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Eye colour and skin pigmentation as significant factors for refractive outcome and residual accommodation in hypermetropic children: a randomized clinical trial using cyclopentolate 1% and tropicamide 1%

Purpose To compare the refractive outcome and residual accommodation with respect to various degrees of iris and skin pigmentation in hypermetropic children using 2 drops of cyclopentolate 1% (C + C) or 1 drop of cyclopentolate 1% and 1 drop of tropicamide 1% (C + T). Methods Two hundred fifty-one hypermetropic children were classified according to iris and skin pigmentation (light, medium, dark) and received randomized and double-blind C + C or C + T. Refractive error (spherical equivalent, SEQ) was determined using the Retinomax-K + 3. In 204 subjects, residual accommodation (RA) was determined using the PlusoptiX PowerRefractor. Results A linear mixed model with a light-irided and light skin-pigmented reference group receiving C + T (mean SEQ +3.10 +/- 1.87D) indicated significant less hypermetropia in subjects with a dark iris having a medium- and dark-pigmented skin in C + T, -1.02 +/- 0.29 (-1.59/-0.45) and -1.53 +/- 0.30 (-2.10/-0.95); and in subjects having a light-, medium- and dark-pigmented skin in C + C, -0.74 +/- 0.34 (-1.41/-0.06), -1.26 +/- 0.30 (-1.85/-0.66) and -1.84 +/- 0.30 (-2.42/-1.26). Similar findings were present for RA. Our model with a light-irided and light skin-pigmented reference group receiving C + T (mean RA +0.84 +/- 0.61D) indicated significantly higher RA in dark-irided subjects with medium- and dark-pigmented skin in C + T, +1.05 +/- 0.19 (+0.67/+1.43) and +1.35 +/- 0.20 (+0.9/+1.74), and in C + C, +1.13 +/- 0.21 (+0.71/+1.55) and +1.90 +/- 0.19 (+1.51/+2.28). Conclusions We found solid evidence that skin pigmentation rather than iris pigmentation is the decisive factor for effectiveness of cycloplegics. Awareness of the limitations of cycloplegic regimens in dark-irided/pigmented children is needed. Our study showed that cyclopentolate 1% combined with tropicamide 1% provides more accurate refractive outcomes both statistically and clinically integrating the factor skin pigmentation for dark-irided subjects.Ophthalmic researc

Evident hypopigmentation without other ocular deficits in Dutch patients with oculocutaneous albinism type 4

To describe the phenotype of Dutch patients with oculocutaneous albinism type 4 (OCA4), we collected data on pigmentation (skin, hair, and eyes), visual acuity (VA), nystagmus, foveal hypoplasia, chiasmal misrouting, and molecular analyses of nine Dutch OCA4 patients from the Bartimeus Diagnostic Center for complex visual disorders. All patients had severely reduced pigmentation of skin, hair, and eyes with iris transillumination over 360 degrees. Three unrelated OCA4 patients had normal VA, no nystagmus, no foveal hypoplasia, and no misrouting of the visual pathways. Six patients had poor visual acuity (0.6 to 1.0 logMAR), nystagmus, severe foveal hypoplasia and misrouting. We found two novel variants in the SLC45A2 gene, c.310C > T; (p.Pro104Ser), and c.1368 + 3_1368 + 9del; (p.?). OCA4 patients of this Dutch cohort all had hypopigmentation of skin, hair, and iris translucency. However, patients were either severely affected with regard to visual acuity, foveal hypoplasia, and misrouting, or visually not affected at all. We describe for the first time OCA4 patients with an evident lack of pigmentation, but normal visual acuity, normal foveal development and absence of misrouting. This implies that absence of melanin does not invariably lead to foveal hypoplasia and abnormal routing of the visual pathways.Ophthalmic researc

Visual impairment due to retinopathy of prematurity and concomitant disabilities in the Netherlands

Aim: Determine incidence of visual impairment due to retinopathy of prematurity (ROP) and concomitant dis-abilities between 2009 and 2018 in the Netherlands and compare data to four former similar studies. Secondly, monitor if infants were missed for ROP-screening since the adoption of stricter, risk factor guided criteria (2013).Methods: Retrospective inventory on anonymous data of infants diagnosed with ROP from Dutch visual impairment-institutes. Data including: best corrected visual acuity, ROP-treatment and concomitant disabilities: bronchopulmonary dysplasia, behavioral abnormalities, epilepsy, hearing deficit, developmental delay, cerebral palsy and cerebral visual impairment. During the study period, lower age limit for neonatal life support (2010) and higher oxygen saturation targets (2014) were implemented.Results: Records of 53 infants were analyzed. Visual impairment incidence due to ROP was 2.02 per 100.000 live births (2000-2009: 1.84, p = 0.643). Compared to earlier periods (1975-2000), a significant decrease was observed. The incidence of concomitant disabilities remained stable. Mean gestational age (GA) continued to decrease to 26.6 & PLUSMN; 1.9 weeks (2000-2009: 27.4 & PLUSMN; 2.0 weeks, p = 0.047). All patients met the screening inclusion criteria.Conclusion: The incidence of visual impairment due to ROP and concomitant disabilities between 2009 and 2018 has not increased, despite lower GA and higher oxygen saturation targets. None of the infants were missed for ROP screening following introduction of more restricted screening inclusion criteria.Ophthalmic researc

Defining inclusion criteria and endpoints for clinical trials: a prospective cross-sectional study in CRB1-associated retinal dystrophies

