Polypoidal Choroidal Vasculopathy Cases Research Articles

Predicting treatment outcomes of intravitreal brolucizumab for polypoidal choroidal vasculopathy through noninvasive assessment of polypoidal lesion blood flow with optical coherence tomography angiography

We investigated the assessment of blood flow within polypoidal lesions using optical coherence tomography angiography (OCTA) to determine intravitreal brolucizumab (IVBr) efficacy for treating polypoidal choroidal vasculopathy (PCV). We retrospectively studied 46 eyes with PCV that completed 1-year IVBr treatment. Blood flow signals within polypoidal lesions were evaluated using OCTA after loading-phase treatment, and 1-year outcomes were compared between eyes in which blood flow signals disappeared versus persisting. After loading-phase treatment, blood flow signals within polypoidal lesions disappeared in 31 eyes and persisted in 15. In the former group, visual acuity improved significantly throughout the year (P < 0.01), while in the latter there was no significant difference between baseline and after 1 year. The total number of injections was significantly lower with than without disappearance of blood flow signals (6.0 vs. 6.9, P < 0.01). The intended injection interval at the last visit was significantly longer in the former than in the latter group (15.7 weeks vs. 12.5 weeks, P < 0.01). These results indicate that PCV cases showing disappearance of blood flow signals within polypoidal lesions by OCTA after loading-phase treatment had favorable 1-year outcomes of IVBr. Therefore, evaluating blood flow within polypoidal lesions by OCTA may allow noninvasive prediction of PCV treatment outcomes.

RISK OF EXUDATION IN EYES WITH NONEXUDATIVE POLYPOIDAL CHOROIDAL VASCULOPATHY.

To investigate the characteristics and natural history of treatment-naive nonexudative polypoidal choroidal vasculopathy (PCV) and to determine biomarkers predicting exudative conversion. Patients diagnosed with nonexudative PCV based on indocyanine green angiography and optical coherence tomography were included. Incidence of exudative conversion in nonexudative PCV patients and cumulative estimates for overall risk were assessed. Indocyanine green angiography and optical coherence tomography imaging-based features were analyzed to identify risk factors for exudative conversion. The study included 42 eyes of 40 patients with nonexudative PCV. The mean follow-up duration was 54.3 ± 35.5 months. Of the 42 eyes with nonexudative PCV, exudative conversion developed in 23 eyes (54.8%) after 42.2 ± 28.3 months (range, 8-103 months). Kaplan-Meier analysis showed that the exudation-free survival at 5 years after baseline was estimated to be 53.6%. Multivariate regression analysis showed that sequentially increased protrusion of retinal pigment epithelium in the polyp area was a significant risk factor for exudation in nonexudative PCV (odds ratio = 10.16; 95% CI 1.78-57.81; P = 0.01). Exudative conversion has been noted in nearly half of the nonexudative PCV cases in 5 years. The progressive protrusion of polypoidal lesions on optical coherence tomography examination may be a significant biomarker for predicting the near-term onset of exudation.

Different Morphology of Branching Neovascular Network in Polypoidal Choroidal Vasculopathy: A Swept-Source Optical Coherence Tomography Angiography Study.

Purpose: To evaluate the classification system of branching neovascular network (BNN) morphology in polypoidal choroidal vasculopathy (PCV) patients based on swept-source optical coherence tomography (SS-OCT) and swept-source optical coherence tomography angiography (SS-OCTA), and analyze the morphological features in each group as potential prognostic features. Methods: A total of 32 PCV eyes were included in this retrospective study. SS-OCT and SS-OCTA images of 6 mm × 6 mm centered on the foveal of each eye were analyzed. PCV cases were classified into three types ("trunk", "glomeruli", and "stick" type) based on the morphological features of BNN. OCT and OCTA features were compared among the three groups. The correlation of OCT/OCTA features with visual acuity at 12 months after anti-VEGF treatment was also analyzed. Results: Type 1 group had the largest BNN area and the largest numbers of polypoidal lesions. Type 2 group has the largest pigment epithelial detachment (PED) area, PED volume, subretinal fluid (SRF) area, and SRF volume. Type 3 group had better baseline BCVA, the smallest BNN area, the smallest PED size, and the smallest SRF size. Type 1 was also featured by a clear break on Bruch's membrane which corresponded to the origin of neovascular tissue. BCVA at 12 months was not significantly different among groups. Baseline BCVA and baseline central macular thickness were correlated with the final BCVA. Conclusions: The current classification system based on BNN morphology on SS-OCTA was highly applicable and revealed distinct characteristics in each group. The BNN type was not correlated with BCVA at 12 months after treatment.

