Chronic choriocapillaris ischemia secondary to choroidal congestion in pachychoroid neovasculopathy

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.

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Chronic choriocapillaris ischemia in dilated vortex vein region in pachychoroid neovasculopathy

Scientific Reports ◽

10.1038/s41598-021-95904-9 ◽

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.

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Multimodal imaging evaluation of combined hamartoma of the retina and retinal pigment epithelium

European Journal of Ophthalmology ◽

10.1177/1120672119831223 ◽

2019 ◽

Vol 30 (3) ◽

pp. 595-599 ◽

Cited By ~ 1

Author(s):

Andrea Scupola ◽

Gabriela Grimaldi ◽

Maria G Sammarco ◽

Paola Sasso ◽

Michele Marullo ◽

...

Keyword(s):

Optical Coherence Tomography ◽

Retinal Pigment Epithelium ◽

Multimodal Imaging ◽

Optical Coherence Tomography Angiography ◽

Pigment Epithelium ◽

Retinal Pigment ◽

Case Series ◽

Variable Degree ◽

Optical Coherence ◽

Imaging Evaluation

Purpose: Combined hamartoma of the retina and retinal pigment epithelium is a rare benign tumor characterized by a variable combination of glial, vascular, and pigmented components. The purpose of our study was to analyze the features of combined hamartoma of the retina and retinal pigment epithelium on optical coherence tomography angiography. Methods: Small case series of two cases of combined hamartoma of the retina and retinal pigment epithelium with macular and optic nerve involvement, evaluated with multimodal imaging including optical coherence tomography, fluorescein angiography, and optical coherence tomography angiography. Results: On optical coherence tomography, combined hamartoma of the retina and retinal pigment epithelium is characterized by disruption of the inner neurosensory retina and a variable degree of involvement of the external retina. Optical coherence tomography angiography showed diffuse alterations of the retinal vessels of the superficial and deeper layers, extended to the peripapillary area. Vessel abnormalities included increased tortuosity and caliber of vessels, vascular traction, and vessel stretching within the lesion. Conclusion: Optical coherence tomography angiography allows in-depth multilayer analysis of tumor vascular network, highlighting the fine abnormalities of retinal vasculature characteristic of combined hamartoma of the retina and retinal pigment epithelium.

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Outcome of half-dose photodynamic therapy in chronic central serous chorioretinopathy with fovea-involving atrophy

Graefe s Archive for Clinical and Experimental Ophthalmology ◽

10.1007/s00417-020-04959-3 ◽

2020 ◽

Author(s):

Thomas J. van Rijssen ◽

Elon H. C. van Dijk ◽

Paula Scholz ◽

Robert E. MacLaren ◽

Sascha Fauser ◽

...

Keyword(s):

Photodynamic Therapy ◽

Central Serous Chorioretinopathy ◽

Multimodal Imaging ◽

Pigment Epithelium ◽

Retinal Pigment ◽

Final Visit ◽

Chronic Central Serous Chorioretinopathy ◽

Half Dose ◽

Rpe Atrophy ◽

The Mean

Abstract Purpose To evaluate the clinical outcomes after half-dose photodynamic therapy (PDT) in chronic central serous chorioretinopathy (cCSC) patients with pre-existent fovea-involving atrophy. Methods In this retrospective study, cCSC patients who had a window defect of the retinal pigment epithelium (RPE) on fluorescein angiography (FA), compatible with RPE atrophy, prior to half-dose PDT were included. Results Thirty-four cCSC eyes with typical findings of cCSC on multimodal imaging, and fovea-involving RPE atrophy on FA, were included. At the first visit after PDT (at a median of 1.8 months after half-dose PDT), 20 eyes (59%) had a complete resolution of SRF (p < 0.001), while this was the case in 19 eyes (56%) at final visit (median of 11.3 months after half-dose PDT; p < 0.001). The mean BCVA in Early Treatment of Diabetic Retinopathy Study letters was 71. 2 ± 15.9 at last visit before PDT, which increased to 74.1 ± 14.1 at first visit after PDT (p = 0.093, compared with baseline), and changed to 73.0 ± 19.1 at final visit (p = 0.392, compared with baseline). Both at first visit after PDT and at final visit, a significant decrease in subfoveal choroidal thickness was observed (p = 0.032 and p = 0.004, respectively). Conclusions Half-dose PDT in cCSC patients with pre-existing fovea-involving atrophy may lead to anatomical changes, but not to functional improvements. Ideally, cCSC should be treated with half-dose PDT before the occurrence of such atrophy.

