Polypoidal choroidal vasculopathy (PCV) is characterized by multiple serosanguineous retinal pigment epithelium (RPE) detachments with abnormal choroidal vasculature as seen on indocyanine green (ICG) angiography.[1,2]
We describe a case of a 52-year-old female with dimness of vision for 7 months in the left eye (best-corrected visual acuity 20/200). Fundus examination showed serosanguineous RPE detachment with orange-red polypoidal lesions [Fig. 1 red arrowheads]. ICG angiography revealed hot spots suggestive of polyps [Fig. 2 red arrowheads] and a large feeder vessel with a branching vascular network (BVN) [Fig. 2 white arrowheads]. Enhanced depth imaging optical coherence tomography (OCT) scan through the BVN showed areas of hyperreflective RPE undulations [Fig. 3 asterisks] representing the vascular network and the presence of moderately hyperreflective polypoidal lesions adherent to its undersurface [Fig. 4 red arrowheads]. The characteristic “double layer sign” was noted between two highly reflective membranes [Fig. 4].[3]
Figure 1.
Color fundus image showing an orange-red polyp (red arrowheads)
Figure 2.
Indocyanine green angiography photo showing feeder vessels with branching vascular network (white arrowheads) with hyperfluorescent polyps (red arrowheads)
Figure 3.
Enhanced depth imaging spectral-domain optical coherence tomography image showing areas of retinal pigment epithelium undulations representing branching vascular network (asterisks)
Figure 4.
Enhanced depth imaging spectral-domain optical coherence tomography image showing polyps adherent to the undersurface of hyperreflective retinal pigment epithelium (red arrowheads) “pearls on a string” appearance with characteristic “double layer sign” noted between two highly reflective layers consisting of the hyperreflective detached retinal pigment epithelium and Bruch membrane
Discussion
PCV is diagnosed by the presence of subretinal focal ICG hyperfluorescence, plus at least one of the angiographic or clinical criteria, i.e., a nodular appearance when viewed stereoscopically, BVN, pulsatile polyp, presence of hypofluorescent halo, orange subretinal nodule, and massive submacular hemorrhage.[4]
BVN is seen as a highly reflective undulation of the RPE on OCT scan with the presence of PCV structures adherent to the undersurface of this elevated pigment epithelial detachment resembling the appearance of “pearls on a string.”[3,4]
PCV lesions and the associated Type 1 neovascular lesions are located within or above Bruch membrane, usually in association with an RPE detachment. The presence of this neovascular tissue between the hyperreflective RPE band and the hyperreflective remainder of Bruch membrane and inner choroid creates the characteristic OCT “double layer sign” as described by Sato et al.[3]
This Photo Essay highlights the salient diagnostic ICG angiography and OCT features in PCV.
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Conflicts of interest
There are no conflicts of interest.
References
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