Correlation of optical coherence tomography and autofluorescence in the outer retina and choroid of patients with choroideremia
PURPOSE. To evaluate the relationships between RPE, photoreceptor, and choroidal degeneration in choroideremia. METHODS. Enhanced-depth imaging optical coherence tomography (EDI-OCT), scanning laser ophthalmoscopy (SLO), and autofluorescence (AF) were performed on 39 patients (78 eyes) with choroide...
| Main Authors: | , , , , , , |
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| פורמט: | Journal article |
| שפה: | English |
| יצא לאור: |
Association for Research in Vision and Ophthalmology
2016
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| _version_ | 1826259020897320960 |
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| author | Xue, K Oldani, M Jolly, J Edwards, T Groppe, M Downes, S Maclaren, R |
| author_facet | Xue, K Oldani, M Jolly, J Edwards, T Groppe, M Downes, S Maclaren, R |
| author_sort | Xue, K |
| collection | OXFORD |
| description | PURPOSE. To evaluate the relationships between RPE, photoreceptor, and choroidal degeneration in choroideremia. METHODS. Enhanced-depth imaging optical coherence tomography (EDI-OCT), scanning laser ophthalmoscopy (SLO), and autofluorescence (AF) were performed on 39 patients (78 eyes) with choroideremia. The edges of surviving outer retina on OCTand residual AF were aligned. The distribution of outer retinal tubulations was mapped over a range of ages (16–71 years), and comparison made between pre- and postsubretinal gene therapy. Subfoveal choroidal thickness (SFCT) was compared between 23 choroideremia patients (42 eyes) and 20 age- and refraction-matched male controls (40 eyes). RESULTS. The edges of RPE AF aligned with a reduction in outer nuclear layer thickness (Spearman’s rho = 0.9992). Correlation was also found between the quality of AF and integrity of ellipsoid zone within islands of surviving retina. Tubulations existed in 71 of 78 (91%) eyes with choroideremia and remained stable following gene therapy. Subfoveal choroidal thickness was reduced at baseline in choroideremia (179.7 ± 17.2 μm) compared with controls (302.0 ± 4.8 μm; P < 0.0001), but did not undergo significant thinning until end-stage retinal degeneration (43.1 ± 6.5 μm). CONCLUSIONS. The data suggest that RPE loss is the primary cause of photoreceptor degeneration in choroideremia. The choroid is thinner than controls from early stages, in keeping with a mild developmental defect. Photoreceptors appear to lose outer segments following loss of underlying RPE and form tubulations at the edges of degeneration. The preservation of tubulations over time and after subretinal injection would be consistent with these structures maintaining attachment to the inner retina and hence being potentially light responsive (ClinicalTrials.gov, NCT01461213). |
| first_indexed | 2024-03-06T18:43:17Z |
| format | Journal article |
| id | oxford-uuid:0da13466-6265-4174-b51f-fc43b617d292 |
| institution | University of Oxford |
| language | English |
| last_indexed | 2024-03-06T18:43:17Z |
| publishDate | 2016 |
| publisher | Association for Research in Vision and Ophthalmology |
| record_format | dspace |
| spelling | oxford-uuid:0da13466-6265-4174-b51f-fc43b617d2922022-03-26T09:41:41ZCorrelation of optical coherence tomography and autofluorescence in the outer retina and choroid of patients with choroideremiaJournal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:0da13466-6265-4174-b51f-fc43b617d292EnglishSymplectic Elements at OxfordAssociation for Research in Vision and Ophthalmology2016Xue, KOldani, MJolly, JEdwards, TGroppe, MDownes, SMaclaren, RPURPOSE. To evaluate the relationships between RPE, photoreceptor, and choroidal degeneration in choroideremia. METHODS. Enhanced-depth imaging optical coherence tomography (EDI-OCT), scanning laser ophthalmoscopy (SLO), and autofluorescence (AF) were performed on 39 patients (78 eyes) with choroideremia. The edges of surviving outer retina on OCTand residual AF were aligned. The distribution of outer retinal tubulations was mapped over a range of ages (16–71 years), and comparison made between pre- and postsubretinal gene therapy. Subfoveal choroidal thickness (SFCT) was compared between 23 choroideremia patients (42 eyes) and 20 age- and refraction-matched male controls (40 eyes). RESULTS. The edges of RPE AF aligned with a reduction in outer nuclear layer thickness (Spearman’s rho = 0.9992). Correlation was also found between the quality of AF and integrity of ellipsoid zone within islands of surviving retina. Tubulations existed in 71 of 78 (91%) eyes with choroideremia and remained stable following gene therapy. Subfoveal choroidal thickness was reduced at baseline in choroideremia (179.7 ± 17.2 μm) compared with controls (302.0 ± 4.8 μm; P < 0.0001), but did not undergo significant thinning until end-stage retinal degeneration (43.1 ± 6.5 μm). CONCLUSIONS. The data suggest that RPE loss is the primary cause of photoreceptor degeneration in choroideremia. The choroid is thinner than controls from early stages, in keeping with a mild developmental defect. Photoreceptors appear to lose outer segments following loss of underlying RPE and form tubulations at the edges of degeneration. The preservation of tubulations over time and after subretinal injection would be consistent with these structures maintaining attachment to the inner retina and hence being potentially light responsive (ClinicalTrials.gov, NCT01461213). |
| spellingShingle | Xue, K Oldani, M Jolly, J Edwards, T Groppe, M Downes, S Maclaren, R Correlation of optical coherence tomography and autofluorescence in the outer retina and choroid of patients with choroideremia |
| title | Correlation of optical coherence tomography and autofluorescence in the outer retina and choroid of patients with choroideremia |
| title_full | Correlation of optical coherence tomography and autofluorescence in the outer retina and choroid of patients with choroideremia |
| title_fullStr | Correlation of optical coherence tomography and autofluorescence in the outer retina and choroid of patients with choroideremia |
| title_full_unstemmed | Correlation of optical coherence tomography and autofluorescence in the outer retina and choroid of patients with choroideremia |
| title_short | Correlation of optical coherence tomography and autofluorescence in the outer retina and choroid of patients with choroideremia |
| title_sort | correlation of optical coherence tomography and autofluorescence in the outer retina and choroid of patients with choroideremia |
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