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Choroidal imaging in inherited retinal disease using the technique of enhanced depth imaging optical coherence tomography

  • Jonathan Yeoh
  • Waheeda Rahman
  • Fred Chen
  • Claire Hooper
  • Praveen Patel
  • Adnan Tufail
  • Andrew R. Webster
  • Anthony T. Moore
  • Lyndon DaCruz
Retinal Disorders

Abstract

Purpose

The aim of this study is to image and describe the in vivo choroidal changes in various retinal dystrophies using the technique of enhanced depth imaging (EDI) optical coherence tomography (OCT) and to correlate these findings with the clinical appearance. Associations between choroidal change and genotype, visual acuity and results of retinal electrophysiology are also explored.

Design

Retrospective observational case series.

Methods

Twenty patients attending the medical retina clinics at Moorfields Eye Hospital underwent EDI OCT choroidal scans as part of the scanning protocol when they underwent OCT imaging with the Spectralis HRA and OCT. The choroidal images were obtained by moving the Spectralis camera close enough to obtain an inverted image of the retina. The scans were read by two experienced OCT readers assessing the choroidal thickness as well as the choroidal contour for focal areas of choroidal thinning corresponding to the areas of RPE/outer retinal atrophy. The spectrum of patients included those with Stargardt macular dystrophy, macular dystrophies secondary to known mutations such as peripherin/RDS, uncharacterised macular dystrophies, Best disease, bifocal chorioretinal atrophy, Bietti crystalline retinal dystrophy and choroideraemia.

Results

The choroidal appearance was symmetrical in all patients who had both eyes scanned. Ten patients showed no choroidal thinning, five had focal mild to moderate choroidal thinning, three had focal severe choroidal thinning, and two patients had diffuse severe choroidal thinning. There was no association between choroidal thinning and visual acuity [Fisher’s exact test, p = 0.350 (right eye), p = 1.000 (left eye)], or extent of retinal dysfunction on electrophysiology (Fisher’s exact test, p = 1.000).

Conclusion

Enhanced depth imaging using spectral domain OCT can be used to identify choroidal changes in inherited retinal disease. The pattern of choroidal change correlates well with the clinical appearance. It appears that the extent and pattern of choroidal thinning is dependent on the stage of the disease in some cases, and in others the causative gene defect.

Keywords

Optical coherence tomography Enhanced depth imaging Choroid Retinal dystrophy Inherited retinal disease 

Notes

Acknowledgments

We would like to acknowledge Ms Genevieve Wright (genetics co-ordinator) and Ms Sophie Devery (genetics counsellor), Moorfields Eye Hospital for their help in obtaining molecular diagnoses of the patients from the database.

Funding

NIHR Biomedical Research Centre, Moorfields Eye Hospital and UCL Institute of Ophthalmology

Financial Disclosures

The Authors report no financial interests in the Spectralis HRA and OCT device used to obtain choroidal scans in the study

Study Approval

The study was approved by the Moorfields Eye Hospital Clinical Governance Committee (Approval Number: YEOHJ1002)

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Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Jonathan Yeoh
    • 1
  • Waheeda Rahman
    • 1
  • Fred Chen
    • 1
    • 2
  • Claire Hooper
    • 1
  • Praveen Patel
    • 1
    • 2
  • Adnan Tufail
    • 1
    • 2
  • Andrew R. Webster
    • 1
    • 2
  • Anthony T. Moore
    • 1
    • 2
  • Lyndon DaCruz
    • 1
    • 2
  1. 1.Moorfields Eye HospitalLondonUK
  2. 2.NIHR Biomedical Research Centre for OphthalmologyMoorfields Eye Hospital and UCL Institute of OphthalmologyLondonUK

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