Clinical and Experimental Ophthalmology最新文献

Background

KCNV2-associated retinopathy is an autosomal recessive inherited retinal disease classically named cone dystrophy with supernormal rod response (CDSRR). This study aims to identify the best biomarker for evaluating the condition.

Methods

A retrospective review of eight patients from seven families with genetically confirmed KCNV2-associated retinopathy was performed. The best corrected visual acuity (BCVA), full-field electroretinogram (ffERG), pattern ERG (pERG), fundus imaging: retinal photograph and fundus autofluorescence (FAF), and optical coherence tomography (OCT) were analysed.

Results

There was a disproportionate increase in b-wave amplitude with a relatively small light intensity increase, especially between the two dimmest stimuli of DA 0.002 and 0.01 (−2.7 and −2.0 log cd.s/m²). The a-wave amplitude was normal. The a-wave peak time was delayed in all stimuli. The b-wave peak time was delayed compared to normal, but the gap tightened as intensity increased. The b:a wave ratio was above or at the upper limit for the reference values. FAF bull's eye maculopathy pattern was prominent and variable foveal disruption on OCT was apparent in all patients. Legal blindness was reached before the age of 25.

Conclusions

We identified three potential electrophysiology biomarkers to assist in evaluating future therapies: the disproportionate b-wave amplitude jump, delayed a-wave and b-wave peak time, and the higher than normal b:a wave ratio. Any of these biomarkers found with photoreceptor ellipsoid zone foveal-perifoveal disruption should prompt consideration for KCNV2 retinopathy. The BCVA natural history data suggests the probable optimum therapeutic window in the first three decades of life.

Background

The aim of this study is to determine the 5-year changes in macular thickness and related factors.

Methods

Data were from the second (2014) and third (2019) phases of the Shahroud Eye Cohort Study. Examinations included measurement of uncorrected and best-corrected visual acuity, non-cycloplegic autorefraction, slit-lamp biomicroscopy, and funduscopy. Participants underwent Cirrus HD-OCT 4000 (Carl Zeiss Meditec, Dublin, CA). imaging.

Results

The 5-year changes (95% confidence interval) of central and overall macular thicknesses were − 3.48 ± 8.16 μ (−3.92, −3.03) and − 0.79 ± 4.06 μ (−1.03, −0.54), respectively.

The median and IQR of 5-year changes in the central subfield thickness were −3 and 10, although they were 0 and 5 in the overall macular thickness, respectively. Multiple regression model showed the central macular thickness (CMT) decreased with a U-shape pattern with increasing age. The 5-year changes in CMT were significantly lower in females compared to males β = −1.55; (−2.78, −0.32) and in smokers compared to non-smokers β = −1.92; (−3.55, −0.28). Moreover, higher body mass index β = −0.12; (−0.22, −0.02) and CMT at baseline β = −0.08; (−0.10, −0.06) were significantly associated with lower CMT changes. The average 5-year changes in overall macular thickness showed a non-linear decrease with age and was significantly higher in females β = 0.93; (0.4, 1.43). These changes were directly related to the anterior chamber depth β = 0.87; (0.10, 1.64) in the baseline.

Conclusions

The macular thickness decreased slightly after 5 years; however, this change is not clinically significant. Demographic factors such as age and sex and refractive errors were significantly related to macular thickness changes.

Background

A rebound in myopia progression following cessation of atropine eyedrops has been reported, yet there is limited data on the effects of stopping 0.01% atropine compared to placebo control. This study tested the hypothesis that there is minimal rebound myopia progression after cessation of 0.01% atropine eyedrops, compared to a placebo.

Methods

Children with myopia (n = 153) were randomised to receive 0.01% atropine eyedrops or a placebo (2:1 ratio) daily at bedtime during the 2-year treatment phase of the study. In the third year (wash-out phase), all participants ceased eyedrop instillation. Participants underwent an eye examination every 6 months, including measurements of spherical equivalent (SphE) after cycloplegia and axial length (AL). Changes in the SphE and AL during the wash-out phase and throughout the 3 years of the study (treatment + wash-out phase) were compared between the treatment and control groups.

Results

During the 1-year wash-out phase, SphE and AL progressed by −0.41D (95% CI = −0.33 to −0.22) and +0.20 mm (95% CI = −0.46 to −0.36) in the treatment group compared to −0.28D (95% CI = 0.11 to 0.16) and +0.13 mm (95% CI = 0.18 to 0.21) in the control group. Progression in the treatment group was significantly faster than in the control group (p = 0.016 for SphE and <0.001 for AL). Over the 3-year study period, the cumulative myopia progression was similar between the atropine and the control groups.

Conclusions

These findings showed evidence of rapid myopia progression following cessation of 0.01% atropine. Further investigations are warranted to ascertain the long-term effects of atropine eyedrops.