Optical Coherence Tomography Angiography in <i>CRB1</i>-Associated Retinal Dystrophies
Aim of the study: To report optical coherence tomography angiography (OCTA) findings in patients affected by <i>CRB1</i>-associated retinal dystrophies. Method: Patients affected by a genetically confirmed <i>CRB1</i>-associated retinal dystrophy were prospectively enrolled i...
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| Format: | Article |
| Language: | English |
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MDPI AG
2023-01-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/12/3/1095 |
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| author | Firuzeh Rajabian Alessandro Arrigo Lorenzo Bianco Alessio Antropoli Maria Pia Manitto Elisabetta Martina Francesco Bandello Jay Chhablani Maurizio Battaglia Parodi |
| author_facet | Firuzeh Rajabian Alessandro Arrigo Lorenzo Bianco Alessio Antropoli Maria Pia Manitto Elisabetta Martina Francesco Bandello Jay Chhablani Maurizio Battaglia Parodi |
| author_sort | Firuzeh Rajabian |
| collection | DOAJ |
| description | Aim of the study: To report optical coherence tomography angiography (OCTA) findings in patients affected by <i>CRB1</i>-associated retinal dystrophies. Method: Patients affected by a genetically confirmed <i>CRB1</i>-associated retinal dystrophy were prospectively enrolled in an observational study, along with age- and sex-matched healthy volunteers as control subjects. All study and control subjects received a complete ophthalmic examination and multimodal retinal imaging, including OCTA. Result: A total of 12 eyes from 6 patients were included in the study. The mean BCVA of patients was 0.42 ± 0.25 logMAR. Two patients showed large central atrophy, with corresponding definite hypo-autofluorescence on fundus autofluorescence (FAF). Another four patients disclosed different degrees of RPE mottling, with uneven FAF. On OCTA, the macular deep capillary plexus and choriocapillaris had a lower vessel density in eyes affected by <i>CRB1</i>-associated retinopathy when compared to healthy controls. On the other hand, vessel density at the peripapillary radial capillary plexus, superficial capillary plexus, and deep capillary plexus was significantly altered with respect to control eyes. Statistical analyses disclosed a negative correlation between the deep capillary plexus and both LogMAR best corrected visual acuity and central retinal thickness. Conclusion: Our study reveals that <i>CRB1</i>-associated retinal dystrophies are characterized by vascular alterations both in the macular and peripapillary region, as assessed by OCTA. |
| first_indexed | 2024-03-11T09:37:55Z |
| format | Article |
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| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-11T09:37:55Z |
| publishDate | 2023-01-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-63891fc62ae34febb41ab766985daf3d2023-11-16T17:11:40ZengMDPI AGJournal of Clinical Medicine2077-03832023-01-01123109510.3390/jcm12031095Optical Coherence Tomography Angiography in <i>CRB1</i>-Associated Retinal DystrophiesFiruzeh Rajabian0Alessandro Arrigo1Lorenzo Bianco2Alessio Antropoli3Maria Pia Manitto4Elisabetta Martina5Francesco Bandello6Jay Chhablani7Maurizio Battaglia Parodi8Department of Ophthalmology, Vita-Salute San Raffaele University, IRCCS Ospedale San Raffaele, 20132 Milan, ItalyDepartment of Ophthalmology, Vita-Salute San Raffaele University, IRCCS Ospedale San Raffaele, 20132 Milan, ItalyDepartment of Ophthalmology, Vita-Salute San Raffaele University, IRCCS Ospedale San Raffaele, 20132 Milan, ItalyDepartment of Ophthalmology, Vita-Salute San Raffaele University, IRCCS Ospedale San Raffaele, 20132 Milan, ItalyDepartment of Ophthalmology, Vita-Salute San Raffaele University, IRCCS Ospedale San Raffaele, 20132 Milan, ItalyDepartment of Ophthalmology, Vita-Salute San Raffaele University, IRCCS Ospedale San Raffaele, 20132 Milan, ItalyDepartment of Ophthalmology, Vita-Salute San Raffaele University, IRCCS Ospedale San Raffaele, 20132 Milan, ItalyDepartment of Ophthalmology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USADepartment of Ophthalmology, Vita-Salute San Raffaele University, IRCCS Ospedale San Raffaele, 20132 Milan, ItalyAim of the study: To report optical coherence tomography angiography (OCTA) findings in patients affected by <i>CRB1</i>-associated retinal dystrophies. Method: Patients affected by a genetically confirmed <i>CRB1</i>-associated retinal dystrophy were prospectively enrolled in an observational study, along with age- and sex-matched healthy volunteers as control subjects. All study and control subjects received a complete ophthalmic examination and multimodal retinal imaging, including OCTA. Result: A total of 12 eyes from 6 patients were included in the study. The mean BCVA of patients was 0.42 ± 0.25 logMAR. Two patients showed large central atrophy, with corresponding definite hypo-autofluorescence on fundus autofluorescence (FAF). Another four patients disclosed different degrees of RPE mottling, with uneven FAF. On OCTA, the macular deep capillary plexus and choriocapillaris had a lower vessel density in eyes affected by <i>CRB1</i>-associated retinopathy when compared to healthy controls. On the other hand, vessel density at the peripapillary radial capillary plexus, superficial capillary plexus, and deep capillary plexus was significantly altered with respect to control eyes. Statistical analyses disclosed a negative correlation between the deep capillary plexus and both LogMAR best corrected visual acuity and central retinal thickness. Conclusion: Our study reveals that <i>CRB1</i>-associated retinal dystrophies are characterized by vascular alterations both in the macular and peripapillary region, as assessed by OCTA.https://www.mdpi.com/2077-0383/12/3/1095<i>CRB1</i>crumbs homolog 1retinal dystrophyoptical coherence tomography angiographyOCTAmultimodal imaging |
| spellingShingle | Firuzeh Rajabian Alessandro Arrigo Lorenzo Bianco Alessio Antropoli Maria Pia Manitto Elisabetta Martina Francesco Bandello Jay Chhablani Maurizio Battaglia Parodi Optical Coherence Tomography Angiography in <i>CRB1</i>-Associated Retinal Dystrophies Journal of Clinical Medicine <i>CRB1</i> crumbs homolog 1 retinal dystrophy optical coherence tomography angiography OCTA multimodal imaging |
| title | Optical Coherence Tomography Angiography in <i>CRB1</i>-Associated Retinal Dystrophies |
| title_full | Optical Coherence Tomography Angiography in <i>CRB1</i>-Associated Retinal Dystrophies |
| title_fullStr | Optical Coherence Tomography Angiography in <i>CRB1</i>-Associated Retinal Dystrophies |
| title_full_unstemmed | Optical Coherence Tomography Angiography in <i>CRB1</i>-Associated Retinal Dystrophies |
| title_short | Optical Coherence Tomography Angiography in <i>CRB1</i>-Associated Retinal Dystrophies |
| title_sort | optical coherence tomography angiography in i crb1 i associated retinal dystrophies |
| topic | <i>CRB1</i> crumbs homolog 1 retinal dystrophy optical coherence tomography angiography OCTA multimodal imaging |
| url | https://www.mdpi.com/2077-0383/12/3/1095 |
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