title: CHOROIDAL ALTERATIONS IN ABCA4-RELATED RETINOPATHY.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.affiliation: *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.
authors.forename: Philipp L
authors.initials: PL
authors.lastname: Müller
authors.forename: Rolf
authors.initials: R
authors.lastname: Fimmers
authors.forename: Martin
authors.initials: M
authors.lastname: Gliem
authors.forename: Frank G
authors.initials: FG
authors.lastname: Holz
authors.forename: Peter
authors.initials: P
authors.lastname: Charbel Issa
mesh_terms: D018528:ATP-Binding Cassette Transporters / Q000235:genetics; D000293:Adolescent; D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D002648:Child; D002829:Choroid / Q000473:pathology*; D003430:Cross-Sectional Studies; D004596:Electroretinography; D005260:Female; D006801:Humans; D008875:Middle Aged; D012164:Retinal Diseases / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D055213:Retinal Pigment Epithelium / Q000473:pathology; D012189:Retrospective Studies; D041623:Tomography, Optical Coherence / Q000379:methods; D014792:Visual Acuity / Q000502:physiology; D055815:Young Adult
chemical_list: C056475:ABCA4 protein, human; D018528:ATP-Binding Cassette Transporters