28.1 Features
Polypoidal choroidal vasculopathy (PCV) is known to be more common in people of Asian and African ancestry. Clinically, PCV is characterized by polypoidal aneurysmal dilations of network vessels originating in the choroid and is considered by some to be one of the subtypes of neovascular age-related macular degeneration (NVAMD), although the precise pathogenesis of PCV is still controversial.
28.1.1 Common Symptoms
Metamorphopsia, blurred vision, or scotoma in the central or paracentral vision. It may be asymptomatic because the lesions of PCV are located beneath the retinal pigment epithelium (RPE) or they may be located outside the macular area.
28.1.2 Exam Findings
Orange–red nodule-like subretinal lesions can be found on fundus examination or color fundus images (▶ Fig. 28.1). Serosanguineous pigment epithelial detachment (PED), serous retinal detachment, and/or subretinal hemorrhage are found in most active cases (▶ Fig. 28.2). Massive subretinal and sub-RPE hemorrhage can also be seen when polypoidal lesions are ruptured.
Fig. 28.1 (a) Orange–red nodule-like subretinal lesions (arrow) in the color fundus image. (b) Fluorescein angiography shows occult-like hyperfluorescence. (c) Indocyanine green angiography delineates multiple nodule-like lesions (arrowhead) with branching vascular network.
Fig. 28.2 (a) Subretinal hemorrhage and hemorrhagic pigment epithelial detachment in the color fundus image. (b) Indocyanine green angiography demonstrates multiple polypoidal lesions (arrows A and B) connected with branching vascular network. Hazed polypoidal lesion in the hemorrhage (arrow C).
28.2 Key Diagnostic Tests and Findings
28.2.1 Optical Coherence Tomography
Anterior sharp protrusion of RPE with relatively moderate internal reflectivity relating to polyps (▶ Fig. 28.3, ▶ Fig. 28.4). Once polyps cause active leakage around them, large serous or hemorrhagic PED can be found. A notch sign in the RPE of a PED or moderate hyperreflective nodule-like lesions along the basal RPE or inside PED are both suggestive of the presence of polypoidal lesions. Round hyporeflective or moderate hyperreflective areas surrounded by hyperreflective rings beneath RPE suggest lumens of polyps (▶ Fig. 28.3). Branching vascular networks (BVN) can be found adjacent to the sharp or large PED as lower RPE elevation filled with moderate hyperreflectivity (▶ Fig. 28.4). These may be best seen with en face optical coherence tomography (OCT) or C-scan review. At the site of lower RPE elevation, a hyperreflective thin line between the RPE and Bruch’s membrane, named the “double-layer sign,” may also be noted. However, the double-layer sign can be seen in not only BVN but also type-1 choroidal neovascularization (CNV). Serous retinal detachment and subretinal hyperreflective material associated with subretinal hemorrhage and/or fibrin are often observed around polypoidal lesions with less intraretinal edema than with NVAMD. Enhanced depth images of spectral-domain OCT or swept-source OCT can be useful in detecting thick choroid called “pachychoroid.” It is reported that choroidal thickness in eyes with PCV was greater than that in eyes with NVAMD. Thus, pachychoroid on OCT imaging is helpful in detecting PCV.
Fig. 28.3 The associated optical coherence tomography image of ▶ Fig. 28.1 shows sharp-peaked pigment epithelial detachment with round lumen of polyps (arrows).
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