Dataset Information

Choroideremia Carriers: Dark-Adapted Perimetry and Retinal Structures.

ABSTRACT:

Purpose

In choroideremia (CHM) carriers, scotopic sensitivity was assessed by dark adapted chromatic perimetry (DACP) and outer retinal structure was evaluated by multimodal imaging.

Methods

Nine carriers (18 eyes) and 13 healthy controls (13 eyes) underwent DACP testing with cyan and red stimuli. Analysis addressed peripapillary (4 test locations closest to the optic disc), macular (52 locations), and peripheral (60 locations outside the macula) regions. Responses were considered to be rod-mediated when cyan relative to red sensitivity was >5 dB. Fundus imaging included spectral domain optical coherence tomography (SD-OCT), short-wavelength (SW-AF), near-infrared (NIR-AF), ultrawide-field (200 degrees) pseudocolor fundus imaging, and quantitative (qAF) fundus autofluorescence.

Results

Detection of the cyan stimulus was rod mediated in essentially all test locations (99.7%). In the macular and peripheral areas, DACP sensitivity values were not significantly different from healthy eyes. In the peripapillary area, sensitivities were significantly decreased (P < 0.05). SD-OCT imaging ranged from hyper-reflective lesions and discontinuities of the outer retinal bands to hypertransmission of signal. SW-AF and NIR-AF images presented with peripapillary atrophy in seven patients (14 eyes). Mosaicism was detectable in SW-AF images in seven patients and in NIR-AF images in five patients. Frank hypo-autofluorescence was visible in eight patients with distinct chorioretinopathy in seven patients. The qAF values were below the 95% confidence interval (CI) of healthy age-matched individuals in 12 eyes.

Conclusions

Rod mediated scotopic sensitivity was comparable to that in control eyes in macular and peripheral areas but was decreased in the peripapillary area where changes in retinal structure were also most severe.

SUBMITTER: Parmann R

PROVIDER: S-EPMC9284471 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:PURPOSE:To examine the feasibility and utility of dark-adapted two-color fundus-controlled perimetry (FCP) in patients with cuticular, reticular, and soft drusen, and to compare FCP data to microstructural spectral-domain optical coherence tomography (SD-OCT) data. METHODS:Forty-four eyes (24 eyes of 24 patients with drusen, age 69.4?±?12.6 years; 20 normal eyes of 16 subjects, 61.7?±?12.4 years) underwent duplicate mesopic, dark-adapted cyan and dark-adapted red FCP within 14° of the central retina (total of 12?936 threshold tests) using the Scotopic Macular Integrity Assessment (S-MAIA, CenterVue, Padova, Italy) device. FCP data were registered to SD-OCT data to obtain outer nuclear layer, inner and outer photoreceptor segment, and retinal pigment epithelium drusen complex (RPEDC) thickness data spatially corresponding to the stimulus location and area (0.43°). Structure-function correlations were assessed using mixed-effects models. RESULTS:Mean deviation values for eyes with cuticular, soft, and reticular drusen were similar for mesopic (-2.1, -3.4, and -3.6?dB) and dark-adapted red (-1.4, -2.6, and -3.3?dB) FCP. For the dark-adapted cyan FCP (0.1, -1.9, and -5.0?dB) and for the cyan-red sensitivity difference (+1.0, +0.5, and -2.4?dB), the mean deviation values differed significantly in dependence of the predominant drusen type (one-way ANOVA; p?<?0.05). RPEDC thickness was associated with reduction of mesopic sensitivity (-0.34?dB/10?µm RPEDC thickening; p?<?0.001), dark-adapted cyan sensitivity (-0.11?dB/10?µm RPEDC thickening; p?=?0.003), and dark-adapted red sensitivity (-0.26?dB/10?µm RPEDC thickening; p?<?0.001). CONCLUSIONS:In contrast to mesopic FCP, dark-adapted two-color FCP allowed for meaningful differential testing of rod and cone function in patients with drusen indicating predominant cone dysfunction in eyes with cuticular drusen and predominant rod dysfunction in eyes with reticular drusen. RPEDC thickness was the strongest predictor of the evaluated SD-OCT biomarkers for point-wise sensitivity.