Philipp L Müller1, Rolf Fimmers, Martin Gliem, Frank G Holz, Peter Charbel Issa. 1. *Department of Ophthalmology, University of Bonn, Bonn, Germany; †Center for Rare Diseases Bonn (ZSEB), University Hospital of Bonn, Bonn, Germany; and ‡Institute of Medical Biometry, Informatics and Epidemiology, University of Bonn, Bonn, Germany.
Abstract
PURPOSE: To investigate choroidal alterations in ABCA4-related retinopathy. METHODS: Mean choroidal thickness and subfoveal choroidal thickness were measured in the right eyes of 40 patients with ABCA4-related retinopathy using enhanced-depth imaging optical coherence tomography. The right eyes of 65 age-matched healthy subjects were used for comparison. RESULTS: Compared with controls, patients with ABCA4-related retinopathy revealed a reduced subfoveal choroidal thickness ([mean ± SEM] 347 ± 10 μm vs. 302 ± 12 μm; P = 0.006) and mean choroidal thickness (315 ± 9 μm vs. 275 ± 10 μm; P = 0.005). This difference was mainly due to choroidal thinning in eyes with reduced photopic and/or scotopic amplitudes on full-field electroretinography. Atrophy of the retinal pigment epithelium (RPE) was associated with a thinner choroid compared with eyes without RPE atrophy (subfoveal choroidal thickness: 277 ± 17 μm vs. 341 ± 16 μm; mean choroidal thickness: 252 ± 13 μm vs. 313 ± 13 μm; both, P ≤ 0.001), but choroidal thinning was not restricted to the area of RPE atrophy. Choroidal thickness was similar to controls when RPE atrophy and functional loss were limited to the central retina. There was no association between visual acuity and choroidal thickness. CONCLUSION: The results indicate choroidal alterations in widespread ABCA4-related retinopathy, especially when associated with atrophy of the RPE. The absence of focal choroidal thinning in areas of RPE atrophy is suggestive for a diffusible factor from the RPE sustaining the choroidal structure.
PURPOSE: To investigate choroidal alterations in ABCA4-related retinopathy. METHODS: Mean choroidal thickness and subfoveal choroidal thickness were measured in the right eyes of 40 patients with ABCA4-related retinopathy using enhanced-depth imaging optical coherence tomography. The right eyes of 65 age-matched healthy subjects were used for comparison. RESULTS: Compared with controls, patients with ABCA4-related retinopathy revealed a reduced subfoveal choroidal thickness ([mean ± SEM] 347 ± 10 μm vs. 302 ± 12 μm; P = 0.006) and mean choroidal thickness (315 ± 9 μm vs. 275 ± 10 μm; P = 0.005). This difference was mainly due to choroidal thinning in eyes with reduced photopic and/or scotopic amplitudes on full-field electroretinography. Atrophy of the retinal pigment epithelium (RPE) was associated with a thinner choroid compared with eyes without RPE atrophy (subfoveal choroidal thickness: 277 ± 17 μm vs. 341 ± 16 μm; mean choroidal thickness: 252 ± 13 μm vs. 313 ± 13 μm; both, P ≤ 0.001), but choroidal thinning was not restricted to the area of RPE atrophy. Choroidal thickness was similar to controls when RPE atrophy and functional loss were limited to the central retina. There was no association between visual acuity and choroidal thickness. CONCLUSION: The results indicate choroidal alterations in widespread ABCA4-related retinopathy, especially when associated with atrophy of the RPE. The absence of focal choroidal thinning in areas of RPE atrophy is suggestive for a diffusible factor from the RPE sustaining the choroidal structure.
Authors: Philipp L Müller; Johannes Birtel; Philipp Herrmann; Frank G Holz; Peter Charbel Issa; Martin Gliem Journal: Transl Vis Sci Technol Date: 2019-12-20 Impact factor: 3.283
Authors: Philipp L Müller; Alexandru Odainic; Tim Treis; Philipp Herrmann; Adnan Tufail; Frank G Holz; Maximilian Pfau Journal: Sci Rep Date: 2021-01-14 Impact factor: 4.379
Authors: Solmaz Abdolrahimzadeh; Martina Formisano; Mariachiara Di Pippo; Manuel Lodesani; Andrew John Lotery Journal: Int J Mol Sci Date: 2022-07-09 Impact factor: 6.208
Authors: Peter M Maloca; Christian Freichel; Christof Hänsli; Philippe Valmaggia; Philipp L Müller; Sandrine Zweifel; Christine Seeger; Nadja Inglin; Hendrik P N Scholl; Nora Denk Journal: Sci Rep Date: 2022-08-02 Impact factor: 4.996
Authors: Malia M Edwards; Vera L Bonilha; Imran A Bhutto; Brent A Bell; D Scott McLeod; Joe G Hollyfield; Gerard A Lutty Journal: Invest Ophthalmol Vis Sci Date: 2020-07-01 Impact factor: 4.799