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Objective: To investigate whether dietary nutrients can reduce the genetic risk of early age-related macular degeneration (AMD) conferred by the genetic variants CFH Y402H and LOC387715 A69S in a nested case-control study. Methods: For 2167 individuals (>= 55 years) from the population-based Rotterdam Study at risk of AMD, dietary intake was assessed at baseline using a semiquantitative food frequency questionnaire and genetic variants were determined using TaqMan assay. Incident early AMD was determined on fundus photographs at 3 follow-up visits (median follow-up, 8.6 years). The synergy index was used to evaluate biological interaction between risk factors; hazard ratios were calculated to estimate risk of early AMD in strata of nutrient intake and genotypes. Results: Five hundred seventeen participants developed early AMD. Significant synergy indices supported the possibility of biological interaction between CFH Y402H and zinc, beta-carotene, lutein/zeaxanthin, and eicosapentaenoic/docosahexaenoic acid (EPA/DHA) and between LOC387715 A69S and zinc and EPA/DHA (all P < .05). Homozygotes of CFH Y402H with dietary intake of zinc in the highest tertile reduced their hazard ratio of early AMD from 2.25 to 1.27. For intakes of beta-carotene, lutein/zeaxanthin, and EPA/DHA, these risk reductions were from 2.54 to 1.47, 2.63 to 1.72, and 1.97 to 1.30, respectively. Carriers of LOC387715 A69S with the highest intake of zinc and EPA/DHA reduced their risk from 1.70 to 1.17 and 1.59 to 0.95, respectively (all P trends

Objectives: To demonstrate how human-machine intelligence can be integrated for efficient image analysis of drusen in age-related macular degeneration and to validate the method in 2 large, independently graded, population- based data sets. Methods: We studied 358 manually graded color slides from the Netherlands Genetic Isolate Study. All slides were digitized and analyzed with a user-interactive drusen detection algorithm for the presence and quantity of small, intermediate, and large drusen. A graphic user interface was used to preprocess the images, choose a region of interest, select appropriate corrective filters for images with photographic artifacts or prominent choroidal pattern, and perform drusen segmentation. Weighted kappa statistics were used to analyze the initial concordance between human graders and the drusen detection algorithm; discordant grades from 177 left-eye slides were subjected to exhaustive analysis of causes of disagreement and adjudication. To validate our method further, we analyzed a second data set from our Columbia Macular Genetics Study. Results: The graphical user interface decreased the time required to process images in commercial software by 60.0%. After eliminating borderline size disagreements and applying corrective filters for photographic artifacts and choroidal pattern, the weighted kappa values were 0.61, 0.62, and 0.76 for small, intermediate, and large drusen, respectively. Our second data set demonstrated a similarly high concordance. Conclusions: Drusen identification performed by our user-interactive method presented fair to good agreement with human graders after filters for common sources of error were applied. This approach exploits a synergistic relationship between the intelligent user and machine computational power, enabling fast and accurate quantitative retinal image analysis.

To evaluate the quality of vision (visual acuity and straylight) in patients with Fuchs dystrophy and the improvement in visual quality after Descemet stripping endothelial keratoplasty (DSEK). There was an observational case series (Amsterdam group) and a prospective interventional case series (Mayo group). Corrected distance visual acuity (CDVA), straylight, and corneal thickness were measured in patients with phakic and pseudophakic eyes with Fuchs dystrophy recruited at the Academic Medical Center, Amsterdam, the Netherlands (99 eyes), and at Mayo Clinic, Rochester, Minnesota (48 eyes). The Mayo group was also examined at 1, 3, 6, and 12 months after DSEK; all these eyes were rendered pseudophakic during DSEK. Eyes with Fuchs dystrophy had decreased CDVA (mean [SD], 0.42 [0.26] logMAR; Snellen equivalent 20/53) and increased straylight (mean [SD], 1.54 [0.24] logarithm of the straylight parameter) compared with normal eyes. Younger patients were affected more by increased straylight than by decreased CDVA. Corrected distance visual acuity (r = 0.26; P = .003; n = 135) and straylight (r = 0.26; P = .003; n = 133) were correlated with corneal thickness. Corrected distance visual acuity and straylight improved at all postoperative examinations (P < .001), and improvement in straylight from before DSEK to 12 months after DSEK correlated with recipient age (r = -0.43; P = .01; n = 33). Improvement in straylight was more predictable than that of CDVA and was associated with preoperative straylight more than 1.33 logarithm of the straylight parameter. Quality of vision is severely impaired in patients with Fuchs dystrophy and improves significantly after DSEK. Straylight improves more in younger than in older eyes after DSEK. Preoperative straylight can be a useful clinical metric to predict postoperative improvement, especially in cases where preoperative visual acuity is close to 20/20

Objective: To compare prospectively 2 perimetric progression detection algorithms for glaucoma, the Early Manifest Glaucoma Trial algorithm (glaucoma progression analysis [GPA]) and a nonparametric algorithm applied to the mean deviation (MD) (nonparametric progression analysis [NPA]). Methods: Patients with a reproducible glaucomatous visual field defect at baseline in at least 1 eye were followed up prospectively using perimetry (Humphrey field analyzer 30-2 Swedish interactive thresholding algorithm). Classifications by GPA and by NPA at the end of the follow-up period were compared. Results: Two hundred twenty-one patients met the inclusion criteria; 1 eye per patient was analyzed. On average, 7.1 reliable fields were available after a mean follow- up period of 5.3 years. The mean MD at baseline was -9.4 dB; the mean MD slope during the follow-up period was -0.25 dB/y. Fifty-six eyes showed progression by GPA and 89 eyes by NPA ( P

Objective: To describe the phenotypes of 5 patients with NR2E3 mutations. Methods: Two patients with familial and 3 with sporadic early-onset nyctalopia and retinal pigment abnormalities were screened for mutations in the NR2E3 gene (OMIM 604485). The clinical course, fundus features, visual field test results, and fluorescein angiographic and electrophysiologic findings were compared. Results: Three different mutations in NR2E3 were identified: R311Q and 2 novel mutations-missense change Q350R and an in-frame deletion of phenylalanine at position 71 (delF71) in exon 2. Three patients who were homozygous for R311Q had posterior subcapsular cataracts and a concentric ring of round pigment clumps. Electroretinograms were extinguished. A fourth patient, a 24-year-old man who was heterozygotic for R311Q and Q350R, had Goldmann-Favre syndrome. A fifth patient, a 10-year-old boy with heterozygotic mutations R311Q and delF71, had diminished foveal reflexes and subtle pigmentary changes, perhaps a forme fruste of Goldmann-Favre syndrome. Both of these patients had an identical spectral electroretinographic pattern characteristic of enhanced S-cone syndrome. Conclusions: Molecular genetic testing is essential for establishing the correct diagnosis in patients with NR2E3 mutations because of the variable phenotype associated with these degenerations. Two novel NR2E3 mutations are described that are associated with Goldmann-Favre syndrome and enhanced S-cone syndrome.

