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Editor,—Idiopathic polypoidal choroidal vasculopathy (IPCV) also known as “posterior uveal bleeding syndrome” or “serosanguineous detachments in black females” is an exudative macular degeneration characterised by a juxtapapillary branching choroidal network with surrounding polypoidal excrescences associated with serous and haemorrhagic detachments of the neurosensory retina and pigment epithelium.1-4 The patients are almost always black females in the fifth to seventh decade.
We describe here the indocyanine green angiographic (ICGA) findings of a white male patient with IPCV, who had been misdiagnosed for exudative age related macular degeneration (AMD).
A 54 year old white male patient was referred for an ICGA examination with the diagnosis of exudative AMD. He complained of decreased vision and metamorphopsia in his left eye of 10 days’ duration. The past ocular history was remarkable for recurrent serosanguineous exudative maculopathy of his right eye over a period of 10 years. He did not have a history of systemic disease. Visual acuity of the right eye was reduced to 10/200. Visual acuity of the left eye was 25/200. There was no evidence of inflammation in either the anterior chamber or vitreous. Fundus examination of the right eye disclosed a pigment epithelial detachment with a meniscus of subretinal blood superior to the macula (Fig 1A). In addition, marked atrophy of the retinal pigment epithelium with visible choroidal vessels was seen in the centre of the macula. The left fundus revealed a neurosensory detachment of the macula associated with subretinal lipid deposits (Fig 2A). There were two serous pigment epithelial detachments inferior and temporal to the centre of the macula.
Fluorescein angiography (FA) of the right eye (Fig 1B and C) revealed a window defect with a visible choroidal vascular net and filling of the pigment epithelial detachment with a blood-fluid level. Early phase ICGA (Fig 1D) demonstrated a subfoveal vascular network in an oval configuration with nodular excrescences in the area of the subpigment epithelial blood. Later in the ICG study (Fig 1E) the vascular network showed staining. The core of the polypoidal structures became hypofluorescent. There was prominent leakage of ICG into the pigment epithelial detachment.
FA (Fig 2B and C) of the left eye revealed subfoveal mottled hyperfluorescence with late staining and filling of the two pigment epithelial detachments suggestive of occult choroidal neovascularisation (CNV). On ICGA (Fig 2D) a well defined hyperfluorescent network of branching vessels with polypoidal lesions at the inferior border became visible. Late phase ICG angiogram (Fig2E) revealed staining of the vascular network and minimal leakage of dye into the pigment epithelial detachment.
AMD is currently the leading cause of severe visual loss in Western societies. The basic mechanism for severe visual loss is the ingrowth of CNV and its consequence: exudation, haemorrhage, and disciform scarring. Our patient demonstrated serous and haemorrhagic detachments of the retinal pigment epithelium and the neurosensory retina associated with lipid deposits, characteristics shared with CNV secondary to AMD. However, ICGA revealed a branching vascular network with polypoidal structures at their border characteristic of IPCV.
IPCV is rarely described. The few reported case series suggest that the exudative and haemorrhagic detachments arise from the polypoidal elements. These orange vascular structures may be difficult to visualise on slit lamp biomicroscopy.5 While the polypoidal elements can be blocked by haemorrhage and fluid of the pigment epithelial detachments on FA as seen in our patient, ICGA clearly demonstrates the underlying pathology.5
Our patient is not typical of the clinical presentation and population in which this condition was originally described. Earlier studies have suggested that IPCV mainly occurs in middle aged black females and that the multifocal bilateral lesions, which initially often present with breakthrough vitreous haemorrhage, are found predominantly in the temporal juxtapapillary region.1-4 Our male patient is white and he has a solitary lesion in the central macula which was unilateral over an extended course without a history of vitreous haemorrhage. However, our patient has features of IPCV with the vascular elements ending in aneurysmal or polypoidal lesions on ICGA which differentiates it from AMD. The ocular history with recurrent serous and haemorrhagic detachments of the retinal pigment epithelium and neurosensory retina is consistent as well. Within follow up the right eye went on to develop vitreous haemorrhage and the inferior serous pigment epithelial detachment of the left resolved. Whereas previously it was thought that IPCV was more or less exclusively in the peripapillary region, isolated lesions in the central macula may occur in 10% of patients. In addition, the lesions may be unilateral over an extended course (Yannuzzi, personal communication).
The differential diagnosis of IPCV includes the various causes of CNV. In our patient there were no “histo spots”, angioid streaks, high myopia, history of trauma, or previous inflammations, nor was there any other systemic disease associated with CNV. No evidence of coexisting AMD such as focal hyperpigmentations or drusen were noted in both eyes of our patient. Our case report also differs from AMD in that the progression of the disease over a period of 10 years was relatively slow and the exudative complications started in his early 40s.
The natural course of IPCV is generally associated with recurrent exudative and haemorrhagic episodes. While AMD causes large disciform scars, resolution of the exudative and haemorrhagic manifestations in IPCV may be associated with good visual outcomes in most cases. Involvement of the central macula, however, may result in severe loss of vision as described in our patient. Treatment of the polypoidal elements by laser photocoagulation may be taken into consideration in cases involving the central macula. In one report laser photocoagulation was performed in nine eyes with six showing resolution of the exudative manifestations. However, final visual acuities of the six treated eyes reached the same level as four of five untreated eyes.1
ICGA with its improved imaging of the choroidal circulation accentuates the vascular polypoidal lesion5 and allows differentiation between IPCV and CNV, an important distinction concerning the natural course, visual prognosis, and management of these diseases. Additional clinical features such as a solitary lesion in the central macula and an unilateral course over an extended period may expand the clinical definition of IPCV.