PMC9587723_01
Multimodal imaging of Best vitelliform macular dystrophy (BVMD). (A) Multicolor image of the right eye shows an elevated macula with yellow subfoveal deposits, giving off the characteristic "egg yolk" appearance of the vitelliform stage of BVMD. (B) Fundus autofluorescence shows a circular ring of hyperautofluorescence surrounding the fovea. (C) Infrared en face and optical coherence tomography (OCT) B scan shows a subfoveal optically hyporeflective space and thickening of the cone outer segments. (D) OCT angiography (OCTA) confirms the absence of complicating neovascular fronds. (E) Multicolor image of the left eye shows subfoveal vitelliform deposits. (F) Fundus autofluorescence shows a circular ring of hyperautofluorescence surrounding the fovea with a tiny inferior juxtafoveal hypoautofluorescent spot, suggestive of pigmentary disruption. (G) Infrared en face and OCT B scan shows a subfoveal optically hyporeflective space and thickening of the cone outer segments with an inferior juxtafoveal localized area of pigmentary elevation suggestive of macular neovascularization (MNV). (H) OCTA confirms an inferior juxtafoveal neovascular frond in the outer retinal and choriocapillaris slabs.
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Electrooculography demonstrated an absent light rise with a significantly low Arden ratio of 1.1, confirming the diagnosis of BVMD (Figure 1). 
One month follow-up after two intravitreal ranibizumab injections. (A) Comparative OCT B scans before (top) and after (bottom) injection reveal a surprising disappearance of the subfoveal optically hyporeflective space. (B) Comparative OCT B scans reveal regression of the MNV after the injections. (C) Comparative OCTA choriocapillaris en face and B scans before (left) and after (right) injection reveal marked shrinkage of the neovascular frond. (D and E) Comparative FAF images before (D) and after (E) injection show disappearance of the inferior juxtafoveal hypoautofluorescent spot, consistent with regression of the MNV.
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MNV demonstrated subjectively clear shrinkage in size and arborization. Moreover, the inferior juxtafoveal hypoautofluorescent corresponding to the MNV appeared smaller (Figure 2). 
Two-year follow-up after two intravitreal ranibizumab injections. (A) Serial comparative 3x3 OCTA choriocapillaris slabs showing baseline (left), 6 months (middle) and 2 years (right) after injection reveal consistent shrinkage of the neovascular frond. (B) Serial comparative 6x6 OCTA choriocapillaris slabs taken from 8 follow-up visits over a 2-year follow-up period confirm the regression of the MNV, and the surprising gradual reaccumulation of the subfoveal deposits which began 2 months after the injections and persisted throughout the follow-up. (C) Comparative Amsler grids at baseline (left) and after 2 years (right) demonstrates the profound and long-lasting improvement of the patient's metamorphopsia.
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No further signs of activity were detected, and the right eye was not complicated by MNVs (Figure 3).