Retinal Pigment Epitheliopathy

  • Destruction of retinal pigment epithelium, migration of black pigment, extreme narrowing of retinal vessels, optic disc pallor
  • Usually caused by hereditary disorders ("retinitis pigmentosa"), rarely by paraneoplastic or other autoimmune disorders, intra-uterine inflammatory, and acquired toxic-metabolic-neurodegenerative disorders
  • Two retinal areas are most vulnerable: mid-peripheral (equatorial) and perifoveal regions
  • Patient usually has constricted visual field and poor night vision ("nyctalopia")
  • Eventually foveal region itself becomes damaged and visual acuity fails
  • Often with family history of retinal degeneration, night vision difficulty, progressive neurologic illness, unusual medication use
  • Look for narrowed retinal vessels and black pigment
  • Black pigment that may be clumpy ("bone spicular"), as in hereditary degenerations, or very fine, as in intra-uterine inflammations like rubella
  • Old retinal artery occlusion, BUT...usually in one eye only
  • Ocular trauma, BUT...usually in one eye only
  • Old retinal detachment, BUT...history of repair
  • Posterior uveitis, BUT...retinal vessels not so narrowed and less pigment migration
  • Refer to ophthalmologist non-urgently unless vision loss is rapidly progressing
  • Genetically-determined retinopathies typically progress slowly
  • There is no treatment, but genetic counseling is important
  • Paraneoplastic retinopathy may progress faster, and there is controversy as to whether discovering underlying tumor or instituting immune-modulating treatment helps slow down visual loss, BUT earlier detection of tumor may improve survival
  • Autoimmune retinopathy may exist in isolation, but there is controversy about whether it is true entity and whether any form of treatment helps