Genetics, Risk Factors and Pathogenesis in the Spectrum of Pachychoroid Diseases

2020 ◽  
pp. 62-66
Keyword(s):  
Risk Factors ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Focal Choroidal Excavation ◽  
Pachychoroid Neovasculopathy ◽  
Choroidal Vessels ◽  
Angiographic Findings ◽  
Choroidal Vascular Hyperpermeability ◽  
Choroidal Hyperpermeability ◽  
Pachychoroid Pigment Epitheliopathy

The spectrum of pachychoroid disease was first described by Warrow et al. in 2013. The characteristics of pachyoroid phenotype are dilatation of the vessels in the Haller layer (pachyvessels), thinning of the layers of Sattler, and choriocapillaris. Dilated choroidal vessels, choroidal hyperpermeability, diffuse or focal choroidal thickness and associated with progressive retinal pigment epithelium (RPE) dysfunction are thought to be in the pathogenesis. Angiographic findings such as choroidal vascular hyperpermeability, especially in the region of RPE changes, and delay in choroidal filling suggest that the primary pathology is associated with choroidal vascular disturbance. However, the exact pathophysiological mechanisms of the disease are not fully known.  Pakikoroid-related diseases include pachychoroid pigment epitheliopathy, central serous chorioretinopathy, pachychoroid neovasculopathy, polypoidal choroidal vasculopathy, focal choroidal excavation, and peripapillary pachychoroid syndrome. These diseases are thought to be reveal different manifestations of common pathological mechanisms. This review highlights the current understanding of genetics, risk factors, and pathogenesis in the spectrum of pachychoroid disease based on the current literature.

Epidemiology, Prevalence And Incidence in Pachychoroid Disease Spectrum

2020 ◽  
pp. 57-91
Keyword(s):  
Risk Factors ◽  
Central Serous Chorioretinopathy ◽  
Polypoidal Choroidal Vasculopathy ◽  
Disease Spectrum ◽  
Focal Choroidal Excavation ◽  
Pachychoroid Neovasculopathy ◽  
Novel Concept ◽  
Pathogenic Process ◽  
Choroidal Vasculopathy ◽  
Pachychoroid Pigment Epitheliopathy

Pachychoroid is a relatively novel concept describing a phenotype characterized by increased choroidal thickening, pathologically dilated veins in the Haller’s layer (pachy-veins), thinning in Sattler’s and choriocapillaris layers. The spectrum comprises the following disease groups: Pachychoroid Pigment Epitheliopathy, Central Serous Chorioretinopathy, Pachychoroid Neovasculopathy, Polypoidal Choroidal Vasculopathy, Focal choroidal excavation, Peripapillary pachychoroid syndrome. These conditions all exhibit the characteristic choroidal alterations and are believed to represent different manifestations of a common pathogenic process. The aim of this review is to exhibit the epidemiology, risk factors, incidence and prevalence of the pachychoroid diseases respectively.

scholarly journals Prevalence of and factors associated with dilated choroidal vessels beneath the retinal pigment epithelium among the Japanese

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yasuki Ito ◽  
Mari Ito ◽  
Takeshi Iwase ◽  
Keiko Kataoka ◽  
Kazuhisa Yamada ◽  
...  
Keyword(s):  
Risk Factors ◽  
Retinal Pigment Epithelium ◽  
Axial Length ◽  
Choroidal Thickness ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Younger Age ◽  
Male Sex ◽  
Associated Risk Factors ◽  
Choroidal Vessels

AbstractPachyvessels are pathologically dilated large choroidal vessels and are associated with the pathogenesis of several pachychoroid-related disorders, including central serous chorioretinopathy. We aimed to investigate the prevalence of and risk factors for pachyvessels in the Japanese population. We included 316 participants (aged ≥ 40 years) with normal right eyes. The presence of pachyvessels (vertical diameter > 300 µm, distance to the retinal pigment epithelium < 50 µm) was determined using 6 × 6 mm macular swept-source optical coherence tomography images, and associated risk factors were investigated. Subfoveal choroidal thickness was measured, and its associated risk factors investigated. The overall prevalence of pachychoroids was 9.5%. Regression analysis showed that a younger age, shorter axial length, male sex, and smoking were significantly associated with the presence of pachyvessels (p = 0.047; odds ratio [OR] 0.96 per year, p = 0.021; OR 0.61 per 1 mm, p = 0.012; OR 3.08 vs. female, and p = 0.011; OR 3.15 vs. non-smoker, respectively) and greater choroidal thickness (p < 0.001, p < 0.001, p < 0.003, and p < 0.017, respectively). The results were consistent with other research findings which showed that pachychoroid-related disorders such as central serous chorioretinopathy were associated with younger age, male sex, shorter axial length, and smoking. Smoking may be associated with choroidal circulatory disturbance in the Japanese population.

