentation and with vitreous hemorrhage as the initial clinical presentation in seven. All of the lesions appeared to arise from the peripapillary choroidal circulation. Similarly, Kleiner and colleagues2 described eight patients who suffered visual loss secondary to multiple recurrent hemorrhages and serous fluid beneath the retinal pigment epithelium and neurosensory retina in the posterior fundus. They termed this entity posterior uveal bleeding syndrome. They described orange subretinal lesions located in the posterior pole that derived most of their circulation from the peripapillary choroidal vasculature. Yannuzzi and colleagues1 described orange subretinal lesions that appeared to be polypoidal dilations arising from a choroidal vascular network. Eleven patients were described, all of whom had peripapillary lesions or lesions that appeared to have their vascular supply arising from the peripapillary choroidal circulation. These seven patients differ from those previously reported in that the location of the polypoidal lesions was in the macula. Furthermore, the circulation leading to these
1384
lesions was the macular choroidal vasculature. The strongest evidence for the macular choroidal origin of these polypoidal lesions is the ICG angiogram of the first patient. This revealed two large polypoidal lesions arising from discrete macular choroidal vessels that had the appearance of grapes hanging from vines. The origin of the subretinal fluid and hard exudates in patients with IPCV is not well understood. If these lesions represent polypoidal dilations or varicosities of the choroidal circulations, they should collapse with digital pressure. They do not.1 With fluorescein angiography, these lesions pool fluorescein but do not tend to leak. These patients also may have leakage from secondary classic or occult choroidal neovascularization.1–7 It may be that these lesions represent an unusual form of choroidal neovascularization. Age-related macular degeneration in white patients usually presents with drusen. Polyps are rare. Classic or occult choroidal neovascularization seen in such patients may have a rapid downhill course, with resultant disciform scarring and severe visual loss. In IPCV, the lesions are polypoidal, not necessarily associated with drusen, and have a relatively more stable long-term course without disciform scarring. This pattern is more commonly seen in younger patients of African or Asian ethnic origin. Only scant histopathologic information is available. In a recent report of a clinicopathologic correlation of a patient with long-standing rubeosis 3 years after the initial findings of bilateral, multiple, recurrent hemorrhagic detachments of the sensory retina and pigment epithelium, microscopic examination disclosed extensive fibrovascular proliferation in the subretinal space and within Bruch’s membrane.5 In this report, most of the changes were located in the posterior
Moorthy et al 䡠 Idiopathic Polypoidal Choroidal Vasculopathy pole and the peripapillary choroid. The authors concluded that recurrent serous and hemorrhagic detachments of the sensory retina and retinal pigment epithelium were secondary to choroidal neovascularization with extensive fibrovascular proliferation within the subretinal space and Bruch’s membrane.5 However, this report describes pathologic changes at the time of end-stage disciform scarring rather than the earlier active stage of polypoidal lesions. In addition, it is the only report of a patient known to have rubeosis as a result of this disorder. Fluorescein angiography reveals evidence of pooling, and sometimes leakage, of fluorescein in the region of the choroidal lesions. ICG angiography shows choroidal vessels that are dilated and polypoidal. This is seen most strikingly in Figure 4. ICG angiographic evidence suggests saccular dilatation of vessels from the choroidal vasculature rather than small classic neovascularization. In six of these seven cases, the ICG angiograms revealed a small network of choroidal new vessels with polypoidal excrescences at the edge of this network. The first case did not show evidence of classic choroidal neovascularization on fluorescein or ICG angiography but clearly showed three large saccular dilations