Purchase this article with an account.
Markus Groppe, Charles Cottriall, Susan Downes, Robert MacLaren; Correlation of retinal sensitivity with the area of normal auto-fluorescence in choroideremia patients. Invest. Ophthalmol. Vis. Sci. 2013;54(15):642.
Download citation file:
© ARVO (1962-2015); The Authors (2016-present)
To investigate whether patients with choroideremia have defects in retinal sensitivity prior to the onset of degeneration and how function correlates to fundus autofluorescence (FAF).
Thirty-eight patients (72 eyes) with choroideremia underwent a complete ophthalmologic examination, including best-corrected visual acuity, microperimetry (Maia system), FAF and spectral-domain optical coherence tomography (Heidelberg Spectralis). The residual area of functioning retina was determined using FAF. The mean retinal sensitivities within the central 2 degrees of visual field were examined.
The area of autofluorescent retina ranged from 0.13 mm2 to 62 mm2 and correlated highly with the central retinal sensitivity (range 1-30 db) and age of the patient (13-63 years) (P <0.01). Central retinal sensitivity was reduced in all patients compared to controls, including those with normal visual acuity.
Choroideremia is a progressive retinal and choroidal degeneration. The size of the area of surviving retinal tissue determined by FAF is a biomarker for disease progression. There is a functional deficit in retinal sensitivity in these patients which exists prior to the onset of degeneration and might therefore be partly reversible following successful gene replacement therapy.
This PDF is available to Subscribers Only