Polypoidal Choroidal Vasculopathy Mimicking as Central Serous Chorioretinopathy

Polypoidal choroidal vasculopathy (PCV) is considered one of the disease entities in the pachychoroid spectrum sharing the common finding of abnormally thickened choroid with Central serous chorioretinopathy (CSCR). Polypoidal choroidal vasculopathy (PCV) is a vascular malformation of the choroid, comprising a network of branching vessels of varying sizes that produce aneurysmal-like enlargements. This disease is generally observed in the macular area. Central serous chorioretinopathy is a retinal disorder characterized by localized serous detachment of the macula. It is secondary to retinal pigment epithelial decomposition and choroidal vascular changes. Fundus photography, optical coherence tomography (OCT), Fundus fluorescein angiography, and Indocyanine green angiography (ICG) aid in the diagnosis and differentiation between these two entities. Here, we report a case of middle aged female presented initially with clinical features suggestive of CSCR and later turned out to be a case of PCV.

Association of HERPUD1 genetic variant rs2217332 with age-related macular degeneration and polypoidal choroidal vasculopathy in an Indian cohort.

Age-related macular degeneration (AMD) and polypoidal choroidal vasculopathy (PCV) are sister diseases and have several similar clinical features and still have few genetic differences. The association of HERPUD1 (homocysteine inducible ER protein with ubiquitin like domain 1) gene variant rs2217332 with PCV is known; however, such association with AMD has not been reported in the Indian population. We analyzed the association of rs2217332 with PCV and AMD to identify the preferential association of this variant with these diseases. This is a population-based case-control study consisting of 422 patients (129 AMD cases; 101 PCV cases, 192 healthy controls) recruited from the vitreoretinal clinic Sankara Nethralaya. The sample size for the study was calculated using appropriate power calculation methods. Genotype was determined using PCR-based Sanger sequencing. The SPSS V23.0 statistical package tool was used to calculate chi-square and ROC to determine the association of rs2217332 with control, AMD, and PCV. Here, we report for the first time the association of this genetic variant (rs2217332) with AMD and PCV in the Indian population. The case-control study shows a significant association of this SNP with PCV (P value = 0.002); however, this variant is not significantly associated with AMD (P value = 0.602). Comparison between AMD (as control) and PCV (as case) also showed significant association of the SNP with PCV (P value = 0.02). Minor allele A conferred to increase the risk of PCV. The study concludes that the genetic variant rs2217332 in HERPUD1 gene is highly significantly associated with PCV and not with AMD in Indian populations.

Characteristics of Retinal Pigment Epithelium Elevations Preceding Exudative Age-Related Macular Degeneration in Japanese

Introduction: Drusen and pigmentary abnormality are found as the hallmark to predict progression of age-related macular degeneration (AMD). In Asian populations, exudative AMD often appears in the absence of drusen but is rather accompanied by pigmentary abnormality. Recently, shallow irregular retinal pigment epithelium (RPE) elevations (SIRE) have been shown as a sign of subclinical nonexudative macular neovascularization. In this study, we aimed to investigate the characteristics of optical coherence tomography (OCT) findings including SIRE before the appearance of exudative AMD. Methods: We retrospectively reviewed 32 cases of exudative AMD that occurred in the fellow eye within the 5-year-observation period. Color fundus photography, OCT, and fluorescein/indocyanine-green angiography at the beginning of observation and at the time when exudative AMD appeared were examined to diagnose SIRE and the subtype of exudative AMD. Results: Exudative AMD was found in 19 eyes with large drusen and 13 eyes without large drusen. Mean sub-foveal choroidal thickness without large drusen was significantly thicker than that with large drusen (336 ± 109 and 220 ± 96 μm, respectively; mean ± SD). Six eyes with pachychoroid neovasculopathy, 4 eyes with type 1 macular neovascularization, and 3 eyes with PCV had occurred in the fellow eye without large drusen. Among those, 6 eyes had been accompanied by SIRE with a greatest transverse linear dimension of 1 mm or more at the beginning of observation period. Besides, small RPE elevations with a longest diameter of less than 1 mm had been observed in other 5 eyes. Three cases of polypoidal choroidal vasculopathy had originated from small RPE elevations. Moreover, pachyvessels, choriocapillaris thinning, or choroidal hyperpermeability were observed with SIRE or small RPE elevations. Conclusions: There is a non-drusen type of exudative AMD that originates from small RPE elevations as well as SIRE.

