title: ULTRAWIDEFIELD AUTOFLUORESENCE IN ABCA4 STARGARDT DISEASE.
abstract: OBJECTIVE
  To report the ultrawidefield fundus autofluorescence (UWF-FAF) patterns in ABC4A Stargardt disease.
  
  
  METHODS
  A retrospective cohort study of patients with a clinical diagnosis of Stargardt disease, confirmed ABCA4 genotype, and ultrawidefield fundus autofluorescence imaging using an Optos P200Tx. Four independent graders evaluated the images. Ultrawidefield fundus autofluorescence images were evaluated for the presence of posterior pole and peripheral findings, and were classified into one of three types (Type I: lesions confined to the macula with no peripheral findings; Type II: macular atrophy with flecks only in the periphery; Type III: macular atrophy and varying degrees of peripheral atrophy).
  
  
  RESULTS
  Ultrawidefield fundus autofluorescence was performed on 58 eyes of 29 patients. Reviews of images revealed the presence of peripheral (outside the 55° view of standard nonwidefield FAF imaging) alterations on UWF-FAF in 76% of eyes. Overall, the UWF-FAF pattern was classified as Type I in 24% eyes (14/58), Type II in 24% (14/58), and Type III in 52% (30/58). The most common genetic mutations were c.2588G>C (6/29 patients, 20.7%), and c.5882G>A (5/29 patients, 17.2%).
  
  
  CONCLUSIONS
  Ultrawidefield fundus autofluorescence reveals peripheral changes in the majority of patients with Stargardt disease. Peripheral FAF changes may have implications for diagnosis, prognosis, and management of individual patients with Stargardt disease.
authors.affiliation: Stein Eye Institute, University of California, Los Angeles, California.
authors.forename: Michael A
authors.initials: MA
authors.lastname: Klufas
authors.affiliation: Stein Eye Institute, University of California, Los Angeles, California.|Doheny Eye Institute, University of California, Los Angeles, California.
authors.forename: Irena
authors.initials: I
authors.lastname: Tsui
authors.affiliation: Doheny Eye Institute, University of California, Los Angeles, California.
authors.forename: SriniVas R
authors.initials: SR
authors.lastname: Sadda
authors.affiliation: Stein Eye Institute, University of California, Los Angeles, California.
authors.forename: Hamid
authors.initials: H
authors.lastname: Hosseini
authors.affiliation: Stein Eye Institute, University of California, Los Angeles, California.
authors.forename: Steven D
authors.initials: SD
authors.lastname: Schwartz
mesh_terms: D018528:ATP-Binding Cassette Transporters / Q000235:genetics*; D000328:Adult; D000368:Aged; D004247:DNA / Q000032:analysis; D004252:DNA Mutational Analysis; D004596:Electroretinography; D005260:Female; D005451:Fluorescein Angiography / Q000379:methods; D005654:Fundus Oculi; D005838:Genotype; D006801:Humans; D008266:Macula Lutea / Q000473:pathology*; D008268:Macular Degeneration / Q000151:congenital* / Q000000981:diagnostic imaging / Q000235:genetics / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009154:Mutation; D009887:Ophthalmoscopy / Q000379:methods; D061848:Optical Imaging / Q000379:methods*; D015203:Reproducibility of Results; D055213:Retinal Pigment Epithelium / Q000473:pathology*; D012189:Retrospective Studies; D000080362:Stargardt Disease; D041623:Tomography, Optical Coherence / Q000379:methods; D014792:Visual Acuity; D055815:Young Adult
chemical_list: C056475:ABCA4 protein, human; D018528:ATP-Binding Cassette Transporters; D004247:DNA