scholarly journals Pachychoroid: current concepts on clinical features and pathogenesis

2020 ◽  
Author(s):  
Veronica Castro-Navarro ◽  
Francine Behar-Cohen ◽  
Woohyok Chang ◽  
Antonia M. Joussen ◽  
Timothy Y. Y. Lai ◽  
...  
Keyword(s):  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Clinical Signs ◽  
Salient Feature ◽  
Posterior Pole ◽  
Geographic Atrophy ◽  
Retinal Disorders ◽  
Pachychoroid Neovasculopathy ◽  
Pachychoroid Pigment Epitheliopathy

Abstract Purpose The term “pachychoroid” refers to a newly described phenotype in which functional and structural choroidal changes are thought to play a key pathogenic role in a spectrum of related retinal disorders. A more detailed understanding of how the choroid is involved within this spectrum and a better knowledge of the most relevant clinical signs of the pachychoroid phenotype are important to differentiate these disorders from other retinal conditions. Our objectives are to provide a literature review of pachychoroid and the commonalities that may be present across pathologies included in the spectrum, and to provide details on the examination, monitoring, and management of these disorders. Methods We searched the PubMed web platform to identify relevant studies using the following keywords: pachychoroid, pachychoroid pigment epitheliopathy, pachychoroid neovasculopathy, aneurysmal type 1 neovascularization, focal choroidal excavation, peripapillary pachychoroid syndrome, vasculopathy pachysclera, pachychoroid geographic atrophy, and pachydrusen. We selected 157 publications and identified the most important features related to pachychoroid. Results The presence of hypertrophic or congested vessels in the choroid, not thickened choroid per se, under an area of reduced or absent choriocapillaris in the posterior pole seems to be the most salient feature of pachychoroid. However, other qualitative/quantitative features are needed to differentiate the uncomplicated pachychoroid from the pathological pachychoroid clinical spectrum, which may be associated with exudation, neovascularization, and/or retinal pigment epithelium and photoreceptor atrophy. Conclusions The most salient feature of pachychoroid appears to be the presence of large vessels under an area of reduced or absent choriocapillaris. Knowledge of the features and pathogenesis of the different disorders in the pachychoroid spectrum may assist in the management of patients.

scholarly journals Chronic choriocapillaris ischemia secondary to choroidal congestion in pachychoroid neovasculopathy

2021 ◽  
Author(s):  
Hidetaka Matsumoto ◽  
Junki Hoshino ◽  
Ryo Mukai ◽  
Kosuke Nakamura ◽  
Shoji Kishi ◽  
...  
Keyword(s):  
Multimodal Imaging ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Case Series ◽  
Retrospective Case ◽  
Rpe Atrophy ◽  
Pachychoroid Neovasculopathy ◽  
Vortex Vein ◽  
Clinical Records ◽  
Choroidal Vessels

Abstract We evaluated choroidal congestion using multimodal imaging in pachychoroid neovasculopathy (PNV). In a retrospective case series of 100 eyes of 99 treatment-naïve PNV patients, their clinical records were reviewed and the corresponding multimodal imaging studies were analyzed. We assessed areas of choriocapillaris filling delay which overlapped with dilated outer choroidal vessels, choroidal neovascularization (CNV), and retinal pigment epithelium (RPE) atrophy. The study subjects were 78 men (78.8%) and 21 women (21.2%). The mean patient age was 67.5 ± 10.5 years. On indocyanine green angiography, all eyes showed choriocapillaris filling delay in the early phase. Dilated outer choroidal vessels were demonstrated in all eyes by en face optical coherence tomography. The areas of choriocapillaris filling delay overlapped extensively with that of dilated outer choroidal vessels. All eyes showed CNV localized within the sites of choriocapillaris filling delay. RPE atrophy was noted in 71 eyes (71.0%), and 68 of these (95.8%) had RPE atrophy within the areas showing choriocapillaris filling delay. These findings indicate chronic choriocapillaris ischemia secondary to vortex vein congestion may lead to CNV development as well as RPE atrophy in eyes with PNV.

