July 2018
Volume 59, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2018
Spatial distribution of preserved autofluorescence in patients with choroideremia
Author Affiliations & Notes
  • Amir H Hariri
    Doheny Eye Inst/UCLA, North Hollywood, California, United States
  • Aniz Girach
    NightStaRx, London, United Kingdom
  • Michael S Ip
    Doheny Eye Inst/UCLA, North Hollywood, California, United States
  • Byron L Lam
    Bascom Palmer Eye Institute, Miami, Florida, United States
  • M Dominik Fischer
    University of Tubingen, Tubingen, Germany
  • Eeva-Marja Kaarina Sankila
    Helsinki University Eye Hospital, Helsinky, Finland
  • Mark E Pennesi
    Casey Eye Institute - OHSU, Astoria, Oregon, United States
  • Frank G. Holz
    University of Bonn, Bonn, Germany
  • David G Birch
    Retina Foundation of the Southwest, Dallas, Texas, United States
  • Robert E MacLaren
    Nuffield Lab of Ophthalmology, London, United Kingdom
  • Ian M MacDonald
    Royal Alexandra Hospital, Edmonton, Alberta, Canada
  • Carel C B Hoyng
    Nijmegen Univ Medical Center, Nijmegen, Netherlands
  • Graeme Black
    Manchester Centre for Genomic Medicine, Manchester, United Kingdom
  • Neil M Bressler
    Wilmer Eye Institute, Baltimore, Maryland, United States
  • Stephen H. Tsang
    Columbia Univ-Harkness Eye Inst , New York, New York, United States
  • Srinivas R. Sadda
    Doheny Eye Inst/UCLA, North Hollywood, California, United States
  • Footnotes
    Commercial Relationships   Amir Hariri, None; Aniz Girach, NightStarX (E); Michael Ip, None; Byron Lam, NightStarX (S); M Dominik Fischer, NightStarX (C); Eeva-Marja Sankila, None; Mark Pennesi, None; Frank Holz, Heidelberg (F), Heidelberg (C), Heidelberg (R), NightStarX (F); David Birch, NightStarX (C), NightStarX (F); Robert MacLaren, NightStarX (F), NightStarX (I), NightStarX (C); Ian MacDonald, None; Carel Hoyng, None; Graeme Black, None; Neil Bressler, None; Stephen Tsang, None; Srinivas Sadda, Heidelberg (C), NightStarX (C)
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science July 2018, Vol.59, 4991. doi:
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    • Get Citation

      Amir H Hariri, Aniz Girach, Michael S Ip, Byron L Lam, M Dominik Fischer, Eeva-Marja Kaarina Sankila, Mark E Pennesi, Frank G. Holz, David G Birch, Robert E MacLaren, Ian M MacDonald, Carel C B Hoyng, Graeme Black, Neil M Bressler, Stephen H. Tsang, Srinivas R. Sadda; Spatial distribution of preserved autofluorescence in patients with choroideremia. Invest. Ophthalmol. Vis. Sci. 2018;59(9):4991.

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      © ARVO (1962-2015); The Authors (2016-present)

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Purpose : To study the spatial distribution of preserved autofluorescence (PAF) in choroideremia (CHM).

Methods : Spectral domain optical coherence tomography (SD-OCT) and blue-light fundus autofluorescence (FAF) images were captured from patients with a confirmed genetic diagnosis of CHM using the Heidelberg Retinal Angiograph or Spectralis. Taking advantage of HEYEX review software, the foveal center was marked on infrared reflectance (IR) images under the guidance of corresponding OCT, followed by application of an ETDRS grid. The IR images were then registered to the FAF images. The boundaries of PAF were manually segmented in each individual ETDRS subfield. To evaluate the regional distribution of PAF, the area of PAF was compared among the various subfields (Figure).

Results : A total of 168 eyes from 84 CHM cases were enrolled. PAF area in the inner nasal subfield was 0.39 ± 0.60 mm2, and was significantly smaller than the PAF area in the other inner subfields (all p values < 0.001). The inner temporal PAF area was 0.81 ± 0.79 mm2, which was significantly larger than the PAF area in the other inner subfields (all p values < 0.05). There was no statistically significant difference in PAF areas between inner inferior and inner superior subfields (0.64 ± 0.71 mm2 and 0.63 ± 0.69 mm2 respectively).
Analogously, in the outer subfields, nasal PAF area was also significantly smaller (0.22 ± 0.56 mm2; all p values < 0.001) than the other outer subfields with no statistically significant differences in PAF areas among other outer subfields.

Conclusions : The asymmetric spatial distribution of PAF in CHM (corresponding to the stellate shaped nature of these regions), suggests that the progression of the degeneration in these cases may be directionally-dependent. This has implications for the design of anatomic endpoints for CHM clinical trials.

This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.



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