期刊
RETINA-THE JOURNAL OF RETINAL AND VITREOUS DISEASES
卷 39, 期 3, 页码 570-580出版社
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/IAE.0000000000001965
关键词
adaptive optics; carriers; heterozygotes; imaging; retinitis pigmentosa; tapetal-like reflex
资金
- National Institute for Health Research (NIHR) Biomedical Research Centre at Moorfields Eye Hospital National Health Service Foundation Trust
- National Eye Institute (NEI) of the National Institutes of Health (NIH) [R01EY017607, R01EY025231, P30EY001931, T32GM080202, T32EY014537, U01EY025477]
- Fight For Sight (United Kingdom)
- Moorfields Eye Hospital Special Trustees [R140032A]
- Moorfields Eye Charity [MEC1512B]
- Foundation Fighting Blindness
- Retinitis Pigmentosa Fighting Blindness
- Research to Prevent Blindness (RPB)
- Wellcome Trust [099173/Z/12/Z]
- FFB Career Development Award
- NATIONAL EYE INSTITUTE [R01EY017607, U01EY025477, P30EY001931, T32EY014537, R01EY025231] Funding Source: NIH RePORTER
- NATIONAL INSTITUTE OF GENERAL MEDICAL SCIENCES [T32GM080202] Funding Source: NIH RePORTER
Purpose: To examine the features of the tapetal-like reflex (TLR) in female carriers of RPGR-associated retinopathy by means of adaptive optics scanning light ophthalmoscopy (AOSLO) and spectral domain optical coherence tomography. Methods: Nine molecularly confirmed RPGR carriers and three healthy controls underwent ocular examination and the following retinal imaging modalities: color photography, near-infrared reflectance, fundus autofluorescence, spectral domain optical coherence tomography, and AOSLO. After identifying TLR areas across all imaging modalities, normalized local contrast of outer retinal bands on spectral domain optical coherence tomography was calculated and AOSLO-acquired photoreceptor mosaic analysis was performed. Results: Seven carriers had TLR areas, which colocalized with increased rod photoreceptor reflectivity on confocal AOSLO and reduced cone photoreceptor densities. Parafoveal TLR areas also exhibited reduced local contrast (i.e., increased reflectivity) of the outer retinal bands on spectral domain optical coherence tomography (inner segment ellipsoid zone and outer segment interdigitation zone). Healthy controls did not show TLR. Conclusion: The cellular resolution provided by AOSLO affords the characterization of the photoreceptor mosaic in RPGR carriers with a TLR. Features revealed include reduced cone density, increased cone inner segment diameter, and increased rod outer segment reflectivity.
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