of the macular choroidal vessels corresponding to the clinical orange polyps. The optimal management of patients with IPCV is unclear. The hemorrhagic lesions may have a fulminant onset with severe vision loss but often show spontaneous resolution. Lesions that are more indolent and leak fluid have a more progressive course if left untreated. The second and third patients in our report required several sessions of laser photocoagulation and had good preservation of visual acuity. The first patient had spontaneous resolution of subretinal fluid but subsequent growth of the polyps. The fourth and fifth cases had spontaneous resolution of hemorrhage and had excellent vision. The sixth patient had visual loss following laser photocoagulation and the development of classic choroidal neovascularization in one eye and had a dense vitreous hemorrhage in the fellow eye. We feel that laser photocoagulation of the polypoidal choroidal lesions should be considered if subretinal fluid, hard exudates, or hemorrhage is present and threatens or involves the fovea. However, this study is limited by the small number of
patients. The recommendations for laser are generalized. The variability in presentation and course demonstrate that each case be evaluated individually. This series of seven patients with unusual macular polypoidal lesions had no evidence of peripapillary lesions. With ICG angiography, the presence of isolated macular lesions arising from the discrete submacular choroidal vessels was clearly identified in six of the seven patients. In macular IPCV, ICG and fluorescein angiography demonstrate macular polypoidal lesions that arise from the macular choroidal circulation. The visual prognosis appears to be good; most of these patients retained visual acuity of 20/80 or better. Vitreous and subretinal hemorrhage, however, can cause severe loss of vision. Acknowledgment. The authors thank John C. Michael, MD, Des Plaines, Illinois, for referring the fifth patient in the series for evaluation and providing the photographs. The authors thank Norman P. Blair, MD, for providing the photographs for the fourth patient in the series.
References 1. Yannuzzi LA, Sorenson JA, Spaide RF, Lipson B. Idiopathic polypoidal choroidal vasculopathy (IPCV). Retina 1990;10: 1– 8. 2. Kleiner RC, Brucker AJ, Johnston RL. The posterior uveal bleeding syndrome. Retina 1990;10:9 –17. 3. Stern RM, Zakov ZN, Zegarra H, Gutman FA. Multiple recurrent serosanguineous retinal pigment epithelial detachments in black women. Am J Ophthalmol 1985;100:560 –9. 4. Perkovich BT, Zakov ZN, Berlin LA, et al. An update on multiple recurrent serosanguineous retinal pigment epithelial detachments in black women. Retina 1990;10:18 –26. 5. MacCumber MW, Dastgheib K, Bressler NM, et al. Clinicopathologic correlation of the multiple recurrent serosanguineous retinal pigment epithelial detachments syndrome. Retina 1994;14:143–52. 6. Spaide RF, Yannuzzi LA, Slakter JS, et al. Indocyanine green videoangiography of idiopathic polypoidal choroidal vasculopathy. Retina 1995;15:100 –10. 7. Guyer DR, Yannuzzi LA, Slakter JS, et al. Digital indocyanine-green videoangiography of occult choroidal neovascularization. Ophthalmology 1994;101:1727–35.
1385
Originally received: April 23, 1997. Revision accepted: January 12, 1998. Manuscript no. 97221 1 Associated Vitreoretinal and Uveitis Consultants, Indianapolis, Indiana. 2 Northwestern University Medical School, Chicago, Illinois. 3 The University of Illinois at Chicago, Chicago, Illinois. 4 Vitreous-Retina-Macula Consultants of New York, PC, New York, New York. Presented in part as a poster at the Association for Research in Vision and Ophthalmology, Fort Lauderdale, Florida, April 1996, and the Annual Meeting of the American Academy of Ophthalmology, Chicago, Illinois, October 1996. Supported in part by an unrestricted grant from Research to Prevent Blindness, Inc., New York, New York (ATL, LMJ), The Illinois Retina Fund, Chicago, Illinois (MFR), and The Macula Foundation of the LuEsther T. Mertz Retinal Research Laboratory, New York, New York (RFS, LAY). Reprint requests to Alice T. Lyon, MD, Department of Ophthalmology, Northwestern University Medical School, Suite 440, 645 N. Michigan Ave, Chicago, IL 60611.
