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(a)-(b) Colour fundus photography showed arteriolar attenuation, peripapillary atrophy, optic disc pallor, macular hemorrhage and typical retinal pigment deposits (bone spicules) involving the mid-peripheral retina (c) Indocyanine green angiography (ICGA) revealed a well-distinct “triangular shape” area of intense hypercyanescence (“hot spot”) corresponding to the neovascularization (d) B-Scan optical coherence tomography angiography revealed high-flow intraretinal neovascularization originated in the superficial layer descending into a tuft-shaped lesion toward the sub-RPE space. (e)–(h) Optical Coherence Tomography Angiography images (6 × 6 mm) revealing the progression of the tuft-shaped lesion from the superficial layer to choroid capillary. (e) Superficial capillary plexus segmentation. (f) Deep capillary plexus segmentation. (g) Outer retinal layer segmentation. (h) Choriocapillaris segmentation. (i)–(j) Spectral Domain-Optical Coherence Tomography at baseline. (k)–(l) Spectral Domain-Optical Coherence Tomography after treatment.
Source publication
Purpose:
To describe the first case of bilateral retinal angiomatous proliferation (RAP) in a patient with a variant of retinitis pigmentosa (RP).
Case report:
An 85-year-old man with RP presented with visual acuity decrease and metamorphopsia in the left eye (LE). Fundus examination revealed typical signs of RP in both eyes, associated with int...
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Background
Electroretinogram (ERG) plays an essential role in the diagnosis of retinal disease. Choosing appropriate methods could extract valuable information from ERG. In this study, a new criterion based on time–frequency domain analysis was proposed to investigate the retina in retinitis pigmentosa (RP) patients.
Materials and methods
The tota...
Citations
... Notably, no lesions compatible to MNV1 or 2 were observed [39]. Likewise, all recent case reports of retinitis pigmentosa using OCT angiography identified only MNV3-like lesions as a sequela [40,41]. This distinguishing retinal neovascularization developed after a primary severe loss of rods reinforces the hypothesis that rod degeneration is an indispensable event for the development of MNV3 lesions [13]. ...
Macular neovascularization type 3 (MNV3) is a multifactorial disease with distinct epidemiological, clinical, pathomorphological and topographical characteristics. This review of the literature discusses the latest experimental and clinical outcomes that could explain the pathogenesis of retinal neovascularization. Although patients with MNV3 are usually older than those with MNV1 or 2, their lesions do not coexist with, precede, or follow other types in the same eye. The regional distribution of MNV3 lesions is characterized as confined to the parafoveal macula without any involvement of the rod-free foveal area. Focal outer retinal atrophy and choroidal non-perfusion are the main structural features that occur prior to the development of retinal neovascularization. Also, histological and experimental studies of MNV3 and other non-neovascular age-related macular degeneration diseases complicated with MNV3-like lesions strongly suggest rod degeneration contributes to the pathogenesis. Therefore, the retinal neovascularization in MNV3 has a different pathogenesis from the choroidal neovascularization in MNV1 and 2 and emerging evidence indicates that choroidal non-prefusion and rod degeneration play a key role in the pathogenesis of MNV3. Accordingly, we suggest a sequence of pathological events that start with choroidal non-perfusion due to advanced age followed by hypoxia of the outer retina at the parafoveal area. This induces a remarkable degeneration of rods that triggers the growth of retinal neovascularization due to the imbalance of the angiogenic factors in the outer retina.
... Choroidal neovascularization (CNV) has been associated with several IRDs: Stargardt disease, Best vitelliform dystrophy, Sjögren reticular dystrophy, pattern dystrophy, gyrate atrophy, Sorsby fundus dystrophy, and RP [10,11]. In RP, CNV is extremely rare [11], with the majority of case reports describing either classic CNV [12][13][14][15], pachychoroid neovasculopathy [12], Genes 2023, 14, 1438 2 of 8 or RAP lesions [16][17][18]. In our study, we present a case report of a patient with RP and a RAP lesion diagnosed using multimodal imaging. ...
... Central vision can also be affected earlier due to the CMO, vitreomacular traction, epiretinal membrane, and macular hole [5, 19,20]. One of the causes of central vision loss in RP could be a RAP lesion, which is extremely rare, having been reported hitherto in only three case reports [16][17][18]. ...
... We were unable to determine whether the RAP lesion in our patient was associated with her RP or whether it occurred within the neovascular AMD spectrum independent of the RP. In all case reports where RAP and RP were described, patients were 60 years of age or older [16][17][18]. By contrast, in the case reports with RP and type 2 CNV, the patients were 40 years old [13,31]. ...
Retinal angiomatous proliferation (RAP) and other types of choroidal neovascularization (CNV) are very rarely reported in patients with retinitis pigmentosa (RP). We present a case report of a 91-year-old patient with an obvious RP phenotype, who presented with a sudden onset of vision worsening and metamorphopsia in the left eye. Genetic testing on the UK inherited retinal disease panel did not identify a pathogenic variant. Multimodal imaging comprising optical coherence tomography (OCT), OCT angiography, and fluorescein and indocyanine green angiography showed a RAP lesion in the left macula. The patient received three treatments of monthly injections of aflibercept, with excellent morphological and functional outcomes. Taking into account the patient’s age at presentation of the RAP lesion, it is not clear whether the RAP was associated or coincidental with RP. This case report highlights the importance of possessing an awareness that RAP lesions can occur in RP. Moreover, due to a good response and potential safety concerns with continuous anti-VEGF injections in RP patients, a pro re nata (PRN) regimen might be the safest option.
