Abstract
This review presents a systematic analysis of the literature regarding the pachychoroid disease spectrum nomenclature, diagnosis criteria and therapeutic options. Pachychoroid related diseases are a new concept, introduced into the ophthalmological nomenclature in 2013 and evolving both as concept and as classification since then. The six disorders included in this phenotype have some common characteristics (thick choroid, pachyvessels, attenuation of the choriocapillaris), but also show individual features. The classification of the pachychoroid spectrum was revised many times, with the recent addition of the focal choroidal excavation (FCE) and peripapillary pachychoroid syndrome (PPS). As the terminology is developing, so is the number of case reports and case series from the initial report in 2013 to 57 reports in 2019. This review takes into account both the current literature and the clinical experience of the authors, emphasizing the understanding of the pathogenesis and aiming to update the therapeutic options available.