Polypoidal Choroidal Vasculopathy

SALIENT FEATURES

Polypoidal choroidal vasculopathy (PCV), described by Yannuzzi in 1982, manifests clinically as dilated and branching inner choroidal vessels terminating in reddish-orange aneurysm-like lesions called “polyps” of varying size.¹
The disease is more common in pigmented races such as Asians and commonly affects people in the sixth to seventh decade of life. Hypertension is a known systemic association.¹
Clinically, it presents as serous and serosanguinous detachments of the neurosensory retina and retinal pigment epithelium (RPE) in the macular and peripapillary area. The disease is broadly classified clinically into hemorrhagic and exudative variants based on the predominant clinical presentation. Spontaneous resolution of acute exudation results in subretinal fibrosis and atrophic degeneration.¹
PCV, owing to structural features identified by multimodal imaging, has been recently described as an aneurysmal type 1 neovascularization.²

Indocyanine green angiography (ICG) is the gold standard for disease detection and characterization.

Early-phase ICG depicts branching vascular network (BVN) as a well-defined distinct neovascular network in the choroid (Figure 24.1A, white circle and Figure 24.2A, white circle).

Polyps appear within the first 6 minutes as hyperfluorescent lesions with a hypofluorescent halo (Figures 24.1B, 24.2, 24.3B, white boxes).

FIGURE 24.1 Multimodal imaging of a patient with polypoidal choroidal vasculopathy (PCV). Indocyanine green angiography shows a well-defined branching vascular network (white circle) in the early phase (A), while the later phase (B) shows a well-defined group of hyperfluorescent polyps (white box) with a subtle hypofluorescent halo. Note the diffuse hyperfluorescence temporally (red arrow) which denotes pachyvessels. Swept-source optical coherence tomography (C and D) depicts signs of active polypoidal choroidal vasculopathy. Horizontal scan passing above the fovea and through the polyps (C) shows a fibrovascular pigment epithelial detachment (PED—white arrow) with hyporeflective polyps under the retinal pigment epithelium (black arrow). The vertical scan (D) shows a QRS complex-shaped PED (white arrow) with fibrovascular and hemorrhagic content (red asterisk). Note the polyp (black arrow) under the PED with the adjoining double-layer sign (red arrow) with hyperreflective content. Both scans show subretinal fluid (white asterisk), subretinal hyperreflective material representing hemorrhage (black asterisk) and features of pachychoroid (blue asterisk). Manually segmented (E; blue lines) optical coherence tomography angiography (OCTA) slab (F) shows a well-defined branching vascular network (white circle) in the trunk pattern.

FIGURE 24.2 The fellow eye of the subject in Figure 24.1. Indocyanine green angiography demonstrates a well-defined branching vascular network (white circle) in the early phase (A) and well-defined hyperfluorescent polyps (white boxes) with a hypofluorescent halo in late phase (B). The vascular network shows increasing hyperfluorescence in the late phase, and this is defined as late geographic hyperfluorescence (red arrow). There is no activity on swept-source optical coherence tomography (C). A double-layer sign is noted (red arrow). Optical coherence tomography angiography (OCTA) (D) delineates the branching vascular pattern as glomerulus-like structure (white circle) with a subtle ring-shaped polyp (white arrow).

In the mid-late ICG phase, choroidal hyperpermeability (Figure 24.1B, red arrow), late leakage from the polyps (Figure 24.2B, white box), and the presence of well-defined geographic hyper fluorescence (Figure 24.2B, red arrow) are noted.³

FIGURE 24.3 Multimodal imaging in polypoidal choroidal vasculopathy. Indocyanine green angiography highlights well-defined subfoveal hyperfluorescent polyps that demonstrate a progressive increase in fluorescence from early (A) to mid-phase (B, white box) and are surrounded by a distinct hypofluorescent halo. Swept-source optical coherence tomography (C and D) demonstrates characteristic fibrovascular pigment epithelial detachments (PEDs) with subretinal fluid (white asterisk). The horizontal scan (C) passing through the fovea shows an M-shaped fibrovascular PED (white arrow), while the vertical scan (D) shows a thumb-shaped PED (white arrow). Note the subtle splitting of retinal pigment epithelium from the Bruch membrane (double-layer sign) adjacent to the thumb-shaped PED denoting the branching vascular network (red arrow). Both scans detect polyps as subtle hyporeflective lesions under the PED (black arrows). Subretinal hyperreflective material is noted at the apices of the PED in both scans (black asterisk). Choroid under the PED shows thickening compared to the adjacent uninvolved area (blue asterisk). Manually segmented (E; blue lines) optical coherence tomography angiography (OCTA) slab (F) demonstrates the polyps as a cluster of increased flow-signal (white box). A peaked PED has resulted in polyps being detected in the superficial retinal slab.

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