scholarly journals Chronic choriocapillaris ischemia in dilated vortex vein region in pachychoroid neovasculopathy

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Hidetaka Matsumoto ◽  
Junki Hoshino ◽  
Ryo Mukai ◽  
Kosuke Nakamura ◽  
Shoji Kishi ◽  
...  
Keyword(s):  
Multimodal Imaging ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Case Series ◽  
Retrospective Case ◽  
Rpe Atrophy ◽  
Pachychoroid Neovasculopathy ◽  
Vortex Vein ◽  
Clinical Records ◽  
Choroidal Vessels

AbstractWe evaluated choroidal congestion using multimodal imaging in pachychoroid neovasculopathy (PNV). In a retrospective case series of 100 eyes of 99 treatment-naïve PNV patients, their clinical records were reviewed and the corresponding multimodal imaging studies were analyzed. We assessed areas of choriocapillaris filling delay which overlapped with dilated outer choroidal vessels, choroidal neovascularization (CNV), and retinal pigment epithelium (RPE) atrophy. The study subjects were 78 men (78.8%) and 21 women (21.2%). The mean patient age was 67.5 ± 10.5 years. On indocyanine green angiography, all eyes showed choriocapillaris filling delay in the early phase. Dilated outer choroidal vessels were demonstrated in all eyes by en face optical coherence tomography. The areas of choriocapillaris filling delay overlapped extensively with that of dilated outer choroidal vessels. All eyes showed CNV localized within the sites of choriocapillaris filling delay. RPE atrophy was noted in 71 eyes (71.0%), and 68 of these (95.8%) had RPE atrophy within the areas showing choriocapillaris filling delay. These findings indicate that chronic choriocapillaris ischemia secondary to vortex vein congestion may lead to CNV development as well as RPE atrophy in eyes with PNV.

scholarly journals Focal Choroidal Excavation in a Case of Choroidal Osteoma Associated with Choroidal Neovascularization

2020 ◽  
Author(s):  
Mahdieh Azimizadeh ◽  
Seyedeh Maryam Hosseini ◽  
Esmaeil Babaei
Keyword(s):  
Choroidal Neovascularization ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Subretinal Fluid ◽  
Retinal Hemorrhage ◽  
Retinal Layers ◽  
Choroidal Osteoma ◽  
Focal Choroidal Excavation ◽  
Neurosensory Retina ◽  
The Right

Purpose: To report a case of choroidal osteoma associated with reactivation of choroidal neovascularization (CNV) and development of focal choroidal excavation (FCE). Case Report: A 34-year-old woman with choroidal osteoma complicated by CNV in the right eye for two years presented with deterioration of visual acuity in her right eye. A small retinal hemorrhage accompanied by subretinal fluid (SRF) was seen in the macular area of the right eye. Optical coherence tomography (OCT) showed that the inner retina was intact, and the outer retinal layers had outward displacement. SRF and a wedge-shaped choroidal depression were also seen. This choroidal excavation was not present on previous OCT images. The integrity of the inner retinal layers was maintained, and an optically clear space was present between the neurosensory retina and the retinal pigment epithelium. Conclusion: Choroidal osteoma can be complicated by CNV and FCE could occur as a consequence. Again, FCE can lead to CNV development. This cascade can deteriorate vision and sometime lead to permanent visual loss.

scholarly journals Central Serous Chorioretinopathy Induced by Work Stress: A Case Report

e-CliniC ◽  
2020 ◽  
Vol 8 (2) ◽  
Author(s):  
Ade J. Nursalim ◽  
Vera Sumual
Keyword(s):  
Risk Factors ◽  
Work Stress ◽  
Central Serous Chorioretinopathy ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Subretinal Fluid ◽  
Blurred Vision ◽  
Reading Text ◽  
Neurosensory Retinal Detachment ◽  
The Right