Objective: To evaluate the preliminary visual results of femtosecond laser-assisted descemet stripping endothelial keratoplasty (FS-DSEK). keratoplasty (FS-DSEK). Methods: We prospectively analyzed results of 20 consecutive patients with Fuchs endothelial dystrophy or aphakic/pseudophakic bullous keratopathy who underwent FS-DSEK. Best spectacle-corrected visual acuity (BSCVA), refraction, corneal topography, and endothelial cell density were measured preoperatively and 3 and 0 months after FS-DSEK. Conical thickness was measured using all optical coherence tomography technique. Results: The average BSCVA of 11 eyes with normal visual potential significantly improved from 20/110 +/- 4 lines to 20/57 +/- 1 line at 6 months (P Conclusions: Femtosecond laser-assisted Descemet stripping endothelial keratoplasty was effective in treating endothelial failure with minimal induced refractive astigmatism, limited improvement of BSCVA, and induction of a hyperopic shift. Endothelial cell count and dislocation rate were significant, which may be related to the surgical technique.

Objective To evaluate the safety, efficacy, predictability, and long-term stability of the Artisan Phakic Intraocular Lens (Ophtec BV, Groningen, the Netherlands) for the correction of high myopia. Methods Prospective analysis of 26 eyes from 15 patients who underwent placement of the Artisan lens for the correction of high and extreme myopia. The mean (SD) preoperative spherical equivalent was − 12.30 (2.69) diopters (D) (range, − 17.25 to − 8.25 D). Results At 5 years, the mean (SD) manifest refraction was − 0.37 (0.69) D, with 95% of eyes within 1 D of attempted correction and 74% of eyes within 0.5 D of the attempted correction. Ninety-five percent of eyes achieved an uncorrected visual acuity of 20/40 or better and 74% achieved an uncorrected visual acuity of 20/20 or better. No eyes experienced a loss of 1 or more lines of best-corrected visual acuity. Sixteen percent of eyes gained 2 or more lines of best-corrected visual acuity. From preoperative measurements, the mean endothelial cell density decreased by 14.05% at 5 years postoperatively. Conclusions Placement of the Artisan lens is predictable, stable, and effective at reducing high and extreme myopia 5 years after implantation. The rate of endothelial cell loss was significantly higher than has been reported in previous studies.

Objective: To investigate whether polymorphisms in the estrogen receptor alpha (ESR1) and beta (ESR2) genes were a risk factor for open-angle glaucoma (OAG). Methods: Participants 55 years and older from the population-based Rotterdam Study underwent, at baseline and at follow-up, the same ophthalmic examination, including visual field screening and stereo optic disc photography. A diagnosis of OAG was based on an algorithm using optic disc measures and visual field loss. Haplotypes of the ESR1 and ESR2 genes were determined. Results: We diagnosed incident OAG in 87 of 3842 participants (2.3%) at risk after a mean follow-up of 6.5 years. We could not detect any association with ESR1 haplotypes. Haplotype 1 of ESR2 showed a 3.6-fold (95% confidence interval, 1.4-9.2) higher risk of incident OAG in men. In women, no association was found between ESR2 and incident OAG. Conclusion: Polymorphisms in the ESR1 gene are unrelated to OAG, but ESR2 polymorphisms seem to lead to increased risk of OAG in men.

Contains fulltext : 71383.pdf (Publisher’s version ) (Closed access) OBJECTIVE: To evaluate the preliminary visual results of femtosecond laser-assisted Descemet stripping endothelial keratoplasty (FS-DSEK). METHODS: We prospectively analyzed results of 20 consecutive patients with Fuchs endothelial dystrophy or aphakic/pseudophakic bullous keratopathy who underwent FS-DSEK. Best spectacle-corrected visual acuity (BSCVA), refraction, corneal topography, and endothelial cell density were measured preoperatively and 3 and 6 months after FS-DSEK. Corneal thickness was measured using an optical coherence tomography technique. RESULTS: The average BSCVA of 11 eyes with normal visual potential significantly improved from 20/110 +/- 4 lines to 20/57 +/- 1 line at 6 months (P < .007). At 6 months, the mean (SD) hyperopic shift was 2.24 (2.3) diopters (D). Preoperative and 6 months postoperative refractive astigmatism were -0.75 (0.9) D and -1.58 (1.1) D (P = .01), but the topographic astigmatism did not change postoperatively (P = .95). Mean (SD) endothelial cell density at 6 months was 1368 (425) cells/mm(2). There was a persistent deswelling of the graft up to 3 months postoperatively. Complications included graft dislocations requiring repositioning (20%), pupillary block glaucoma (5%), epithelial ingrowth (5%), and primary graft failure (5%). CONCLUSIONS: Femtosecond laser-assisted Descemet stripping endothelial keratoplasty was effective in treating endothelial failure with minimal induced refractive astigmatism, limited improvement of BSCVA, and induction of a hyperopic shift. Endothelial cell count and dislocation rate were significant, which may be related to the surgical technique.

Objective: To investigate whether polymorphisms in the estrogen receptor alpha (ESR1) and beta (ESR2) genes were a risk factor for open-angle glaucoma (OAG). Methods: Participants 55 years and older from the population-based Rotterdam Study underwent, at baseline and at follow-up, the same ophthalmic examination, including visual field screening and stereo optic disc photography. A diagnosis of OAG was based on an algorithm using optic disc measures and visual field loss. Haplotypes of the ESR1 and ESR2 genes were determined. Results: We diagnosed incident OAG in 87 of 3842 participants (2.3%) at risk after a mean follow-up of 6.5 years. We could not detect any association with ESR1 haplotypes. Haplotype 1 of ESR2 showed a 3.6-fold (95% confidence interval, 1.4-9.2) higher risk of incident OAG in men. In women, no association was found between ESR2 and incident OAG. Conclusion: Polymorphisms in the ESR1 gene are unrelated to OAG, but ESR2 polymorphisms seem to lead to increased risk of OAG in men.

