Download fulltext HTML
1.
Sabbaghi H, Ahmadieh H, Jalili J, Behnaz N, Fakhri M, Suri F, Rajabpour M, Entezari M, Daftarian N. Choroidal Thickness in Different Types of Inherited Retinal Dystrophies. JOVR [Internet]. 2020Jul.29 [cited 2024Apr.25];15(3):351–361. Available from: https://knepublishing.com/index.php/JOVR/article/view/7454

https://doi.org/10.18502/jovr.v15i3.7454

statistics

  • 790 Abstract Views
  • 522 PDF Views
  • 0 HTML Views
  • Cited by 2 articles

authors

  • Hamideh Sabbaghi

    Hamideh Sabbaghi

    Ophthalmic Epidemiology Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  • Hamid Ahmadieh

    Hamid Ahmadieh

    Ophthalmic Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  • Jalil Jalili

    Jalil Jalili

    edical Physics and Biomedical Engineering Department, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
  • Nazanin Behnaz

    Nazanin Behnaz

    Ophthalmic Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  • Maryam Fakhri

    Maryam Fakhri

    Ophthalmic Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  • Fatemeh Suri

    Fatemeh Suri

    Ophthalmic Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  • Mojtaba Rajabpour

    Mojtaba Rajabpour

    Department of Optometry, School of Rehabilitation, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  • Morteza Entezari

    Morteza Entezari

    Ophthalmic Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  • Narsis Daftarian

    Narsis Daftarian

    Ocular Tissue Engineering Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran

Published Date

  • Jul 29, 2020
This journal is publishing all the articles under Creative Commons Attribution License CC BY 4.0.

Choroidal Thickness in Different Types of Inherited Retinal Dystrophies

Journal of Ophthalmic and Vision Research (JOVR) / July–September 2020, Volume 15, Issue 3 / Pages 351–361

Abstract

Purpose: To compare the choroidal thickness among eyes with retinitis pigmentosa (RP), Stargardt disease, Usher syndrome, cone-rod dystrophy, and healthy eyes of sex- and age-matched individuals.


Methods: In this comparative study, 503 eyes with RP (n = 264), cone-rod dystrophy (n = 109), Stargardt disease (n = 76), and Usher syndrome (n = 54) were included. To validate the data, 109 healthy eyes of 56 sex- and age-matched individuals were studied as controls. Choroidal imaging was performed using enhanced depth imaging-optical coherence tomography. Choroidal thickness was measured manually using MATLAB software at 13 points in nasal and temporal directions from the foveal center with the interval of 500 μm and the choroidal area encompassing the measured points was calculated automatically.


Results: The mean age was 36.33 ± 13.07 years (range, 5 to 72 years). The mean choroidal thickness at 13 points of the control eyes was statistically significantly higher than that in eyes with RP (P < 0.001) and Usher syndrome (P < 0.05), but not significantly different from that in eyes with Stargardt disease and cone-rod dystrophy. Among different inherited retinal dystrophies (IRDs), the choroidal thickness was the lowest in eyes with RP (P < 0.001). Choroidal thickness in the subfoveal area correlated negatively with best-corrected visual acuity (r = −0.264, P < 0.001) and the duration of ocular symptoms (r = −0.341, P < 0.001) in all studied IRDs. No significant correlation was observed between the subfoveal choroidal thickness and central macular thickness (r = −0.24, P = 0.576).


Conclusion: Choroidal thinning in four different types of IRDs does not follow a similar pattern and depends on the type of IRD and the duration of ocular symptoms. A larger cohort is required to verify these findings.

Keywords:

Choroidal Thickness, Cone-rod Dystrophy, Enhanced Depth Optical Coherence Tomography, Inherited Retinal Dystrophy, Retinitis Pigmentosa, Stargardt Disease, Usher Syndrome

References
1. Srinivasan VJ, Dubra A. Noninvasive imaging of the photoreceptor mosaic response to light stimulation. Proc Natl Acad Sci USA 2016;113:12902–12903.

2. Rutkowski P, May CA. Nutrition and vascular supply of retinal ganglion cells during human development. Front Neurol 2016;7:49.

3. Hartong DT, Berson EL, Dryja TP. Retinitis pigmentosa. Lancet 2006;368:1795–1809.

4. Ayton LN, Guymer RH, Luu CD. Choroidal thickness profiles in retinitis pigmentosa. Clin Exp Ophthalmol 2013;41:396–403.

5. Sodi A, Bacherini D, Lenzetti C, Caporossi O, Murro V, Mucciolo DP, et al. EDI OCT evaluation of choroidal thickness in Stargardt disease. PLoS One 2018;13:e0190780.