... The angiogenic network usually originates from the retinal vessels at first and extends deeply through the retinal pigment epithelium to the subretinal space and anastomose with the underlying choroidal neovessels eventually [1,2]. Many crucial differences from the other two MNV types have recently been raised, for example, its tendency to distribute in the temporal half of the macula, the contribution of the cilioretinal artery (CRA), and the possible role of disturbance of choroidal blood perfusion and rod damage on its pathogenesis process [3][4][5][6]. Moreover, this type affects patients older than those with type 1 or 2 and can be bilateral in up to all cases [7][8][9]. ...
Purpose
To report on the morphological characteristics and regional distribution of multifocal macular neovascularization type 3 (mMNV3).
Methods
Twenty-two consecutive eyes of 21 patients with mMNV3 were included using multimodal imaging. The count and stage of lesions of all MNV types and the existence of exudate and hemorrhage were determined. Also, we addressed the regional distribution of MNV3 lesions between the superior-inferior and the nasal-temporal halves of the macula, and the range of the distance of the lesions from the central fovea. Furthermore, we explored the number of feeding vessels including the cilioretinal artery.
Results
We found 51 lesions in 22 eyes of 21 patients. They were bifocal in 16 (73%) eyes, trifocal in 5 (23%), and quadrifocal in one (4%). No lesion of MNV1 or 2 was found. Fifteen (68%), 2 (9%), and 16 (73%) eyes were associated with retinal hard exudate, subretinal pigment epithelium exudate, and intraretinal hemorrhage, respectively. Thirty (59%) lesions were located in the temporal half of the macula, whereas 21 (41%) were located nasally ( p = 0.07). One (2%) lesion was closer than 500 µm, 49 (96%) between 500 and 1500 µm, and one (2%) between 1500 and 3000 µm. The lesions were supplied by one arteriole in one (4%) eye, two arterioles in 16 (73%) eyes, and 3 arterioles in 5 (23%) eyes. The CRA contributed as a feeding vessel in 5 (23%) eyes.
Conclusion
The multifocal variant of MNV3 has specific morphological and topographical characteristics. Multimodal imaging allows the understanding of the pathomorphological condition in more detail.
... Furthermore, oxidative stress or inflammatory processes in the retina may induce VEGF in these diseases Oltra et al. 2020). In RP, neovascularization and cystoid edemas have occasionally been reported and can be treated with anti-VEGF therapy (Yuzbasioglu et al. 2009;Sayadi et al. 2017;Miyata et al. 2018;Aloe et al. 2019; Montoya Delgado, R ıos Nequis, and Ram ırez Estudillo 2019). However, neovascularization is not a common feature of RP. ...
We rely on vision more than on any other sense to obtain information about our environment. Hence, the loss or even impairment of vision profoundly affects our quality of life. Diet or food components have already demonstrated beneficial effects on the development of retinal diseases. Recently, there has been a growing interest in resources from marine animals and plants for the prevention of retinal diseases through nutrition. Especially fish intake and omega-3 fatty acids have already led to promising results, including associations with a reduced incidence of retinal diseases. However, the underlying molecular mechanisms are insufficiently explained. The aim of this review was to summarize the known mechanistic effects of marine resources on the pathophysiological processes in retinal diseases. We performed a systematic literature review following the PRISMA guidelines and identified 107 studies investigating marine resources in the context of retinal diseases. Of these, 46 studies described the underlying mechanisms including anti-inflammatory, antioxidant, antiangiogenic/vasoprotective, cytoprotective, metabolic, and retinal function effects, which we critically summarize. We further discuss perspectives on the use of marine resources for human nutrition to prevent retinal diseases with a particular focus on regulatory aspects, health claims, safety, and bioavailability.
Inherited retinal diseases (IRDs) are the most common cause of blindness in working-age adults. Macular neovascularisation (MNV) may be a presenting feature or occur as a late-stage complication in several IRDs. We performed an extensive literature review on MNV associated with IRDs. MNV is a well-known complication of Sorsby fundus dystrophy and pseudoxanthoma elasticum. Those with late-onset Stargardt disease may masquerade as exudative age-related macular degeneration (AMD) when MNV is the presenting feature. Peripherinopathies may develop MNV that responds well to a short course of anti-vascular endothelial growth factor (anti-VEGF) therapy, while bestrophinopathies tend to develop MNV in the early stages of the disease without vision loss. Enhanced S-cone syndrome manifests type 3 MNV which typically regresses into a subfoveal fibrotic nodule. MNV is only a rare complication in choroideraemia and rod-cone dystrophies. Most IRD-related MNV exhibit a favourable visual prognosis requiring less intensive regimens of anti-VEGF therapy as compared to AMD. We discuss the role of key imaging modalities in the diagnosis of MNV across a wide spectrum of IRDs and highlight the gaps in our knowledge with respect to the natural history and prognosis to pave the way for future directions of research.
Optical coherence tomography angiography (OCTA) is a noninvasive new imaging modality that can be used to diagnose and monitor progression of retinitis pigmentosa (RP). Cohorts and case series have shown correlation between OCTA findings and visual function and disease severity. Although an early use of the technology is promising, there are concerns about segmentation errors and artifacts. There is also a paucity of data on genotype and how that correlates with OCTA findings. Despite its limitations, OCTA remains a useful tool for clinicians managing retinitis pigmentosa patients.Key wordsOptical coherence tomography angiographyRetinitis pigmentosaTraditional intravenous fluorescein angiographyIndocyanine green angiographyVasculatureChoriocapillaris
