PMC8595029_01
(a) and (b) Multicolour images (a) showing retinal pigment epithelial (RPE) alterations with vitelliform deposits in both macula simulating pattern dystrophy. Spectral-domain optical coherence tomography (SD-OCT) showing hyperreflective lesions in subretinal space ((c), (d):arrows) with a shallow PED in the subfoveal region of the right eye ((c):arrowhead) and similar deposition in the left eye (e) and focal extrafoveal DLS ((e) red arrowhead). Note the pachychoroid features in both eyes.
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fig1
Fundus examination showed patterned retinal pigment epithelial (RPE) alterations in OD and nonspecific RPE changes in OS, seen better on multicolor imaging (Figures 1(a) and 1(b)). Vitelliform-like deposits were also seen in OU (Figures 1(a) and 1(b)). Spectral-domain optical coherence tomography (SD-OCT) revealed pachychoroid features with hyperreflective lesions in subretinal space and a shallow pigment epithelial detachment (PED) in OD (Figures 1(c) and 1(d)) and similar hyperreflective material extending into outer retinal layers and a focal extra-foveal double-layer sign (DLS; Figure 1(e)) in OS.
Blue peak autofluorescence imaging revealed increased autofluorescence from vitelliform deposits in both eyes ((a) and (b)) while infrared images showed corresponding increased reflectance ((c) and (d)).
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fig2
Blue peak autofluorescence (BAF) imaging revealed increased autofluorescence from vitelliform deposits in both eyes (Figures 2(a) and 2(b)), while infrared images showed corresponding increased reflectance (Figures 2(c) and 2(d)).
(a)-(h) Early and late phase combined fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) showing window defects and blocked fluorescence corresponding to vitelliform deposits with no evidence of neovascularization network. Note the abnormal network evident on OCTA (i) in the left eye suggestive of nonexudative choroidal neovascular membrane (CNVM).
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fig3
Combined fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) showed window defects with foci of blocked fluorescence corresponding to the vitelliform lesions without any evidence of neovascularization (Figures 3(a)-3(h)). OCT angiography (OCTA) however revealed a nonexudative CNVM in OS corresponding to the focal DLS (Figure 3(i)).
Serial infrared reflectance (IR) images demonstrating in the right eye (first row) central migration and gradual disappearance of vitelliform lesions and the left eye (bottom row) even though lesions disappeared initially, it reappeared at different locations in the macula.
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fig4
Serial infrared reflectance (IR; Figure 4) and SD-OCT (Figure 5) imaging over the next two years showed a gradual flattening of the PED with central migration of the vitelliform deposits and absorption in OD. OS, the vitelliform deposits initially decreased only to reappear at different locations (Figure 4) with the onset of subretinal fluid (SRF; Figure 5). On colocalizing the vitelliform deposits with OCTA, we noted an abnormal network in the avascular slabs with dilated vessel complex in choroidal slabs suggestive of pachychoroid neovasculopathy (PNV; Figure 6).
Serial spectral-domain optical coherence tomography (SD-OCT) images of right eye revealing flattening of pigment epithelial detachment (PED) (a) and central clumping of vitelliform deposits (b) and later resorption ((c) and (d)) while the left eye revealed focal double-layer sign (DLS; (e)) and development of extrafoveal fluid (f) managed conservatively and later new vitelliform deposition (g) followed by submacular fluid (h) necessitating treatment. Note that these new hyperreflective lesions appear before the onset of exudation (g).
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fig5
Serial infrared reflectance (IR; Figure 4) and SD-OCT (Figure 5) imaging over the next two years showed a gradual flattening of the PED with central migration of the vitelliform deposits and absorption in OD. OS, the vitelliform deposits initially decreased only to reappear at different locations (Figure 4) with the onset of subretinal fluid (SRF; Figure 5). On colocalizing the vitelliform deposits with OCTA, we noted an abnormal network in the avascular slabs with dilated vessel complex in choroidal slabs suggestive of pachychoroid neovasculopathy (PNV; Figure 6).
Optical coherence tomography angiography (OCTA) images of left eye corresponding to hyperreflective spot on infrared reflectance (IR) image showing an abnormal network in avascular layer and presence of prominent underlying and adjoining large dilated choroidal vascular complex.
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fig6
OS, the vitelliform deposits initially decreased only to reappear at different locations (Figure 4) with the onset of subretinal fluid (SRF; Figure 5). On colocalizing the vitelliform deposits with OCTA, we noted an abnormal network in the avascular slabs with dilated vessel complex in choroidal slabs suggestive of pachychoroid neovasculopathy (PNV; Figure 6).
Comparison of baseline optical coherence tomography angiography (OCTA; nonexudative state) and OCTA at the time of submacular fluid (exudative state). Note the slight increase in the size of the vascular network in avascular slabs and the appearance of new dilated vessels in choroidal slabs.
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fig7
The size of the network had increased marginally as compared to baseline (Figure 7).
Pre- and posttreatment spectral-domain optical coherence tomography (SD-OCT) images ((a) and (b)) showing resolution of subretinal fluid and persistent reflective deposits on infrared reflectance (IR) imaging.
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fig8
Since the patient was symptomatic in OS with a decrease in BCVA to 20/40, she received two monthly doses of intravitreal ranibizumab injection and subsequently had complete resolution of SRF with BCVA improving to 20/20 (Figure 8).