Visual prognosis of submacular hemorrhage secondary to age-related macular degeneration: A retrospective multicenter survey.

PurposeTo investigate the clinical features, treatment options, and visual outcomes of submacular hemorrhage (SMH) secondary to neovascular age-related macular degeneration (nAMD).DesignA retrospective, observational case series.MethodsSetting: Multicenter institutional setting. Patient Population: A total of 127 patients (127 eyes; 88 men, 39 women; (mean age, 74.2 years)) diagnosed with AMD-associated SMHs exceeding 2 disc diameters involving the fovea. Observation: The AMD types, previous treatments, treatment options, anatomic findings, and best-corrected visual acuity (BCVA) were assessed. Main Outcome Measures: Clinical features, treatment options, and visual outcomes of SMHs secondary to nAMD.ResultsThirty-two eyes had typical AMD, 94 eyes polypoidal choroidal vasculopathy (PCV), and one eye retinal angiomatous proliferation. Eighty-five eyes were treatment-naïve; 42 eyes were treated previously: anti-vascular endothelial growth factor (VEGF) therapy (n = 26), photodynamic therapy (n = 3), and combined therapy (n = 13). Treatment of SMHs included vitrectomy (36 eyes), pneumatic displacement (49 eyes), and anti-VEGF monotherapy (42 eyes). The final BCVA improved significantly in treatment-naïve cases from 0.86 to 0.62 logarithm of the minimal angle of resolution (logMAR) unit (Snellen equivalent from 20/145 to 20/83) and from 0.80 to 0.56 (Snellen equivalent from 20/126 to 20/73) in PCV cases. Meanwhile, the BCVA logMAR values improved from 1.15 to 0.75 (Snellen equivalent from 20/283 to 20/112) and from 0.87 to 0.63 (Snellen equivalent from 20/148 to 20/85) in eyes that underwent vitrectomy or pneumatic displacement, respectively. In eyes with BCVAs between 20/133 to 20/40 at SMH onset, the final VA in the pneumatic displacement group was better than in the anti-VEGF monotherapy group. One eye had a retinal detachment and 1 eye had a macular hole in the vitrectomy group, and 5 eyes had a vitreous hemorrhage in the pneumatic displacement group.ConclusionsThe recommended treatment for SMHs secondary to nAMD exceeding 2 disc area and with BCVA below 20/40 is vitrectomy or pneumatic displacement for visual improvement.

Distinguishing retinal angiomatous proliferation from polypoidal choroidal vasculopathy with a deep neural network based on optical coherence tomography

This cross-sectional study aimed to build a deep learning model for detecting neovascular age-related macular degeneration (AMD) and to distinguish retinal angiomatous proliferation (RAP) from polypoidal choroidal vasculopathy (PCV) using a convolutional neural network (CNN). Patients from a single tertiary center were enrolled from January 2014 to January 2020. Spectral-domain optical coherence tomography (SD-OCT) images of patients with RAP or PCV and a control group were analyzed with a deep CNN. Sensitivity, specificity, accuracy, and area under the receiver operating characteristic curve (AUROC) were used to evaluate the model’s ability to distinguish RAP from PCV. The performances of the new model, the VGG-16, Resnet-50, Inception, and eight ophthalmologists were compared. A total of 3951 SD-OCT images from 314 participants (229 AMD, 85 normal controls) were analyzed. In distinguishing the PCV and RAP cases, the proposed model showed an accuracy, sensitivity, and specificity of 89.1%, 89.4%, and 88.8%, respectively, with an AUROC of 95.3% (95% CI 0.727–0.852). The proposed model showed better diagnostic performance than VGG-16, Resnet-50, and Inception-V3 and comparable performance with the eight ophthalmologists. The novel model performed well when distinguishing between PCV and RAP. Thus, automated deep learning systems may support ophthalmologists in distinguishing RAP from PCV.

