ABSTRACT
Polypoidal choroidal vasculopathy (PCV) is a slow growing complex form of neovascularization that has a branching vascular network (BVN) with aneurysmal dilations at the outer border of the network. PCV accounts for a minority of cases of choroidal neovascularization (CNV) seen among Caucasian patients with age-related macular degeneration (AMD), but accounts for a much larger proportion of eyes with neovascular manifestations in Asian and Black populations. The BVN in younger patients or in eyes with long standing disease appears as interconnected, reddish-orange dilated vessels. In older patients, particularly in Caucasians, the BVN can have an appearance indistinguishable from occult CNV. The BVN commonly expands laterally over years. The aneurysmal changes at the outer border of the lesion are more dynamic and can grow, disappear, or be replaced by network vessels over time. The aneurysmal changes are the source of the more dramatic neovascular manifestations such as serous or serosanguineous detachments of the retinal pigment epithelium (RPE) and the retina. There may be impressive amounts of lipid deposition in some cases. PCV may start at a relatively early age, with some patients being in their 40s. The disease can persist for years before threatening the macula or producing enough neovascular manifestations to be symptomatic. The eyes with PCV do not necessarily have the common ophthalmoscopic precursors seen in AMD such as large drusen or focal hyperpigmentation. Although PCV generally appears in fl uorescein angiography with occult characteristics, PCV shows a good treatment response to photodynamic therapy in contrast with eyes harboring occult CNV secondary to AMD. Treatment with agents directed against vascular endothelial growth factor (VEGF) may cause improved visual acuity without much change in the underlying anatomic appearance of the neovascularization. Proper recognition of PCV is important because it can threaten visual function and has different demographic features, appearance, and treatment approaches than does typical CNV in AMD.
