Last reviewed:October 2019
Last updated:November  2019

Summary

Definition

History and exam

Key diagnostic factors

  • family history of vision loss
  • history of retinitis pigmentosa
  • congenital stationary night blindness
  • past medical history of vitamin A deficiency or malignancy

Other diagnostic factors

  • decreased visual acuity in dim light
  • variable pupillary responses (depending on underlying cause)
  • retinal vascular attenuation
  • visual field loss
  • peripheral chorioretinal degeneration
  • history of drugs that interfere with vitamin A metabolism

Risk factors

  • retinitis pigmentosa
  • congenital stationary night blindness
  • vitamin A deficiency
  • cancer-associated retinopathy
  • melanoma-associated retinopathy
  • gyrate atrophy
  • choroideremia

Diagnostic investigations

1st investigations to order

  • electroretinography (ERG)
  • slit-biomicroscopy and indirect ophthalmoscopy
Full details

Treatment algorithm

Contributors

Consultant Ophthalmologist

Sunderland Eye Infirmary

Sunderland

UK

Disclosures

JS has received sponsorship from Novartis, Bayer, and DORC for meeting travel and accommodation. JS was awarded a research grant in 2017 by the British Vitreoretinal Society.

Consultant Ophthalmologist

Sunderland Eye Infirmary

Sunderland

UK

Disclosures

WA declares that she has no competing interests.

The authors would like to gratefully acknowledge the assistance of Ms Karen Bradshaw. KB declares that she has no competing interests.

Peer reviewersVIEW ALL

Associate Professor

Department of Ophthalmology

Nagoya University Graduate School of Medicine

Nagoya

Japan

Disclosures

MN declares that he has no competing interests.

Klara Spinks Fleming Fellow

Jules Stein Eye Institute

UCLA

Los Angeles

CA

Disclosures

EA declares that he has no competing interests.

Emeritus Professor and Chairman

Department of Ophthalmology

University of California

San Francisco

CA

Disclosures

CSH declares that he has no competing interests.

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