1380
changes in this syndrome,5 have emphasized the peripapillary location of these lesions and propose a peripapillary choroidal origin of the vascular abnormalities. There has been one study of the indocyanine green (ICG) angiographic findings of IPCV in 12 patients.6 This revealed a temporal peripapillary choroidal origin of a vascular network with polypoidal structures at the border. We describe the clinical and ICG angiographic characteristics of seven patients with IPCV in whom the primary lesions were located solely within the macula. There was no clinical or angiographic evidence of peripapillary choroidal origin of these vascular lesions.
Case Reports Case 1. A 49-year-old black man presented with loss of vision in the right eye due to trauma. His visual acuity was light perception in the right eye and 20/30 in the left eye. In the left eye, polypoidal lesions with a branching vascular network were present on clinical examination (Fig 1). Eleven years later, the patient complained of blurry vision in the left eye for 4 weeks. He had a history of hypertension and diabetes mellitus, which were under good control. His visual acuity was light perception in the right eye and 20/60 in the left eye. The right eye had iris atrophy, 360 degrees of posterior synechiae, and a dense cataract that prevented fundus examination. The left eye had a
Moorthy et al 䡠 Idiopathic Polypoidal Choroidal Vasculopathy
Figure 1. Case 1. Fundus photograph, left eye, with polypoidal lesions at ends of the choroidal vascular network.
1
2
Figure 2. Case 1. Fundus photograph, left eye, 13 years after Figure 1, with resolution of initial lesion leaving a scar and presence of new polypoidal lesions. These are interconnected by a large choroidal vascular network, as can be seen on the fluorescein (Fig 3) and ICG (Fig 4) angiograms. Figure 3. Case 1. Fluorescein angiogram, left eye, revealing a large branching choroidal vascular network originating from macular choroidal vessels.
3
4
Figure 4. Case 1. ICG angiogram, left eye, revealing a large branching choroidal vascular network originating from macular choroidal vessels. Figure 5. Case 2. Initial fundus photograph, left eye, with neurosensory fluid and exudates.
5
6
Figure 6. Case 2. ICG angiogram with hypofluorescent laser scars following laser of polypoidal lesions temporally, with additional polypoidal excrescences at the edge of the choroidal vessels nasally. Figure 7. Case 2. Final fundus photograph, left eye, showing photocoagulation scars and resolution of fluid and exudates. Figure 8. Case 4. Fundus photograph, right eye, of two hemorrhagic retinal pigment epithelial detachments and one chorioretinal scar.
7
8
normal anterior segment, except for mild nuclear sclerotic changes in the lens. Intraocular pressures were normal. Fundus examination of the left eye revealed two discrete, elevated, orange subretinal lesions with overlying subretinal fluid, which extended into the fovea. One lesion was located inferonasal and the other slightly temporal to the fovea. Subretinal hard exudates were present. Fluorescein angiography results revealed three well-defined hyperfluorescent lesions inferonasal, tempo-
ral, and superotemporal to the fovea, two of which leaked. Because of the presence of subretinal fluid in the fovea and the decreased vision, the patient was offered laser photocoagulation. However, on his return approximately 5 weeks later, the fluid had resolved spontaneously. Two polypoidal lesions were still visible. The vision improved to 20/50. Results of ICG angiography at this time revealed evidence of two macular dilated polypoidal lesions in the choroidal vasculature. These
1381
Ophthalmology Volume 105, Number 8, August 1998 lesions appeared to arise from discrete choroidal vessels and did not arise from the peripapillary choroidal circulation. One year later, the two polypoidal lesions were unchanged, but the neurosensory detachment of the fovea had resolved. The visual acuity was 20/25 in the left eye. Thirteen years after initial presentation, the vision declined to 20/50, the temporal lesion was larger, and a new polyp was present inferior to the fovea (Fig 2). Fluorescein angiography revealed leakage from the two polyps inferior to the fovea and the larger polyp temporal to the fovea (Fig 3). ICG angiography clearly demonstrated branching choroidal vessels in the macula that feed the three polypoidal lesions (Fig 4). There was fluid in the fovea, which lessened, and the vision returned to 20/25 within 3 months. Case 2. A 50-year-old white woman presented with a complaint of decreased vision in both eyes for 6 months. Visual acuity was 20/25 in the right eye and 20/60 in the left eye. The right fundus was normal. In the left eye, there was a shallow neurosensory macular detachment surrounded by a rim of subretinal lipid exudate (Fig 5). ICG angiography revealed a network of choroidal vessels with polypoidal extensions at its edge. Several of the small