Clinical Features and Spectral-Domain Optical Coherence Tomography Findings of Complete Congenital Stationary Night Blindness Patients

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Türkiye Klinikleri Oftalmoloji Dergisi, vol.27, no.4, pp.267-272, 2018 (Peer-Reviewed Journal) identifier


ABSTRACT Objective: To describe clinical findings and spectral domain optical coherence tomography(SD-OCT) features of our complete congenital stationary night blindness (CSNB) patients.Material and Methods: This retrospective study included 12 eyes of six patients diagnosed with completetype CSNB. Patients were evaluated with SD-OCT, fundus autofluorescence (FAF) and electrophysiologicaltests. Segmentation of retinal layers was performed on SD-OCT images of all CSNBpatients and compared with 6 age matched, normal myopic controls. Results: All patients' anteriorsegments findings were normal and none of our patients had nystagmus or strabismus. Pale optic discwas observed in two patients. On FAF imaging eyes with CSNB demonstrated unremarkable fundiincluding normal distribution of autofluorescence. Full-field electroretinography (ERG) demonstratedb-wave to a-wave amplitude ratio of less than one in the combined rod–cone response whichdescribes electronegative ERG. SD-OCT segmentation revealed statistically significant thinning of thetotal retina, retinal nerve fiber layer (RNFL), inner plexiform layer (IPL) and inner retinal thicknessesin the CSNB group compared to control group (respectively p=0.015, p=0.011, p=0.017 andp=0.021). In the other layers of retina, we observed thinning in the CSNB group, but this differencewas not statistically significant. Conclusion: In our study we observed selective thinning of RNFL andIPL in our patients. We thought that thinning of the IPL and RNFL and possibly optic disc palenessin complete CSNB patients suggests bipolar cell dysfunction or synaptogenesis defect between bipolarcells and ganglion cells and possibly reduced number of these cells.