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Acute Zonal Occult Outer Retinopathy (AZOOR) Results from a Clinicopathological Mechanism Different from Choriocapillaritis Diseases: A Multimodal Imaging Analysis

Diagnostics ◽

10.3390/diagnostics11071184 ◽

2021 ◽

Vol 11 (7) ◽

pp. 1184

Author(s):

Carl P. Herbort ◽

Ilir Arapi ◽

Ioannis Papasavvas ◽

Alessandro Mantovani ◽

Bruno Jeannin

Keyword(s):

Visual Field ◽

Multimodal Imaging ◽

Pigment Epithelium ◽

Retinal Pigment ◽

Immunosuppressive Treatment ◽

Case Series ◽

Fundus Photography ◽

Ganglion Cell Complex ◽

Ophthalmological Examination ◽

Sequence Of Events

Background and aim: AZOOR is a rare disease characterized by loss of zones of outer retinal function, first described by J Donald Gass in 1993. Symptoms include acute onset photopsias and subjective visual field losses. The syndrome is characterized by a normal fundus appearance, scotomas and electroretinographic changes pointing towards outer retinal dysfunction. Evolution, response to immunosuppressive treatment and outcome are difficult to predict. The aim of this small case series was to identify the morphological changes and sequence of events in AZOOR thanks to multimodal imaging. Methods: Charts of AZOOR patients seen in the Centre for Ophthalmic Specialized care (COS, Lausanne, Switzerland) were analyzed by multimodal imaging including fundus photography, fluorescein angiography (FA), indocyanine green angiography (ICGA), blue light fundus autofluorescence (BL-FAF) and spectral domain optical coherence tomography (SD-OCT) in addition to a complete ophthalmological examination including visual field testing and microperimetry, as well as OCT angiography (OCT-A) and ganglion-cell complex analysis when available. Cases and Results: Three AZOOR patients with a mean follow-up of 47 ± 25.5 months were included following the clinical definitions laid down by J Donald Gass. The primary damage was identified at the level of the photoreceptor outer segments with an intact choriocapillaris and retinal pigment epithelium (RPE) layer, these structures being only secondarily involved with progression of the disease. Conclusion: Although AZOOR has often been included within white dot syndromes, some of which are now known to be choriocapillaris diseases (choriocapillaritis entities), our findings clearly commend to differentiate AZOOR from entities such as MEWDS (Multiple evanescent white dot syndrome), APMPPE (Acute Posterior Multifocal Placoid Pigment Epitheliopathy), MFC (Multifocal Choroiditis) and others, as the damage to photoreceptors is primary in AZOOR (a retinopathy) and secondary in choriocapillaritis (a choriocapillaropathy).

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The Spectrum of Maculopathy in Mitochondrial DNA A3243G Mutation: A Case Series of Six Patients

Klinische Monatsblätter für Augenheilkunde ◽

10.1055/a-1386-5826 ◽

2021 ◽

Vol 238 (04) ◽

pp. 414-417

Author(s):

Eirini Kaisari ◽

François-Xavier Borruat

Keyword(s):

