Peripapillary Pachychoroid Syndrome

Phasukkijwatana, Nopasak; Freund, K. Bailey; Dolz-Marco, Rosa; Al-Sheikh, Mayss; Keane, Pearse A.; Egan, Catherine; Randhawa, Sandeep; Stewart, Jay M.; Liu, Qingyun; Hunyor, Alex P.; Kreiger, Allan E.; Nagiel, Aaron; Lalane, Robert; Rahimi, Masoud; Lee, Won Ki; Jampol, Lee M.; Sarraf, David

doi:10.1097/iae.0000000000001907

Cited by 123 publications

(136 citation statements)

References 28 publications

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“…Increased choroidal thickness is thought to result predominantly from dilatation of choroidal vessels in Haller's layer. Increased diameter of choroidal vessels has been observed as larger hyporeflective lumen in cross-sectional OCT in eyes with CSC, PCV, focal choroidal excavation (FCE), and PPS [22,23]. In histological sections in eyes with PCV, dilated choroidal vessels with diameter of up to 300 µm have been observed.…”

Section: Pachyvesselsmentioning

confidence: 97%

“…Peripapillary pachychoroid syndrome was recently described by Phasukkijwatana and colleagues as distinct variant of the pachychoroid disease spectrum in which maximal choroidal thickness occurs close to the optic nerve rather than subfovealy [23]. These patients typically present with nasal macular intraretinal and/or SRF and occasional optic nerve edema.…”

Section: Peripapillary Pachychoroid Syndromementioning

confidence: 99%

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Pachychoroid disease

Cheung

Lee

Koizumi

et al. 2018

Eye

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Pachychoroid is a relatively novel concept describing a phenotype characterized by attenuation of the choriocapillaris overlying dilated choroidal veins, and associated with progressive retinal pigment epithelium dysfunction and neovascularization. The emphasis in defining pachychoroid-related disorders has shifted away from simply an abnormally thick choroid (pachychoroid) toward a detailed morphological definition of a pathologic state (pachychoroid disease) with functional implications, which will be discussed in this review. Several clinical manifestations have been described to reside within the pachychoroid disease spectrum, including central serous chorioretinopathy, pachychoroid pigment epitheliopathy, pachychoroid neovasculopathy, polypoidal choroidal vasculopathy/aneurysmal type 1 neovascularization, 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. This review is based on both the current literature and the clinical experience of our individual authors, with an emphasis on the clinical and imaging features, management considerations, as well as current understanding of pathogenesis of these disorders within the context of the recent findings related to pachychoroid disease.

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Section: Pachyvesselsmentioning

confidence: 97%

Section: Peripapillary Pachychoroid Syndromementioning

confidence: 99%

Pachychoroid disease

Cheung

Lee

Koizumi

et al. 2018

Eye

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533

525

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“…Based on multimodal imaging studies, pachychoroid has been postulated to be associated with increased hydrostatic pressure in the choroid causing disruption of Bruch's membrane, RPE dysfunction, and inner choroidal ischemia [8], and has been considered an underlying cause of a disease spectrum that includes pachychoroid pigment epitheliopathy, central serous chorioretinopathy, and pachychoroid neovasculopathy (type 1 neovascularization associated with shallow irregular RPE detachment with or without polyps). It is likely that pachychoroid is also associated with focal choroidal excavation [9], geographic atrophy (pachychoroid geographic atrophy [10]), peripapillary exudative changes (peripapillary retinoschisis [11] and peripapillary pachychoroid syndrome [12]) and drusen with a peculiar shape (pachydrusen [13]), although there is some debate as to whether pachychoroid is a primary cause of these conditions.…”

Section: Pachychoroid and Pcvmentioning

confidence: 99%

Asian age-related macular degeneration: from basic science research perspective

Yanagi

Foo

Yoshida

2018

Eye

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In Asian populations, polypoidal choroidal vasculopathy (PCV), a distinct phenotype of neovascular age-related macular degeneration (AMD), is more prevalent than Caucasians. Recently, there has been significant focus on how PCV differs from typical AMD. Although typical AMD and PCV share a variety of mechanisms by which abnormal angiogenic process occurs at the retinochoroidal interface, PCV has different clinical characteristics such as aneurysm-like dilation at the terminal of choroidal neovascular membranes, less frequent drusen and inner choroidal degeneration due to the thickened choroid. Recent studies support an important role for inflammation, angiogenesis molecules and lipid metabolism in the pathogenesis of neovascular AMD. Furthermore, although less attention has been paid to the role of the choroid in AMD, accumulating evidence suggests that the choriocapillaris and choroid also play a pivotal role in drusenogenesis, typical AMD and PCV. This review discusses the basic pathogenic mechanisms of AMD and explores the difference between typical AMD and PCV.

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“…Pathologically dilated vessels in Haller's layer (pachyvessels) with overlying choriocapillaris attenuation are an essential feature required to define pachychoroid . This feature was originally described in the first article that introduced pachychoroid and has consistently been described in subsequent articles on pachychoroid disorders . Since it is a pathognomonic finding that defines the condition, it is considered to be related to the pathogenic mechanism of the diseases.…”

Section: Introductionmentioning

confidence: 99%

“…1,2 This feature was originally described in the first article that introduced pachychoroid and has consistently been described in subsequent articles on pachychoroid disorders. [3][4][5] Since it is a pathognomonic finding that defines the condition, it is considered to be related to the pathogenic mechanism of the diseases.…”

mentioning

confidence: 99%

Choroidal morphology under pachydrusen

Baek

Lee

Chung

et al. 2018

Clinical Exper Ophthalmology

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Importance To reveal choroidal morphological profiles under pachydrusen. Background Drusen in pachychoroid disorders show certain differences from conventional drusen and are recently named as “pachydrusen.” This study analysed the specific choroidal morphology under pachydrusen. Design This study is a cross‐sectional case series. Participants Enrolled were 136 eyes with pachydrusen from 134 patients. Methods The presence of pachydrusen in fundus area covered by a 12 × 9 mm2 optical coherence tomography (OCT) image was accessed in eyes with pachychoroid‐related diseases or their fellow eyes using colour fundus photography and swept source OCT. Choroidal morphology under the pachydrusen was analysed using OCT B‐scans and en face images. Main Outcome Measures Choroidal thicknesses and topographical correlation between pachydrusen and pachyvessels. Results A total of 225 pachydrusen in 136 eyes were analysed. The mean number of pachydrusen was 1.65 ± 1.07 per eye. Most pachydrusen were located para‐ or perifoveally (37 subfoveal, 86 parafoveal and 102 perifoveal). The proportion of Haller's layer to total choroidal thickness was higher at the area of the pachydrusen compared to the subfovea (0.881 ± 0.081 vs 0.765 ± 0.111, P < 0.001). In multimodal image analysis, 90.1% of pachydrusen identified using fundus photography and OCT B‐scan were located at the area of a dilated Haller vessel (pachyvessel) seen on en face images. Conclusions and Relevance Choroidal morphology under the pachydrusen showed increased Haller's layer thickness with an attenuated choriocapillaris layer, which is the hallmark of pachychoroid definition. Topographically, their locations correlated with the underlying pachyvessel.

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Peripapillary Pachychoroid Syndrome

Cited by 123 publications

References 28 publications

Pachychoroid disease

Pachychoroid disease

Asian age-related macular degeneration: from basic science research perspective

Choroidal morphology under pachydrusen

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