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Histopathologic Study of Autosomal Dominant Vitreoretinochoroidopathy in a 26-Year-Old Woman

Histopathologic Study of Autosomal Dominant Vitreoretinochoroidopathy in a 26-Year-Old Woman Abstract The clinicopathologic findings were obtained from enucleated eyes, obtained post mortem, of a 26-year-old woman with autosomal dominant vitreoretinochoroidopathy. Light microscopy demonstrated atrophic, disorganized peripheral retina with retinal blood vessels obscured by pigmented cells surrounding periodic acid-Schiff-positive deposits. Peripheral retinal pigment epithelial cells showed multilayering and pigmentation, with a thickened basal lamina. By electron microscopy, the peripheral retinal vessel endothelium was replaced by an arrangement of morphologically polarized pigmented cells of presumed retinal pigment epithelial origin oriented with their basal surfaces toward a fibrillar matrix occupying the vessel lumen. The similarity of the findings in this young patient to those of an aged patient described previously suggest that autosomal dominant vitreoretinochoroidopathy is an early-onset dystrophy of the peripheral retina with minimal subsequent progression, characterized by a retinal pigment epithelial response that includes marked intraretinal migration and extracellular matrix deposition. References 1. Kaufman SJ, Goldberg MF, Orth DH, Fishman GA, Tessler H, Mizuno K. Autosomal dominant vitreoretinochoroidopathy . Arch Ophthalmol . 1982;100:272-278.Crossref 2. Blair NP, Goldberg MF, Fishman GA, Salzano T. Autosomal dominant vitreoretinochoroidopathy (ADVIRC) . Br J Ophthalmol . 1984;68:2-9.Crossref 3. Han DP, Lewandowski MF. Electro-oculography in autosomal dominant vitreoretinochoroidopathy . Arch Ophthalmol . 1992;110:1563-1567.Crossref 4. Traboulsi EI, Payne JW. Autosomal dominant vitreoretinochoroidopathy: report of the third family . Arch Ophthalmol . 1993;111:194-196.Crossref 5. Goldberg MF, Lee F-L, Tso MOM, Fishman GA. Histopathologic study of autosomal dominant vitreoretinochoroidopathy: peripheral annular pigmentary dystrophy of the retina . Ophthalmology . 1989;96:1736-1746.Crossref 6. Santos-Anderson RM, Tso MOM, Fishman GA. A histopathologic study of retinitis pigmentosa . Ophthalmol Paediatr Genet . 1982;1:151-168.Crossref 7. Kolb H, Gouras P. Electron microscopic observations of human retinitis pigmentosa, dominantly inherited . Invest Ophthalmol . 1974;13:487-498. 8. Gartner S, Henkind P. Pathology of retinitis pigmentosa . Ophthalmology . 1982;89:1425-1432.Crossref 9. Wolter R. Retinitis pigmentosa: a histopathologic study with a new technique . Arch Ophthalmol . 1957;57:539-553.Crossref 10. Cogan DG. Symposium: primary chorioretinal aberrations with night blindness: pathology . Trans Am Acad Ophthalmol Otolaryngol . 1950;54:629-661. 11. Li ZY, Possin DE, Milam AH. Histopathology of bone spicule pigmentation in retinitis pigmentosa. Ophthalmology. In press. 12. Wang AZ, Ojakian GK, Nelson WJ. Steps in the morphogenesis of a polarized epithelium, I: uncoupling the roles of cell-cell and cell-substratum contact in establishing plasma membrane polarity in multicellular epithelial (MDCK) cysts . J Cell Sci . 1990;95( (pt 1) ):137-151. 13. Foos RY. Vitreoretinal juncture—simple epiretinal membranes . Albrecht von Graefes Arch Klin Exp Ophthalmol . 1974;189:231-250.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Ophthalmology American Medical Association

Histopathologic Study of Autosomal Dominant Vitreoretinochoroidopathy in a 26-Year-Old Woman

