Retinitis pigmentosa

Author: Nat Pernick, M.D. (see Authors page)

Revised: 21 May 2018, last major update February 2014

Copyright: (c) 2004-2018, PathologyOutlines.com, Inc.

PubMed Search: Retinitis pigmentosa [title] review[ptyp] pathology

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Cite this page: Pernick, N. Retina: retinitis pigmentosa. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/eyeretinaretinitispigmentosa.html. Accessed December 18th, 2018.
Definition / general
  • Various inherited retinal disorders that are not actually inflammatory
  • Common (incidence of 1/3600) with variable inheritance or as part of Refsum disease
  • Usually causes loss of rods and cones to apoptosis and focal proliferation of retinal pigment epithelium
  • Loss of rods causes early night blindness and restricted visual fields (bilateral loss of peripheral vision)
  • Loss of cones affects central visual acuity
  • Retinal atrophy causes constriction of retinal vessels, optic nerve head atrophy and accumulation of retinal pigment around blood vessels
Microscopic (histologic) description
  • Migration of macrophages and retinal pigment epithelial cells with melanin into retina, particularly around vessels
  • Also atrophy of photoreceptors in retina and choriocapillaris