Macular spatial distribution of preserved autofluorescence in patients with choroideremia

TY - JOUR

T1 - Macular spatial distribution of preserved autofluorescence in patients with choroideremia

AU - Hariri, Amir H

AU - Ip, Michael S

AU - Girach, Aniz

AU - Lam, Byron L

AU - Fischer, M Dominik

AU - Sankila, Eeva-Marja

AU - Pennesi, Mark Edward

AU - Holz, Frank G

AU - Maclaren, Robert E

AU - Birch, David G

AU - Hoyng, Carel B

AU - MacDonald, Ian M

AU - Black, Graeme C

AU - Tsang, Stephen H

AU - Bressler, Neil M

AU - Stepien, Kimberly E

AU - Larsen, Michael

AU - Gorin, Michael B

AU - Meunier, Isabelle

AU - Webster, Andrew R

AU - Sadda, SriniVas

AU - For Natural History of the Progression of Choroideremia (NIGHT) Study Group

N1 - © Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.

PY - 2019/7/1

Y1 - 2019/7/1

N2 - 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.

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.

U2 - 10.1136/bjophthalmol-2018-312620

DO - 10.1136/bjophthalmol-2018-312620

M3 - Journal article

C2 - 30297337

SN - 0007-1161

VL - 103

SP - 933

EP - 937

JO - British Journal of Ophthalmology

JF - British Journal of Ophthalmology

IS - 7

ER -