Purpose: To investigate the retinal structure and function in patients with CRB1-associated retinal dystrophies (RD) and to explore potential clinical endpoints. Methods: In this prospective cross-sectional study, 22 patients with genetically confirmed CRB1-RD (aged 6–74 years), and who had a decimal best-corrected visual acuit

The spectrum of structural and functional abnormalities in female carriers of pathogenic variants in the RPGR gene

PURPOSE. The purpose of this study was to investigate the phenotype and long-term clinical course of female carriers of RPGR mutations. METHODS. This was a retrospective cohort study of 125 heterozygous RPGR mutation carriers from 49 families. RESULTS. Eighty-three heterozygotes were from retinitis pigmentosa (RP) pedigrees, 37 were from cone-/cone-rod dystrophy (COD/CORD) pedigrees, and 5 heterozygotes were from pedigrees with mixed RP/CORD or unknown diagnosis. Mutations were located in exon 1-14 and in ORF15 in 42 of 125 (34%) and 83 of 125 (66%) subjects, respectively. The mean age at the first examination was 34.4 years (range, 2.1 to 86.0 years). The median follow-up time in heterozygotes with longitudinal data (n = 62) was 12.2 years (range, 1.1 to 52.2 years). Retinal pigmentary changes were present in 73 (58%) individuals. Visual symptoms were reported in 51 (40%) cases. Subjects with both symptoms and pigmentary fundus changes were older than the other heterozygotes (P = 0.01) and had thinner foveal outer retinas (P = 0.006). Complete expression of the RP or CORD phenotype was observed in 29 (23%) heterozygotes, although usually in milder forms than in affected male relatives. Best-corrected visual acuity (BCVA) was <20/40 and <20/400 in at least one eye in 45 of 116 (39%) and 11 of 116 (9%) heterozygotes, respectively. Myopia was observed in 74 of 101 (73%) subjects and was associated with lower BCVA (P = 0.006). Increasing age was associated with lower BCVA (P = 0.002) and decreasing visual field size (P = 0.012; I4e isopter). CONCLUSIONS. RPGR mutations lead to a phenotypic spectrum in female carriers, with myopia as a significantly aggravating factor. Complete disease expression is observed in some individuals, who may benefit from future (gene) therapeutic options

Increase in treatment of retinopathy of prematurity in the Netherlands from 2010 to 2017

Purpose: Compare patients treated for Retinopathy of Prematurity (ROP) in two consecutive periods. Methods: Retrospective inventory of anonymized neonatal and ophthalmological data of all patients treated for ROP from 2010 to 2017 in the Netherlands, subdivided in period (P)1: 1-1-2010 to 31-3-2013 and P2: 1-4-2013 to 31-12-2016. Treatment characteristics, adherence to early treatment for ROP (ETROP) criteria, outco

Neutral Density Filters as a Tool for Cycloplegic Plusoptix-Photorefractor Measurements: An Explorative Study

Purpose:The purpose of this study is to investigate the usefulness of neutral-density (ND) filters in cycloplegic-Plusoptix-photorefractor measurements.Methods:No-filter and ND-filter 0.04, 0.1 and 0.2 cycloplegic-Plusoptix-photorefractor measurements were made in 42 hypermetropic eyes. Sphere, cylinder, spherical equivalent (SEQ), J0, and J45 values were compared.Results:Mean Plusoptix-photorefractor pupil sizes were 7.7 +/- 0.68 and 7.7 +/- 0.72 mm The no-filter failure rate was 16%, with 87% in pupils >7.8 mm. Mean no-filter sphere, cylinder, SEQ, J0 and J45 values were +0.34 +/- 0.35D, -0.29 +/- 0.22D, +0.20 +/- 0.36, -0.00 +/- 0.15, and +0.02 +/- 0.11, respectively. Only ND-filter-0.04 provided 5% more successful measurements and a clinically significant alteration in the percentage of values exceeding 0.5D for sphere and SEQ (-10% and -20%), but not for cylinder (+5%). Despite the increased accuracy, 21% of the spherical outcome exceeded 0.50D. Furthermore, the single-measure-intraclass-correlation-coefficient between no-filter and ND-filter-0.04 outcome was moderate (sphere 0.78 (0.62-0.87), cylinder 0.59 (0.35-0.75), SEQ 0.68 (0.48-0.82), J0 0.73 (0.54-0.84) and J45 0.57 (0.50-0.86)) and indicated significant individual variation. Bland-Altman-analyses indicated significant bias for sphere and SEQ; p=0.038 and p=0.030.Conclusion:ND-filter-0.04 resulted in a larger proportion of successful measurements and an increased accuracy. However, an unacceptable percentage of inaccuracy was still present compared to retinoscopy. There could be validity issues with the ND-filter 0.04 or the baseline no-filter readings at the start. We conclude that cycloplegic Plusoptix-photorefraction, even with the use of a 0.04 ND filter, is not a suitable method for exact objective refraction purposes in children.Ophthalmic researc

Considerations for future studies on the effect of late phase oxygen strategies on retinopathy of prematurity

Strategies to ensure high intraocular oxygen delivery to the developing retina after 32 weeks gestational age, such as higher saturation targets and/or higher hemoglobin levels, are hypothesized to prevent ophthalmological treatment for retinopathy of prematurity (ROP). This short report summarizes the current evidence of these strategies, and discusses possibilities of future studies. A large sample size would be required and therefore the feasibility of a future randomized controlled trial is questioned

A randomized clinical trial using atropine, cyclopentolate, and tropicamide to compare refractive outcome in hypermetropic children with a dark iris; skin pigmentation and crying as significant factors for hypermetropic outcome

Ophthalmic researc