Abstract：
:Autosomal dominant vitreoretinochoroidopathy (ADVIRC), a recently described disease, is clinically characterized by a slowly progressive or stationary circumferential peripheral pigmentary retinopathy with fibrillar condensation of the vitreous. Histopathologic study of an 88-year-old patient with this disease showed disorganization of the peripheral retina with focally atrophic retinal pigment epithelium (RPE). Altered pigment epithelial cells surrounded retinal blood vessels and lined the internal limiting membrane. At the equator, a remarkable and possibly unique multifocal loss of photoreceptor cells was seen. An extensive preretinal membrane, consisting of condensed vitreous with cellular debris and layers of Müller cells, was demonstrated by electron microscopic examination and immunohistochemistry. Histologically, this entity has some similarities to and some differences from retinitis pigmentosa. The clinical features are distinctive.
journal_name
Ophthalmologyjournal_title
Ophthalmologyauthors
Goldberg MF,Lee FL,Tso MO,Fishman GAdoi
10.1016/s0161-6420(89)32663-7subject
Has Abstractpub_date
1989-12-01 00:00:00pages
1736-46issue
12eissn
0161-6420issn
1549-4713pii
S0161-6420(89)32663-7journal_volume
96pub_type
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