The clinical spectrum of polypoidal choroidal vasculopathy in Caucasian patients: a retrospective multicenter cohort study

Purpose: To describe clinical characteristics of polypoidal choroidal vasculopathy (PCV) in a large Caucasian cohort.

Design: Multicenter retrospective cohort study in 3 tertiary referral centers in the Netherlands.

Subjects: Caucasian patients with an indocyanine green angiography-confirmed diagnosis of PCV in one or both eyes.

Methods: The medical charts and multimodal imaging (MMI) of the included patients were assessed retrospectively by 2 independent assessors. Any discrepancies between graders were resolved by a senior retinal specialist. A predefined set of phenotypic characteristics were graded on MMI, including optical coherence tomography, color fundus photography, fundus fluorescein angiography, and indocyanine green angiography.

Main outcome measures: PCV patients were distributed among 4 phenotypically different types, based on a previously published description: PCV-AMD: PCV with drusenoid age-related macular degeneration (AMD; type A); PCV-BNN: PCV without drusen but with a branching neovascular network (BNN; type B); PCV-i: isolated PCV without drusen or a BNN (type C); PCV-CSC: PCV with a background of central serous chorioretinopathy (CSC; type D).

Results: We included 332 eyes of 305 PCV patients, with 179 out of 305 patients being female (58.7%). The average age at diagnosis was 73 years. The included eyes had the following types: PCV-AMD in 188 eyes (58.4%); PCV-BNN in 61 eyes (18.9%); PCV-i in 15 eyes (4.7%); PCV-CSC in 58 eyes (18.0%). Patients with PCV-AMD were older and more often female than patients with PCV-CSC. The median best-corrected visual acuity of affected eyes was 0.30 logMAR (interquartile range: 0.10 - 0.52), with a large range in each type. A median of 2 polypoidal lesions per eye was found (range: 1 - 12), with no significant differences between types. The choroidal thickness beneath the fovea and beneath polypoidal lesions was significantly higher in PCV-CSC than in PCV-AMD (both p<0.001).

Conclusions: PCV in Caucasian patients comprises a spectrum of different phenotypes: it may present with signs of drusenoid AMD, with a background of CSC, or without signs of either diseases. We found a different phenotype distribution when compared to published findings in Asian patients with PCV.