More than a quarter century has passed since the original description of polypoidal choroidal vasculopathy (PCV) in 1982 as a peculiar hemorrhagic disorder involving the macula characterized by recurrent subretinal pigment epithelial bleeding. In the ensuing years, numerous reports have described the expanded clinical spectrum of this entity. PCV is the principal vascular composition of patients of pigmented races experiencing neovascular maculopathies, particularly African Americans and Asians. This form of neovascularization is now known to occur in white patients with or without concomitant drusen, and the site of involvement has extended from the peripapillary area to the peripheral fundus. Indocyanine green angiography has made detection of these abnormal vascular changes more reliable and definitive. More precise diagnosis has also led to a better understanding of specific clinical features that distinguish PCV from more typical proliferations of abnormal choroidal vessels. We review the nature of PCV, including its genetic basis, demographic features, histopathology, clinical manifestations, natural course, response to treatments, and the histopathological and genetic bases. We emphasize multimodal ophthalmic imaging of these vessels, in particular fluorescein and indocyanine green angiography and optical coherence tomography.
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