Polypoidal choroidal vasculopathy in Caucasians

Vrije Universiteit Brussel, Bruxelles, Brussels Capital, Belgium
Albrecht von Graæes Archiv für Ophthalmologie (Impact Factor: 1.91). 10/2000; 238(9):752-9. DOI: 10.1007/s004170000180
Source: PubMed


To study the prevalence of polypoidal choroidal vasculopathy (PCV) in Caucasian patients with occult choroidal neovascularization (CNV); to study the clinical spectrum of PCV in Caucasians and the outcome after laser photocoagulation of such lesions.
(1) A consecutive series of 374 eyes of Caucasian patients at least 58 years old, presenting occult CNV, presumed to have age-related macular degeneration (AMD) on fluorescein angiography (FA) were further characterized by indocyanine green angiography (ICGA) to determine the frequency of PCV. (2) The funduscopic, FA and ICGA findings in a cohort of 36 Caucasian patients with PCV were analyzed. (3) The outcome after laser photocoagulation was studied in 14 PCV eyes with a minimum follow-up of 6 months.
(1) Fourteen of 374 eyes (4%) presenting occult CNV in patients at least 58 years old were diagnosed as PCV by means of ICG-A. (2) A polypoidal lesion was found in the macula in 22 of 45 PCV eyes, in the peripapillary area in 16 of 45, under the temporal vascular arcade in 6 of 45 and in the midperiphery in 6 of 45. Large or soft drusen were observed in 15 of 45 eyes with PCV. (3) Regression of fundus signs without persisting polyps 6 months after laser photocoagulation was obtained in 5 of 5 treated peripapillary lesions but in only 5 of 9 treated macular or arcade lesions.
Polypoidal choroidal vasculopathy is not rare in Caucasian patients presenting with occult choroidal neovascularization. The fundus abnormalities seen in such eyes overlap with the typical manifestations of AMD. Whereas the prognosis after photocoagulation of peripapillary polypoidal lesions appears to be relatively good, it is more guarded for macular or arcade lesions.

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    • "Evidence of PCV on ICGA was only observed in 3.8% of patients who received their first six injections over a ≥12-month interval. This relatively low prevalence was similar to the overall 4% prevalence of PCV previously reported in a large case series (374 eyes) of VEGF inhibitor-naive Caucasian patients initially diagnosed with occult CNV.25 "
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    ABSTRACT: To determine the prevalence of polypoidal choroidal vasculopathy (PCV) in patients with presumed neovascular age-related macular degeneration (AMD) who were considered poor responders to ranibizumab. Caucasian patients with suspected neovascular AMD, presumed to be choroidal neovascularisation, previously treated with ≥8 intravitreal injections of ranibizumab 0.5 mg (Lucentis; Novartis AG, Basel, Switzerland) administered as required during optical coherence tomography-guided dosing were retrospectively included. Eyes were categorised according to the time from injection 1 to injection 6 (group 1: <12 months; group 2: ≥12 months). Indocyanine green angiography (ICGA) was used to re-evaluate eyes for PCV. Suitable candidates received reduced-fluence photodynamic therapy/ranibizumab combination therapy supplemented by ranibizumab monotherapy, as required. 202 eyes were included (group 1: 73.8%; group 2: 26.2%). The prevalence of PCV in group 1 (21.5%) was significantly higher than in group 2 (3.8%; p=0.003). After initiation of combination therapy, 16 eyes with PCV received 3.1±2.5 ranibizumab injections/year vs 8.4±2.4 injections/year before initiation of combination therapy (p<0.001). In Caucasian patients with presumed neovascular AMD, PCV prevalence is increased in eyes that respond poorly to ranibizumab monotherapy. ICGA improved PCV diagnosis in poor responders; combination therapy may be beneficial for eyes with PCV.
    Full-text · Article · Nov 2013 · The British journal of ophthalmology
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    • "It can affect both genders, although more often in men than women and in Asian populations.6,7 The prevalence of PCV varies among different ethnic groups and has a higher prevalence in Asians and Blacks as compared to Caucasians.2 PCV accounts for 25-50% of Asian patients presenting with presumed neovascular AMD,7-10 whereas it is seen in only 5-10% of Caucasian patients with neovascular AMD.11-13 "
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    ABSTRACT: Polypoidal choroidal vasculopathy (PCV) is a retinal disease involving the choroidal vasculature characterized by the presence of polypoidal lesions with or without branching vascular network best seen on indocyanine green angiography (ICGA). Clinical features of PCV include recurrent subretinal hemorrhage; serosanguineous pigment epithelial detachment, subretinal exudation and serous retinal detachment. PCV is more prevalent among Asians and Blacks as compared to Caucasians and has been found to account for 25 to 50% of cases of presumed neovascular age-related macular degeneration in Asian patients. Treatment is indicated in patients with symptomatic PCV due to potentially irreversible visual loss. Various treatment modalities for symptomatic PCV have been described in the literature, including thermal laser photocoagulation, ICGA-guided photodynamic therapy (PDT) with verteporfin, anti-vascular endothelial growth factor (VEGF) therapy, and combined PDT and anti-VEGF therapy. This review aims to provide an update on the therapeutic options for PCV, with particular reference to recent studies published in the past two years.
    Full-text · Article · Oct 2013
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    • "PCV was first described by Yannuzzi and associates in 1990 as an idiopathic PCV presenting polypoidal and choroidal vascular lesions [1]. The incidences of PCV among Chinese and Japanese populations with fundus characteristics of neovascular age-related macular degeneration (nAMD) are 24.5% and 54.7%, respectively, which are much higher than the incidence noted in Caucasians [2], [3], [4]. Distinct from the clinical features of AMD, PCV is characterized on indocyanine green (ICG) by a network of branching abnormal choroid vessels and polypoidal vascular dilations. "
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    ABSTRACT: Multiple genetic studies have suggested that high-temperature requirement serine protease (HTRA1) is associated with polypoidal choroidal vasculopathy (PCV). To date, no functional studies have investigated the biological effect of HTRA1 on vascular endothelial cells, essential vascular components involved in polypoidal vascular abnormalities and arteriosclerosis-like changes. In vitro studies were performed to investigate the effect of HTRA1 on the regulation of fibronectin, laminin, vascular endothelial growth factor (VEGF), platelet derived growth factor receptor (PDGFR) and matrix metalloparoteinases 2 (MMP-2) and the role of HTRA1 in choroid-retina endothelial (RF/6A) and human umbilical vein endothelial (HUVEC) cells. Lentivirus-mediated overexpression of HTRA1 was used to explore effects of the protease on RF/6A and HUVEC cells in vitro. HTRA1 overexpression inhibited the proliferation, cell cycle, migration and tube formation of RF/6A and HUVEC cells, effects that might contribute to the early stage of PCV pathological lesions. Fibronectin mRNA and protein levels were significantly down-regulated following the upregulation of HTRA1, whereas the expressions of laminin, VEGF and MMP-2 were unaffected by alterations in HTRA1 expression. The decreased biological function of vascular endothelial cells and the degradation of extracellular matrix proteins, such as fibronectin, may be involved in a contributory role for HTRA1 in PCV pathogenesis.
    Preview · Article · Oct 2012 · PLoS ONE
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