title: Assessment of estimated retinal atrophy progression in Stargardt macular dystrophy using spectral-domain optical coherence tomography.
abstract: OBJECTIVE
  To estimate disease progression based on analysis of macular volume measured by spectral-domain optical coherence tomography (SD-OCT) in patients affected by Stargardt macular dystrophy (STGD1) and to evaluate the influence of software errors on these measurements.
  
  
  METHODS
  58 eyes of 29 STGD1 patients were included. Numbers and types of algorithm errors were recorded and manually corrected. In a subgroup of 36 eyes of 18 patients with at least two examinations over time, total macular volume (TMV) and volumes of all nine Early Treatment of Diabetic Retinopathy Study (ETDRS) subfields were obtained. Random effects models were used to estimate the rate of change per year for the population, and empirical Bayes slopes were used to estimate yearly decline in TMV for individual eyes.
  
  
  RESULTS
  6958 single B-scans from 190 macular cube scans were analysed. 2360 (33.9%) showed algorithm errors. Mean observation period for follow-up data was 15 months (range 3-40). The median (IQR) change in TMV using the empirical Bayes estimates for the individual eyes was -0.103 (-0.145, -0.059) mm3per year. The mean (±SD) TMV was 6.321±1.000 mm3at baseline, and rate of decline was -0.118 mm3per year (p=0.003). Yearly mean volume change was -0.004 mm3in the central subfield (mean baseline=0.128 mm3), -0.032 mm3in the inner (mean baseline=1.484 mm3) and -0.079 mm3in the outer ETDRS subfields (mean baseline=5.206 mm3).
  
  
  CONCLUSIONS
  SD-OCT measurements allow monitoring the decline in retinal volume in STGD1; however, they require significant manual correction of software errors.
authors.affiliation: The Wilmer Eye Institute, Johns Hopkins University, Baltimore, USA.|Department of Ophthalmology, Medical University Graz, Graz, Austria.|Department of Ophthalmology, General Hospital Linz, Linz, Austria.
authors.forename: Rupert W
authors.initials: RW
authors.lastname: Strauss
authors.affiliation: The Wilmer Eye Institute, Johns Hopkins University, Baltimore, USA.
authors.forename: Beatriz
authors.initials: B
authors.lastname: Muñoz
authors.affiliation: The Wilmer Eye Institute, Johns Hopkins University, Baltimore, USA.
authors.forename: Yulia
authors.initials: Y
authors.lastname: Wolfson
authors.affiliation: The Wilmer Eye Institute, Johns Hopkins University, Baltimore, USA.
authors.forename: Raafay
authors.initials: R
authors.lastname: Sophie
authors.affiliation: The Wilmer Eye Institute, Johns Hopkins University, Baltimore, USA.|Department of Ophthalmology, Cheltenham General Hospital, Gloucestershire Hospitals NHS Foundation Trust, Cheltenham, UK.
authors.forename: Emily
authors.initials: E
authors.lastname: Fletcher
authors.affiliation: The Wilmer Eye Institute, Johns Hopkins University, Baltimore, USA.
authors.forename: Millena G
authors.initials: MG
authors.lastname: Bittencourt
authors.affiliation: The Wilmer Eye Institute, Johns Hopkins University, Baltimore, USA.
authors.forename: Hendrik P N
authors.initials: HP
authors.lastname: Scholl
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D018450:Disease Progression; D005260:Female; D006801:Humans; D008266:Macula Lutea / Q000473:pathology*; D008268:Macular Degeneration / Q000151:congenital* / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D015203:Reproducibility of Results; D012189:Retrospective Studies; D000080362:Stargardt Disease; D041623:Tomography, Optical Coherence / Q000379:methods*; D014792:Visual Acuity; D055815:Young Adult
keywords: Dystrophy; Imaging; Macula; Retina