To report 3 cases of pachychoroid neovasculopathy, a form of Type 1 (sub-retinal pigment epithelium) neovascularization, occurring over areas of increased choroidal thickness and dilated choroidal vessels.
A retrospective observational case series of three patients who underwent comprehensive ophthalmic examination and multimodal imaging with fundus photography, fundus autofluorescence, spectral domain optical coherence tomography, enhanced depth imaging optical coherence tomography, fluorescein angiography, and indocyanine green angiography.
In all 3 eyes of 3 patients, aged 55 years to 63 years, there was Type 1 neovascularization overlying a localized area of choroidal thickening and dilated choroidal vessels seen with enhanced depth imaging optical coherence tomography. With indocyanine green angiography, there were large choroidal veins and choroidal hyperpermeability seen beneath the area of the neovascular tissue in all three eyes. No eyes had evidence of submacular exudative detachment or autofluorescence changes to suggest antecedent acute or chronic central serous chorioretinopathy. No eyes had drusen or degenerative changes to suggest age-related macular degeneration or other degenerative diseases. In one patient, the fellow unaffected eye demonstrated retinal pigment epithelium abnormalities, best seen with fundus autofluorescence, overlying focal dilated choroidal vessels seen with enhanced depth imaging optical coherence tomography and associated choroidal hyperpermeability seen with indocyanine green angiography, consistent with the diagnosis of pachychoroid pigment epitheliopathy. All three eyes showed the appearance of polypoidal structures within the neovascular tissue.
Pachychoroid neovasculopathy falls within a spectrum of diseases associated with choroidal thickening that includes pachychoroid pigment epitheliopathy, central serous chorioretinopathy, and polypoidal choroidal vasculopathy and should be considered as a possible diagnosis in eyes with features of Type 1 neovascularization and choroidal thickening in the absence of characteristic age-related macular degeneration or degenerative changes. Pachychoroid neovasculopathy may occur as a focal abnormality within the macula, even in myopic eyes with normal subfoveal choroidal thickness. Pachychoroid neovasculopathy can ultimately progress to the development of polypoidal choroidal vasculopathy.
Pachychoroid neovasculopathy is Type 1 (subretinal pigment epithelium) choroidal neovascularization associated with choroidal thickening and dilated choroidal vessels, which occurs in the absence of overt central serous chorioretinopathy, age-related macular degeneration, or other degenerative diseases and may ultimately lead to polypoidal choroidal vasculopathy. Pachychoroid neovasculopathy lies within a spectrum of pachychoroid-driven diseases including pachychoroid pigment epitheliopathy, central serous chorioretinopathy, and polypoidal choroidal vasculopathy.
*Vitreous, Retina, Macula Consultants of New York, New York, New York;
†LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear and Throat Hospital, New York, New York; and
‡Department of Ophthalmology, New York University School of Medicine, New York, New York.
Reprint requests: K. Bailey Freund, MD, Vitreous, Retina, Macula Consultants of New York, 460 Park Avenue, Fifth Floor, New York, NY 10022; e-mail: firstname.lastname@example.org
Supported by The Macula Foundation, Inc, New York, NY.
K. B. Freund is a consultant for Genentech, Regeneron, Optos, Bayer HealthCare, and Heidelberg Engineering. The other author has no conflicting interests to disclose.