Choroidal imaging in inherited retinal disease using the technique of enhanced depth imaging optical coherence tomography
- 573 Downloads
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.
Retrospective observational case series.
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.
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).
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.
KeywordsOptical coherence tomography Enhanced depth imaging Choroid Retinal dystrophy Inherited retinal disease
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.
NIHR Biomedical Research Centre, Moorfields Eye Hospital and UCL Institute of Ophthalmology
The Authors report no financial interests in the Spectralis HRA and OCT device used to obtain choroidal scans in the study
The study was approved by the Moorfields Eye Hospital Clinical Governance Committee (Approval Number: YEOHJ1002)
- 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 46:310–316CrossRefPubMedGoogle Scholar