OBJECTIVE: To estimate the prevalence of age-related maculopathy in an older population from 7 European countries. METHODS: Randomly sampled people 65 years and older were invited to an eye examination in centers across 7 European countries (Norway, Estonia, United Kingdom, France, Italy, Greece, and Spain). Fundus images of each eye were graded at a single reading center. Prevalence rates were calculated for stage of age-related maculopathy with 95% confidence intervals (CIs) estimated for clustered data. RESULTS: Of 5040 participants (45% response rate), 4753 (2128 men and 2625 women) had gradable fundus images. The prevalences were grade 0, 47.59% (95% CI, 43.53%-51.65%); grade 1, 36.48% (95% CI, 32.66%-40.30%); grade 2, 10.14% (95% CI, 8.92% to 11.37%); grade 3, 2.46% (95% CI, 1.79%-3.13%); and grade 4 (age-related macular degeneration [AMD]), 3.32% (95% CI, 2.52%-4.13%) and large drusen only (> or = 125 microm), 15.41% (95% CI, 13.61%-17.21%). The prevalence of geographic atrophic AMD was 1.2% (95% CI, 0.8%-1.7%) and of neovascular AMD, 2.3% (95% CI, 1.7%-2.9%). The prevalence of bilateral AMD was 1.4% (95% CI, 1.0%-1.8%). CONCLUSION: Age-specific prevalences of age-related maculopathy in the European Eye Study (EUREYE) are similar to other population-based studies in Western populations.

Item does not contain fulltext OBJECTIVE: To describe the clinical and genetic findings in a family with a peculiar autosomal dominant macular dystrophy with peripheral deposits. METHODS: All family members underwent an ophthalmic examination, and their genomic DNA was screened for mutations in the human retinal degeneration slow (peripherin/RDS) and rhodopsin genes. In selected cases, fluorescein angiography and electrophysiologic testing were performed. RESULTS: The age at onset of the disease was between the third and fourth decades of life, starting with mild visual acuity loss and periods of metamorphopsia. Clinical signs included subretinal yellowish macular deposits evolving into geographic atrophy and retinal hypopigmentation and hyperpigmentation. Electroretinography demonstrated rod dysfunction, and electro-oculograms were mildly to severely disturbed. All affected members were found to carry a 3-base pair deletion affecting codon 169 of the peripherin/RDS gene. This mutation resulted in an asparagine (Asn) deletion in the peripherin/RDS protein and was not found in 155 control individuals. CONCLUSION: A deletion of Asn169 in the peripherin/RDS protein causes a peculiar form of autosomal dominant macular dystrophy in a large family from the Netherlands. CLINICAL RELEVANCE: Characterizing the phenotype and genotype in this family may, in the long term, result in a better understanding of the precise mechanism underlying this retinal degeneration.

To evaluate the natural course of age-related maculopathy (ARM) and to assess the incidence and absolute risk of its final stage, age-related macular degeneration (AMD). In a population-based prospective cohort study of 6418 persons 55 years and older, we studied the incidence and natural course of ARM. Subjects underwent identical examinations, including stereoscopic fundus photography, at baseline and at 2.0 and 6(1/2) years' follow-up. Age-related maculopathy was graded according to the International Classification and Grading System for ARM and AMD, and stratified into 5 exclusive stages. Incidence was expressed in rates and 5-year absolute risks. At follow-up, 47 new cases of AMD were identified, with a ratio of neovascular-atrophic AMD of 1.4:1. The 5-year risk of AMD increased with more severe stages to 28.0% for subjects 55 years and older with indistinct drusen and pigmentary irregularities (stage 3). Age, but not sex, independently increased this risk to a maximum of 42.0% for subjects with stage 3 ARM who were 80 years and older. Individual ARM fundus signs that predicted best the development of AMD were 10 or more large drusen (> or =125 microm) and 10% or more of the grid area covered by drusen. Subjects who developed atrophic AMD showed no significant (P =.25) differences in baseline fundus signs and natural course compared with subjects who developed neovascular AMD. We provided the absolute risk of AMD as a function of age and early ARM fundus signs, and showed that both are prominent independent risk factors. The progression of ARM stages follows, after the appearance of the first soft drusen, a distinct course at a gradual pace that accelerates with increasing age

Objectives: To investigate the effect of visual field defects on driving performance, and to predict practical fitness to drive. Methods: The driving performance of 87 subjects with visual field defects due to ocular abnormalities was assessed on a driving simulator and during an on-road driving test. Outcome Measures: The final score on the on-road driving test and simulator indexes, such as driving speed, viewing behavior, lateral position, time-headway, and time to collision. Results: Subjects with visual field defects showed differential performance on measures of driving speed, steering stability, lateral position, time to collision, and time-headway. Effective compensation consisted of reduced driving speed in cases of central visual field defects and increased scanning in cases of peripheral visual field defects. The sensitivity and specificity of models based on vision, visual attention, and compensatory viewing efficiency were increased when the distance at which the subject started to scan was taken into account. Conclusions: Subjects with visual field defects demonstrated differential performance on several driving simulator indexes. Driving examiners considered reduced speed and increased scanning to be valid compensation for central and peripheral visual field defects, respectively. Predicting practical fitness to drive was improved by taking driving simulator indexes into account.

Objective To investigate whether nutritional factors and possible risk factors for cataract influence the lens optical density (LOD). Design Three hundred seventy-six subjects, aged 18 to 75 years, were recruited. In a cross-sectional design, serum was analyzed for lutein, zeaxanthin, vitamin C, -tocopherol, and cholesterol levels. Adipose tissue (n = 187) was analyzed for lutein level. The LOD and the macular pigment optical density (MPOD) were measured by spectral fundus reflectance. Results The mean ± SD LOD at 420 nm was 0.52 ± 0.17. It showed a significant association with age ( = .008, P

Objective To study the prevalence and causes of blindness and visual impairment in various age categories of a large population-based study. Methods For the study, 6775 subjects aged 55 years or older underwent an extensive ophthalmologic screening examination, including measurements of visual acuity and the visual field and fundus photography. The causes of blindness or visual impairment were determined using all screening information and medical records. Results The prevalence of blindness, according to World Health Organization criteria, ranged from 0.1% in subjects aged 55 to 64 years to 3.9% in subjects aged 85 years or older; the prevalence of visual impairment ranged from 0.1% to 11.8%. For persons younger than 75 years, myopic degeneration and optic neuropathy were the most important causes of impaired vision. For persons aged 75 years or older, age-related macular degeneration was the major cause of the increased prevalence of blindness, whereas age-related cataract predominantly caused the increased prevalence of visual impairment. Conclusions The hierarchy of causes of blindness and visual impairment is highly determined by age. As yet, little can be done to reduce the exponential increase of blindness; however, adequate implementation of surgery to treat cataract could reduce visual impairment by one third. Underuse of ophthalmologic care is a prominent cause of the high frequency of untreated cataracts among the elderly.

