PMC5244733_01
The evolution of clinical findings in the right fundus. a The right fundus showed mild temporal pallor of the optic disc. b The right eye, at six weeks after initial presentation, showed thinning of the retina at the posterior pole, with inferior intraretinal (arrow) and subretinal (star) hemorrhage. c The ultrawide field fluorescein angiogram (UWFA) of the right eye revealed hyperfluorescence along the inferior arcade and blocked fluorescence due to the hemorrhage. Peripherally there were dropouts of vessels (arrow) and arteriovenous loops (star). d The hemorrhage resolved leaving behind a moth-eaten retina with conspicuous retinal vessels and inferior peripheral subretinal deposits
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Fig1
Only mild pallor of the right optic disc was discernible (Fig. 1a). Ultrawide field imaging (UWFI) of the RE showed disc pallor, thinning at the macula, sclerosed vessels at the macula and inferotemporal retina along with a hemorrhage in the inferior half of the fundus (Fig. 1b). The ultrawide fluorescein angiogram (UWFA) of the RE revealed leak from vessels at the posterior pole and paravascular staining, capillary non-perfusion, drop out of retinal vessels (arrow) and arteriovenous loop (star) formation in the temporal periphery (Fig. 1c). The OCT of the RE showed a continuous thin strip of the inner retina with underlying fluid and absence of outer retinal layers along with macular atrophy (Fig. 1d).
Follow-up of the changes in the left eye and the electroencephalogram. a The left eye at the first ophthalmic evaluation showed macular scar with pigment splinters (arrow). Small irregular whitish lesions with some pigmentation around the macular scar suggest distinct areas of chorioretinal atrophy. b The ultrawide field fundus photograph of the left eye 6 weeks after the initial presentation to the Ophthalmology department showed similar findings as in the presentation. c The ultrawide field fluorescein angiogram done at that time showed window defect at the macular, nasal capillary non-perfusion, and paravascular staining. d The electroencephalogram showed generalized pseudo-periodic epileptiform discharges at variable intervals of 6-30 s (compressed image at paper speed of 10 mm/s)
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Fig2
The left optic disc was pale and the macula showed a subretinal scar with radial pigment splinters (Fig. 2a, arrow). Electroencephalogram (EEG) showed generalized pseudo-periodic complexes (Fig. 2d). The LE maintained status quo (Fig. 2b). The LE showed a window defect at the macula and capillary non-perfusion and staining of few nasal peripheral veins (Fig. 2c).
Optical coherence tomography features of the eyes. a The optical coherence tomography (OCT) of the right eye at six weeks after initial presentation showed thinning of the retina with a detached internal limiting membrane. b The left eye revealed severe retinal thinning at the posterior pole. c The OCT of the right eye one week after the appearance of hemorrhage showed necrosis of the outer retina with resulting cavitation. d The OCT of the inferior retina of the right eye after the intraretinal and subretinal hemorrhage resolved (3 months) showed a persistence of a continuous thin inner retinal layer with subretinal fluid
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Fig3
An optical coherence tomography (OCT) inferior to the fovea of the RE showed retinal atrophy with an overlying detached internal limiting membrane (Fig. 3a). The LE revealed retinal thinning of the macula (Fig. 3b). Within one week, the inferotemporal part of the right macula showed cavitary changes in the outer retina suggesting a necrotizing outer retinitis (Fig. 3c). The subretinal hemorrhage completely resolved in the RE by 3 months leaving a moth-eaten thinned out retina, conspicuous retinal vessels, and peripheral subretinal deposits (Fig. 3d).