PMC5778589_01
Fundus photograph of Case 1 showing a subfoveal deposit in the right eye and foveal scar in the left eye (top). Fluorescein angiography showed a central hypofluorescence with a halo of hyperfluorescence in the right eye and a central tiny hyperfluorescence with surrounding hypofluorescence in the left eye (bottom)
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F1
Fundus examination revealed subfoveal yellowish deposit in the right eye and foveal scar in the left eye [Fig. 1]. The right eye FFA showed a central hypofluorescence with a halo of hyperfluorescence and the left eye showed a central small area of hyperfluorescence with surrounding hypofluorescence [Fig. 1].
Fundus autofluorescence and corresponding spectral domain-optical coherence tomography scans of right eye (Case 1) showing a brightly hyperautofluorescent lesion in the center, surrounded by a border of hypoautofluorescence, with spectral domain-optical coherence tomography showing a hyperreflective complex in the outer retinal layers, anterior to the retinal pigment epithelium (arrow) (a). Two years later, the lesion now appeared completely hypoautofluorescent with disappearance of the yellowish deposits, and the spectral domain-optical coherence tomography scan showed disappearance of the previously accumulated hyperreflective material, baring of Bruch's membrane, and discontinuity of photoreceptor-retinal pigment epithelium complex (arrow) (b). Three years later, a new hyperautofluorescent material (dashed arrow) appeared adjacent and temporal to previous lesion, appearing as new hyperreflective deposits beneath the photoreceptor-retinal pigment epithelium complex (dashed arrow), at the border of previously identified region of missing photoreceptor-retinal pigment epithelium complex (solid arrow) (c). The left eye too underwent similar changes and aperture of retinal pigment epithelium was noted 5 years after presentation (d)
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F2
The SD-OCT showed a hyperreflective complex in the outer retinal layers that extended into the Henle's layer with aperture of the ellipsoid zone and the outer limiting membrane [Fig. 2a]. The SD-OCT scan showed disappearance of the previously accumulated hyperreflective material, baring of the Bruch's membrane, and discontinuity of photoreceptor (PR)-RPE complex [Fig. 2b]. Three years later, the right eye fundoscopy showed the reappearance of the yellowish material (adjacent to the previous atrophic lesion), which was hyperautofluorescent and corresponded to new hyperreflective deposits beneath the ellipsoid zone, at the border of previously identified region of missing PR-RPE complex [Fig. 2c]. The left eye too underwent similar changes with the RPE aperture noted 5 years after presentation [Fig. 2d].
PMC5778589_02
Right eye fundus photograph (left) of Case 2 showing a hypopigmented, subfoveal lesion with hyperautofluorescence on fundus autofluorescence (right)
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F3
The right eye had a hypopigmented, yellowish subfoveal lesion that was hyperautofluorescent on FAF, suggestive of AFVD [Fig. 3].
Line scan (case 2) through the lesion shows hyperreflective material anterior to pigment epithelium detachment (a). Two years later, a retinal pigment epithelium aperture developed (arrow) (b). Three years later, it disappeared and became hypoautofluorescent, corresponding to retinal pigment epithelium aperture (dashed arrow) with collapse of pigment epithelium detachment and baring of Bruch's membrane (solid arrow) (c). Three years later, hyperautofluorescence (solid arrow, left panel) is seen corresponding to the newly appeared subretinal hyperreflective material (solid arrow, right panel) (d). The aperture (dashed arrows) had widened, with baring of Bruch's membrane (block arrow)
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F4
The SD-OCT line scan passing through the lesion showed a pigment epithelium detachment (PED) with hyperreflective material in the outer retinal layers (anterior to PED) that was still persisting 2 years later [Fig. 4a] and developed a small RPE aperture 6 months later [Fig. 4b]. The PED had collapsed with baring of the Bruch's membrane [Fig. 4c]. Still, 3 years later, the yellowish material, brightly hyperautofluorescent reaccumulated next to the region of RPE aperture and corresponded to the newly appeared hyperreflective material under the ellipsoid zone [Fig. 4d].
PMC5778589_03
Fundus photograph (left) and fluorescein angiography (middle) of left eye (Case 3) showing a hyperfluorescent subfoveal lesion, and retinal pigment epithelium aperture on spectral domain-optical coherence tomography (right) with hyporeflectivity anterior (dashed arrow), as well as posterior (solid arrow), to retinal pigment epithelium aperture, suggesting a completely reabsorbed vitelliform material (a). Three years later, the pigment epithelium detachment increased, with appearance of new hyperreflective material under the disrupted retinal pigment epithelium (arrow) (b). Two years later, spectral domain-optical coherence tomography showed the complete disappearance of the hyperreflective material both above and below retinal pigment epithelium, with focal choroidal excavation, due to increase in the pigment epithelium detachment (c). Two years later, the spectral domain-optical coherence tomography findings remained the same through the center of the hypoautofluorescent lesion (d)
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F5
The left eye yellowish subfoveal lesion was hyperfluorescent on FFA and corresponded to the RPE aperture on SD-OCT with hyporeflectivity anterior, as well as posterior, to RPE aperture, suggesting a completely reabsorbed vitelliform material [Fig. 5a]. Three years later, the RPE aperture and PED increased, with appearance of new hyperreflective material under the disrupted RPE [Fig. 5b]. Over the next 2 years, fundus examination and SD-OCT showed complete disappearance of the yellowish material both above and below RPE [Fig. 5c]. Two years later, whereas the SD-OCT findings remained the same through the center of the hypoautofluorescent atrophic lesion [Fig. 5d], a new hyperautofluorescent halo appeared around this lesion, with a corresponding hyperreflective material both above and below the RPE [Fig. 6].
PMC5778589_04
Fundus photograph (left) and autofluorescence (right) of left eye of Case 4 showing a subfoveal lesion with a mixed pattern of autofluorescence (a). Two years later, the lesion showed an increasing hypoautofluorescence (arrow) (b)
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F7
The FAF imaging showed a mixed pattern of the FAF [Fig. 7a]. Two years later, the lesion showed an increasing hypoautofluorescence [Fig. 7b].
The spectral domain-optical coherence tomography scan revealed three different stages of the disease at the same time point within the lesion showing varying levels of autofluorescence, i.e., vitelliform stage through the hyperautofluorescent part of the lesion (a), vitelliruptive stage through the junction of hyper- and hypoautofluorescent parts (b), and retinal pigment epithelium aperture (arrows) through the completely hypoautofluorescent part of the lesion (c)
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F8
The SD-OCT scan revealed three different stages of the disease as the lesion showed varying levels of AF, i.e., vitelliform stage through the hyperautofluorescent part of the lesion [Fig. 8a], vitelliruptive stage through the junction of hyper- and hypoautofluorescent parts [Fig. 8b], and RPE aperture through the completely hypoautofluorescent part of the lesion [Fig. 8c].