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Case Reports and Small Case Series
March 1998

Idiopathic Polypoidal Choroidal Vasculopathy: A Peripheral Lesion

Arch Ophthalmol. 1998;116(3):382-383. doi:

Idiopathic polypoidal choroidal vasculopathy (IPCV) is a peculiar vascular abnormality of the inner choroid, composed of 2 components, a network of branching vessels of variable dimension, terminating in aneurysmal-like enlargements with episodic serosanguineous detachments of the retinal pigment epithelium and neurosensory retina.13 The network of vessels usually emanates from the peripapillary area or less commonly as an isolated macular lesion.3 While sometimes clinically evident beneath atrophic pigment epithelium as reddish orange, spheroidal or polyplike structures, the vascular abnormality is most clearly discernible using indocyanine green angiography for enhanced choroidal imaging.2,3 We report, to our knowledge, the first case of a peripheral IPCV seen with subretinal hemorrhage.

Report of a Case

A 58-year-old white woman with no previous ocular or systemic history was noted to have a focal area of subretinal hemorrhage of several disc diameters in the inferior temporal fundus (Figure 1). The far peripheral retina and the macular region revealed no associated abnormalities. A fluorescein angiogram showed a patchy area of subretinal staining of undetermined origin, along with hypofluorescence or blockage of the choroidal circulation by blood. An indocyanine green videoangiogram revealed the presence of an inner choroidal vascular abnormality, ending in multiple small, hyperfluorescent polyps, characteristic of the IPCV abnormality (Figure 2). There was no vascular abnormality of the inner choroid in the fellow eye.

Figure 1.
Clinical composite shows hemorrhagic detachment of the retina with lipid deposition and multiple polypoidal vascular lesions. The macula was clinically flat.

Clinical composite shows hemorrhagic detachment of the retina with lipid deposition and multiple polypoidal vascular lesions. The macula was clinically flat.

Figure 2.
Composite image of the late indocyanine green angiogram reveals multiple polypoidal lesions; the largest one is leaking beneath the hemorrhage. The hyperfluorescent area just superior nasal to the foveal avascular zone corresponds to an area of thin or hypopigmented retinal pigment epithelium.

Composite image of the late indocyanine green angiogram reveals multiple polypoidal lesions; the largest one is leaking beneath the hemorrhage. The hyperfluorescent area just superior nasal to the foveal avascular zone corresponds to an area of thin or hypopigmented retinal pigment epithelium.

Comment

The vascular abnormality in IPCV is associated with multiple, recurrent serosanguineous detachments of the retinal pigment epithelium and neurosensory retina secondary to leakage, and bleeding from the polypoidal component of the vascular lesion.13 An expanded spectrum of this IPCV has recently been reported in characterizing the demographic features of age, sex, and race, along with the nature of the vascular lesion and the natural course. Although IPCV usually occurs bilaterally in the peripapillary region in 50- to 60-year-old women of pigmented races, it may be seen clinically with a much broader demographic and clinical spectrum. Most recently, an isolated macular lesion has been described, simulating central serous chorioretinopathy or age-related macular degeneration with choroidal neovascularization.3 Our patient, to our knowledge, is the first to be seen with IPCV in the midperipheral fundus without involvement of the peripapillary or central macula areas. In the absence of trauma, a peripheral subretinal hemorrhage, such as the one seen in our patient, is usually diagnosed as eccentric disciform process from choroidal neovascularization. The differential diagnosis includes other entities, such as acquired vasoproliferative disease, metastatic lesions to the choroid, a focal posterior scleritis, or even a primary tumor such as a choroidal osteoma. Our case suggests that IPCV should also be suspected in any case seen in the peripheral retina with serosanguineous subretinal manifestations. Consistent with a previous report,2 indocyanine green angiography is essential in making the diagnosis of IPCV with reasonable certainty; by establishing a definitive diagnosis of the disorder, an appropriate course of treatment can be instituted, consistent with the clinical expectations noted in the disorder.2,3

Since submitting the report, in November, we have seen 2 additional cases of IPCV with peripheral lesions, 1 in a white male and 1 in a black female.

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Article Information

Corresponding author: Lawrence A. Yannuzzi, MD, LuEsther T. Mertz Retinal Research Department, Manhattan Eye, Ear, and Throat Hospital, 210 E 64th St, New York, NY 10021.

References
1.
Yannuzzi  LASorenson  JSpaide  RFLipson  B Idiopathic polypoidal choroidal vasculopathy. Retina. 1990;101- 8Article
2.
Spaide  RFYannuzzi  LASlakter  JSSorenson  JAOrlock  DA Indocyanine green angiography of idiopathic choroidal vasculopathy. Retina. 1995;15100- 110Article
3.
Yannuzzi  LACiardella  ASpaide  RF  et al.  The expanding clinical spectrum of idiopathic polypoidal choroidal vasculopathy. Arch Ophthalmol. 1997;115478- 485Article
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