polypoidal lesions were photocoagulated in an effort to reduce the chronic exudation. Six weeks after laser photocoagulation, her vision was stable at 20/50. Eighteen months after presentation, an area of thickening with more lipid exudation was present inferior to the center of the macula of the left eye. This appeared to arise from polypoidal lesions along the inferonasal border of the original lesion (Fig 6). These polypoidal lesions were photocoagulated. The patient’s visual acuity improved to 20/20 in the left eye 2 years after initial presentation. No other polypoidal lesions were evident on fundus examination (Fig 7) or ICG angiography. The right eye remained asymptomatic. Case 3. A 72-year-old white woman presented with complaints of decreased vision in the left eye. Visual acuity was 20/30 in the right eye and 20/70 in the left eye. There was an exudative detachment of the macula of the left eye, with an orange-red nodule along the nasal portion of the detachment. This red nodule was treated with krypton laser photocoagulation, with resolution of subretinal fluid. Her visual acuity was stable for several months. An untreatable area of typical discrete choroidal neovascularization with massive subretinal hemorrhage developed in the right eye, which evolved into a disciform scar. Seven years after presentation, the patient noted a gradual decline of vision in her left eye to 20/200. She received additional laser photocoagulation directed toward polypoidal structures at the border of a larger plaque-like lesion evident with ICG angiography. Several ICG angiograms revealed no evidence of peripapillary choroidal vascular involvement. She had fluctuating vision for several months after the laser treatment, and her visual acuity was 20/400 in the left eye 8 years after presentation. Fundus examination revealed additional polypoidal lesions along the inferior margin of an area of central subretinal fluid. ICG angiography confirmed a recurrence of polypoidal lesions along the inferior border of the central plaque-like lesion. There was again no evidence of peripapillary involvement. Laser photocoagulation was directed toward these polypoidal excrescences. Two and one-half years later (10 years after initial presentation), her visual acuity was 20/80 in the left eye. Case 4. A 45-year-old white woman presented with a visual acuity of 20/20 in both eyes. There was an atrophic extramacular scar in each eye approximately 1⁄2 disc diameter in size. On examination 2 years later, she had what appeared to be two hemorrhagic retinal pigment epithelial detachments in the right eye (Fig 8). These blocked fluorescence. Over the next few months these detachments disappeared without symptoms. Three years after initial presentation, the two lesions were not clinically evident (Fig 9). Fluorescein angiography revealed three window defects (Fig 10). On ICG angiography, there was a network of vessels with small polypoidal lesions in the region of the originally hemorrhagic lesions (Fig 11). The visual acuity was 20/20.
1382
Case 5. A 54-year-old man from Thailand presented with distorted vision in the right eye. The visual acuity was 20/25 in the right eye and counting fingers in the left eye. There was an old macular scar from prior inflammation in the left eye. In the right eye, a small polypoidal lesion was noted at the edge of a hemorrhagic pigment epithelial detachment (Fig 12). There was leakage from the polypoidal lesion with fluorescein angiography. One month later, there was a large subretinal hemorrhage (Fig 13). Fluorescein angiography revealed window defects (Fig 14). ICG angiography revealed a network of vessels with a small polypoidal lesion (Fig 15). Six months after initial presentation, a new larger polypoidal lesion was present in the right eye; the hemorrhage was resolving; and the visual acuity was 20/20 (Fig 16) without treatment. Case 6. A 50-year-old black woman noted decreased vision in the right eye. Her visual acuity was 20/20 in each eye. On examination there was a polyp temporal to the macula, with neurosensory fluid extending into the macula (Fig 17). The left eye had multiple polyps with a connecting vascular network in the superotemporal macula (Fig 18). Laser photocoagulation was performed in the right eye. Subsequently, a classic choroidal neovascular membrane developed, and the vision dropped to 20/200. The left eye developed a dense vitreous hemorrhage, with hand motions vision. Ultrasound examination revealed that the retina was attached and the hemorrhage appeared to arise from the polypoidal lesions. Case 7. A 52-year-old white woman presented with decreased vision in the right eye. Her visual acuity was 20/300 in the right eye and 20/30 in the left eye. The left eye was normal. There was a macular hemorrhage in the right eye. Fluorescein and ICG angiography revealed a network of vessels with polypoidal excrescences in the right eye (Fig 19). No subsequent evaluation was available.
