PMC5015622_01
Fundus photograph at presentation showing sub macular yellowish lesion, one-third disc diameter in size, not associated with any vascular changes, pigmentation or CNV in the RE (a), RPE hypo pigmentation over fovea with minimal graying and telangiectasia in the LE (b). Fluorescein angiography showing ill defined hyper fluorescence sub foveally in the RE (c) and temporal parafoveal leakage in the LE (d). Spectral domain Optical Coherence Tomography (OCT) of the RE showing hyper reflective clump of echoes sub foveally, normal morphology of all the retinal layers around the lesion and no features of CNV (e). In the LE there is foveal thinning, small sub foveal lamellar hole and internal limiting membrane (ILM) drape with disruption of outer retinal layers temporally (f). Disruption of the ellipsoid line and intact RPE are noted in BE.
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F1
LE had retinal pigment epithelial (RPE) depigmentation and minimal telangiectasia parafoveally. (Figure 1 a, b (Fig. 1)). FFA showed ill defined hyperflourescence subfoveally in the RE and temporal parafoveal leakage in the LE (Figure 1 c, d (Fig. 1)). OCT of LE showed foveal thinning with CFT of 180 microns, multiple lamellar holes, break in the IS/OS layer and intact RPE (Figure 1 e, f (Fig. 1)).
At 15 months follow up the sub retinal lesion in the RE remaining unchanged on fundus (a) and OCT (c) whereas LE fundus showing increased parafoveal RPE depigmentation and a small pigmented plaque along the right angled venule temporally (b). OCT of the LE showing multiple lamellar holes sub foveally increased disruption of the outer retinal layers with hyper reflective echoes temporally suggestive of progression (d). mfERG recording (e) showing normal response in the RE and diminished amplitudes in the inner rings in the LE. At her final visit at 3 years, no significant changes are seen ophthalmoscopically or on OCT in the RE (f, h), but LE is showing further progression with increased RPE atrophy and pigmented RPE plaques (g). OCT showing enlarged lamellar hole and RPE hyperplasia causing shadowing temporal to fovea (i).
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F2
Fundus and OCT remained unchanged in the RE, but LE showed increased RPE depigmentation and pigmented plaque along the dilated venule (Figure 2 a-d (Fig. 2)). Arden's ratio was 2.7 in the RE and 4.3 in the LE. mfERG amplitudes were normal in the RE and diminished in the inner rings in the LE (Figure 2 e (Fig. 2)). At her next visit in July 2013 BCVA had deteriorated to 6/36 in the RE and 6/18 in the LE with worsening of cataract in BE. Fundus and OCT evaluation showed RE to be status quo whereas there was progressive RPE atrophy, pigmented plaques and cavitation in the LE (Figure 2 f-i (Fig. 2)).