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Arch Ophthalmol. 2002;120:502-508.

Idiopathic polypoidal choroidal vasculopathy (IPCV), a peculiar hemorrhagic disorder of the macula involving serosanguineous detachments of the retinal pigment epithelium (RPE) and neurosensory retina, was first described by Yannuzzi¹ at a meeting of the Macula Society in 1982. At the American Academy of Ophthalmology in 1984, Kleiner and coworkers² described 7 patients with varying degrees of visual loss secondary to multiple recurrent hemorrhages or serous fluid beneath the RPE and neurosensory retina in the posterior fundus, and coined the phrase posterior uveal bleeding syndrome for this condition. In 1985, Stern and coworkers³ published the first report on "multiple recurrent serosanguineous RPE detachments" in 3 middle-aged black women. In his 1987 atlas, Gass⁴ described 3 black women with subretinal bleeding from multicentric neovascular networks with orange sub-RPE plaques or nodules in the peripapillary region.

Several additional reports and case series^5-11 and 2 clinicopathologic studies^12-13 have been published. The previously published pathologic reports as well as the current case may represent end-stage disease in IPCV. Herein, we describe and correlate the clinicopathologic findings in an enucleated eye with IPCV.

Report of a Case
A 46-year-old black woman was referred to one of us (J.L.D.) for evaluation of a mass in the left eye on November 20, 1995. Her medical history was significant for hypertension for 10 years and diabetes mellitus for 6 years (insulin-dependent for 3 years). The patient had smoked half a pack of cigarettes per day for nearly 30 years. The patient's only complaint was occasional burning in both eyes with otherwise normal vision. The best-corrected visual acuity was 20/25 OU. The visual fields were full to finger counting in both eyes. No afferent pupillary defect was found. The motility was full. Slitlamp examination results were unremarkable. The intraocular pressure was 21 mm Hg OU. Ophthalmoscopic examination results revealed multiple areas of retinal-choroidal elevation with red-orange sinuous and tubular patterns within the peripapillary and macular regions in the left eye (Figure 1). Superotemporal to the optic nerve head, a dark red hemorrhagic pigment epithelial detachment was found adjacent to a red-orange nodular or polypoidal lesion. Inferotemporally, a hemorrhagic retinal detachment appeared as an orange subretinal mass with focal hard exudate. The right fundus was unremarkable. A fluorescein angiogram disclosed mottled hypofluorescence and hyperfluorescence throughout the posterior pole in the early frames (Figure 2A) and blockage of fluorescein transmission in the region of the inferotemporal hemorrhagic retinal detachment and the superotemporal hemorrhagic RPE detachment. Mild early hyperfluorescence was present in the regions corresponding to the elevated red-orange sinuous and tubular lesions in the peripapillary and macular regions. Hyperfluorescent polypoidal lesions with minimal fluorescein leakage were at the posterior margins of the hemorrhagic detachments (Figure 2B). An indocyanine green (ICG) angiogram was recommended; however, the patient reported a reaction to iodine after a hysterosalpingogram in 1980 and was advised not to take iodine. The patient was diagnosed as having IPCV, and observation was recommended.

View larger version (25K): [in this window] [in a new window] Figure 1. Montage fundus photograph of left eye (October 11, 1995) reveals peripapillary red-orange nodular lesions contiguous with elevated, sinuous, tubular lesions extending through the macular region. Note subretinal pigment epithelial hemorrhage superotemporal to the optic disc and the red-orange nodular or polypoidal lesions just posterior to the hemorrhage (arrow). Also note the serosanguineous retinal detachment and few hard exudates inferotemporally.

View larger version (26K): [in this window] [in a new window] Figure 2. A, Early fluorescein angiogam (20.8 seconds) of the left eye (October 11, 1995) shows irregular linear and nodular areas of hyperfluorescence (arrowheads) in the macular region corresponding to red-orange tubular and polypoidal lesions, respectively, in Figure 1. B, Late fluorescein angiogram (304.2 seconds) of the left eye shows hyperfluorescence without leakage corresponding to the tubular and nodular lesions in the macula. More intense hyperfluorescence is present superotemporal to the optic disc and along the inferotemporal arcade just posterior to the hemorrhagic retinal pigment epithelium and retinal detachments, respectively. Note the polypoidal configuration of the inferotemporal lesion (arrowhead).

