Latest Developments in Polypoidal Choroidal Vasculopathy: Epidemiology, Etiology, Diagnosis, and Treatment

Abstract

Polypoidal choroidal vasculopathy (PCV) is a condition characterized by multiple, recurrent, serosanguineous pigment epithelial detachments, and neurosensory retinal detachments due to abnormal aneurysmal neovascular lesions. It is generally considered as a variant of neovascular age-related macular degeneration, but there are some differences between the clinical presentation, natural history, and treatment response between patients with PCV and typical neovascular age-related macular degeneration patients. Over the past decade, new research and technological advancements have greatly improved our understanding of the PCV disease process and the management of PCV. This review aims to summarize the recent research findings to highlight the epidemiology, pathogenesis, genetics, the application of various diagnostic tools for PCV, and the available treatment options for PCV.

FIGURE 1

Left eye of a PCV patient presented with serous maculopathy with lipid exudates and fibrovascular PED (arrowhead) (A). FA showed stippled hyperfluorescent staining of fibrovascular PED (arrowhead) (B). ICGA revealed focal hypercyanescent polypoidal lesion (arrowhead) with hypofluorescent halo and adjacent BVN (C). B-scan OCT across the polypoidal lesion showed highly suggestive features of PCV including sharply-peaked PED, notched PED, and hyperreflective ring underneath the PED, along with subretinal and intraretinal fluid (D). BVN indicates branching vascular network. FA indicates fluorescein angiography; ICGA, indocyanine green angiography; OCT, optical coherence tomography; PCV, polypoidal choroidal vasculopathy; PED, pigment epithelial detachment.

FIGURE 2

Right eye of a PCV patient with massive hemorrhagic maculopathy (A). FA showed stippled hyperfluorescent staining of fibrovascular PED (arrowhead) surrounded by hypofluorescent blockage due to hemorrhage (B). ICGA revealed a grape-like hypercyanescent polypoidal lesion (arrowhead) surrounded by hypofluorescent halo (C). B-scan OCT across the lesion showed highly suggestive features of PCV including multiple notched PED and hyperreflective ring underneath the PED (arrow), along with subretinal fluid and hemorrhage (D). FA indicates fluorescein angiography; ICGA, indocyanine green angiography; OCT, optical coherence tomography; PCV, polypoidal choroidal vasculopathy; PED, pigment epithelial detachment.