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Macular spatial distribution of preserved autofluorescence in patients with choroideremia

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  • Amir H Hariri
  • Michael S Ip
  • Aniz Girach
  • Byron L Lam
  • M Dominik Fischer
  • Eeva-Marja Sankila
  • Mark Edward 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 Webster
  • SriniVas Sadda
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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.

OriginalsprogEngelsk
Artikelnummer1468-2079
TidsskriftThe British journal of ophthalmology
Vol/bind103
Udgave nummer7
Sider (fra-til)933-937
Antal sider4
ISSN0007-1161
DOI
StatusUdgivet - jul. 2019

ID: 56583982