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R. W. S. Chen, J. P. Greenberg, J. C. Hwang, S. H. Tsang; Evaluation of Individuals With Congenital Stationary Night Blindness (CSNB) and Retinitis Pigmentosa (RP) Using Spectral Domain Optical Coherence Tomography and Autofluorescence Imaging. Invest. Ophthalmol. Vis. Sci. 2010;51(13):3273.
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© ARVO (1962-2015); The Authors (2016-present)
To test the hypothesis that evaluation of retinal structure can have diagnostic and prognostic value in congenital stationary night blindness (CSNB) and retinitis pigmentosa (RP).
Ten eyes of five patients with incomplete CSNB (iCSNB) and six eyes of three patients with RP (2 autosomal dominant, 1 autosomal recessive) were evaluated with spectral/Fourier domain optical coherence tomography (SDOCT) and fundus autofluorescence (FAF). Diagnoses of iCSNB and RP were confirmed by electroretinography.
Patients with iCSNB had specific findings in SDOCT and FAF that were distinct from those found in RP. In patients with iCSNB, the photoreceptor IS/OS junction was preserved using SDOCT, while this junction was lost in RP patients. In addition, iCSNB patients had normal FAF images, while the patients with RP demonstrated a ring of increased autofluorescence in the macula.
SDOCT and FAF have distinct features in CSNB and RP and may be helpful in differentiating the two disease entities. RP is distinct from CSNB in that it is progressive and carries with it a worse prognosis; however, the fundus appearance is these two entities may appear similar, especially in early stages. Although ERG testing remains the gold standard for the diagnosis of RP and CSNB, it is often difficult to perform the test in young children in which these diseases first manifest. Because SDOCT and FAF have shorter study acquisition times and are less invasive, the two tests in combination may help to distinguish patients with CSNB from patients with RP when ERG studies are not feasible.
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