PT  - JOURNAL ARTICLE
AU  - Amir H Hariri
AU  - Michael S Ip
AU  - Aniz Girach
AU  - Byron L Lam
AU  - M. Dominik Fischer
AU  - Eeva-Marja Sankila
AU  - Mark Edward Pennesi
AU  - Frank G Holz
AU  - Robert E Maclaren
AU  - David G Birch
AU  - Carel B Hoyng
AU  - Ian M MacDonald
AU  - Graeme C Black
AU  - Stephen H Tsang
AU  - Neil M Bressler
AU  - Kimberly E Stepien
AU  - Michael Larsen
AU  - Michael B Gorin
AU  - Isabelle Meunier
AU  - Andrew R Webster
AU  - SriniVas Sadda
ED  - ,
TI  - Macular spatial distribution of preserved autofluorescence in patients with choroideremia
AID  - 10.1136/bjophthalmol-2018-312620
DP  - 2019 Jul 01
TA  - British Journal of Ophthalmology
PG  - 933--937
VI  - 103
IP  - 7
4099  - http://bjo.bmj.com/content/103/7/933.short
4100  - http://bjo.bmj.com/content/103/7/933.full
SO  - Br J Ophthalmol2019 Jul 01; 103
AB  - 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.