Retinal Angiomatous Proliferation (RAP)

A 74-year-old woman presented with decreased vision in her left eye. Her medical history was unremarkable except for systemic hypertension. Ophthalmic history revealed that she had previously received multiple intravitreal anti-VEGF injections in the right eye for the treatment of neovascular age-related macular degeneration (nAMD). On ophthalmic examination, the BCVA) was hand motions in the right eye and 0.5 (Snellen equivalent) in the left eye.

Fundus examination of the right eye revealed a well-demarcated macular scar with intraretinal haemorrhages consistent with end-stage nAMD.

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Structural OCT of the right eye confirmed a fibrotic macular scar with severe disruption of the outer retinal layers and the retinal pigment epithelium, with intraretinal fluid and degeneration.

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In the left eye, multicolor imaging demonstrated distinct macular changes, including mild hemorrhagic alterations accompanied by surrounding drusenoid deposits and intraretinal exudation.

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Optical coherence tomography angiography (OCTA) of the left eye demonstrated a well-circumscribed, vertically oriented vascular complex originating from the deep capillary plexus and extending toward the outer retina. En face OCTA images showed clear flow signals within this lesion, while the superficial capillary plexus remained largely preserved. No definite neovascular network was identified at the level of the outer retina or choriocapillaris. The lesion exhibited distinct flow on OCTA, associated with surrounding intraretinal fluid without subretinal fluid.

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Based on the multimodal imaging findings—particularly the history of advanced neovascular disease in the fellow eye, the presence of a deep intraretinal neovascular complex with intraretinal fluid, absence of a classic subretinal neovascular membrane, and flow signals detected on deep-layer OCTA—the diagnosis of retinal angiomatous proliferation (RAP, type 3 neovascularization) was established in the left eye.

Retinal angiomatous proliferation (RAP) is a distinct subtype of neovascular age-related macular degeneration characterized by intraretinal neovascularization with progressive involvement of the subretinal space and choroidal circulation. RAP typically affects elderly patients and is often bilateral, with a high risk of fellow-eye involvement. Clinically, intraretinal hemorrhages, retinal edema, exudate, and pigment epithelial detachment may be observed, sometimes with subtle funduscopic findings in early stages. Multimodal imaging is essential for accurate diagnosis: OCT reveals intraretinal cysts and hyperreflective foci, OCTA demonstrates intraretinal vascular complexes, and fluorescein/ICG angiography helps delineate the vascular origin and staging. On OCTA, early RAP lesions typically appear as small, high-flow intraretinal vascular tufts located in the deep capillary plexus, often with a focal “hot spot” configuration. As the disease progresses, vertical flow signals connecting the deep retinal plexus to the outer retina and choriocapillaris—so-called retinal–choroidal anastomoses—may be visualized. En face OCTA often shows a compact, well-circumscribed vascular complex rather than the classic sub-RPE neovascular network seen in type 1 MNV. Projection-resolved and carefully segmented slabs are essential to avoid misinterpretation. RAP lesions are known for their aggressive course and tendency toward recurrence. Anti-VEGF therapy remains the mainstay of treatment and can achieve good anatomical and functional outcomes, particularly when initiated early. Prompt recognition and close follow-up are crucial to preserve visual function in these patients.

Credit: Kemal Tekin, M.D., from Ulucanlar Eye Training and Research Hospital

Instagram accounts: @retina.academy and @dr.kemaltekin

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