Polypoidal Choroidal Vasculopathy and Neovascularized Age-related Macular Degeneration

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Abstract
FOR NEARLY 2 decades, ophthalmologists have been aware of a peculiar hemorrhagic disorder of the macula, called idiopathic polypoidal choroidal vasculopathy (IPCV).1-9 It was originally described as an inner choroidal vascular abnormality with 2 distinct components: (1) a network of branching vessels external to the choriocapillaris, and (2) terminal aneurysmal dilations sometimes seen clinically as reddish-orange, spheroidal, polyplike structures or polypoidal vascular lesions (Figure 1). Patients with this disorder are known to be at risk of experiencing large serous and hemorrhagic detachments of the retinal pigment epithelium (RPE) and the neurosensory retina (Figure 2, A). Idiopathic polypoidal choroidal vasculopathy is typically described as a bilateral, peripapillary disorder of middle-aged black women (Figure 2, B). If the polypoidal lesions are large enough, and there is sufficient atrophy of the overlying RPE, they may sometimes be detectable clinically or with fluorescein angiography; however, they are best identified with indocyanine green (ICG) angiography, which can more clearly image the choroid through the RPE and the secondary serosanguineous complications.8,9 It is generally accepted that ICG angiography is the diagnostic adjunct of choice in identifying the vascular components of the IPCV abnormality with increased sensitivity and specificity.7 Recently, Yannuzzi et al,9 in an update on the clinical spectrum of IPCV, reported that choroidal vasculopathy may also occur as an isolated entity in the macula in individuals of all races and sexes with a broad age range and a marked variability in the morphology and natural course of the abnormal vessels (Figure 3). Also in this report, it was concluded that the pathogenesis of the vascular abnormality in IPCV was most likely a variant of choroidal neovascularization (CNV) with a separate or independent set of demographic risk factors, clinical features, angiographic characteristics, natural course, and visual prognosis. Numerous other reports have subsequently confirmed and expanded on these observations, clearly establishing the existence of a geographically widespread condition.10-20