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Noriyuki Unoki, Audina M. Berrocal, Sander R. Dubovy; Clinicopathologic Features of Epiretinal Proliferation in Familial Exudative Vitreoretinopathy. Invest. Ophthalmol. Vis. Sci. 2011;52(14):3157.
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The aim of this study is to describe the histopathologic features of a unique macular epiretinal proliferation in two patients with familial exudative vitreoretinopathy (FEVR).
The clinical and histopathologic findings of two patients (1 year-old, 7 year-old) that presented with atypical epiretinal proliferation in addition to FEVR are described. Clinical studies included fundus photography, fluorescein angiography (FA) and optic coherence tomography (OCT). Both patients underwent pars plana vitrectomy with histopathologic evaluation of the excised tissues.
The fundus findings of both patients disclose tan white tissue extending from the superior vascular arcade to the superonasal aspect of the fovea. The fovea is distorted and thickened, which is identified both in the fundus photograph and OCT. The OCT findings demonstrate a thickened epiretinal tissue causing vitreoretinal traction with a relatively broad macular insertion and underlying retinal thickening. The FA findings disclose an area of avascular retina in the mid and far periphery and no leakage at the posterior pole. The histopathologic findings of the excised tissue disclose dense collagenous tissue, which stains positively with Mallory one step trichrome, and glial tissue, which stains positively with neural specific enolase.
We describe a novel epiretinal proliferation in two childhood patients with FEVR that is composed of fibrous and glial tissue. The clinical and histopathologic findings may be useful to elucidate the mechanism of proliferative change in FEVR.
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