On August 5, 1996 (about 10 months after the initial examination), the patient had an acute loss of vision in the left eye. She had eaten "bad cream cheese" and subsequently developed nausea and vomiting. She awoke the next morning with markedly decreased vision and pain in the left eye. The visual acuity was 20/20 OD and hand motions OS. The intraocular pressure was 17 mm Hg OU. Ophthalmoscopic examination results revealed extensive subretinal and sub-RPE hemorrhage in the central macula extending beyond the vascular arcades in the left eye (Figure 3). Surgical removal of the hemorrhage was considered but rejected because of the extensive sub-RPE hemorrhage.

View larger version (31K): [in this window] [in a new window] Figure 3. Left eye (August 5, 1996) with extensive subretinal and subretinal pigment epithelial hemorrhage. Note the elevated red-orange peripapillary lesions.

On October 13, 1997 (about 2 years after the initial examination), the visual acuity was 20/20 OD and light perception OS. Ophthalmoscopic examination results of the left eye disclosed hazy media secondary to vitreous hemorrhage, persistence of the peripapillary elevated red-orange tubular lesions, and a disciform scar with subretinal fibrosis in the inferotemporal macula (Figure 4). Fluorescein angiography results revealed early hyperfluorescence with late leakage of fluorescein in the region of the disciform scar and RPE transmission defects throughout the posterior pole in the left eye.

View larger version (19K): [in this window] [in a new window] Figure 4. Left eye (October 13, 1997) with a disciform macular scar and persistent elevated, red-orange, nodular, and tubular peripapillary lesions.

On March 19, 1998, nearly 2 years after the initial examination, the patient returned with marked pain and redness in the left eye for 1 week. The visual acuity was 20/25 OD and no light perception OS. The intraocular pressure was 45 mm Hg OS. Gonioscopy revealed 270° of angle closure without neovascularization and anterior displacement of the iris-lens diaphragm in the left eye. Results of slitlamp examination revealed marked conjunctival hyperemia, microcystic and stromal corneal edema, a shallow anterior chamber with rare cells, no rubeosis iridis, and a clear lens in the left eye. Ophthalmoscopic examination results revealed a hemorrhagic retinal detachment in the left eye. Echography showed dense opacities beneath hemorrhagic kissing choroidal vs retinal/RPE detachments and a posterior disciform lesion in the left eye. The patient underwent multiple procedures in the left eye, including anterior chamber paracentesis, diode laser cyclophotocoagulation, and retrobulbar alcohol injection. The pain could not be managed adequately, the intraocular pressure rose to 84 mm Hg OS, and the eye was enucleated, with placement of a hydroxyapatite orbital implant. On December 29, 1998, the visual acuity in the right eye was stable at 20/25 + 1, and no diabetic retinopathy or IPCV was found in the right eye.

Gross examination revealed a left eye of firm consistency measuring 23 x 23.5 x 21.5 mm, with 3 mm of attached optic nerve. The cornea was hazy and measured 11.5 x 11 mm in diameter. The pupil measured 6 mm in diameter. There were no transillumination defects. The optic nerve was sectioned close to the sclera. A horizontal cut was made and the superior cap was removed. On sectioning, the anterior chamber was flat, and there was anterior displacement of the iris-lens diaphragm by dense vitreous hemorrhage and a bullous hemorrhagic retinal detachment (Figure 5). Focal areas of sub-RPE and suprachoroidal hemorrhage were found. The sclera was grossly unremarkable. The optic nerve head was not visualized.

View larger version (65K): [in this window] [in a new window] Figure 5. Photomacrograph of left eye shows bullous serosanguineous retinal detachment (arrowhead) and hemorrhagic retinal pigment epithelium detachment (asterisk). Note also the posterior disciform scar.