Results Eight eyes of seven patients with macular lesions of IPCV were reviewed. The patients ranged in age from 45 to 72 years, and six were less than 55 years of age. Five were female and two were male. Four were black, two were white, and one was of Asian ancestry. In each case, subretinal, orange-red polypoidal structures were present in the macular region of at least one eye. The lesions were bilateral in one patient and unilateral in two patients. Four others were possibly bilateral cases as well. Usually more than one polypoidal lesion was evident when such lesions were present. Follow-up was available on six patients and ranged from 2 months to 13 years (average, 3.6 years). Visual acuity ranged from 20/20 to 20/300 at presentation. Final visual acuity ranged from 20/20 to hand motions. Three eyes were treated with laser photocoagulation, and five eyes received no treatment. The ICG angiogram was used to determine which areas to photocoagulate. Visual loss was associated with subretinal fluid, exudate, or hemorrhage. The lesions were variable in appearance. Neurosensory detachment with exudate was the most prominent feature in two eyes. Orange-red vascular polypoidal lesions associated with a deep network of branching choroidal vessels were striking features in three eyes. Hemorrhagic retinal pigment epithelial detachments occurred in two eyes. In two eyes, there was subretinal hemorrhage. One eye developed a dense vitreous hemorrhage. One patient had a small chorioretinal scar in each eye and later developed polypoidal lesions that spontaneously regressed. With time, there was considerable variation in the lesions: the polyps grew, regressed, bled, and leaked, and new lesions developed. In two cases, the exudative component involving the macula responded to repeated laser photocoagulation with improvement in vision. In two cases, the hemorrhage cleared spontaneously and with minimal residual visual defect.
Moorthy et al 䡠 Idiopathic Polypoidal Choroidal Vasculopathy
Figure 9. Case 4. Fundus photograph, right eye, with spontaneous resolution of hemorrhagic pigment epithelial detachments.
9
Figure 10. Case 4. Fluorescein angiogram following resolution of hemorrhagic pigment epithelial detachment, with window defects corresponding to those regions.
10
Figure 11. Case 4. ICG angiogram following resolution of hemorrhagic pigment epithelial detachments, with two areas of vascular channels corresponding to the lesions.
11
Figure 12. Case 5. Initial fundus photograph, right eye, with small orange lesion at the superior edge of the hemorrhagic pigment epithelial detachment.
12
Figure 13. Case 5. Fundus photograph 1 month after Figure 12, with a large hemorrhagic pigment epithelial detachment involving the fovea.
13
Figure 14. Case 5. Fluorescein angiogram with ill-defined leakage at the superior margin of the hemorrhage.
14
Figure 15. Case 5. ICG angiogram with leakage from polypoidal lesion at the superior edge of the hemorrhage. Figure 16. Case 5. Fundus photograph 6 months following presentation, with marked reduction of the hemorrhage and good preservation of the macula.
15
16
With fluorescein angiography, the polypoidal lesions pooled fluorescein and some leaked. Such lesions tended to have more associated subretinal fluid. Window defects were seen that corresponded to regressed lesions. In other cases, the regressed lesions left no visible defect. In six patients the deep choroidal vessels feeding the polyps were evident. The choroidal origin of these vessels was well demonstrated with ICG angiography. In all six patients who underwent ICG angiography, there was a network of choroidal vessels originating in the macular area with terminal polypoidal excrescences. In five
cases, there was a fine network of vessels with small polypoidal extensions at the border. In one case, the macular choroidal vessels were well delineated with large polypoidal lesions at the ends.
