PMC4363966_01
(a) Diffuse intraretinal hyperfluorescent spots overlying retinal vessels (arrows) on indocyanine green angiography; (b) whitish amyloid deposit being removed during pars plana vitrectomy (PPV); (c) amyloid material in the anterior chamber of the right eye; (d) glass wool-like vitreous opacities prior to complete left PPV
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F1
Indocyanine green angiography (ICGA) showed late diffuse hyperfluorescent spots overlying retinal vessels [Fig. 1a]. Pathologic examination of amorphous material from a gastric mucosa biopsy and from a 23-gauge nonextensive pars plana vitrectomy (PPV) in the right eye [Fig. 1b] showed positive Congo red staining [Fig. 2] and confirmed the diagnosis of nonfamilial amyloidosis. PPV, thick grayish material consistent with amyloid deposit was found in the right anterior chamber [Fig. 1c] and her right visual acuity had deteriorated to counting fingers within 8 weeks, along with moderate vitreous haze. Predominantly glass wool-like vitreous opacities [Fig. 1d] required a 23-gauge extensive PPV with IOL implantation in the left eye.
(a and b) Spectral domain optical coherence tomography shows bilateral epimacular layer of amyloid material and bilateral reflective spots (arrows), mostly located in the inner plexiform layer. (c) Note markedly reduced rim area and markedly increased C/D ratio in the right eye
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F3
Spectral domain optical coherence tomography (SD-OCT) evidenced a bilateral epimacular layer of amyloid deposits and numerous reflective spots within the inner retina [Fig. 3a and b]; average cup/disk (C/D) ratio was 0.89 and 0.44 in the right and left eye, respectively. Rim area was markedly reduced in the right eye and normal in the left; whereas only the retinal nerve fiber layer (RNFL) thickness in the left eye appeared reduced [Fig. 3c].