Fundus autofluorescence and ellipsoid zone (EZ) line width can be an outcome measurement in RHO-associated autosomal dominant retinitis pigmentosa.


Department of Ophthalmology, Columbia University, New York, NY, USA. [Email]


OBJECTIVE : To evaluate the progression of retinitis pigmentosa (RP) due to mutations in rhodopsin (RHO) by measuring the short-wavelength autofluorescence (SW-AF) increased autofluorescence ring and ellipsoid zone (EZ)-line width.
METHODS : Fundus autofluorescence (FAF) and spectral domain optical coherence tomography (SD-OCT) images were obtained from 10 patients with autosomal dominant RP due to mutations in the RHO gene. Measurements of ring area on FAF images, as well as the EZ line width on SD-OCT images and horizontal, vertical diameter, were performed by two independent masked graders.
RESULTS : The ring area decreased by a rate of 0.6 ± 0.2 mm2 per year. We observed that the EZ line width decreased by an average of 152 ± 37 μm per year, while the horizontal and vertical diameters decreased by 106 ± 35 μm and 125 ± 29 μm per year, respectively. Progression rates were similar between eyes.
CONCLUSIONS : We observed SW-AF ring constriction and a progressive loss of EZ line width over time.


Autosomal dominant,Disease progression,RHO,Retinitis pigmentosa,