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Case Reports
. 2015 Jan;63(1):72-4.
doi: 10.4103/0301-4738.151481.

Bilateral optic neuropathy and intraretinal deposits after pars plana vitrectomy in amyloidosis

Alberto Rossetti, Luigi Spedicato, Ambrogio Fassina, Daniele Doro
Case Reports

Bilateral optic neuropathy and intraretinal deposits after pars plana vitrectomy in amyloidosis

Alberto Rossetti et al. Indian J Ophthalmol. 2015 Jan.

Erratum in

Abstract

Pathological examination of material from a nonextensive pars plana vitrectomy (PPV) in the right eye provided a diagnosis of nonfamilial amyloidosis in a 68-year-old woman, who presented with bilateral glass wool-like vitreous opacities. Genetic testing revealed a Tyr114Cys mutation in the transthyretin gene. Six months after PPV, perimetry showed intense constriction with a temporal island and central scotoma in the right eye. An extensive PPV was performed in the left eye. Spectral domain optical coherence tomography evidenced bilateral epimacular amyloid deposits and unreported reflective spots within the inner retina. One year later, visual acuity had decreased to 20/400 in the left eye, with mild vitreous opacity, pale cupped optic disc and inferior altitudinal field defect. Bilateral diurnal intraocular pressure, transiently increased after PPV, never exceeded 16 mmHg with medication. Our patient presented optic nerve blood supply impairment, due to amyloidosis, which caused optic atrophy. Epiretinal and intraretinal deposit detection could aid in diagnosing patients with suspected amyloidosis.

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Conflict of interest statement

Conflict of Interest: None declared.

Figures

Figure 1
Figure 1
(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
Figure 2
Figure 2
Vitrectomy specimen of the right eye showing Congo red stained amyloid fibrils
Figure 3
Figure 3
(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
See this image and copyright information in PMC

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