Mitochondrial Dna ◽

Point Mutation ◽

Pigment Epithelium ◽

Retinal Pigment ◽

Case Series ◽

Progressive External Ophthalmoplegia ◽

Ocular Involvement ◽

Mitochondrial Dna Mutation ◽

Female Patients ◽

Rpe Atrophy

Abstract Background The mitochondrial DNA (mtDNA) A3243G point mutation encompasses a heterogenous group of disorders including mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), maternally inherited diabetes and deafness (MIDD), and, rarely, chronic progressive external ophthalmoplegia (CPEO). Regardless of the clinical phenotype, a specific retinopathy has been associated with the presence of this mitochondrial DNA mutation. We present six female patients exhibiting retinopathy of the A3243G point mutation at various stages. History and Signs Six female patients (37 – 70 years old) with the A3243G point mutation (four MELAS, one MIDD, and one CPEO) exhibited a maculopathy. Visual acuity ranged from 1/60 to 10/10. Visual field abnormalities varied from minimal decreased sensitivity to absolute central scotomas. They all exhibited, at various degrees, a characteristic pattern of perimacular and peripapillary retinal pigment epithelium (RPE) alterations, with mottled dys-autofluorescence and RPE atrophy and deposits on OCT. Therapy and Outcome The level of visual impairment depended on the foveal involvement and the extension of RPE atrophy. The severity of the maculopathy was not related to age. In the only long-term follow-up (15 years), evolution was slowly progressive. Conclusions A single mtDNA point mutation at locus 3243 can result in a variety of clinical presentations (MELAS, MIDD, or CPEO). Ocular involvement may manifest as a perimacular/peripapillary RPE atrophy/deposit, which can variably impact central visual function (from asymptomatic to legal blindness). The discovery of such a maculopathy should prompt the ophthalmologist to complete the personal and family history, namely, asking for the presence of diabetes mellitus and/or deafness.

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Choroidal congestion mouse model: Could it serve as a pachychoroid model?

PLoS ONE ◽

10.1371/journal.pone.0246115 ◽

2021 ◽

Vol 16 (1) ◽

pp. e0246115

Author(s):

Hidetaka Matsumoto ◽

Ryo Mukai ◽

Junki Hoshino ◽

Mai Oda ◽

Toshiyuki Matsuzaki ◽

...

Keyword(s):

Mouse Model ◽

Pigment Epithelium ◽

Retinal Pigment ◽

Posterior Pole ◽

Fundus Photography ◽

Control Group ◽

Choroidal Thickening ◽

Tissue Sections ◽

Vortex Vein ◽

Choroidal Vessels

Pachychoroid spectrum diseases have been described as a new clinical entity within the spectrum of macular disorders. “Pachychoroid” is defined as choroidal thickening associated with dilated outer choroidal vessels often showing retinal pigment epithelium (RPE) degeneration. Although various clinical studies on the pachychoroid spectrum diseases have been conducted, the pathophysiology of pachychoroid has yet to be fully elucidated. In this study, we attempted to establish a mouse model of pachychoroid. We sutured vortex veins in eyes of wild type mice to imitate the vortex vein congestion in pachychoroid spectrum diseases. Fundus photography and ultra-widefield indocyanine green angiography showed dilated vortex veins from the posterior pole to the ampulla in eyes after induction of choroidal congestion. Optical coherence tomography and tissue sections presented choroidal thickening with dilatation of choroidal vessels. The RPE-choroid/retina thickness ratios on the tissue sections in the treated day 1 and day 7 groups were significantly greater than that in the control group (0.19±0.03 and 0.16±0.01 vs. 0.12±0.02, P<0.05 each). Moreover, immunohistochemistry using RPE flatmount revealed focal RPE degeneration in the treated eyes. Furthermore, inflammatory response-related genes were upregulated in eyes with choroidal congestion induction, and macrophages migrated into the thickened choroid. These results indicated that vortex vein congestion triggered some pachychoroid features. Thus, we have established a choroidal congestion mouse model by suturing vortex veins, which would potentially be useful for investigating the pathophysiology of pachychoroid spectrum diseases.

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Degenerative lamellar macular holes: tractional development and morphological alterations

International Ophthalmology ◽

10.1007/s10792-020-01674-0 ◽

2021 ◽

Author(s):

Andreas Bringmann ◽

Jan Darius Unterlauft ◽

Renate Wiedemann ◽

Thomas Barth ◽

Matus Rehak ◽

...