Results of microscopic examination of 250 serial stepped sections revealed a cornea with intraepithelial edema. The Bowman layer, the stroma, and Descemet membrane were unremarkable. There was moderate attenuation of the endothelium. The iris and lens were displaced forward by extensive intraocular hemorrhage, with the iris lining the posterior surface of the cornea. The angle was closed. Hemorrhagic necrosis with rounding up of pigment and thickening of the basement membrane of the pigmented ciliary epithelium were seen in the region of the ciliary body. The lens was cataractous with cortical globule formation and posterior migration of the lens epithelium. Focal necrosis of the lens epithelium was present anteriorly. Sub-RPE and intra–Bruch membrane choroidal neovascularization (Figure 6A-D) were noted in the peripapillary region nasal and temporal to the optic nerve head. The RPE overlying the intra–Bruch membrane choroidal neovascularization was relatively intact with apparent focal vacuolation (Figure 6B-D). Thin-walled, cavernous vascular channels (Figure 6A-C) that originated from branches of the short posterior ciliary arteries through defects in Bruch membrane (Figure 6D and E) in the peripapillary region were found in the intra–Bruch choroidal neovascular membrane. Similar cavernous vascular channels without an apparent muscular layer were also in the peripapillary choroid (Figure 6C). The vessels closest to the optic nerve head exhibited a muscularis layer of 1 to 3 cells thick (Figure 6F). Branches of the short posterior ciliary arteries focally abutted Bruch membrane in the peripapillary region (Figure 6E). A few nodular and calcified drusen were in the peripapillary region. Focal calcium deposition was in Bruch membrane, particularly in the peripapillary region. A disciform scar with marked subretinal fibrosis, focal RPE hyperplasia in a tubuloacinar configuration, focal bone formation, and a few blood vessels (subretinal and sub-RPE choroidal neovascularization) were in the macular region and detached by hemorrhage (Figure 6G). There were extensive hemorrhagic retinal and bullous RPE detachments with hemorrhagic necrosis (Figure 6A and B). Focal aggregates of chronic inflammatory cells were found in the choroid, choroidal neovascular membrane, inferior oblique muscle, and episclera. There was focal arteriolar sclerosis within the retina and choroid. Longitudinal sections of the optic nerve disclosed a focal area of retrolaminar cavernous optic atrophy. Cross-sections of the optic nerve disclosed a focal area of hemorrhagic necrosis involving the superior or inferior nerve fiber bundles.

View larger version (161K): [in this window] [in a new window] Figure 6. A, Peripapillary intra-BM CNV (between arrowheads) composed of thin-walled cavernous vascular channels. Note subretinal hemorrhage (asterisk) (HE; x40). B, Subretinal RPE (intra-BM x10 is between arrowheads) cavernous vascular channels (asterisks) at the posterior margin of hemorrhagic RPE detachment. The upper arrowhead identifies the RPE and inner aspect of BM; the lower arrowhead identifies the outer aspect of BM (PAS; x100). C, Higher-power view of intra-BM CNV (between arrowheads) with thin-walled cavernous vascular channels. The upper arrowhead identifies the RPE and inner aspect of BM; the lower arrowhead identifies the outer aspect of BM. Note the intact overlying RPE with apparent vacuolation and the cavernous vessel in the choroid (asterisk) (PAS; x100). D, Intra-BM CNV with arteriole traversing defect (between arrows) in the peripapillary BM (PAS; x400). E, Branches (asterisks) of a short posterior ciliary artery in juxtapapillary choroid extending to BM (PAS; x250). F, Juxtapapillary intra-BM membrane arteriole (asterisk) with muscularis of 1-3 cell layers thick (PAS; x250). G, Disciform macular scar (asterisk) with focal bone formation (arrow), RPE hyperplasia in a tubuloacinar configuration (arrowhead), and few blood vessels. Note the adjacent subretinal hemorrhage to the right of the disciform scar (HE; x40).

