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TitleColour vision deficiency in Stargardt disease
Abstract Nr.P12
PurposeTo investigate whether the colour vision deficiency (CVD) in patients with Stargardt disease (STD) is predominantly in the red-green axis (R/G) as is always described. If present, whether the R/G defect is of the Verriest type 1. And to establish whether there is a correlation between best-corrected visual acuity (BCVA) loss and degree of CVD.
MethodsA retrospective, cross-sectional study of 30 patients (60 eyes) with STD who underwent extensive colour vision (CV) testing using Ishihara, Tritan Plate & AO-HRR pseudo-isochromatic (PIC) plates, Panel D-15 & Desaturated Panel D-15 tests under standardised CIE-light conditions. In addition, 9 patients underwent anomaloscopy. All patients had a clinical diagnosis of STD, which was confirmed by the presence of mutations in ABCA4.
ResultsCV was normal in 10/60 eyes (16,7%) with BCVA between 0.8-1.0. In 50 eyes with BCVA below 0.8 (<0.05 - 0.8), R/G defects were more prominent with decreasing BCVA. Eyes with BCVA <0.1 showed severe R/G defects with additional blue-yellow CVD, pending towards scotopisation. Overall, in these 50 eyes, PIC plates & PD-15 tests showed R/G CVD without predominance of either Verriest type 1 or type II. Anomaloscopy in 9 patients revealed pseudoprotanomaly in 8 and pseudodeuteranomaly in 1.
ConclusionCV is normal in patients with STD and good BCVA. In those with BCVA below 0.8, CVDs are initially and predominantly of the R/G type, with additional B/Y defects manifesting as BCVA declines, eventually leading to scotopisation. A predominance of Verriest type 1 R/G CVD is evident only on anomaloscopy.
Author 1
Last nameUVIJLS
InitialsA
DepartmentDept Ophth, Ghent Univ Hosp
CityGhent
Author 2
Last nameBREUSEGEM
InitialsCM
DepartmentDept Ophth, Ghent Univ Hosp
CityGhent
Author 3
Last nameKESTELYN
InitialsP
DepartmentDept Ophth, Ghent Univ Hosp
CityGhent
Author 4
Last nameDE LAEY
InitialsJJ
DepartmentDept Ophth, Ghent Univ Hosp
CityGhent
Author 5
Last nameDE BAERE
InitialsE
DepartmentDept Ophth, Ghent Univ Hosp / Ctr Med Genet, Ghent Univ Hosp
CityGhent
Author 6
Last nameLEROY
InitialsBP
DepartmentDept Ophth, Ghent Univ Hosp / Ctr Med Genet, Ghent Univ Hosp
CityGhent
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