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Published in: Graefe's Archive for Clinical and Experimental Ophthalmology 2/2021

Open Access 01-02-2021 | Retinal Disorders

The spectrum of polypoidal choroidal vasculopathy in Caucasians: clinical characteristics and proposal of a classification

Authors: Elon H. C. van Dijk, Danial Mohabati, Simona Veselinovic, Wing H. Chung, Greet Dijkman, Camiel J. F. Boon

Published in: Graefe's Archive for Clinical and Experimental Ophthalmology | Issue 2/2021

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Abstract

Purpose

To describe the clinical characteristics and outcome of polypoidal choroidal vasculopathy (PCV), also known as aneurysmal type 1 (sub-retinal pigment epithelium (RPE)) neovascularization, in Caucasian patients.

Methods

Single-centre study in 66 Caucasian patients with a diagnosis of PCV based on optical coherence tomography scan and indocyanine green angiography. Clinical characteristics and multimodal imaging were collected and assessed by an experienced retina specialist.

Results

This study involved 74 eyes of 66 patients with PCV, with a mean age at onset of 73 years and a female preponderance of 66%. The mean number of polypoidal lesions per eye was 1 (range: 1–5 lesions), out of which 75% was located in the macula and 19% in the peripapillary region. Of the 74 eyes, 37 eyes (50%) had PCV associated with a drusenoidal neovascular age-related macular degeneration (AMD) phenotype (PCV-AMD) and 18 eyes (24%) had PCV associated with non-polypoidal type 1 choroidal neovascularization/branching vascular network (PCV-BVN) without signs of drusenoidal AMD, while 19 eyes (26%) had idiopathic, isolated PCV (iPCV). The mean subfoveal choroidal thickness measured in 22 patients was 245 μm (range: 71–420 μm). In 51% of patients, the initially performed therapy showed good anatomical recovery (resolution of intra- and subretinal fluid).

Conclusions

A spectrum of PCV (aneurysmal type 1/sub-RPE neovascularization) can be seen in Caucasian patients. PCV associated with a drusenoidal neovascular AMD phenotype in Caucasians is phenotypically and presumably pathophysiologically more associated with neovascular AMD (PCV-AMD: type A PCV). However, this may not be the case for patients with PCV with non-polypoidal type 1 choroidal neovascularization or BVN and no signs of drusenoidal AMD (PCV-BVN: type B PCV), and for patients with idiopathic PCV without associated drusen or BVN (iPCV; type C PCV). Most patients have a thin choroid, even when drusen are absent. For the entire patient group, a moderate anatomical recovery was observed after treatment.
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Metadata
Title
The spectrum of polypoidal choroidal vasculopathy in Caucasians: clinical characteristics and proposal of a classification
Authors
Elon H. C. van Dijk
Danial Mohabati
Simona Veselinovic
Wing H. Chung
Greet Dijkman
Camiel J. F. Boon
Publication date
01-02-2021
Publisher
Springer Berlin Heidelberg
Published in
Graefe's Archive for Clinical and Experimental Ophthalmology / Issue 2/2021
Print ISSN: 0721-832X
Electronic ISSN: 1435-702X
DOI
https://doi.org/10.1007/s00417-020-04844-z

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