Abstract
PurposeTo 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.MethodsSingle-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.ResultsThis 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).ConclusionsA 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.
Highlights
Idiopathic polypoidal choroidal vasculopathy (PCV) was first described in 1990 by Yannuzzi et al as peculiar polypoidal subretinal lesions that were associated with haemorrhagic detachments of the retinal pigment epithelium (RPE) [1]
To the best of our knowledge, we describe the largest group of Caucasian PCV patients to date
B3, indocyanine green angiography (ICGA) shows 3 solitary polypoidal lesions associated with a vascular network nasally
Summary
Idiopathic polypoidal choroidal vasculopathy (PCV) was first described in 1990 by Yannuzzi et al as peculiar polypoidal subretinal lesions that were associated with haemorrhagic detachments of the retinal pigment epithelium (RPE) [1]. Graefes Arch Clin Exp Ophthalmol (2021) 259:351–361 that polypoidal lesions in PCV are often associated with a branching vascular network (BVN) of subretinal neovascularization between the RPE and Bruch’s membrane, indicating that PCV could be a variant of type 1 neovascularization of any origin [4, 7]. Patients with type 1 (sub-RPE) subretinal neovascularization can have an aneurysmal dilation (the ‘polyp’), often at the edge of the neovascularization. This aneurysmal dilation is known with the term PCV, which strictly speaking is a misnomer because we have learned that neovascular lesions that characterize PCV do not arise directly from the choroid [3]. Some authors have suggested that the term PCV should be reserved for patients in whom findings characteristic for AMD, such as drusen, pigmentary changes, and geographic atrophy, are absent [7, 10, 11]
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