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TitleBest-Corrected Visual Acuity and Foveal Location in Patients with Congenital Stationary Night Blindness
Abstract Nr.1058
PurposeTo evaluate best-corrected visual acuity and foveal location in patients with congenital stationary night blindness (CSNB) with Schubert-Bornschein type electroretinographies (ERG), of the Miyake Complete (cCSNB) and Incomplete (iCSNB) types.
MethodsIn this mixed retro- and prospective study, data on 22 cCSNB and 14 Miyake iCSNB patients were obtained. All available ophthalmological clinical findings, in addition to Goldmann visual field tests, full-field and ON-OFF ERGs, white, infrared and blue light fundus pictures, OCT images and genetic testing results were studied.
ResultsData on 72 eyes of 35 X-linked patients (21 cCSNB & 14 iCSNB) and 1 AR cCSNB patient were included in the study. Mean decimal best-corrected visual acuities were 0,43 ± 0,23 for RE and 0,37 ± 0,24 for LE, mean refractive values were -7,28 ± 4,42D for RE and -7,31 ± 5,06D for the LE. Nineteen patients had nystagmus, 14 patients had either strabismus or had undergone strabismus surgery. Foveal ectopia was observed in 30 eyes (14 cCNSB and 8 iCSNB). Macular staphylomata were seen in 14 eyes, foveal hypoplasia was noted in 4 eyes.
ConclusionBest-corrected visual acuity is significantly lower in CSNB patients. Nearly half of the patients had either foveal ectopia or maldevelopment.
Authors 1
Last nameKARABULUT
InitialsE
DepartmentDept of Ophthalmology Ghent University Hospital; Ophthalmology Clinic,Elmadag State Hospital
CityGhent, Belgium; Ankara, Turkey
Authors 2
Last nameWALRAEDT
InitialsS
DepartmentDept of Ophthalmology Ghent University Hospital
CityGhent, Belgium
Authors 3
Last nameDE ZAEYTIJD
InitialsJ
DepartmentDept of Ophthalmology Ghent University Hospital
CityGhent, Belgium
Authors 4
Last nameDELBEKE
InitialsP
DepartmentDept of Ophthalmology Ghent University Hospital
CityGhent, Belgium
Authors 5
Last nameLEROY
InitialsB.P
DepartmentDept of Ophthalmology & Ctr for Medical Genetics, Ghent University Hospital;Div of Ophthalmology &
CityGhent, Belgium;
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