Progression of Rare Inherited Retinal Dystrophies May Be Monitored by Adaptive Optics Imaging
Inherited retinal dystrophies (IRDs) are bilateral genetic conditions of the retina, leading to irreversible vision loss. This study included 55 eyes afflicted with IRDs affecting the macula. The diseases examined encompassed Stargardt disease (STGD), cone dystrophy (CD), and cone–rod dystrophy (CRD...
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MDPI AG
2023-09-01
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| Series: | Life |
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| Online Access: | https://www.mdpi.com/2075-1729/13/9/1871 |
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| author | Katarzyna Samelska Jacek Paweł Szaflik Barbara Śmigielska Anna Zaleska-Żmijewska |
| author_facet | Katarzyna Samelska Jacek Paweł Szaflik Barbara Śmigielska Anna Zaleska-Żmijewska |
| author_sort | Katarzyna Samelska |
| collection | DOAJ |
| description | Inherited retinal dystrophies (IRDs) are bilateral genetic conditions of the retina, leading to irreversible vision loss. This study included 55 eyes afflicted with IRDs affecting the macula. The diseases examined encompassed Stargardt disease (STGD), cone dystrophy (CD), and cone–rod dystrophy (CRD) using adaptive optics (Rtx1™; Imagine Eyes, Orsay, France). Adaptive optics facilitate high-quality visualisation of retinal microstructures, including cones. Cone parameters, such as cone density (DM), cone spacing (SM), and regularity (REG), were analysed. The best corrected visual acuity (BCVA) was assessed as well. Examinations were performed twice over a 6-year observation period. A significant change was observed in DM (<inline-formula><math xmlns="http://www.w3.org/1998/Math/MathML" display="inline"><semantics><mrow><mn>1282.73</mn></mrow></semantics></math></inline-formula>/mm<inline-formula><math xmlns="http://www.w3.org/1998/Math/MathML" display="inline"><semantics><msup><mrow></mrow><mn>2</mn></msup></semantics></math></inline-formula> vs. 10,073.42/mm<inline-formula><math xmlns="http://www.w3.org/1998/Math/MathML" display="inline"><semantics><msup><mrow></mrow><mn>2</mn></msup></semantics></math></inline-formula>, <inline-formula><math xmlns="http://www.w3.org/1998/Math/MathML" display="inline"><semantics><mrow><mi>p</mi><mo><</mo></mrow></semantics></math></inline-formula> 0.001) and SM (9.83 <inline-formula><math xmlns="http://www.w3.org/1998/Math/MathML" display="inline"><semantics><mi mathvariant="sans-serif">μ</mi></semantics></math></inline-formula>m vs. 12.16 <inline-formula><math xmlns="http://www.w3.org/1998/Math/MathML" display="inline"><semantics><mi mathvariant="sans-serif">μ</mi></semantics></math></inline-formula>m, <inline-formula><math xmlns="http://www.w3.org/1998/Math/MathML" display="inline"><semantics><mrow><mi>p</mi><mo><</mo></mrow></semantics></math></inline-formula> 0.001) during the follow-up. BCVA deterioration was also significant (<inline-formula><math xmlns="http://www.w3.org/1998/Math/MathML" display="inline"><semantics><mrow><mn>0.16</mn></mrow></semantics></math></inline-formula> vs. <inline-formula><math xmlns="http://www.w3.org/1998/Math/MathML" display="inline"><semantics><mrow><mn>0.12</mn></mrow></semantics></math></inline-formula>, <i>p</i> = 0.001), albeit uncorrelated with the change in cone parameters. No significant difference in REG was detected between the initial examination and the follow-up (<i>p</i> = 0.089). |
| first_indexed | 2024-03-10T22:33:05Z |
| format | Article |
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| institution | Directory Open Access Journal |
| issn | 2075-1729 |
| language | English |
| last_indexed | 2024-03-10T22:33:05Z |
| publishDate | 2023-09-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Life |
