Polypoidal Choroidal Vasculopathy and Pachychoroid Neovasculopathy Represent Different Manifestations of the Same Disease

The pachychoroid spectrum has various clinical manifestations. There are three major characteristics, all of which have an unknown etiology and controversial pathogenesis: pachychoroid, presence of pachyvessels at the external choroid, and inner choroidal attenuation. This study describes a patient with clinical and multimodal manifestations in the pachychoroid spectrum, in which different clinical variants are presented in both eyes simultaneously. Specifically, the patient had an acute visual loss and massive hemorrhagic maculopathy in the right eye, and a chronic decrease in visual acuity and metamorphopsia in the left eye accompanied by pigmentary changes and subretinal fluid at the geometric center of the fovea. The patient underwent a complete ophthalmological examination and multimodal imaging and was diagnosed with polypoidal choroidal vasculopathy (PCV) and pachychoroid neovasculopathy (PNV); two different manifestations of the same disease spectrum occur simultaneously.

Owing to the active nature of the disease, the patient received three doses of intravitreal antiangiogenic agents in each eye. Many different degenerative etiologies have been considered, such as pathological choroidal neovascularization due to age-related macular degeneration (AMD) and pachychoroid spectrum. Evaluation of the choroid vasculature using swept-source optical coherent tomography (SS-OCT) and OCT angiography (OCT-A) revealed the origin of the disease to be idiopathic. PCV and PNV are considered to represent a single end-stage of the pachychoroid spectrum with different manifestations; the former presents with aneurysmatic characteristics, whereas the latter lacks this anomaly. 

Keywords: Central serous chorioretinopathy, Choroidal neovascularization, Hemorrhagic maculopathy, Pachychoroid neovasculopathy, Pachychoroid spectrum, Peripapillary pachychoroid syndrome, Pachychoroid neovasculopathy polypoidal choroidal vasculopathy, Polypoidal choroidal neovascularization, Retinal angiomatous proliferation.