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

  • Retinal Disorders
  • Published:
Graefe's Archive for Clinical and Experimental Ophthalmology Aims and scope Submit manuscript

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.

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References

  1. Oh KT, Weleber R, Stone E, Oh D, Rosenow J, Billingslea A (2004) Electroretinographic findings in patients with Stargardt disease and fundus flavimaculatus. Retina 24(6):920–928

    Article  PubMed  Google Scholar 

  2. Ashton N (1953) Central areolar choroidal sclerosis; a histopathological study. Br J Ophthalmol 37:140–147

    Article  CAS  PubMed  Google Scholar 

  3. Ferry AP, Llovera I, Shafer DM (1972) Central areolar choroidal dystrophy. Arch Ophthalmol 88:39–43

    CAS  PubMed  Google Scholar 

  4. Voo I, Glasgow BJ, Flannery J, Udar N, Small KW (2001) North Carolina Macular Dystrophy: clinicopathological correlation. Am J Ophthalmol 132(6):933–935

    Article  CAS  PubMed  Google Scholar 

  5. Mura M, Sereda C, Jablonski MM, MacDonald IM, Iannaccone A (2007) Clinical and functional findings in choroideraemia due to complete deletion of the CHM gene. Arch Ophthalmol 125(8):1107–1113

    Article  CAS  PubMed  Google Scholar 

  6. Ayata A, Tatlipinar S, Unal M, et Ersanli D, Bilge AH (2008) Autofluorescence and OCT features of Bietti’s crystalline dystrophy. Br J Ophthalmol 92:718–720

    Article  CAS  PubMed  Google Scholar 

  7. Korte GE, Reppucci V, Henkind P (1984) RPE destruction causes choriocapillary atrophy. Invest Ophthalmol Vis Sci 25:1135–1145

    CAS  PubMed  Google Scholar 

  8. Neuhardt TH, May CA, Wilsch C, Eichorn M, Lutjen-Drecoll E (1999) Morphological changes of retinal pigment epithelium and choroid in mice. Exp Eye Res 68:75–83

    Article  CAS  PubMed  Google Scholar 

  9. Saint-Geniez N, Kurihara T, Sekiyama E, Maldonado A, D’Amore P (2009) An essential role for RPE-derived soluble VEGF in the maintainance of the choriocapillaris. Proc Natl Acad Sci U S A 106(44):18751–18756, Epub

    Article  CAS  PubMed  Google Scholar 

  10. Wirtitsch MG, Ergun E, Hermann B, Unterhuber A, Stur M, Schloda C, Sattman HH, Ko TH, Fujimoto JG, Drexler W (2005) Ultrahigh resolution optical coherence tomography in macular dystrophy. Am J Ophthalmol 140(6):976–983

    Article  PubMed  Google Scholar 

  11. Ergun E, Hermann B, Wirtitsch M, Unterhuber A, Ko TH, Sattmann H, Scholda C, Fujimoto JG, Stur M, Drexler W (2005) Assessment of central visual function in Stargardt’s disease/fundus flavimaculatus with ultrahigh-resolution optical coherence tomography. Invest Ophthalmol Vis Sci 46:310–316

    Article  PubMed  Google Scholar 

  12. Lim JL, Tan O, Fawzi AA, Hopkins JJ, Gil-Flamer JH, Huang D (2008) A pilot study of Fourier-domain optical coherence tomography of retinal dystrophy patients. Am J Ophthalmol 146(3):417–426

    Article  PubMed  Google Scholar 

  13. Margolis R, Spaide RF (2009) A pilot study of enhanced depth imaging optical coherence tomography of the choroid in normal eyes. Am J Ophthalmol 147(5):811–815

    Article  PubMed  Google Scholar 

  14. Spaide RF, Koizumi H, Pozzoni MC (2008) Enhanced depth imaging spectral domain optical coherence tomography. Am J Ophthalmol 146(4):496–500

    Article  PubMed  Google Scholar 

  15. Povazay B, Hermann B, Hofer B, Kajic V, Simpson E, Bridgford T, Drexler W (2009) Wide-field optical coherence tomography of the choroid in vivo. Invest Ophthalmol Vis Sci 50:1856–1863

    Article  PubMed  Google Scholar 

  16. Ikuno Y, Kawaguchi K, Nouchi T, Yasuno Y (2010) Choroidal thickness in healthy Japanese subjects. Invest Ophthalmol Vis Sci 51:2173–2176

    Article  PubMed  Google Scholar 

  17. Ramrattan R, Van Der Schaft T, Mooy C, Bruijin W, Mulder P, de Jong P (1994) Morphometric analysis of Bruch’s membrane, the choriocapillaris and the choroid in aging. Invest Ophthalmol Vis Sci 35:2857–2864

    CAS  PubMed  Google Scholar 

  18. Godley BF, Tiffin PA, Evans K, Kelsell RE, Hunt DM, Bird AC (1996) Cinical features of progressive bifocal chorioretinal atrophy: a retinal dystrophy linked to chromosome 6q. Ophthalmology 103(6):893–898

    CAS  PubMed  Google Scholar 

  19. MacDonald I, Russell L, Chi-Chao C (2009) Choroideraemia: new findings from ocular pathology and review of recent literature. Surv Ophthalmol 54:401–407

    Article  PubMed  Google Scholar 

  20. Cideciyan AV, Aleman TS, Swinder M et al (2004) Mutations in ABCA4 result in accumulation of lipofuschin before slowing of the retinoid cycle. A reappraisal of the human disease sequence. Hum Mol Genet 13:525–534

    Article  CAS  PubMed  Google Scholar 

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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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Authors and Affiliations

Authors

Corresponding author

Correspondence to Jonathan Yeoh.

Appendix

Appendix

Table 3 Relationship between visual acuity and choroidal change on EDI OCT for both right and left eyes
Table 4 Relationship between extent of retinal dysfunction on electrophysiology and choroidal change on EDI OCT.

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Yeoh, J., Rahman, W., Chen, F. et al. Choroidal imaging in inherited retinal disease using the technique of enhanced depth imaging optical coherence tomography. Graefes Arch Clin Exp Ophthalmol 248, 1719–1728 (2010). https://doi.org/10.1007/s00417-010-1437-3

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  • DOI: https://doi.org/10.1007/s00417-010-1437-3

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