Background: The theoretical benefits of synthetic keratophakia over conventional corneal lamellar procedures are the elimination of donor concerns and superior refractive predictability Additionally, synthetic material can be inspected for optical quality and power, and it can be sterilized. Furthermore, visual recovery should be more rapid since epithelium is not removed from the central part of the cornea and the need for keratocyte repopulation is eliminated. Objective: To present results on patients who received an intracorneal implant (Kerato-Gel, Allergan Medical Optics, Irvine, Calif) that was made from lidofilcon A, a glucose-permeable hydrogel with an equilibrium water content of 68%. Methods: The intracorneal implants were implanted in 35 adult patients for correction of aphakia. Inclusion criteria excluded patients with aphakia who were candidates for intraocular lenses. Results: A total of 19 patients were followed up through 2 years postoperatively. For 16 patients with 2-year postoperative refractive data, the average spherical equivalent was -0.63 +/- 2.07 diopters (D). At 2 years, 88% of patients were within +/-3.00 D of plano and 50% were within +/-1.00 D. The mean change in Snellen's line for corrected visual acuity was -3.25 lines at 2 years for an patients and -2.0 lines for a subgroup of five patients who were free of vision-limiting preoperative disease. Conclusions: Results suggest that this intracorneal implant is well tolerated by the cornea and can provide predictable refractive results in patients with high-risk aphakia. Limitations of the procedure are uneven microkeratome resections, loss of best-corrected visual acuity, and irregular astigmatism in some patients. Although these data show good evidence of biocompatibility of the implant material, technical surgical progress is needed to advance this procedure into clinical therapeutic practice.

To determine the prevalence of chorioretinal lesions and optic disc edema after heart transplantation and to study potential associations.One hundred one patients who had undergone heart transplantation at one institution and 19 patients prior to heart transplantation underwent ophthalmological examination, including fundus photography. The prevalence of fundus lesions was then compared between the two groups.With a standardized protocol, the presence of optic disc edema, chorioretinal hyperpigmentation and depigmentation, retinal hemorrhages, cotton-wool lesions, and arteriovenous nicking was graded on color transparencies.The prevalence of optic disc edema and hypopigmentations was significantly higher among the transplant recipients than among the patients prior to heart transplantation (31% vs 5%, P = .01, and 55% vs 11%, P.001, respectively). Hyperpigmentation was only present in patients after transplantation (15% vs 0%, P = .06). Heart transplant recipients showed an increased risk of hyperpigmentations after 2.5 years. Acute rejection episodes were not associated with posterior pole lesions.Common posterior pole lesions after heart transplantation are optic disc edema and pigmentary changes. Although visual acuity does not seem severely impaired, further longitudinal study is necessary to evaluate the long-term significance of these lesions.

To present and evaluate a new combined index of structure and function (CSFI) for staging and detecting glaucomatous damage.Observational study including 333 glaucomatous eyes (295 with perimetric glaucoma and 38 with preperimetric glaucoma) and 330 eyes of healthy subjects. All the eyes were tested with standard automated perimetry and spectral domain optical coherence tomography within 6 months. Estimates of the number of retinal ganglion cells (RGCs) were obtained from standardautomated perimetry and spectral domain optical coherence tomography and a weighted averaging scheme was used to obtain a final estimate of the number of RGCs for each eye. The CSFI was calculated as the percent loss of RGCs obtained by subtracting estimated from expected RGC numbers. The performance of the CSFI for discriminating glaucoma from normal eyes and the different stages of disease was evaluated by receiver operating characteristic curves.The mean CSFI, representing the mean estimated percent loss of RGCs, was 41% and 17% in the perimetric and preperimetric groups, respectively (P.001). They were both significantly higher than the mean CSFI in the healthy group (P.001). The CSFI had larger receiver operating characteristic curve areas than isolated indexes of structure and function for detecting perimetric and preperimetric glaucoma and differentiating among early, moderate, and advanced stages of visual field loss.An index combining structure and function performed better than isolated structural and functional measures for detection of perimetric and preperimetric glaucoma as well as for discriminating different stages of the disease.

Objective: To examine peripapillary choroidal thickness in healthy controls and in patients with glaucoma who have focal, diffuse, and sclerotic optic disc damage. Methods: Healthy controls (n=92) and patients with glaucoma who have focal (n=34), diffuse (n=35), and sclerotic (n=34) optic disc damage were imaged with spectral-domain optical coherence tomography (12 circular scan protocol centered on optic nerve head). Peripapillary choroidal thickness was measured as the distance between the automatically segmented retinal pigment epithelium/Bruch's membrane and the manually outlined interface between the posterior choroid and the anterior border of the sclera in eyes in which the anterior scleral border was visible over more than 85% of the scan circumference. Results: The anterior scleral border was visible in 76 controls (83%) and 89 patients (86%). Peripapillary choroidal thickness in healthy controls decreased linearly with age (-11 mu m/decade; P < .001; r(2)=0.16), with a predicted value of 137 mu m at age 70 years (95% prediction interval, 62-212 mu m). While this value was similar in patients with focal and diffuse optic disc damage (126 and 130 mu m, respectively; P=.22 compared with controls), it was approximately 30% lower in patients with sclerotic optic disc damage (96 mu m; P

To determine whether mutations in different Bardet-Biedl syndrome (BBS) genes result in different ocular phenotypes.Thirty-seven patients from 31 families were enrolled who met the clinical criteria for BBS and for whom a BBS mutation had been identified. Seventeen patients harbored mutations in BBS1, 10 in BBS10, and 10 in other genes (BBS2, BBS3, BBS5, BBS7, and BBS12). All the patients underwent ocular examination; 36 patients had computerized full-field electroretinograms (ERGs).Visual acuity was significantly better in BBS1 patients than in patients with other BBS mutations (P=.01), and a larger proportion of BBS1 patients had good (≥20/50) visual acuity (P=.01). The ERG amplitudes were significantly higher in BBS1 patients than in patients with other BBS mutations in response to 0.5-Hz and 30-Hz flashes (P.001 for both). All the BBS1 patients harbored at least 1 missense mutation compared with only 45% of patients with mutations in other BBS genes (P.001); the rest harbored only null alleles. However, multivariate analysis demonstrated that visual acuity or ERG amplitude did not depend on the type of mutation present (missense or null) when controlling for BBS gene. Prevalences of bone spicule pigmentation and cataract were comparable in BBS subtypes.Patients with BBS1 mutations had a milder phenotype than did patients with mutations in other BBS genes. Clinically, this manifested as significantly better visual acuity and larger ERG amplitudes.These phenotypic differences can help guide genetic testing and genetic counseling for patients with this syndrome.

