Macular spatial distribution of preserved autofluorescence in patients with choroideremia

Amir H. Hariri, Michael S. Ip, Aniz Girach, Byron L. Lam, M. Dominik Fischer, Eeva Marja Sankila, Mark Pennesi, Frank G. Holz, Robert E. Maclaren, David G. Birch, Carel B. Hoyng, Ian M. Macdonald, Graeme C. Black, Stephen H. Tsang, Neil M. Bressler, Kimberly E. Stepien, Michael Larsen, Michael B. Gorin, Isabelle Meunier, Andrew R. WebsterSrinivas Sadda

Research output: Contribution to journalArticle

3 Scopus citations

Abstract

Background/Aims: To better understand the pattern of degeneration progression in cases with choroideremia. Methods: A cohort of genotypically confirmed choroideremia cases who underwent optical coherence tomography (OCT) and fundus autofluorescence (FAF) imaging was studied. Using HEYEX review software, the foveal centre was marked on FAF images under guidance of corresponding OCT images, followed by application of an ETDRS grid. The boundaries of preserved autofluorescence (AF) were manually segmented in each individual ETDRS subfield. The regional distribution of preserved AF was assessed by comparing its area among the various subfields. Results: A total of 168 eyes from 84 choroideremia cases were enrolled. There was a statistically significant difference in the amount of preserved AF area between inner subfields as determined by one-way analysis of variance (F (3,668)=9.997, p<0.001) and also between outer subfields (F (3,668)=8.348, p<0.001). A Tukey posthoc test revealed that the preserved AF area in the nasal subfields in both the inner and outer subfields was significantly smaller compared with analogue subfields. Conclusion: The asymmetric spatial distribution of preserved AF in choroideremia (corresponding to the stellate shaped nature of these regions) suggests that the progression of degeneration has directional preference.

Original languageEnglish (US)
JournalBritish Journal of Ophthalmology
DOIs
Publication statusAccepted/In press - Jan 1 2018

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Keywords

  • choroideremia
  • ellipsoid zone
  • fundus autofluorescence
  • optical coherence tomography
  • preserved autofluorescence
  • retinal pigment epithelium

ASJC Scopus subject areas

  • Ophthalmology
  • Sensory Systems
  • Cellular and Molecular Neuroscience

Cite this

Hariri, A. H., Ip, M. S., Girach, A., Lam, B. L., Fischer, M. D., Sankila, E. M., ... Sadda, S. (Accepted/In press). Macular spatial distribution of preserved autofluorescence in patients with choroideremia. British Journal of Ophthalmology. https://doi.org/10.1136/bjophthalmol-2018-312620