Perifoveal exudative anomalous complex (PEVAC) is clinically defined by the presence of a unilateral, isolated, perifoveal aneurysm with surrounding exudation in an otherwise healthy patient.1 Multimodal imaging has described these lesions in detail.2 Structural optical coherence tomography (OCT) demonstrates PEVAC as round, hyperreflective lesions with a hyporeflective lumen. OCT angiography (OCTA) shows a defined aneurysmal lesion with detectable flow in the superficial vascular plexus (SVP).3
Suspended scattered particles in motion (SSPiM) is a newly described OCTA signal corresponding to hyperreflective intraretinal fluid (IRF) in exudative retinopathies, including diabetic retinopathy (DR), retinal vein occlusion (RVO), and neovascular age-related macular degeneration (AMD).4 Areas of SSPiM have increased OCTA signal and appear as oval or circular regions of flow on cross-sectional and en face images.5 The “suspended particles” seen on OCTA are suspected to be particulate in IRF, which eventually form hard retinal exudates.6,7 We present, to our knowledge, the first case of PEVAC exhibiting the OCTA finding of SSPiM.
A 47-year-old male presented with a 2-day history of a painless scotoma in the left eye. He had no known medical problems, no history of chronic medication use, and no history ocular procedures or treatments. Best-corrected visual acuity was 20/20 in both eyes. Anterior segment exam was unremarkable in both eyes.
Fundus examination of the left eye showed a perifoveal aneurysm with a surrounding ring of exudation and IRF (figure 1). IVFA demonstrated an early hyperfluorescence corresponding with IRF (Figure 2A) and a late, discrete punctate area of leakage defining the aneurysmal lesion (Figure 2B). OCT showed a hyperreflective perifoveal aneurysmal lesion with a hyporeflective lumen (Figure 2A) and associated cystic IRF (Figure 2B). Discreet hyperreflective opacities representing exudates can also be seen on OCT.
Color fundus photo of the left eye. Note presence of intraretinal edema and perifoveal hard exudates.
(A, B) Fluorescein angiography (FA) images (left) with corresponding optical coherence tomography (OCT) images (right). (A) Note early hyperfluorescence on FA corresponding with cystic intraretinal fluid on OCT (yellow arrows). (B) Punctate area of late leakage defines perifoveal exudative vascular anomalous complex lesion on FA. On OCT, it is seen as an ovoid hyperreflective perifoveal aneurysm with a hyporeflective lumen (red arrow).
OCTA B-scans with flow revealed an isolated dilatation matching the PEVAC lesion with detectable flow in the SVP (Figure 3). En face imaging of the SVP demonstrates a poorly defined flow signal that represents SSPiM associated with cystic intraretinal cavities (Figure 4). The B-scan image of the cystic cavity demonstrates hyperreflective “dots” in the inner border of the cystoid space (Figure 5). As the cystic fluid resolves, there is an increase in intraretinal hyperreflective material. The patient was observed during the course of 8 months with spontaneous resolution of IRF and hyperreflective material and improved symptoms.
Optical coherence tomography (OCT) angiography B-scans with flow reveal an isolated dilation matching the perifoveal exudative vascular anomalous complex lesion (red arrow; OCT in lower left corner for comparison) with detectable flow in the superficial vascular plexus. Associated exudation (yellow arrow) can also be seen in this image with evidence of suspended scattering particles in motion.
The B-scan image (above) of the cystic cavity demonstrates punctate flow signal in the inner border of the cystoid space. En face imaging of the superficial vascular plexus (below) demonstrate a poorly defined flow signal that represent suspended scattering particles in motion associated with cystic intraretinal cavities. Segmentation artifact prevents visualization on perifoveal exudative vascular anomalous complex lesion.
Color fundus photos (left) and optical coherence tomography (OCT) (right) of left eye at presentation (A), week 7 (B), week 15 (C), and month 8 (D). Presence of intraretinal fluid decreased spontaneously over time. In panels A and B, as fluid decreased on OCT, hyperreflective material and hard exudates increased. Over time, hyperreflective material on OCT and hard exudates on examination improved.
PEVAC was recently described as an isolated aneurysmal lesion, occurring in one eye of patients without any systemic or ocular condition associated with aneurysms. Though the pathogenesis of PEVAC is not clearly understood, OCTA imaging has been useful in identifying and describing lesions in detail. In reported case series detailing OCTA findings of PEVAC,2,10 such lesions may be located in the superficial or deep vascular plexi or, more often, in between. OCTA can demonstrate the characteristic aneurysmal dilation of PEVAC, as well as the rarefaction of retinal capillaries in the perilesional area. In our case, segmentation artifact on the en face image limits the visualization of the PEVAC lesion, though it is clearly demonstrated on OCTA B-scan and OCT.
We add SSPiM, a hyperreflective OCTA finding best demonstrated on en face imaging, to the description of PEVAC. Cases of SSPiM of any cause tend to have a preference for vascular-avascular junctions,4 as seen in our case bordering the fovea avascular zone (FAZ). The pathophysiology and resolution of such hyperreflective fluid has been speculated; however, Taarnhøj et al.8 and Kashani et al.9 describe the kinetics of liporoteinaceous debris in the retina. Resorption of fluid containing hyperreflective material by the retinal pigment epithelium and intraretinal capillaries leads to precipitation of macromolecules forming exudates on clinical exam. In our case, as IRF resolved, presence of exudates on clinical exam became more apparent (Figure 4). Eventually, focal exudation spontaneously improved. We extrapolate that the discrete punctate flow signal found in the IRF as demonstrated by OCTA B-scan images correspond to suspended lipids or proteinaceous aggregates which eventually form intraretinal exudates. Though the significance of SSPiM in PEVAC is unknown, better qualification and quantification of the finding may be meaningful in determining the level of resulting exudation.
Treatment of exudation secondary to PEVAC is not clearly established. Similar to the case presented, spontaneous resolution of exudative fluid secondary PEVAC lesions has been reported.3 Thermal laser has also been reported as a viable treatment option; however, laser photocoagulation near the fovea should be considered with caution due to the risk of permanent paracentral scotoma.3,10 Literature supporting the responsiveness to anti-vascular endothelial growth factor (VEGF) agents appears to be mixed. 3,10 Additional studies are needed to further investigate PEVAC, its course and efficacy of anti-VEGF injections or other therapies.
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