Surgical outcomes of vitrectomy for breakthrough vitreous hemorrhage in eyes with exudative age-related macular degeneration

To report the outcomes of pars plana vitrectomy in cases with breakthrough vitreous hemorrhage (VH) secondary to exudative age-related macular degeneration (AMD) and polypoidal choroidal vasculopathy. We also investigated the relationship between the preoperative b-mode ultrasonographic findings and the postoperative visual acuity to determine if the ultrasonographic findings can predict the surgical outcome after pars plana vitrectomy. This was a retrospective, interventional, case series. Twenty eyes of 20 patients were studied. The associations between the pre- and intraoperative factors and the final best-corrected visual acuity (BCVA) were determined. Recombinant tissue-plasminogen activator (tPA) was used in cases with massive hemorrhagic retinal detachment. Ten eyes with polypoidal choroidal vasculopathy (PCV), two eyes with choroidal neovascularization (CNV), and eight eyes with an unknown type of AMD were studied. The mean BCVA was 0.73 ± 0.57 logarithm of the minimum angle of resolution (logMAR) units before developing the VH, 2.25 ± 0.45 logMAR units before the surgery, and 1.52 ± 0.87 logMAR units after the surgery. The BCVA improved significantly after the surgery (P = 0.004) but was significantly worse than that before developing the VH (P = 0.012). The cases of PCV had better final BCVA than cases of CNV (P = 0.043, Mann-Whitney test). The preoperative presence of a subretinal elevation at the macula detected by ultrasonography was significantly associated with a poorer final BCVA (P = 0.031). Vitrectomy significantly improved visual function in the eyes with VH associated with exudative AMD. The eyes with PCV and no macular subretinal elevation on ultrasonography had a better visual prognosis.

One-year Results of Aflibercept Treatment for Polypoidal Choroidal Vasculopathy with Good Visual Acuity.

PurposeTo evaluate the one-year efficacy of intravitreal aflibercept injection in polypoidal choroidal vasculopathy (PCV) with best-corrected visual acuity (BCVA) of 20 / 40 or better.MethodsThis was a multicenter retrospective study. The medical records of patients diagnosed with treatment-naïve PCV were retrospectively reviewed. Patients with an initial BCVA of 20 / 40 or better and who had undergone intravitreal aflibercept injection were included. Patients were treated with three consecutive monthly injections, followed by pro re nata regimen according to the clinician’s discretion at variable interval visits. The proportions of eyes for which BCVA was maintained (≤ 0.2 logarithm of the minimum angle of resolution change) or improved at 12 months were evaluated. The changes of BCVA, central subfield macular thickness (CSMT), pigment epithelial detachment, and subretinal fluid also were assessed.ResultsA total of 86 eyes were included. The mean number of injections for 12 months of treatment was 5.4 ± 1.7. BCVA was maintained or improved in 94.2% (81 / 86) of cases. Mean BCVA (logarithm of the minimum angle of resolution) had changed from the baseline (0.23 ± 0.09) at 3 months (0.21 ± 0.14), 6 months (0.24 ± 0.22), and 12 months (0.20 ± 0.18), but with no statistical significance. CSMT had improved significantly from the baseline (336.1 ± 97.3 μm) at 3 months (223.6 ± 47.22 μm), 6 months (239.6 ± 64.2 μm), and 12 months (223.8 ± 47.9 μm). Pigment epithelial detachment was observed in 93% of cases at the baseline, 72.1% at 3 months, and 69.8% at 12 months, showing a significant decrease at all observation points. Subretinal fluid was observed in 91.9% of cases at the baseline, 20.9% at 3 months, and 29.1% at 12 months, showing a significant decrease at all observation points.ConclusionsIn cases of PCV with good visual acuity, intravitreal aflibercept injections decreased CSMT and were effective in maintaining visual acuity.