Objective To describe the associations of visual impairment and major age-related eye diseases with cognitive function in an older Asian population. Methods A population-based, cross-sectional study of 1179 participants aged 60 to 80 years from the Singapore Malay Eye study was conducted. Visual acuity was measured using the logMAR vision chart. Cataract and age-related macular degeneration were graded using the Wisconsin Cataract Grading System and the Wisconsin Age-Related Maculopathy Grading System, respectively. Glaucoma was diagnosed using the International Society Geographical and Epidemiological Ophthalmology criteria. Diabetic retinopathy was graded using the modified Airlie House classification system. Cognitive dysfunction was defined as a locally validated Abbreviated Mental Test using education-based cutoff scores. Results After adjusting for age, sex, education level, income, and type of housing, persons with visual impairment before refractive correction (odds ratio [OR] = 2.59; 95% CI, 1.89-3.56) or after refractive correction (OR = 1.96; 95% CI, 1.27-3.02) and those with visual impairment due to cataract (OR = 2.75; 95% CI, 1.35-5.63) were more likely to have cognitive dysfunction. Only moderate to severe diabetic retinopathy was independently associated with cognitive dysfunction (OR = 5.57; 95% CI, 1.56-19.91) after controlling for concurrent age-related eye diseases. No significant independent associations were observed between cataract, age-related macular degeneration, or glaucoma and cognitive dysfunction. Conclusions Older persons with visual impairment, particularly those with visual impairment due to cataract, were more likely to have cognitive dysfunction. Furthermore, among the major age-related eye diseases, only diabetic retinopathy was associated with cognitive dysfunction.

We describe a new surgical technique for treating traumatic aniridia with aphakia and its results in a small consecutive case series. We attached a 3-piece acrylic intraocular lens through the haptics to a customized silicone iris prosthesis. The combined implant was inserted through a 5-mm incision and fixated with a transscleral suture in the ciliary sulcus using a knotless technique (Z suture). In all patients, the combined implant stayed firmly fixed within the sulcus and showed a stable and centered position without any tilt or torque during follow-up. Thus, managing posttraumatic aniridia with aphakia by means of haptic fixation of a foldable intraocular lens on a custom-tailored iris prosthesis is a promising approach for visual rehabilitation and cosmetic improvement.

To investigate the longitudinal changes in flicker perimetry in patients with age-related macular degeneration (AMD) as the condition progresses from early AMD to geographic atrophy (GA) or choroidal neovascularization (CNV).Patients with AMD and control subjects were recruited from a longitudinal study of retinal function in early AMD consisting of 187 participants. Only those who completed at least 4 consecutive, 6-monthly flicker perimetry tests were selected for this study. Study groups consisted of everyone who went on to develop GA (n = 16) or CNV (n = 5), controls (n = 24), and the high-risk, early- AMD participants whose eyes did not progress to GA or CNV (drusen125 μm; n = 18). The flicker sensitivity was determined, and its rate of change during the 18 months before the clinical detection of late AMD was calculated.Eyes that went on to develop GA or CNV had a significantly reduced mean (SD) flicker sensitivity in the months before clinical detection of GA (15.8 [5.6] dB) or CNV (19.1 [3.8] dB) compared with control eyes (22.9 [3.0] dB) (P.001) and with eyes that did not progress to GA or CNV (21.4 [3.4] dB) (P.001). The rate of change in flicker sensitivity was significantly increased in GA eyes (-0.07 dB/mo) (P.001) but not in CNV eyes (0.006 dB/mo) (P = .56) compared with the control eyes (-0.003 dB/mo).Flicker sensitivity is reduced in eyes that go on to develop late AMD. The rate of change in flicker sensitivities over time was particularly useful in predicting eyes and areas within the eye that subsequently develop GA.

Objective To determine the levels of vascular endothelial growth factor (VEGF) in both the aqueous humor and Tenon tissue in patients with primary open-angle glaucoma (POAG) and the associations between the VEGF and outcomes of glaucoma surgery. Methods The study involved 19 patients with POAG who were scheduled to undergo glaucoma surgery owing to uncontrolled intraocular pressure (IOP) and 17 control subjects who were scheduled to undergo cataract surgery. At the time of surgery, about 0.1 mL of aqueous humor was collected through an anterior chamber paracentesis and a 4 × 4-mm Tenon tissue sample was cut from the eye. Concentrations of VEGF were analyzed by enzyme-linked immunosorbent assay. Spearman correlation and regression analysis were used to assess the relationship with VEGF level to the clinical characteristics and postoperative IOP. Results The VEGF in Tenon tissue was significantly elevated in patients with POAG compared with control subjects (P = .001). When patients with POAG were divided into success or failure groups 1 year following surgery, the VEGF levels were significantly higher in the Tenon tissue of the failure group compared with the success group (P = .014). The preoperative IOP was significantly related to the VEGF level in Tenon tissue in both the univariate (P = .001) and multivariate (P = .012) regressions. Conclusions The VEGF level in Tenon tissue at the time of surgery was significantly related to 1-year surgical outcomes of glaucoma surgery, and it was significantly associated with the final IOP in patients with POAG.

Optimal management of optic pit–related maculopathy remains to be determined. The fluid source for the maculopathy also remains controversial. In this article, we present a unique surgical technique for internal drainage of the intraretinal fluid and describe the intraoperative use of spectral-domain optical coherence tomography to assist in the surgical management of this condition. Pars plana vitrectomy was performed with elevation of the posterior hyaloid. Following an air-fluid exchange, aspiration over the optic nerve pit was performed. Following aspiration, intraoperative spectral-domain optical coherence tomography demonstrated collapse of the retinoschisis, strongly suggesting a connection between the vitreous cavity and the intraretinal fluid.

Objective To evaluate the interaction of intraocular pressure (IOP)–lowering medications with physiologic day and night changes in aqueous humor dynamics in participants with ocular hypertension. Methods Thirty participants were enrolled in this double-masked, randomized, crossover study. Each participant underwent aqueous humor dynamics measurements at baseline and at 2 weeks of dosing in random order with latanoprost in the evening and placebo in the morning, timolol maleate twice daily, and dorzolamide hydrochloride twice daily. Measurements included central corneal thickness by ultrasound pachymetry, anterior chamber depth by A-scan, seated and habitual IOP by pneumatonometry, blood pressure by sphygmomanometry, episcleral venous pressure by venomanometry, and aqueous flow by fluorophotometry. Outflow facility was assessed by fluorophotometry and by tonography. Uveoscleral outflow was mathematically calculated using the Goldmann equation. Results Latanoprost use significantly decreased IOP during the day and night. It increased daytime uveoscleral outflow by a mean (SD) of 0.90 (1.46) μL/min (P = .048), but a nighttime increase of 0.26 (1.10) μL/min (P = .47) did not reach statistical significance. Timolol use decreased IOP during the day by reducing aqueous flow by 25%. Dorzolamide use lowered IOP only at the noon measurement and reduced daytime aqueous flow by 16%. Neither dorzolamide nor timolol use added to the physiologic 47% reduction in nighttime aqueous flow. Conclusions The daytime IOP-lowering effects of latanoprost are mediated by an increase in uveoscleral outflow, and those of timolol and dorzolamide are mediated by aqueous flow suppression. Nighttime physiologic changes in uveoscleral outflow limit the nighttime pharmacodynamic efficacy of latanoprost. Aqueous flow suppression with timolol and dorzolamide was ineffective in obtaining IOP lowering at night. Trial Registration clinicaltrials.gov Identifier: NCT00572936