| spelling | doaj.art-74bd8cae65d942c2bee4a088d723435b2023-11-19T11:37:27ZengMDPI AGLife2075-17292023-09-01139187110.3390/life13091871Progression of Rare Inherited Retinal Dystrophies May Be Monitored by Adaptive Optics ImagingKatarzyna Samelska0Jacek Paweł Szaflik1Barbara Śmigielska2Anna Zaleska-Żmijewska3Department of Ophthalmology, Medical University of Warsaw, 02-091 Warsaw, PolandDepartment of Ophthalmology, Medical University of Warsaw, 02-091 Warsaw, PolandDepartment of Ophthalmology, Medical University of Warsaw, 02-091 Warsaw, PolandDepartment of Ophthalmology, Medical University of Warsaw, 02-091 Warsaw, PolandInherited retinal dystrophies (IRDs) are bilateral genetic conditions of the retina, leading to irreversible vision loss. This study included 55 eyes afflicted with IRDs affecting the macula. The diseases examined encompassed Stargardt disease (STGD), cone dystrophy (CD), and cone–rod dystrophy (CRD) using adaptive optics (Rtx1™; Imagine Eyes, Orsay, France). Adaptive optics facilitate high-quality visualisation of retinal microstructures, including cones. Cone parameters, such as cone density (DM), cone spacing (SM), and regularity (REG), were analysed. The best corrected visual acuity (BCVA) was assessed as well. Examinations were performed twice over a 6-year observation period. A significant change was observed in DM (<inline-formula><math xmlns="http://www.w3.org/1998/Math/MathML" display="inline"><semantics><mrow><mn>1282.73</mn></mrow></semantics></math></inline-formula>/mm<inline-formula><math xmlns="http://www.w3.org/1998/Math/MathML" display="inline"><semantics><msup><mrow></mrow><mn>2</mn></msup></semantics></math></inline-formula> vs. 10,073.42/mm<inline-formula><math xmlns="http://www.w3.org/1998/Math/MathML" display="inline"><semantics><msup><mrow></mrow><mn>2</mn></msup></semantics></math></inline-formula>, <inline-formula><math xmlns="http://www.w3.org/1998/Math/MathML" display="inline"><semantics><mrow><mi>p</mi><mo><</mo></mrow></semantics></math></inline-formula> 0.001) and SM (9.83 <inline-formula><math xmlns="http://www.w3.org/1998/Math/MathML" display="inline"><semantics><mi mathvariant="sans-serif">μ</mi></semantics></math></inline-formula>m vs. 12.16 <inline-formula><math xmlns="http://www.w3.org/1998/Math/MathML" display="inline"><semantics><mi mathvariant="sans-serif">μ</mi></semantics></math></inline-formula>m, <inline-formula><math xmlns="http://www.w3.org/1998/Math/MathML" display="inline"><semantics><mrow><mi>p</mi><mo><</mo></mrow></semantics></math></inline-formula> 0.001) during the follow-up. BCVA deterioration was also significant (<inline-formula><math xmlns="http://www.w3.org/1998/Math/MathML" display="inline"><semantics><mrow><mn>0.16</mn></mrow></semantics></math></inline-formula> vs. <inline-formula><math xmlns="http://www.w3.org/1998/Math/MathML" display="inline"><semantics><mrow><mn>0.12</mn></mrow></semantics></math></inline-formula>, <i>p</i> = 0.001), albeit uncorrelated with the change in cone parameters. No significant difference in REG was detected between the initial examination and the follow-up (<i>p</i> = 0.089).https://www.mdpi.com/2075-1729/13/9/1871adaptive opticscone dystrophycone–rod dystrophyinherited retinal diseasesinherited retinal dystrophiesocular imaging |
| spellingShingle | Katarzyna Samelska Jacek Paweł Szaflik Barbara Śmigielska Anna Zaleska-Żmijewska Progression of Rare Inherited Retinal Dystrophies May Be Monitored by Adaptive Optics Imaging Life adaptive optics cone dystrophy cone–rod dystrophy inherited retinal diseases inherited retinal dystrophies ocular imaging |
| title | Progression of Rare Inherited Retinal Dystrophies May Be Monitored by Adaptive Optics Imaging |
| title_full | Progression of Rare Inherited Retinal Dystrophies May Be Monitored by Adaptive Optics Imaging |
| title_fullStr | Progression of Rare Inherited Retinal Dystrophies May Be Monitored by Adaptive Optics Imaging |
| title_full_unstemmed | Progression of Rare Inherited Retinal Dystrophies May Be Monitored by Adaptive Optics Imaging |
| title_short | Progression of Rare Inherited Retinal Dystrophies May Be Monitored by Adaptive Optics Imaging |
| title_sort | progression of rare inherited retinal dystrophies may be monitored by adaptive optics imaging |
| topic | adaptive optics cone dystrophy cone–rod dystrophy inherited retinal diseases inherited retinal dystrophies ocular imaging |
| url | https://www.mdpi.com/2075-1729/13/9/1871 |
| work_keys_str_mv | AT katarzynasamelska progressionofrareinheritedretinaldystrophiesmaybemonitoredbyadaptiveopticsimaging AT jacekpawełszaflik progressionofrareinheritedretinaldystrophiesmaybemonitoredbyadaptiveopticsimaging AT barbarasmigielska progressionofrareinheritedretinaldystrophiesmaybemonitoredbyadaptiveopticsimaging AT annazaleskazmijewska progressionofrareinheritedretinaldystrophiesmaybemonitoredbyadaptiveopticsimaging |