Pachychoroid disease

• Published in: Eye (London) Vol. 33; no. 1; pp. 14 - 33
• Main Authors: Cheung, Chui Ming Gemmy, Lee, Won Ki, Koizumi, Hideki, Dansingani, Kunal, Lai, Timothy Y. Y., Freund, K. Bailey
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 01-01-2019; Nature Publishing Group
• Subjects: 692/308/409; 692/699/3161/1626; Aneurysm; Choroid - pathology; Choroid Diseases - diagnosis; Disease; Epithelium; Fluorescein Angiography - methods; Fundus Oculi; Genotype & phenotype; Humans; Laboratory Medicine; Medicine; Medicine & Public Health; Morphology; Ophthalmology; Pathogenesis; Pharmaceutical Sciences/Technology; Phenotypes; Retina; Retinal pigment epithelium; Retinal Pigment Epithelium - pathology; Review; Review Article; Surgery; Surgical Oncology; Tomography; Tomography, Optical Coherence - methods; Vascular diseases; Vascularization
• ISSN: 0950-222X; 1476-5454; 1476-5454
• DOI: 10.1038/s41433-018-0158-4

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.