Olivia Xerri1, Federico Bernabei2, Elise Philippakis3, Cyril Burin-Des-Roziers2,4, Pierre-Olivier Barale5, Olivier Laplace5, Claire Monin5, Dominique Bremond-Gignac1,4, Gilles Guerrier6, Sophie Valleix4,7, Antoine Brezin2,4, Pierre-Raphaël Rothschild8,9. 1. Service d'Ophtalmologie, Hôpital Necker-Enfants Malades, AP-HP, F-75014, Paris, France. 2. Service d'Ophtalmologie, Ophtalmopôle de Paris, Hôpital Cochin, AP-HP, 27 rue du Faubourg Saint Jacques, 75014, Paris, France. 3. Service d'Ophtalmologie, Hôpital Lariboisière, AP-HP, F-75014, Paris, France. 4. Université de Paris, Centre de Recherche des Cordeliers, INSERM, UMR_1138, F-75006, Paris, France. 5. Service d'Ophtalmologie, Hôpital des Quinze-Vingts, Paris, France. 6. Anaesthetic and Intensive Care Department, Hôpital Cochin, Paris Descartes university, 75014, Paris, France. 7. Laboratoire de Génétique Moléculaire, Faculté de Médecine Paris, Hôpital Necker-Enfants Malades, Université de Paris, AP-HP; Inserm, U_1163, Institut IMAGINE, F-75014, Paris, France. 8. Service d'Ophtalmologie, Ophtalmopôle de Paris, Hôpital Cochin, AP-HP, 27 rue du Faubourg Saint Jacques, 75014, Paris, France. pierreraphaelrothschild@hotmail.com. 9. Université de Paris, Centre de Recherche des Cordeliers, INSERM, UMR_1138, F-75006, Paris, France. pierreraphaelrothschild@hotmail.com.
Abstract
BACKGROUND: To compare different clinical and Spectral-Domain Optical Coherence Tomography (SD-OCT) features of high myopic eyes with Stickler syndrome (STL) with matched controls. METHODS: Patients with genetically confirmed STL with axial length ≥ 26 mm and controls matched for axial length were included. The following data were obtained from SD-OCT scans and fundus photography: choroidal and retinal thickness (respectively, CT and RT), peripapillary atrophy area (PAA), presence of posterior staphyloma (PS). RESULTS: Twenty-six eyes of 17 patients with STL and 25 eyes of 19 controls were evaluated. Compared with controls, patients with STL showed a greater CT subfoveally, at 1000 μm from the fovea at both nasal and temporal location, and at 2000 and 3000 μm from the fovea in nasal location (respectively, 188.7±72.8 vs 126.0±88.7 μm, 172.5±77.7 vs 119.3±80.6 μm, 190.1±71.9 vs 134.9±79.7 μm, 141.3±56.0 vs 98.1±68.5 μm, and 110.9±51.0 vs 67.6±50.7 μm, always P< 0.05). Furthermore, patients with STL showed a lower prevalence of PS (11.5% vs 68%, P< 0.001) and a lower PAA (2.2±2.1 vs 5.4±5.8 mm2, P=0.03), compared with controls. CONCLUSIONS: This study shows that high myopic patients with STL show a greater CT, a lower PAA and a lower prevalence of PS, compared with controls matched for axial length. These findings could be relevant for the development and progression of myopic maculopathy in patients with STL.
BACKGROUND: To compare different clinical and Spectral-Domain Optical Coherence Tomography (SD-OCT) features of high myopic eyes with Stickler syndrome (STL) with matched controls. METHODS:Patients with genetically confirmed STL with axial length ≥ 26 mm and controls matched for axial length were included. The following data were obtained from SD-OCT scans and fundus photography: choroidal and retinal thickness (respectively, CT and RT), peripapillary atrophy area (PAA), presence of posterior staphyloma (PS). RESULTS: Twenty-six eyes of 17 patients with STL and 25 eyes of 19 controls were evaluated. Compared with controls, patients with STL showed a greater CT subfoveally, at 1000 μm from the fovea at both nasal and temporal location, and at 2000 and 3000 μm from the fovea in nasal location (respectively, 188.7±72.8 vs 126.0±88.7 μm, 172.5±77.7 vs 119.3±80.6 μm, 190.1±71.9 vs 134.9±79.7 μm, 141.3±56.0 vs 98.1±68.5 μm, and 110.9±51.0 vs 67.6±50.7 μm, always P< 0.05). Furthermore, patients with STL showed a lower prevalence of PS (11.5% vs 68%, P< 0.001) and a lower PAA (2.2±2.1 vs 5.4±5.8 mm2, P=0.03), compared with controls. CONCLUSIONS: This study shows that high myopic patients with STL show a greater CT, a lower PAA and a lower prevalence of PS, compared with controls matched for axial length. These findings could be relevant for the development and progression of myopic maculopathy in patients with STL.
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