Comment
The phrase "idiopathic polypoidal choroidal vasculopathy" was first used by Yannuzzi.¹ Other phrases used to describe this condition include "posterior uveal bleeding syndrome"⁶ and "multiple recurrent serosanguineous RPE detachments."³ In 1990, Yannuzzi et al,⁵ Kleiner et al,⁶ and Perkovich et al⁷ each published 3 small case series of this condition. A total of 28 patients (8-11 patients in each study) were described by the above authors. Demographic features included women (96%), black patients (79%), age range of 40 to 79 years (mean age, 58 years), bilateral findings (68%), hypertension (43%), and diabetes mellitus (14%). Clinically, each of the patients had predominantly peripapillary orange-red polypoidal subretinal choroidal lesions that appeared to be nodular protrusions emanating from the choroid and that were associated with serosanguineous detachments of the RPE and neurosensory retina. Fluorescein angiography typically revealed early mottled hyperfluorescence, with late pooling of fluorescein and occasional late leakage in the region of the polypoidal lesions. The lesions appeared somewhat smaller angiographically than clinically.⁵

The natural course of the disease involved chronic and recurrent hemorrhagic detachments of the RPE and retina, with retention of good vision in most eyes with or without treatment. Vitreous hemorrhage occurred in 12 patients (43%). Laser photocoagulation was associated with resolution of the serosanguineous detachments and stabilization or improvement in vision in 6 of 9 patients.⁵ The large disciform macular scars commonly seen in age-related macular degeneration (ARMD) were not characteristic of this condition. In addition, drusen and other vascular, proliferative, and inflammatory diseases of the eye were not typically seen in these patients.

Indocyanine green videoangiography has shown 2 basic choroidal vascular patterns in IPCV: (1) a branching network of variably sized vessels in the inner choroid (most often in the peripapillary region); and (2) vascular dilations at the border of the network of vessels.¹⁴ The vessels in the network do not follow expected choroidal lobular patterns and generally appear more numerous than expected from the clinical examination results. Spaide and coworkers¹⁴ emphasized the fact that the hemorrhagic and serous elevation of the RPE and retina appear to arise from the edge of the polypoidal lesions. Ross and coworkers¹⁵ reported 2 elderly black women with IPCV, retinal arterial macroaneurysms, and hypertensive retinopathy and suggested that the pathophysiology of IPCV might be analogous to hypertensive changes in the choroidal and retinal vasculature.

In 1997, Yannuzzi and coworkers⁹ reported on the expanding clinical spectrum of IPCV and added 20 additional cases to the 45 cases previously reported in the literature. The average age at initial consultation was 60 years, with patients typically first seeking examination between ages 50 and 65 years. Idiopathic polypoidal choroidal vasculopathy was most commonly found in deeply pigmented (4.2 pigmented: 1 white) women (5 women: 1 man). Black persons and Asians seem to be at greatest risk for developing IPCV. However, among the Japanese, the condition is unilateral in 91% and affects elderly men in 69% of cases.¹⁶ Age-related macular degeneration is also more common in elderly Japanese men than in women.¹⁶ Isolated IPCV lesions have been reported in the peripheral fundus¹⁰ and macula¹¹ without apparent peripapillary during involvement.

Clinical observations reveal that the lesions in IPCV typically progress with conversion of the polypoidal nodules into enlarging tubular components (as seen in our patient) and associated serosanguineous RPE and retinal detachments. The RPE detachment eventually flattens and extends tangentially in the plane of the inner choroid.⁹ Subsequent variable RPE atrophy may ensue. Indocyanine green angiography generally discloses early choroidal vascular hyperfluorescence, with uniform washout in the later stages, except when the polypoidal lesions are actively leaking. The ICG characteristics typically observed in classic or occult choroidal neovascularization in ARMD are not seen in IPCV.⁹

