Pachychoroid diseases of the macula: an update.

Background: The pachychoroid disease spectrum comprises a group of chorioretinal disorders characterized by structural and hemodynamic alterations of the choroid that may ultimately lead to dysfunction of the retinal pigment epithelium, choroidal neovascularization, and vision loss. This narrative review aims to summarize the recent advances in the understanding of pachychoroid spectrum diseases, with particular emphasis on pathophysiology, multimodal imaging, and current therapeutic approaches. Methods: Relevant English-language literature on pachychoroid spectrum disorders published up to 31 May 2026 was reviewed through PubMed/MEDLINE, supplemented by Google Scholar to identify additional relevant studies; relevant conference abstracts presented by that date and published subsequently were also included. Focus was on recent developments in disease mechanisms, imaging characteristics, clinical phenotypes, and management strategies. Search terms included "pachychoroid" and individual pachychoroid spectrum entities in combination with terms related to imaging, pathophysiology, diagnosis, and treatment. Relevant Medical Subject Headings (MeSH) terms were also used where applicable. Particular emphasis was placed on recently published studies reflecting current concepts and advances in pachychoroid disease. Earlier landmark studies with substantial scientific and historical relevance were additionally included to provide the original descriptions and foundational evidence underlying the pachychoroid spectrum. Results: The pachychoroid spectrum is increasingly recognized as a continuum of disorders associated with choroidal vascular remodeling, venous congestion, and choriocapillaris attenuation. Characteristic multimodal imaging findings include pachyvessels, choroidal hyperpermeability, intervortex venous anastomoses, and variable inner choroidal thinning, while increased choroidal thickness alone may not reliably define the pachychoroid phenotype. Pachychoroid pigment epitheliopathy may occur as an isolated manifestation or coexist with other pachychoroid entities. Central serous chorioretinopathy represents a pivotal entity within the pachychoroid spectrum and may progress toward neovascular complications, including pachychoroid neovasculopathy and polypoidal choroidal vasculopathy. Advances in multimodal imaging, particularly optical coherence tomography angiography and ultra-widefield indocyanine green angiography, have substantially improved the detection of subclinical vascular alterations and disease progression. Although anti-vascular endothelial growth factor therapy and photodynamic therapy remain central treatment approaches, emerging modalities, including no-dose photodynamic therapy and photobiomodulation, are being investigated as potential therapeutic options, but current evidence does not support their routine clinical use. Conclusions: Pachychoroid spectrum disorders share common choroidal vascular and hemodynamic abnormalities despite considerable phenotypic variability. Ongoing advances in multimodal imaging and evolving therapeutic strategies continue to refine the understanding and management of these disorders.