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References (14)

Publisher
American Medical Association
Copyright
Copyright © 1995 American Medical Association. All Rights Reserved.
ISSN
0003-9950
eISSN
1538-3687
DOI
10.1001/archopht.1995.01100120093017
Publisher site
See Article on Publisher Site

Abstract

Abstract The clinicopathologic findings were obtained from enucleated eyes, obtained post mortem, of a 26-year-old woman with autosomal dominant vitreoretinochoroidopathy. Light microscopy demonstrated atrophic, disorganized peripheral retina with retinal blood vessels obscured by pigmented cells surrounding periodic acid-Schiff-positive deposits. Peripheral retinal pigment epithelial cells showed multilayering and pigmentation, with a thickened basal lamina. By electron microscopy, the peripheral retinal vessel endothelium was replaced by an arrangement of morphologically polarized pigmented cells of presumed retinal pigment epithelial origin oriented with their basal surfaces toward a fibrillar matrix occupying the vessel lumen. The similarity of the findings in this young patient to those of an aged patient described previously suggest that autosomal dominant vitreoretinochoroidopathy is an early-onset dystrophy of the peripheral retina with minimal subsequent progression, characterized by a retinal pigment epithelial response that includes marked intraretinal migration and extracellular matrix deposition. References 1. Kaufman SJ, Goldberg MF, Orth DH, Fishman GA, Tessler H, Mizuno K. Autosomal dominant vitreoretinochoroidopathy . Arch Ophthalmol . 1982;100:272-278.Crossref 2. Blair NP, Goldberg MF, Fishman GA, Salzano T. Autosomal dominant vitreoretinochoroidopathy (ADVIRC) . Br J Ophthalmol . 1984;68:2-9.Crossref 3. Han DP, Lewandowski MF. Electro-oculography in autosomal dominant vitreoretinochoroidopathy . Arch Ophthalmol . 1992;110:1563-1567.Crossref 4. Traboulsi EI, Payne JW. Autosomal dominant vitreoretinochoroidopathy: report of the third family . Arch Ophthalmol . 1993;111:194-196.Crossref 5. Goldberg MF, Lee F-L, Tso MOM, Fishman GA. Histopathologic study of autosomal dominant vitreoretinochoroidopathy: peripheral annular pigmentary dystrophy of the retina . Ophthalmology . 1989;96:1736-1746.Crossref 6. Santos-Anderson RM, Tso MOM, Fishman GA. A histopathologic study of retinitis pigmentosa . Ophthalmol Paediatr Genet . 1982;1:151-168.Crossref 7. Kolb H, Gouras P. Electron microscopic observations of human retinitis pigmentosa, dominantly inherited . Invest Ophthalmol . 1974;13:487-498. 8. Gartner S, Henkind P. Pathology of retinitis pigmentosa . Ophthalmology . 1982;89:1425-1432.Crossref 9. Wolter R. Retinitis pigmentosa: a histopathologic study with a new technique . Arch Ophthalmol . 1957;57:539-553.Crossref 10. Cogan DG. Symposium: primary chorioretinal aberrations with night blindness: pathology . Trans Am Acad Ophthalmol Otolaryngol . 1950;54:629-661. 11. Li ZY, Possin DE, Milam AH. Histopathology of bone spicule pigmentation in retinitis pigmentosa. Ophthalmology. In press. 12. Wang AZ, Ojakian GK, Nelson WJ. Steps in the morphogenesis of a polarized epithelium, I: uncoupling the roles of cell-cell and cell-substratum contact in establishing plasma membrane polarity in multicellular epithelial (MDCK) cysts . J Cell Sci . 1990;95( (pt 1) ):137-151. 13. Foos RY. Vitreoretinal juncture—simple epiretinal membranes . Albrecht von Graefes Arch Klin Exp Ophthalmol . 1974;189:231-250.Crossref

Journal

Archives of Ophthalmology – American Medical Association

Published: Dec 1, 1995

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