Discussion Idiopathic polypoidal choroidal vasculopathy, a term coined by Yannuzzi and colleagues1 in 1990, was originally de-
1383
Ophthalmology Volume 105, Number 8, August 1998
Figure 17. Case 6. Fundus photograph, right eye, with isolated orange polyp and surrounding neurosensory retinal detachment. Figure 18. Case 6. Fundus photograph, left eye of same patient as in Figure 17, with multiple polyps temporally.
17
18
Figure 19. Case 7. ICG angiogram, right eye, revealing network of macular choroidal vessels with polypoidal excrescences and extensive subretinal hemorrhage.
19 scribed in 1985.3 Investigators have reported that the lesions arise from the peripapillary region.1–5 In the macular variant of IPCV in this report, the origin of these polypoidal lesions was solely from the macular choroidal circulation. This macular variant may be mistaken for age-related macular degeneration more readily than the peripapillary variant. Macular IPCV is important to identify because the course, management, and visual prognosis are distinctly different from age-related macular degeneration. Stern and colleagues3 first described multiple recurrent serosanguineous retinal pigment epithelial detachments in black women. In a follow-up report, Perkovich and colleagues4 described nine other female patients with a similar presentation and with vitreous hemorrhage as the initial clinical presentation in seven. All of the lesions appeared to arise from the peripapillary choroidal circulation. Similarly, Kleiner and colleagues2 described eight patients who suffered visual loss secondary to multiple recurrent hemorrhages and serous fluid beneath the retinal pigment epithelium and neurosensory retina in the posterior fundus. They termed this entity posterior uveal bleeding syndrome. They described orange subretinal lesions located in the posterior pole that derived most of their circulation from the peripapillary choroidal vasculature. Yannuzzi and colleagues1 described orange subretinal lesions that appeared to be polypoidal dilations arising from a choroidal vascular network. Eleven patients were described, all of whom had peripapillary lesions or lesions that appeared to have their vascular supply arising from the peripapillary choroidal circulation. These seven patients differ from those previously reported in that the location of the polypoidal lesions was in the macula. Furthermore, the circulation leading to these
1384
lesions was the macular choroidal vasculature. The strongest evidence for the macular choroidal origin of these polypoidal lesions is the ICG angiogram of the first patient. This revealed two large polypoidal lesions arising from discrete macular choroidal vessels that had the appearance of grapes hanging from vines. The origin of the subretinal fluid and hard exudates in patients with IPCV is not well understood. If these lesions represent polypoidal dilations or varicosities of the choroidal circulations, they should collapse with digital pressure. They do not.1 With fluorescein angiography, these lesions pool fluorescein but do not tend to leak. These patients also may have leakage from secondary classic or occult choroidal neovascularization.1–7 It may be that these lesions represent an unusual form of choroidal neovascularization. Age-related macular degeneration in white patients usually presents with drusen. Polyps are rare. Classic or occult choroidal neovascularization seen in such patients may have a rapid downhill course, with resultant disciform scarring and severe visual loss. In IPCV, the lesions are polypoidal, not necessarily associated with drusen, and have a relatively more stable long-term course without disciform scarring. This pattern is more commonly seen in younger patients of African or Asian ethnic origin. Only scant histopathologic information is available. In a recent report of a clinicopathologic correlation of a patient with long-standing rubeosis 3 years after the initial findings of bilateral, multiple, recurrent hemorrhagic detachments of the sensory retina and pigment epithelium, microscopic examination disclosed extensive fibrovascular proliferation in the subretinal space and within Bruch’s membrane.5 In this report, most of the changes were located in the posterior
