title: STARGARDT DISEASE: Beyond Flecks and Atrophy.
abstract: OBJECTIVE
  To identify changes in the outer retina in areas without atrophy or flecks of Stargardt disease (STGD) using spectral-domain optical coherence tomography.
  
  
  METHODS
  Twenty-three STGD patients and 26 control subjects were assessed for outer retina (from the outer border of Bruch membrane [BrM] to the inner border of the inner segment ellipsoid zone [EZ]), BrM-retinal pigment epithelium apex, the EZ thickness, and apical process interdigitation zone.
  
  
  RESULTS
  Patients with STGD had increased BrM-EZ thickness in areas without apparent disease versus control subjects at 1,000, 1,500, 2,000, and 2,500 μm superior and 1,500 μm, 2,000 μm, and 2,500 μm inferior to the fovea (P < 0.05 to P < 0.001), greatest difference (3.4 μm) at 2,500 μm superiorly. The BrM-retinal pigment epithelium segment showed larger fractional contribution of 0.48 to 0.51 to the overall BrM-EZ thickness compared with 0.35 to 0.42 in control subjects. The thickness of EZ and the interspace between the retinal pigment epithelium apex and EZ were smaller in the STGD patients (P < 0.05 to P < 0.001). Patients with STGD displayed an interrupted interdigitation zone in 16 (84.2%) of 19 eyes versus 6 (23.1%) of 26 eyes of the control subjects (P < 0.001). The BrM-EZ segment of the outer retina of STGD patients lacked the typical normal trilaminar pattern.
  
  
  CONCLUSIONS
  Subtle changes are present within the BrM-EZ segment of the outer retina of STGD patients in areas that are devoid of atrophy and flecks. These findings suggest that pathologic changes in STGD are more widespread than that seen by clinical examination.
authors.affiliation: Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland.
authors.forename: Jacob G
authors.initials: JG
authors.lastname: Light
authors.affiliation: Farabi Eye Institute, Tehran University, Tehran, Iran.
authors.forename: Masoud A
authors.initials: MA
authors.lastname: Fard
authors.affiliation: Farabi Eye Institute, Tehran University, Tehran, Iran.
authors.forename: Mehdi
authors.initials: M
authors.lastname: Yaseri
authors.affiliation: Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland.
authors.forename: Paul
authors.initials: P
authors.lastname: Aiyetan
authors.affiliation: Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland.
authors.forename: James T
authors.initials: JT
authors.lastname: Handa
authors.affiliation: Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland.|Mason Eye Institute, University of Missouri, Columbia, Missouri, Columbia, Missouri.
authors.forename: Katayoon B
authors.initials: KB
authors.lastname: Ebrahimi
mesh_terms: D000293:Adolescent; D000328:Adult; D001284:Atrophy; D016570:Bruch Membrane / Q000473:pathology*; D002648:Child; D018450:Disease Progression; D042241:Early Diagnosis; D005260:Female; D005451:Fluorescein Angiography / Q000379:methods*; D005500:Follow-Up Studies; D005544:Forecasting; D005584:Fovea Centralis / Q000473:pathology; D005654:Fundus Oculi; D006801:Humans; D008268:Macular Degeneration / Q000151:congenital* / Q000175:diagnosis / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D055214:Retinal Photoreceptor Cell Outer Segment / Q000473:pathology*; D012189:Retrospective Studies; D000080362:Stargardt Disease; D041623:Tomography, Optical Coherence / Q000379:methods*; D014792:Visual Acuity; D055815:Young Adult