Objective To evaluate whether a diet high in long-chain ω-3 fatty acids can slow the rate of visual acuity loss among patients with retinitis pigmentosa receiving vitamin A palmitate. Methods We calculated dietary intake from questionnaires completed annually by 357 adult patients from 3 randomized trials who were all receiving vitamin A, 15 000 IU/d, for 4 to 6 years. Rates of visual acuity decline were compared between those with high (≥0.20 g/d) vs low ( Results Mean rates of decline of acuity were slower among those with high ω-3 intake: Early Treatment Diabetic Retinopathy Study distance acuity: high intake = 0.59 letter per year, low intake = 1.00 letter per year, P = .001; Snellen retinal acuity: high intake = 1.5% per year, low intake = 2.8% per year, P = .03. Conclusions We conclude that mean annual rates of decline in distance and retinal visual acuities in adults with retinitis pigmentosa receiving vitamin A, 15 000 IU/d, are slower over 4 to 6 years among those consuming a diet rich in ω-3 fatty acids. To our knowledge, this is the first report that nutritional intake can modify the rate of decline of visual acuity in retinitis pigmentosa.

Pupillometric Quantification of Residual Rod and Cone Activity in Leber Congenital Amaurosis

Objective To investigate whether the severity of cystoid macular edema (CME) in neonates who were 31 to 36 weeks' postmenstrual age, as viewed by spectral-domain optical coherence tomography (SD-OCT) imaging, predicts the severity of retinopathy of prematurity (ROP) or is related to systemic health. Design Of 62 prematurely born neonates in a prospective institutional review board–approved study, 42 met the following inclusion criteria: at least 1 SD-OCT imaging session prior to 37 weeks' postmenstrual age and prior to ROP laser treatment, if a laser treatment was performed, and an ophthalmic ROP examination at or after 41 weeks' postmenstrual age, evidence of complete retinal vascularization in zone III, or documentation through telephone report of such information after transfer of care. Measures of CME severity, including central foveal thickness, retinal layer thicknesses, and foveal-to-parafoveal thickness ratio in 1 eye per subject, were compared with ROP outcomes: laser treatment, maximum plus disease, and maximum ROP stage. Systemic health factors were also correlated. Results Cystoid macular edema was present in 50% of neonates. Multiple elongated cystoid structures within the inner nuclear layer were most common. The presence of CME was not associated with ROP outcomes. The central foveal thickness, the thickness of the inner retinal layers, and the foveal-to-parafoveal thickness ratio were higher in eyes that required laser treatment or that developed plus disease or ROP stage 3. Cystoid macular edema was not clearly associated with systemic factors. Conclusions Cystoid macular edema is common in premature infants screened for ROP before 37 weeks' postmenstrual age, with the most common SD-OCT phenotype of a bulging fovea from multiple elongated cystoid spaces. Detection of CME is not associated with ROP severity; however, tomographic thickness measurements could potentially predict a higher risk of requiring laser treatment or developing plus disease or ROP stage 3. Systemic health factors are probably not related to the development of CME.

Objective: Children with congenital esotropia, amblyopia, or early visual deprivation have persistent asymmetric monocular pursuit, as measured by optokinetic nystagmus (OKN), and favor targets moving in a temporal to nasal direction. Previous studies suggest that binocular visual development is necessary for the development of symmetric monocular OKN. We recently treated patients with congenital esotropia with unconventionally early surgery to establish good binocularity. We wished to determine if mature symmetric OKN responses could develop in patients with congenital esotropia following the acquisition of good stereopsis. Methods: Electro-oculographic recordings documented horizontal eye movements in response to an electronic OKN stimulus. Patients: The recordings of three patients with congenital esotropia that had been surgically aligned before age 20 weeks and who had achieved high-grade stereopsis with random dot and Titmus stereographic testing were studied. We compared this group of children with groups of age-matched controls, including the following: (1) three patients with congenital esotropia who underwent surgical alignment after age 6 months and who had no amblyopia but poor stereopsis; (2) three children with accommodative esotropia, good ocular alignment when wearing spectacles, and good stereopsis; and (3) three normal children. Results: The normal children and those with accommodative esotropia demonstrated normal symmetrical OKN. The patients with congenital esotropia showed poor nasal to temporal OKN regardless of the degree of stereopsis or timing of surgery. Conclusions: Our results indicate that good binocularity, as measured by stereopsis, is not sufficient for the development of symmetric OKN in patients with congenital esotropia.

Eighteen patients developed an acute corneal decompensation following normal intraocular surgery (cataract extraction in 17 patients), characterized by star-shaped endothelial folds, a twofold increase in corneal thickness, and a visual acuity of counting fingers during several postoperative days. In some cases, there was an additional iritis and transient hypotony. There was no effect of topical and/or subconjunctival corticosteroids on the course of the decompensation. Endothelial morphometric analysis showed a mean endothelial cell loss of 72%. Endothelial wound healing, as determined by coefficient of variation and percentage hexagonals, stabilized 6 months postoperatively. We coined the term toxic endothelial cell destruction for this syndrome. Epidemiological evaluation revealed the toxic endothelial cell destruction syndrome to be linked with the 10-fold increase of a detergent solution in the ultrasonic bath for cleaning the surgical instruments

Twenty-eight paired human corneas were preserved in minimal essential medium at 31-degrees-C and in McCarey-Kaufman medium at 4-degrees-C. These grafts were then transplanted in pairs of patients with keratoconus who were age matched as closely as possible. These pairs received donor corneas from the same donor, so for each pair the donor age and time from death to preservation were the same. Visual acuity, central corneal thickness, and endothelial cell counts were compared. During the 1- to 2-year study period, no statistically significant difference in visual acuity, corneal thickness, or endothelial cell density was found between grafts stored in minimal essential medium and those stored in McCarey-Kaufman medium.