Abstrak: Central serous chorioretinopathy (CSC) adalah terkumpulnya cairan serosa di bawah lapisan epitel pigmen retina yang mengakibatkan terlepasnya retina neurosensorik (detachment). Keadaan ini dapat disebabkan oleh berbagai faktor risiko, termasuk stres psikologik. Jenis kelamin laki-laki merupakan salah satu faktor risiko CSC. Terapi kondisi psikologik merupakan pena-nganan utama CSC yang diinduksi oleh stres kerja. Kami melaporkan seorang laki-laki berusia 27 tahun yang datang ke Poliklinik Mata RSUP Prof. Dr. R. D. Kandou Hospital dengan keluhan kekaburan mata kanan yang mendadak sejak seminggu lalu tanpa didahului oleh nyeri pada mata. Pasien juga mengalami kesulitan dalam membaca teks dan mengenali wajah bila hanya menggunakan mata kanannya. Pasien telah pergi ke optik tetapi tidak mendapatkan ukuran kacamata yang sesuai. Pasien bekerja sebagai tenaga administrasi dan akhir-akhir ini mendapatkan beban kerja berlebihan. Pasien mengakui bahwa ia mengalami kesulitan dalam mengatur aktivitasnya, dan merasakan kelelahan sepanjang hari serta kehilangan minat kerja. Hasil pemeriksaan mata mendapatkan visus 6/15 untuk mata kanan dan visus 6/6 untuk mata kiri, dan metamorphopsia sedangkan hasil pemeriksaan oftalmoskopi memperlihatkan adanya edema dengan lingkaran kekuningan dan berbatas tidak jelas pada mata kanan. Pemeriksaan lanjut dengan OCT menunjukkan adanya cairan subretinal pada mata kanan disertai terlepasnya lapisan epitel pigmen. Saat kontrol setelah 36 hari, visus mata kanan telah membaik 6/6F2 dan peme-riksaan OCT menunjukkan penurunan tebal makula dari 289 μm pada kunjugan pertama men-jadi 190 μm, serta tidak tampak adanya cairan subretinal. Simpulan kasus ini ialah central serous chorioretinopathy yang diinduksi oleh stres kerja, dengan penanganan utama ialah terapi psikologik untuk memperbaiki kualitas hidup.Kata kunci: central serous chorioretinopathy (CSC), stres kerja Abstract: Central serous chorioretinopathy (CSC) is a condition where serous fluid builds up in the retinal pigment epithelium layer which causes neurosensory retinal detachment. This condition is affected by many risk factors, including psychological stress. Male gender is one of the risk factors for CSC. Treatment to the patient's psychological condition can be the main therapy in handling CSC induced by work stress. We reported a 27-year-old male came to the eye clinic at Prof. dr. R. D. Kandou Hospital complaining of sudden blurred vision in his right eye a week ago without initial pain in the eye. The patient also experienced difficulty in reading text and recognizing people’s face using the right eye. The patient went to an optic store but he did not find suitable glasses. The patient works as an administrative employee and is currently getting a heavy workload at his workplace. The patient admitted that he had issues in managing his life in a day. Patients felt tired throughout the day and had no interest in making any activities. The results of the eye examination showed vision 6/15 in the right eye and 6/6 in the left eye, metamorphopsia, edema with a yellowish circle with an unclear border on the right eye using ophthalmoscopy examination, and a subretinal fluid image in the patient's right eye with epithelial detachment pigment acquired through the examination with OCT. After 36 days from the first visit, the right eye vision was improved to 6/6 F2 and OCT examination resulted in a decrease in macula thickness from 289 μm at the first visit to 190 μm, and there was no subretinal fluid. In conclusion, this was a CSC case induced by work stress, and the main treatment was psychological therapy in order to improve the quality of life (QoL).Keywords: central serous chorioretinopathy (CSC), work stress