6. Öner A. Recent advancements in gene therapy for hereditary retinal dystrophies. Turk J Ophthalmol 2017;47:338–343.

7. Ecklund Winters J. Retinitis pigmentosa: distribution of inheritance patterns in a VA blind rehabilitation center population. Clin Eye Vis Care 2000;12:107–112.

8. Bertelsen M, Jensen H, Bregnhøj JF, Rosenberg T. Prevalence of generalized retinal dystrophy in Denmark. Ophthalmic Epidemiol 2014;21:217–223.

9. Oh KT, Weleber RG, Stone EM, Oh DM, Rosenow J, Billingslea AM. Electroretinographic findings in patients with Stargardt disease and fundus flavimaculatus. Retina 2004;24:920–928.

10. Yeoh J, Rahman W, Chen F, Hooper C, Patel P, Tufail A, et al. Choroidal imaging in inherited retinal disease using the technique of enhanced depth imagingoptical coherence tomography. Graefes Arch Clin Exp Ophthalmol 2010;248:1719–1728.

11. Al-Saikhan FI. The gene therapy revolution in ophthalmology. Saudi J Ophthalmol 2013;27:107–111.

12. Mrejen S, Spaide RF. Optical coherence tomography: imaging of the choroid and beyond. Surv Ophthalmol 2013;58:387–429.

13. Dhoot DS, Huo S, Yuan A, Xu D, Srivistava S, Ehlers JP, et al. Evaluation of choroidal thickness in retinitis pigmentosa using enhanced depth imaging optical coherence tomography. Br J Ophthalmol 2013;97:66–69.

14. Heckenlively JR, Yoser SL, Friedman LH, Oversier JJ. Clinical findings and common symptoms in retinitis pigmentosa. Am J Ophthalmol 1988;105:504–511.

15. Laviers H, Zambarakji H. Enhanced depth imaging-OCT of the choroid: a review of the current literature. Graefes Arch Clin Exp Ophthalmol 2014;252:1871–1883.

16. Sodi A, Lenzetti C, Murro V, Caporossi O, Mucciolo DP, Bacherini D, et al. EDI-OCT evaluation of choroidal thickness in retinitis pigmentosa. Eur J Ophthalmol 2018;28:52–57.

17. Adhi M, Read SP, Ferrara D, Weber M, Duker JS, Waheed NK. Morphology and vascular layers of the choroid in Stargardt disease analyzed using spectral-domain optical coherence tomography. Am J Ophthalmol 2015;160:1276– 1284.e1.

18. Ayyildiz O, Ozge G, Kucukevcilioglu M, Ozgonul C, Mumcuoglu T, Durukan AH, et al. Is there a relationship between outer retinal destruction and choroidal changes in cone dystrophy? Arq Bras Oftalmol 2016;79:315–318.

19. Chhablani J, Jonnadula GB, Srinivasa Rao P, Venkata A, Jalali S. Choroidal thickness profile in Retinitis Pigmentosa - correlation with outer retinal structures. Saudi J Ophthalmol 2016;30:9–13.

20. Falsini B, Anselmi GM, Marangoni D, D’Esposito F, Fadda A, Di Renzo A, et al. Subfoveal choroidal blood flow and central retinal function in retinitis pigmentosa. Invest Ophthalmol Vis Sci 2011;52:1064–1069.

21. Konieczka K, Flammer AJ, Todorova M, Meyer P, Flammer J. Retinitis pigmentosa and ocular blood flow. EPMA J 2012;3:17.

22. Sorrentino FS, Bonifazzi C, Perri P. The role of the endothelin system in the vascular dysregulation involved in Retinitis Pigmentosa. J Ophthalmol 2015;2015:405234.

23. Ford KM, Saint-Geniez M, Walshe T, Zahr A, D’Amore PA. Expression and role of VEGF in the adult retinal pigment epithelium. Invest Ophthalmol Vis Sci 2011;52:9478–9487.

24. Yoshida N, Ikeda Y, Notomi S, Ishikawa K, Murakami Y, Hisatomi T, et al. Laboratory evidence of sustained chronic inflammatory reaction in retinitis pigmentosa. Ophthalmology 2013;120:e5–e12.

25. Martínez-Fernández de la Cámara C, Salom D, Sequedo MD, Hervás D, Marín-Lambíes C, et al. Altered antioxidantoxidant status in the aqueous humor and peripheral blood of patients with retinitis pigmentosa. PLoS One 2013;8:e74223.