INJEKSI INTRAVITREAL RANIBIZUMAB PADA POLYPOIDAL CHOROIDAL VASCULOPATHY (PCV)

&lt;p&gt;&lt;strong&gt;&lt;em&gt;Introduction: &lt;/em&gt;&lt;/strong&gt;&lt;em&gt;Polypoidal choroidal vasculopathy (PCV) is a peculiar form of choroidal neovascularization characterized by protrusion of the aneurysm at the end of the choroidal blood vessels. The aim of this study is to report a case of PCV in a young woman who treated with intravitreal ranibizumab injection. &lt;strong&gt;Method: &lt;/strong&gt;The patient undergoes general ophthalmic examinations, amsler grid, funduscopic photography and OCT analysis. The repeated examination performed after intravitreal injection of ranibizumab to assess the effect of therapy.&lt;/em&gt; &lt;strong&gt;&lt;em&gt;Result: &lt;/em&gt;&lt;/strong&gt;&lt;em&gt;Two weeks after intravitreal ranibizumab injection, the visual acuity was improved and on OCT examination revealed decreasing in macular thickness. &lt;strong&gt;Conclusion:&lt;/strong&gt; Intravitreal ranibizumab injection can give good result, but in this case the effectiveness hasn’t so evident because of the administration only once and follow-up only in two weeks.&lt;/em&gt;&lt;/p&gt;

Prevalence of polypoidal choroidal vasculopathy in Indian population: Risk factors, clinical and imaging characteristics.

AimTo assess prevalence, clinical presentation and multimodal imaging characteristics of polypoidal choroidal vasculopathy (PCV) in a hospital-based setting in South India.MethodsElectronic medical records (EMR) of new patients presenting with suspected clinical signs of wet age-related macular degeneration (AMD) in a tertiary hospital from January to December 2016 were retrospectively analyzed using keywords and filtered for patient who underwent multimodal imaging. Clinical presentations were categorized into predominantly hemorrhagic, exudative or mixed pattern. The imaging features were compared in these clinical groups. The multimodal images were graded by two masked graders and discrepancies between them were settled by a senior arbitrator.ResultsOf the 147 clinically suspicious cases of PCV out of 785 patients with clinical presentation of AMD as recorded in the EMR, 73 (49.7%) patients had a multimodal imaging diagnosis of PCV. There was no difference in the demography, distribution of polyps, ICGA and OCT characteristics in eyes presenting with hemorrhagic, exudative or mixed clinical features.ConclusionApproximately half of South Asian patients presenting with clinical features of neovascular AMD harbor PCV irrespective of their clinical presentation and so we recommend that multimodal imaging is done in all cases of suspicious neovascular AMD in Indian population.

Using optical coherence tomography angiography in assessment of the anti-vascular endothelial growth factor effect for pathological vascular tissue in age-related macular degeneration and polypoidal choroidal vasculopathy.

Using optical coherence tomography angiography to assess and compare changes in pathological vascular tissue, including choroidal neovascularization in neovascular age-related macular degeneration and polypoidal complex in polypoidal choroidal vasculopathy, after treatment with anti-vascular endothelial growth factor. This is a retrospective observational case series study. Clinical data were collected, including that on the best-corrected visual acuity and images of spectrum domain optical coherence tomography and optical coherence tomography angiography of consecutive patients with macula-involved lesions, active pathological vascular tissue in neovascular age-related macular degeneration, and polypoidal complex in polypoidal choroidal vasculopathy who were treated with anti-vascular endothelial growth factor injection. The primary outcome measures were the lesion area, flow density, and flow area of the pathological vascular tissue obtained in optical coherence tomography angiography before treatment, as well as week-1 (W1) and week-5 (W5) after treatment. The secondary outcome measures were the best-corrected visual acuity and the anatomic changes in spectrum domain optical coherence tomography at the same periods. A total of 86 eyes in 79 patients (mean age: 73.10 ± 10.10 (range = 50-91) years, 45 males (57%), of which two eyes were treatment-naïve) underwent one section of intravitreal treatment. Of which 44 eyes (40 patients) were diagnosed as typical neovascular age-related macular degeneration and 42 eyes (39 patients) as polypoidal choroidal vasculopathy. The sensitivity for detecting choroidal neovascularization in neovascular age-related macular degeneration and polypoidal complex in polypoidal choroidal vasculopathy was 75.00% (33/44) and 69.05% (29/42), respectively. There was no significant difference in the detection rate between neovascular age-related macular degeneration and polypoidal choroidal vasculopathy (p = 0.54). In the detectable group, there were significant decrease in lesion area and flow area in the optical coherence tomography angiography images after anti-vascular endothelial growth factor treatment in both the neovascular age-related macular degeneration group (lesion area: W1 = -26.94 ± 19.50%, W5 = -35.52 ± 30.85%, all ps < 0.001; flow area: W1 = -26.22 ± 25.23%, W5 = -32.24 ± 32.07%, all ps < 0.001) and the polypoidal choroidal vasculopathy group (lesion area: W1 = -25.19 ± 20.27%, W5 = -31.55 ± 27.04%, all ps < 0.001; flow area: W1 = -21.83 ± 26.29%, W5 = -28.31 ± 30.72%, all ps < 0.001). The central subfield retinal thickness in spectrum domain optical coherence tomography also showed similar amelioration in both groups. However, the flow density in optical coherence tomography angiography image and the visual outcome did not reveal any significant difference before or after intravitreal injections, and neither were there significant differences between the neovascular age-related macular degeneration and polypoidal choroidal vasculopathy groups. Concerning the effect on the optical coherence tomography angiography images of pathological vascular tissue, there were no statistical differences among different anti-vascular endothelial growth factor agents (i.e. aflibercept, ranibizumab, and bevacizumab). Our study revealed that optical coherence tomography angiography can be used noninvasively and quantitatively to assess the detailed pathologic vascular structures in both neovascular age-related macular degeneration and polypoidal choroidal vasculopathy. Our study also demonstrated that anti-vascular endothelial growth factor could effectively decrease the lesion size and flow area of both the choroidal neovascularization in neovascular age-related macular degeneration cases and the polypoidal complex in polypoidal choroidal vasculopathy cases; the effects were similar in both diseases.