Moorthy et al 䡠 Idiopathic Polypoidal Choroidal Vasculopathy pole and the peripapillary choroid. The authors concluded that recurrent serous and hemorrhagic detachments of the sensory retina and retinal pigment epithelium were secondary to choroidal neovascularization with extensive fibrovascular proliferation within the subretinal space and Bruch’s membrane.5 However, this report describes pathologic changes at the time of end-stage disciform scarring rather than the earlier active stage of polypoidal lesions. In addition, it is the only report of a patient known to have rubeosis as a result of this disorder. Fluorescein angiography reveals evidence of pooling, and sometimes leakage, of fluorescein in the region of the choroidal lesions. ICG angiography shows choroidal vessels that are dilated and polypoidal. This is seen most strikingly in Figure 4. ICG angiographic evidence suggests saccular dilatation of vessels from the choroidal vasculature rather than small classic neovascularization. In six of these seven cases, the ICG angiograms revealed a small network of choroidal new vessels with polypoidal excrescences at the edge of this network. The first case did not show evidence of classic choroidal neovascularization on fluorescein or ICG angiography but clearly showed three large saccular dilations of the macular choroidal vessels corresponding to the clinical orange polyps. The optimal management of patients with IPCV is unclear. The hemorrhagic lesions may have a fulminant onset with severe vision loss but often show spontaneous resolution. Lesions that are more indolent and leak fluid have a more progressive course if left untreated. The second and third patients in our report required several sessions of laser photocoagulation and had good preservation of visual acuity. The first patient had spontaneous resolution of subretinal fluid but subsequent growth of the polyps. The fourth and fifth cases had spontaneous resolution of hemorrhage and had excellent vision. The sixth patient had visual loss following laser photocoagulation and the development of classic choroidal neovascularization in one eye and had a dense vitreous hemorrhage in the fellow eye. We feel that laser photocoagulation of the polypoidal choroidal lesions should be considered if subretinal fluid, hard exudates, or hemorrhage is present and threatens or involves the fovea. However, this study is limited by the small number of
patients. The recommendations for laser are generalized. The variability in presentation and course demonstrate that each case be evaluated individually. This series of seven patients with unusual macular polypoidal lesions had no evidence of peripapillary lesions. With ICG angiography, the presence of isolated macular lesions arising from the discrete submacular choroidal vessels was clearly identified in six of the seven patients. In macular IPCV, ICG and fluorescein angiography demonstrate macular polypoidal lesions that arise from the macular choroidal circulation. The visual prognosis appears to be good; most of these patients retained visual acuity of 20/80 or better. Vitreous and subretinal hemorrhage, however, can cause severe loss of vision. Acknowledgment. The authors thank John C. Michael, MD, Des Plaines, Illinois, for referring the fifth patient in the series for evaluation and providing the photographs. The authors thank Norman P. Blair, MD, for providing the photographs for the fourth patient in the series.
References 1. Yannuzzi LA, Sorenson JA, Spaide RF, Lipson B. Idiopathic polypoidal choroidal vasculopathy (IPCV). Retina 1990;10: 1– 8. 2. Kleiner RC, Brucker AJ, Johnston RL. The posterior uveal bleeding syndrome. Retina 1990;10:9 –17. 3. Stern RM, Zakov ZN, Zegarra H, Gutman FA. Multiple recurrent serosanguineous retinal pigment epithelial detachments in black women. Am J Ophthalmol 1985;100:560 –9. 4. Perkovich BT, Zakov ZN, Berlin LA, et al. An update on multiple recurrent serosanguineous retinal pigment epithelial detachments in black women. Retina 1990;10:18 –26. 5. MacCumber MW, Dastgheib K, Bressler NM, et al. Clinicopathologic correlation of the multiple recurrent serosanguineous retinal pigment epithelial detachments syndrome. Retina 1994;14:143–52. 6. Spaide RF, Yannuzzi LA, Slakter JS, et al. Indocyanine green videoangiography of idiopathic polypoidal choroidal vasculopathy. Retina 1995;15:100 –10. 7. Guyer DR, Yannuzzi LA, Slakter JS, et al. Digital indocyanine-green videoangiography of occult choroidal neovascularization. Ophthalmology 1994;101:1727–35.
1385