Pachychoroid disease

Abstract

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.

Clinical Characteristics of Polypoidal Choroidal Vasculopathy and Anti-Vascular Endothelial Growth Factor Treatment Response in Caucasians

“…Many eyes with PCV in Asian patients have been noted to have abnormal choroidal architecture with a dilated Haller's layer and attenuated choriocapillaris [26]. This anatomic finding was noted in 29.8% (14/ mean subfoveal thickness of fellow eyes without PCV was 195.7 µm (range 97-363 µm).…”

“…As seen in other series of PCV, RPED was frequent, occurring in 51.9% of cases. RPED has been noted to be more common in PCV than in typical exudative AMD and this represented a distinguishing factor on OCT [26]. Serous retinal detachment was noted in 66.7% of cases with PCV.…”

Five-Year Follow-Up of Unaffected Fellow Eyes in Patients with Polypoidal Choroidal Vasculopathy

“…Ac-cordingly, even with no active polyps, eyes with BVN may develop into a nAMD and should be closely monitored. Ueta et al [11] reported that abnormal choroidal vascular networks were found in more than half of the PCV patients before appearance of active form, with prominent vascular hyperpermeability [27]. Kim et al [31] reported that late geographic hyperfluorescence (LGH) on ICGA might arise in eyes with immature BVN.…”

Choroidal morphology under pachydrusen

“…Previous studies have revealed that eyes with CSC tend to exhibit more prominent, consistent and diffuse thickening with diffusely distributed pachvessels. PCV eyes have a wide variation in choroidal thickness and distribution pattern of pachyvessels . Even among the eyes with thinner choroids, pachyvessels accompanied by attenuation of the choriocapillaris are topographically associated with new vessel ingrowth sites .…”

“…PCV eyes have a wide variation in choroidal thickness and distribution pattern of pachyvessels. [16][17][18][19][20] Even among the eyes with thinner choroids, pachyvessels accompanied by attenuation of the choriocapillaris are topographically associated with new vessel ingrowth sites. 4,21 Pachydrusen showed similar qualitative choroidal changes.…”