Different imaging characteristics between unilateral and bilateral polypoidal choroidal vasculopathy.

To investigate the different imaging characteristics between unilateral and bilateral polypoidal choroidal vasculopathy (PCV) cases, based on confocal scanning laser ophthalmoscope assessment. For this retrospective case series study, diagnostic indocyanine green angiography (ICGA) and spectral domain optical coherence tomography (SD-OCT) were performed to assess the eligible PCV eyes. Among the 53 patients at baseline, 14 showed bilateral PCV lesions, including two cases of branching vessel network (BVN) without leakage. Concerning the subfoveal choroidal thickness (SFCT), unilateral PCV eyes (326 [155-547] μm) were statistically comparable to their fellow eyes (330 [163-477] μm) (p = 0.257). However, the SFCT (228[141-273] μm) from the bilateral PCV group was significantly lower compared with both the PCV (p = 0.002) and fellow eyes (p < 0.001) from the unilateral group. Moreover, ICGA related hyperfluorescent spots were shown to have a significant positive correlation with SFCT in the unilateral PCV eyes and their fellow eyes, other than bilateral PCV cases. In addition, the drusens tended to prevail in the fellow eyes of the unilateral PCV group (46.2%), compared with bilateral cases. Our results indicate that SFCT, ICGA related hyperfluorescent spots, and drusen were the three main imaging characteristic differences between unilateral and bilateral PCV cases.

Characterizing Branching Vascular Network Morphology in Polypoidal Choroidal Vasculopathy by Optical Coherence Tomography Angiography

This study analyze the morphological characteristics of branching vascular networks (BVN) in polypoidal choroidal vasculopathy (PCV) using optical coherence tomography angiography (OCTA), and correlate imaging characteristics with clinical presentations. We presented a retrospective observational case series for fifty cases of PCV confirmed by indocyanine green angiography. Macular OCTA were done by the AngioVue. The PCV cases were classified by distinct morphologic patterns of BVN by two retina specialists and clinical features were analyzed. The sensitivity of polyp detection by OCTA was 86% after manual segmentation and that of BVN was 90%. Three distinct morphologic patterns of BVN were identified. (1) The “Trunk” pattern (47%) exhibited major vessel trunk with features including presence of drusens, thin choroid, and larger BVN area. (2) The “Glomeruli” pattern (33%) showed anastomotic vascular network without major trunk. (3) The “Stick” pattern (20%) had localized BVN and the thickest choroid. Subtypes 2 and 3 held higher recurrence rate. In conclusions, the precise visualization of BVN on OCTA supported that OCTA might be a noninvasive tool to study the morphology of BVN in PCV, which exhibits three different morphological types. Identifying the morphology of BVN has the potential to prognosticate outcomes in PCV patients.

