Multi-platform imaging in ABCA4-Associated Disease

Sci Rep. 2019 Apr 23;9(1):6436. doi: 10.1038/s41598-019-42772-z.

Abstract

Fundus autofluorescence (FAF) imaging is crucial to the diagnosis and monitoring of recessive Stargardt disease (STGD1). In a retrospective cohort study of 34 patients, we compared FAF imaging platforms varying in field size (30° and 55°: blue/SW-AF and NIR-AF; 200°: ultrawide-field, UWF-AF), excitation wavelength (488 nm, blue/SW-AF; 532 nm, UWF-AF and 787 nm, NIR-AF) and image processing. Due to reduced absorption of 532 nm and 787 nm light by macular pigment, foveal sparing was more readily demonstrable by green/UWF-AF and NIR-AF imaging. Prominent in green/UWF-AF images is a central zone of relatively elevated AF that is continuous inferonasal with a demarcation line bordering lower AF nasally and higher AF temporally. This zone and border are more visible in STGD1 than in healthy eyes and more visible with green/UWF-AF. With the development of AF flecks, inferonasal retina is initially spared. Central atrophic areas were larger in NIR-AF images than in blue/SW-AF and green/UWF-AF images and the presence of a contiguous hyperAF ring varied with imaging modality. Flecks visible as hyperAF foci in blue/SW-AF images were also visible in green/UWF-AF but were often hypoAF in NIR-AF. Since disease in STGD1 often extends beyond the 30° and 55° fields, green/UWF-AF has advantages including for pediatric patients. The imaging platforms examined provided complementary information.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • ATP-Binding Cassette Transporters / deficiency
  • ATP-Binding Cassette Transporters / genetics*
  • Adolescent
  • Adult
  • Aged
  • Child
  • Female
  • Fovea Centralis / diagnostic imaging*
  • Fovea Centralis / metabolism
  • Fovea Centralis / pathology
  • Fundus Oculi
  • Gene Expression
  • Humans
  • Male
  • Middle Aged
  • Optical Imaging / methods*
  • Retinal Pigment Epithelium / diagnostic imaging*
  • Retinal Pigment Epithelium / metabolism
  • Retinal Pigment Epithelium / pathology
  • Retrospective Studies
  • Stargardt Disease / diagnostic imaging*
  • Stargardt Disease / genetics*
  • Stargardt Disease / metabolism
  • Stargardt Disease / pathology

Substances

  • ABCA4 protein, human
  • ATP-Binding Cassette Transporters