To determine the extent of visual acuity and visual field impairment in patients with types 1 and 2 Usher syndrome.The records of 53 patients with type 1 and 120 patients with type 2 Usher syndrome were reviewed for visual acuity and visual field area at their most recent visit. Visual field areas were determined by planimetry of the II4e and V4e isopters obtained with a Goldmann perimeter. Both ordinary and logistic regression models were used to evaluate differences in visual acuity and visual field impairment between patients with type 1 and type 2 Usher syndrome.The difference in visual acuity of the better eye between patients with type 1 and type 2 varied by patient age (P=.01, based on a multiple regression model). The maximum difference in visual acuity between the 2 groups occurred during the third and fourth decades of life (with the type 1 patients being more impaired), while more similar acuities were seen in both younger and older patients. Fifty-one percent (n=27) of the type 1 patients had a visual acuity of 20/40 or better in at least 1 eye compared with 72% (n=87) of the type 2 patients (age-adjusted odds ratio, 3.9). Visual field area to both the II4e (P=.001) and V4e (P.001) targets was more impaired in the better eye of type 1 patients than type 2 patients. A concentric central visual field greater than 20 degrees in at least 1 eye was present in 20 (59%) of the available 34 visual fields of type 1 patients compared with 70 (67%) of the available 104 visual fields of type 2 patients (age-adjusted odds ratio, 2.9) with the V4e target and in 6 (21%) of the available 29 visual fields of type 1 patients compared with 36 (38%) of the available 94 visual fields of type 2 patients (age-adjusted odds ratio, 4.9) with the II4e target. The fraction of patients who had a visual acuity of 20/40 or better and a concentric central visual field greater than 20 degrees to the II4e target in at least 1 eye was 17% (n=5) in the type 1 patients and 35% (n=33) in the type 2 patients (age-adjusted odds ratio, 3.9).Visual acuity and visual field area were more impaired in patients with type 1 than type 2 Usher syndrome. Of note, 27 of 53 type 1 (51%) and 87 of 120 type 2 (72%) patients had a visual acuity of 20/40 or better in at least 1 eye. These data are useful for overall counseling of patients with Usher syndrome.

Patients with intermittent exotropia may have an increase in their angle of strabismus in the distance when the angle is measured either after 1 hour of monocular occlusion or while the patients fixate on a distant target outdoors. The hypothesis that surgery should be performed for this larger deviation has been suggested but not tested.To test the hypothesis that surgery should be performed for the increased angle of strabismus in the distance in patients with intermittent exotropia and to investigate the factors that influence the angle of misalignment.A prospective, clinical trial was conducted of patients with intermittent exotropia in whom the angle of misalignment in the distance increased after 1 hour of monocular occlusion or while the patients fixated on an outdoor target. The study group underwent surgery for the largest deviation measured; the control group underwent surgery for the initial angle measured at 6 m. All patients in whom the angle of misalignment increased while the patients were looking at an outdoor target were additionally measured in indoor illumination at 24 m and also at 6 m under floodlights that simulated outdoor illumination. Ninety patients undergoing surgery were randomized.Forty-three (86.0%) of the 50 patients undergoing surgery for the largest angle measured had a satisfactory outcome vs 25 (62.5%) of the 40 patients in the control group (P.001). The mechanism for the increase in exotropia while fixating on an outdoor target was studied in 76 patients, and the results were variable.The angle of strabismus in patients with intermittent exotropia undergoing surgery should be measured while the patients fixate on an outdoor target and after 1 hour of monocular occlusion. Surgery should be performed for the largest angle measured.

Objective To evaluate the efficacy and safety of transpupillary thermotherapy in treating choroidal melanoma. Methods and Patients To perform transpupillary thermotherapy, infrared diode laser energy at 810 nm was used with a beam diameter of about 3 mm and 1-minute exposure time. All 50 patients had choroidal melanoma. We performed transpupillary thermotherapy in 21 tumors that had responded insufficiently to 800 Gy ruthenium 106 brachytherapy; it was combined with 800 Gy 106 Ru brachytherapy for 10 tumors greater than 5 mm in height and with 600 Gy for 19 tumors 5 mm or less in height. Results All but 1 tumor exhibited reduction in tumor height within a mean follow-up of 20.5 months (range, 6-49 months). In 41 eyes (82%), the tumor flattened completely. Visual acuity was 20/60 or better in 43 eyes (86%) before treatment and in 14 eyes (28%) at the last examination because of radiation vasculopathy. Neovascular glaucoma developed in 1 eye, and total retinal detachment developed in 2 eyes. Tumor recurrence was observed in 1 patient. Conclusions Although long-term results are necessary to properly appraise this new therapy, transpupillary thermotherapy may be useful as a complementary modality to brachytherapy.

Objective To determine whether laser-cured fibrinogen glue can close bleb leaks in rabbits. Methods Full-thickness filtration surgery with intraoperative mitomycin and a sutured limbus-based conjunctival flap was performed in 1 eye each of 19 New Zealand albino rabbits. On the second postoperative day, a 2- to 3-mm hole was made in the bleb. In 9 rabbits, the hole was glued using fibrinogen glue with indocyanine green dye added. The glue was "cured" with a diode laser. Eyes that had been glued and developed a subsequent leak had the glue reapplied on the day the leak was detected. Results The glue remained on the conjunctiva for an average (mean±SD) of 1.9±1.8 days (range, 0-5 days). The last day of bleb leak for the rabbits with glued eyes was 1.6±2.4 days; for the control rabbits, it was 8.0±4.4 days ( P =.001, Mann-Whitney U test). Conclusion Laser-cured fibrinogen glue is effective in closing bleb leaks in rabbits.

Objective To estimate the incidence of nuclear, cortical, and posterior subcapsular cataract in the Beaver Dam Eye Study cohort. Design A population-based study of the prevalence of age-related eye diseases was conducted between 1988 and 1990 (n=4926), and a follow-up study was conducted between 1993 and 1995 (n=3684). The evaluations included photographic documentation of the lens. Slit-lamp photographs were taken to assess nuclear opacity, and retroillumination photographs were taken to assess cortical and posterior subcapsular cataract. The grading of photographs was done in a masked manner by trained graders using the same protocols for baseline and follow-up photographs. The graders were the same for both evaluations. Participants Persons aged 43 to 86 years who were identified through a private census conducted from 1987 to 1988 of the population of Beaver Dam, Wis, were invited for a baseline examination held between 1988-1990 and again for a follow-up examination held between 1993-1995. Results Incident nuclear cataract occurred in 13.1%, cortical cataract in 8.0%, and posterior subcapsular cataract in 3.4% of right eyes. The cumulative incidence of nuclear cataract in right eyes increased from 2.9% in persons aged 43 to 54 years at baseline to 40.0% in those aged 75 years or older. For cortical and posterior subcapsular cataract, the corresponding values were 1.9% and 21.8% and 1.4% and 7.3%, respectively. Women were more likely than men to have nuclear cataract even after adjusting for age. Conclusions Incident cataracts are common age-related problems, and incidence increases with increasing age at baseline. These data will help in planning for future care (eg, cataract surgery and change in spectacle correction) and in investigating the importance of risk factors.