Polypoidal Choroidal Vasculopathy

Polypoidal choroidal vasculopathy (PCV) is a disease of the choroidal vasculature that may result in sub-retinal hemorrhage and serous detachment of the retinal pigmented epithelium (RPE), leading to sub-retinal brosis and, sometimes, permanent vision loss. This report describes a case of PCV in an African-American female over the course of 1 year and demonstrates the progression of PCV, from being relatively asymptomatic to the development of a visually significant sub-retinal hemorrhage. She is currently being treated with Avastin intravitreal injections with some resolution of her symptoms and a reduction of sub-retinal bleeding.

Association of IGFN1 variant with polypoidal choroidal vasculopathy.

Polypoidal choroidal vasculopathy (PCV) and neovascular age-related macular degeneration (nAMD) share a similar phenotype but are different in their clinical manifestations, responses to treatment and prognosis. Whether PCV is a subtype of AMD or a distinct entity from nAMD remains unknown. Therefore, we performed a whole-exome sequencing based association analysis to compare the genetic architecture of PCV and nAMD in Han Chinese. Whole-exome sequencing analysis was performed on 21 nAMD cases, 20 PCV cases and 20 healthy controls. As a follow-up validation, 145 nAMD cases, 160 PCV cases and 193 controls were genotyped using the Sequenom MassARRAY platform (Sequenom, San Diego, CA, USA). A novel variant, c.6196A>G in the IGFN1 gene, was significantly associated with only PCV (combined p = 7.1 × 10-11 , odds ratio = 9.44), but not with nAMD (combined p = 0.683, odds ratio = 1.30). The minor allele G conferred an increased risk of PCV. The findings of the present study indicate that, although some of the susceptibility loci are shared between PCV and nAMD, a unique genetic signature may decide the pathogenesis of PCV.

A case of polypoidal choroidal vasculopathy in an asymptomatic Nigerian patient

A case of polypoidal choroidal vasculopathy in an asymptomatic Nigerian patient

A Case of Polypoidal Choroidal Vasculopathy and Serous Retinal Detachment in a Bilateral Dome-shaped Macula

A Case of Polypoidal Choroidal Vasculopathy and Serous Retinal Detachment in a Bilateral Dome-shaped Macula

Comparison of Progression to Advanced Stage between Polypoidal Choroidal Vasculopathy and Age-Related Macular Degeneration in Korea

To compare the rate of progression to advanced stage in the fellow eye of patients with typical age-related macular degeneration (AMD) and polypoidal choroidal vasculopathy (PCV) in a South Korean cohort. This is an observational, consecutive retrospective case series. Patients with unilateral advanced stage AMD (n = 288; 180 typical AMD patients and 108 PCV patients). Clinical assessment included detailed eye examination, including fundus photography, fluorescein angiography, and indocyanine green angiography. Five-year progression rate to advanced stage in the fellow eye based on initial Age-Related Eye Disease Study (AREDS) score and the correlation between the initial AREDS score and progression to advanced disease in the fellow eye according to types of AMD. Five-year progression to advanced disease in the fellow eye was similar between typical AMD and PCV cases (11.1% vs. 14.8 %, respectively; P= 0.466, log-rank test). Among patients with initial AREDS score of 2 (normal macula or small drusen on the fellow eye), a higher proportion of patients progressed to advanced disease in the PCV group compared with typical AMD patients (10.4% vs. 2.4 %, respectively; P= 0.0042, log-rank test). Initial AREDS score correlated significantly with progression of the fellow eye to advanced stage in the typical AMD group, after adjusting for age, gender, and other comorbidities (hazard ratio [HR], 9.5; 95% confidence interval [CI], 2.80-32.12; P= 0.0003). However in the PCV group, initial AREDS score did not correlate with progression to advanced stage in the fellow eye (HR, 1.84; 95% CI, 0.83-4.05; P= 0.13). Unlike typical AMD, PCV progresses without typical features such as drusen or pigmentary abnormality. Baseline AREDS score was less likely to predict progression of the fellow eye to advanced-stage disease in PCV compared with typical AMD. Therefore, the globally recognized risk-scoring AREDS system may not be applicable in Asia, where PCV is a prevalent subtype of AMD.