Two possibly related histopathologic studies have been published. MacCumber and coworkers¹² described a 58-year-old white man with a medical history of insulin-dependent diabetes mellitus for 38 years, hypertension for 6 years, and high myopia (7-8 diopters), who developed bilateral, multiple recurrent hemorrhagic detachments of the sensory retina and RPE that eventually led to rubeosis and blindness in one eye. The peripapillary polypoidal choroidal nodules characteristic of IPCV were not apparent in the clinical photographs published by MacCumber et al.¹² The visual acuity was hand motions to light perception for 3 years prior to enucleation. Histopathologic examination results revealed extensive fibrovascular proliferation in the subretinal space and within Bruch membrane, 23 choroidal blood vessels traversing defects in Bruch membrane, and marked lymphocytic infiltration of the choroid and fibrovascular tissue with both T and B cells. Based on the pathologic findings, the patient was treated with immunosuppressive therapy (prednisone and cyclophosphamide). The patient subsequently developed vitreous hemorrhage in the remaining eye and at least 2 new areas of serosanguineous RPE detachment, with a decrease in visual acuity to 20/200 during the ensuing 2 years despite immunosuppressive therapy. The intra–Bruch thin-walled cavernous vascular channels noted in the current case (Figure 6A-C) were not observed. The histopathologic findings might represent end-stage disciform scarring associated with IPCV, ARMD, or some other entity, such as multifocal choroiditis associated with progressive subretinal fibrosis¹⁷ or progressive subretinal fibrosis and uveitis syndrome.¹⁸

Spraul and coworkers¹³ described a 47-year-old black woman who had a sub-RPE hemorrhage in the right eye that was associated with a reddish-orange subretinal lesion in both eyes in the superotemporal peripapillary region. The patient subsequently developed extensive choroidal hemorrhage that led to enucleation of the right eye. Histopathologic examination results revealed fibrovascular membranes within Bruch membrane and between Bruch membrane and the RPE. Numerous breaks in Bruch membrane were observed. Focal serosanguineous detachment of the RPE and total detachment of the retina were revealed. The demographic and historical features as well as the findings of intra-Bruch membrane choroidal neovascularization (CNV), a disciform scar, and extensive serosanguineous retinal detachment described by Spraul and coworkers¹³ were very similar to the current case. The histopathologic findings might represent end-stage disciform scarring associated with IPCV. The one published photomicrograph shows apparent arteriolar sclerosis within the choroid but no large, thin-walled, cavernous vascular channels within Bruch membrane, as in the current case.

A histopathologic report of a lesion in a patient with known IPCV was recently published by Shiraga and coworkers.¹⁹ A subfoveal choroidal neovascular membrane developed 8 months after pars plana vitrectomy, tissue plasminogen activator, and sulfur hexafluoride (SF₆) injection for a submacular hemorrhage associated with IPCV in a Japanese patient. The patient underwent repeated submacular surgery, and results of the histopathologic examination of the excised membrane disclosed fibrovascular tissue between the retina and RPE without the thin-walled cavernous vascular channels described in our patient.

Lafaut and colleagues²⁰ reported the histopathologic findings of an excised choroidal neovascular membrane in a patient with ARMD whose ICG angiogram disclosed a polypoidal choroidal vascular pattern. Pathologic examination results revealed a thick sub-RPE intra–Bruch membrane fibrovascular membrane with diffuse drusen and dilated thin-walled vessels that appeared saccular on serial sections and were located just external to the RPE and diffuse drusen. In the published photomicrographs, no muscularis is apparent in the thin-walled vessels. In addition, the cavernous configuration of the vessels that was found in our case was not observed by Lafaut and colleagues.²⁰

The histopathologic findings in our case further clarify the natural history of IPCV. The flat orange-red vessels noted clinically in the peripapillary region in IPCV appear to be branches of the short posterior ciliary arteries abutting or causing effacement of the RPE (Figure 6E and F). The peripapillary network of vessels observed on results of ICG angiography is derived from these branches of the posterior ciliary arteries. Iijima and coworkers²¹ reported the findings on optical coherence tomography in 2 Japanese patients with IPCV and showed anterior protrusion of an orange subretinal mass corresponding to a polypoidal structure noted on ICG angiography and contiguity between the orange mass (nodule) and hemorrhagic detachment of the RPE. The authors suggested that the RPE and Bruch membrane overlying the polypoidal structure might receive sustained compression from the underlying mass, leading to thinning and defects in the RPE and Bruch membrane. Age-related changes in Bruch membrane, particularly calcium deposition and drusen formation, in the peripapillary region (as found in the current case) may also contribute to defects or cause gaps in Bruch membrane. These defects or gaps in Bruch membrane may permit the proliferation of choroidal neovascularization, which is a common histopathologic finding in each of the reported pathologic studies of possible IPCV cases.

