Structural assessment of hyperautofluorescent ring in patients with retinitis pigmentosa.
OBJECTIVE
To analyze the retinal structure underlying the hyperautofluorescent ring visible on fundus autofluorescence in patients with retinitis pigmentosa.


METHODS
Twenty-four eyes of 13 patients with retinitis pigmentosa, aged 13 years to 67 years, were studied. The integrity of the photoreceptor cilia, also known as the inner/outer segment junction of the photoreceptors, the outer nuclear layer, and retinal pigment epithelium, was evaluated outside, across, and inside the ring with spectral-domain optical coherence tomography (OCT).


RESULTS
Inside the foveal area, fundus autofluorescence did not detect abnormalities. Outside the ring, fundus autofluorescence revealed hypoautofluorescence compatible with the photoreceptor/retinal pigment epithelium degeneration. Spectral-domain OCT inside the ring, in the area of normal foveal fundus autofluorescence, revealed an intact retinal structure in all eyes and total retinal thickness values that were within normal limits. Across the ring, inner/outer segment junction disruption was observed and the outer nuclear layer was decreased in thickness in a centrifugal direction in all eyes. Outside the hyperautofluorescent ring, the inner/outer segment junction and the outer nuclear layer appeared to be absent and there were signs of retinal pigment epithelium degeneration.


CONCLUSIONS
Disruption of the inner/outer segment junction and a decrease in outer retinal thickness were found across the central hyperautofluorescent ring seen in retinitis pigmentosa. Outer segment phagocytosis by retinal pigment epithelium is necessary for the formation of an hyperautofluorescent ring.
Lima
Luiz H
LH
Vitreous, Retina, Macula Consultants of New York, New York, USA.
Cella
Wener
W
Greenstein
Vivienne C
VC
Wang
Nan-Kai
NK
Busuioc
Mihai
M
Smith
R Theodore
RT
Yannuzzi
Lawrence A
LA
Tsang
Stephen H
SH
D000293:Adolescent; D000328:Adult; D000368:Aged; D003430:Cross-Sectional Studies; D005260:Female; D005453:Fluorescence; D005654:Fundus Oculi; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012160:Retina / Q000473:pathology / Q000503:physiopathology; D055214:Retinal Photoreceptor Cell Outer Segment / Q000473:pathology; D012174:Retinitis Pigmentosa / Q000473:pathology* / Q000503:physiopathology*; D041623:Tomography, Optical Coherence / Q000379:methods; D055815:Young Adult