Pachychoroid Neovasculopathy; Findings, Diagnosis, and Treatment

2020 ◽  
pp. 112-117
Keyword(s):  
Optical Coherence Tomography ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Current Treatment ◽  
Age Related Macular Degeneration ◽  
Optical Coherence ◽  
Age Related ◽  
Pachychoroid Neovasculopathy ◽  
Sign Type

Pachychoroid neovasculopathy (PNV) is a novel clinical entity with typical pachychoroid features accompanied by type 1 choroidal neovascularization (CNV). Typical optical coherence tomography finding of the disease is flat irregular pigment epithelial detachments (double layer sign). Type 1 CNV between retinal pigment epithelium and Bruch membrane is seen as a tangled network on optical coherence tomography angiography. Differential diagnosis of PNV from neovascular age-related macular degeneration is especially important because of the different epidemiological, genetic, and therapeutic features of these diseases. Current treatment approaches include intravitreal anti-vascular endothelial growth factor injections and photodynamic therapy.

Intrachoroidal Dye Leakage in Indocyanine Green Fundus Angiography after Experimental Commotio Retinae

1992 ◽  
Vol 2 (2) ◽  
pp. 79-82 ◽  
Author(s):  
T. Miki ◽  
K. Kitashoji ◽  
T. Kohno
Keyword(s):  
Indocyanine Green ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Molecular Size ◽  
Sodium Fluorescein ◽  
Blood Retinal Barrier ◽  
Rabbit Eye ◽  
Icg Angiography ◽  
Commotio Retinae ◽  
Choroidal Vessels

Dye leakage in sodium fluorescein (FLUO) fundus angiography indicates damage to the blood-retinal barrier. However, dye leakage in indocyanine green (ICG) fundus angiography does not mean the same, because of the larger molecular size of the dye and impermeability of the choroidal vessels to it. The possibility of dye leakage in ICG angiography has not yet been revealed in an experimental study in which the blood-retinal barrier is undamaged. We report here that intrachoroidal dye leakage may occur in ICG angiography in an experimental model of the traumatic retinal opacity of the rabbit eye, even when the blood-retinal barrier is undamaged. This mechanism of dye leakage in ICG angiography is quite different from the leakage of FLUO angiography. Pathological choroidal vessels with increased permeability, such as choroidal neovascularization under the retinal pigment epithelium, can be observed using ICG angiography.

Evolution of retinal changes measured by optical coherence tomography in the assessment of hydroxychloroquine ocular safety in patients with systemic lupus erythematosus

Lupus ◽  
2019 ◽  
Vol 28 (4) ◽  
pp. 555-559 ◽  
Author(s):  
D Martín-Iglesias ◽  
J Artaraz ◽  
A Fonollosa ◽  
A Ugarte ◽  
A Arteagabeitia ◽  
...  
Keyword(s):  
Risk Factors ◽  
Optical Coherence Tomography ◽  
Lupus Erythematosus ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Optical Coherence ◽  
The Mean ◽  
Sd Oct

Objective The objective of this report is to analyse retinal changes over a five-year period, assessed by spectral domain-optical coherence tomography (SD-OCT), in patients from the Lupus-Cruces cohort treated with hydroxychloroquine (HCQ). Methods SD-OCT screening was performed annually between 2012 and 2017. Average macular thickness (AMT), ganglion cell layer thickness (GCLT) and qualitative data of retinal pigment epithelium (RPE) and external retina (ExtR) were collected prospectively. We compared data from 2012 (first) and 2017 (second) SD-OCT. Results We studied 110 patients and 195 eyes. No cases of HCQ toxicity were detected. At the time of the second SD-OCT, 99% patients had taken a daily dose of HCQ ≤5 mg/kg/day. The median time on HCQ was 133 months. The mean AMT and GCLT were significantly lower in both eyes at the second SD-OCT; however, all the differences were clinically insignificant at less than 1%. Qualitative analysis of RPE and ExtR showed no significant changes. Similar results were found among patients with risk factors for retinopathy. The comparison of patients with and without risk factors showed no differences. Conclusions This study shows clinically irrelevant retinal changes in an SLE cohort on HCQ treatment over a five-year follow-up. Our findings support the safety of long-term HCQ at doses ≤5 mg/kg/day.

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