In the original descriptions of cases of IPCV,^{3, 5-6} less than 50% of the total number of patients with IPCV were documented to have systemic hypertension. However, in a subsequent report and update by Perkovich and coworkers⁷ in a small case series, 8 (89%) of 9 patients had a history of hypertension. The 5-year cumulative incidence of CNV in the fellow eye of patients with definite hypertension and juxtafoveal or subfoveal CNV secondary to ARMD in the Macular Photocoagulation Study was approximately 50%—indicating a statistically significant systemic risk factor for CNV.²² Additionally, in the argon laser study²³ and in the krypton laser study,²⁴ approximately 60% and 55% of the patients, respectively, had definite hypertension. The prevalence of hypertension increases with age and is higher in African Americans compared with white Americans.²⁵

Histopathologic features of chronic hypertension were noted in the retina and choroid in the current case and in the case reported by Spraul and coworkers.¹³ The choroidal vascular tone is controlled by autonomic (sympathetic) input in contrast to the autoregulation of the retinal vasculature.^{15, 26} Perhaps increased perfusion pressure secondary to hypertension within the arteriolar branches of the short posterior ciliary arteries and associated vascular shunts (arterial-arterial, arterial-venular, or venular-venular)²⁷ stimulates the prolapse and protrusion of these vessels through defects or gaps in Bruch membrane in the peripapillary region. With persistently elevated arterial blood pressure, the branches of the short posterior ciliary arteries become dilated (cavernous) distally along the course of the vessels and internal to the defect or gap in Bruch membrane, leading to the development of the elevated peripapillary polypoidal lesions. The polypoidal and subsequent tubular lesions as described by Yannuzzi and coworkers⁹ correspond histopathologically to the large thin-walled cavernous vascular channels (Figure 6A-C) and accompanying choroidal neovascularization observed within Bruch membrane in the current case. In the early stages, the lack of significant fluorescein leakage and/or staining in IPCV may be due to the relatively intact RPE (Figure 6B-D) overlying the intra–Bruch membrane choroidal neovascularization and polypoidal lesions. Dilatation and attenuation of the blood vessel wall with accompanying disruption of the endothelium in the polypoidal lesions secondary to elevated arterial blood pressure may lead to the leakage noted on ICG angiography and to the serosanguineous detachments of the retina and RPE.

The true relationship between IPCV and hypertension is unknown, and other pathogenetic factors are possible. Perhaps the abnormal cavernous choroidal vessels represent congenital or acquired vascular anomalies or malformations or anatomic variation. The demographic distribution of the disease in patients typically older than 50 years, the possible association with hypertension,^22-24 and the histologic finding of intra–Bruch membrane choroidal neovascularization might suggest that IPCV is a form or variant of ARMD. Occasional drusen and focal calcification within Bruch membrane, as observed in our case, are pathologic findings that have also been described in studies of eyes with ARMD.

In summary, the network of peripapillary vessels seen on fluorescein and ICG angiography in IPCV corresponds histopathologically to branches of the short posterior ciliary arteries. The elevated polypoidal and tubular choroidal lesions correspond to large thin-walled cavernous vascular channels and accompanying choroidal neovascularization within the Bruch membrane. Continuity between the ciliary arteries and the intra–Bruch membrane vascular channels may explain the potential catastrophic intraocular hemorrhage in IPCV. Hypertension in conjunction with age-related or degenerative changes in Bruch membrane may play a role in the development of IPCV. The predilection for deeply pigmented individuals is unexplained.

AUTHOR INFORMATION
This study was supported in part by the Florida Lions Eye Bank, Miami.

This study was presented in part at the meeting of the Verhoeff-Zimmerman Society, Portland, Ore, April 24, 1999.

Robert H. Rosa, Jr, MD; Janet L. Davis, MD; Charles W. G. Eifrig, MD
Temple, Tex

Corresponding author and reprints: Robert H. Rosa, Jr, MD, Division of Ophthalmology, Scott and White Clinic, 2401 S 31st St, Temple, TX 76508 (e-mail: rrosa{at}swmail.sw.org).

REFERENCES
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SECTION EDITOR: W. RICHARD GREEN, MD

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