Keyword(s):

Surgical Treatment ◽

Wound Repair ◽

Pigment Epithelium ◽

Retinal Pigment ◽

Case Series ◽

Retrospective Case ◽

Epiretinal Membranes ◽

Morphological Alterations ◽

Macular Holes ◽

Foveal Center

Abstract Purpose The development of degenerative lamellar macular holes (DLH) is largely unclear. This study was aimed at documenting with spectral-domain optical coherence tomography the tractional development and morphological alterations of DLH. Methods A retrospective case series of 44 eyes of 44 patients is described. Results The development of DLH is preceded for months or years by tractional deformations of the fovea due to the action of contractile epiretinal membranes (ERM) and/or the partially detached posterior hyaloid, or by cystoid macular edema (CME). DLH may develop after a tractional stretching and thickening of the foveal center, from a foveal pseudocyst, after a detachment of the foveola from the retinal pigment epithelium, a disruption of the foveal structure due to CME, and after surgical treatment of tractional lamellar or full-thickness macular holes (FTMH). The foveal configuration of a DLH can be spontaneously reestablished after short transient episodes of CME and a small FTMH. A DLH can evolve to a FTMH by traction of an ERM. Surgical treatment of a DLH may result in an irregular regeneration of the foveal center without photoreceptors. Conclusions Tractional forces play an important role in the development of DLH and in the further evolution to FTMH. It is suggested that a DLH is the result of a retinal wound repair process after a tractional disruption of the Müller cell cone and a degeneration of Henle fibers, to prevent a further increase in the degenerative cavitations.

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

Güncel Retina Dergisi (Current Retina Journal) ◽

10.37783/crj-0198 ◽

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.

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Small Choroidal Melanoma: Correlation of Growth Rate with Pathology

Ocular Oncology and Pathology ◽

10.1159/000517203 ◽

2021 ◽

pp. 1-10

Author(s):

Vishal Raval ◽

Shiming Luo ◽

Emily C. Zabor ◽

Arun D. Singh

Keyword(s):

Growth Rate ◽

Retinal Pigment ◽

Choroidal Melanoma ◽

Case Series ◽

Subretinal Fluid ◽

Categorical Variables ◽

Continuous Variables ◽

Basal Diameter ◽

Orange Pigment ◽

Rpe Atrophy

Purpose: The aim of the study was to evaluate equivalence of growth rate and pathologic confirmation in small choroidal melanoma (SCM). Design: This study is a case series. Subjects, Participants, and Controls: A total of 61 patients with a choroidal melanocytic tumor of size 5.0–16.0 mm in the largest basal diameter and 1.0–2.5 mm in thickness were classified into the pathology-confirmed group (n = 19), growth-confirmed group (n = 30), and with combined observations (n = 12). Methods: Distribution of clinical variables (age, gender, laterality, tumor dimensions, tumor location, and presence of orange pigment, subretinal fluid, drusen, and retinal pigment epithelial [RPE] atrophy) between the groups was analyzed. Patient and disease characteristics were summarized as the median and interquartile range for continuous variables and the frequency and percentage for categorical variables. Comparisons were made using the Wilcoxon rank sum test for continuous variables and either Fisher’s exact test or the χ2 test for categorical variables with a p value threshold of 0.05 for statistical significance. Growth rate (change in basal dimension/12 months) diagnostic of SCM was quantified. Main Outcome Measures: The primary aim of this study was to test the hypothesis that “growth” was diagnostic of SCM with the secondary aim of quantifying the malignant “growth rate” (growth rate of SCM). Results: The clinical characteristics among all 3 groups were similar except more patients with symptoms (68 vs. 20 vs. 42%, p = 0.004) and juxtapapillary location (p = 0.03) were in the pathology group than in the growth-confirmed group. Those in the combined and growth-confirmed groups had more patients with drusen (11 vs. 60 vs. 50%, p = 0.003) and RPE atrophy (11 vs. 23 vs. 67%, p = 0.003), respectively, than in the pathology group. The median time to detect growth was 9 months (range 3–26 months). The mean growth rate in basal dimension was 1.8 mm/12 months (range, 0.0–7.4 mm; [95% CI: 1.32–2.28]). Conclusions and Relevance: Choroidal melanocytic lesions exhibiting a defined growth rate can be clinically diagnosed as SCM without a need for biopsy.

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