Objective To compare baseline characteristics and treatment response of participants with hemiretinal vein occlusion (HRVO) with those of participants with branch retinal vein occlusion (BRVO) or central retinal vein occlusion (CRVO) in the Standard Care vs COrticosteroid for REtinal Vein Occlusion (SCORE) Study. Methods Eyes were randomized to standard care, 1 mg intravitreal triamcinolone acetonide, or 4 mg intravitreal triamcinolone acetonide. Standard care was observation in the SCORE-CRVO trial and grid photocoagulation in the SCORE-BRVO trial. The HRVO eyes were enrolled in the SCORE-BRVO trial. Baseline characteristics, changes in visual acuity and center point thickness, safety outcomes, and number of treatments were compared among HRVO, BRVO, and CRVO participants. Results At baseline, HRVO eyes were intermediate between BRVO and CRVO eyes in area of retinal thickening, area of fluorescein leakage, visual acuity, and center point thickness. No differences in visual acuity change from baseline to 1 year were noted between standard care groups for HRVO and BRVO. Within triamcinolone-treated eyes, HRVO eyes did not differ from BRVO eyes in visual acuity change, but HRVO eyes fared better than CRVO eyes. There were no differences in center point thickness change between standard care groups for HRVO and BRVO, nor were there differences across the 3 disease entities for triamcinolone-treated eyes. There were no differences in frequency of protocol treatments and adverse events. Conclusions The HRVO participants were similar to BRVO and CRVO participants regarding most demographic characteristics, with fundus findings intermediate between BRVO and CRVO. In the SCORE Study, HRVO was treated as BRVO; HRVO eyes responded to treatment similarly to BRVO eyes, and there was no difference among the 3 disease entities in frequency of protocol treatments and adverse events. Trial Registration clinicaltrials.gov Identifier: NCT00105027

Objective To examine the safety, tolerability, and reproducibility of intraocular pressure (IOP) patterns during repeated continuous 24-hour IOP monitoring with a contact lens sensor. Methods Forty patients suspected of having glaucoma (n = 21) or with established glaucoma (n = 19) were studied. Patients participated in two 24-hour IOP monitoring sessions (S1 and S2) at a 1-week interval (SENSIMED Triggerfish CLS; Sensimed AG). Patients pursued daily activities, and sleep behavior was not controlled. Incidence of adverse events and tolerability (visual analog scale score) were assessed. Reproducibility of signal patterns was assessed using Pearson correlations. Results The mean (SD) age of the patients was 55.5 (15.7) years, and 60% were male. Main adverse events were blurred vision (82%), conjunctival hyperemia (80%), and superficial punctate keratitis (15%). The mean (SD) visual analog scale score was 27.2 (18.5) mm in S1 and 23.8 (18.7) mm in S2 (P = .22). Overall correlation between the 2 sessions was 0.59 (0.51 for no glaucoma medication and 0.63 for glaucoma medication) (P = .12). Mean (SD) positive linear slopes of the sensor signal from wake to 2 hours into sleep were detected in both sessions for the no glaucoma medication group (S1: 0.40 [0.34], P Conclusions Repeated use of the contact lens sensor demonstrated good safety and tolerability. The recorded IOP patterns showed fair to good reproducibility, suggesting that data from continuous 24-hour IOP monitoring may be useful in the management of patients with glaucoma. Trial Registration clinicaltrials.gov Identifier: NCT01319617

OBJECTIVE To estimate and compare the incidence of operable cataract and the desired cataract surgery rates required to eliminate cataract-related visual impairment in several Latin American settings. METHODS We obtained raw data on age-specific cataract prevalence from standardized population-based surveys. We used the data in a previously described model to estimate the incidence of operable cataract at 11 sites in 10 countries across Latin America. Age-specific incidence rates were then multiplied by corresponding population data to calculate the desired cataract surgery rates needed to eliminate cataract-related visual impairment in eyes in each country. Age-standardized incidence was also calculated to explore potential non-age-related differences in incidence among the countries. RESULTS The desired cataract surgery rates ranged from 3441 to 8935 in the 11 sites. Much of the variation was owing to differing age structures, but there may be important variation in age-specific incidence rates as well. CONCLUSIONS Age structure has a major effect on the number of cataract surgeries needed in different countries of Latin America, and it is essential to consider this when planning cataract surgical services. Potential differences in non-age-related risk factors for cataract among different populations also deserve further study.

A patient presented with acute solar retinopathy after gazing directly at the sun. Best-corrected visual acuities were 6/12 and 6/18 in the left and right eyes, respectively. Optical coherence tomography (OCT) demonstrates full-thickness hyperreflective areas (A and B) corresponding to the hypopigmented areas observed on examination (C and D). At 3 months follow-up, the patient’s vision had improved to 6/9 in both eyes. The hypopigmented areas on the maculae had faded (E and F), and OCT shows hyporeflectivity in the outer retinal layers (G and H).

OBJECTIVE To compare Early Treatment Diabetic Retinopathy Study visual acuity outcome with retinal structural outcome at the 6-year follow-up examination of infants randomized in the Early Treatment for Retinopathy of Prematurity study. METHODS We compared the results in 606 eyes of subjects in whom both functional (visual acuity) and retinal structural assessments were obtained at age 6 years. Visual acuity assessments were performed by masked testers, and retinal examinations were performed by certified ophthalmologists. MAIN OUTCOME MEASURES Visual acuity and retinal structure at age 6 years. RESULTS Concordant outcomes occurred in 462 eyes (76.2%): 402 eyes had favorable functional and structural outcomes and 60 eyes had unfavorable functional and structural outcomes. Discordant outcomes occurred in 92 eyes (15.2%): 86 eyes had unfavorable functional and favorable structural outcomes and 6 eyes had favorable functional and unfavorable structural outcomes. Of the 86 eyes with unfavorable functional and favorable structural outcomes, 43 had optic atrophy (23 eyes) and/or retinal abnormalities that were less severe than those considered to be unfavorable (32 eyes). In 52 eyes (8.6%), retinal structure could not be assessed or the visual acuity was untestable. CONCLUSION Posterior pole appearance correlates well with visual acuity in 6-year-old infants with a history of advanced retinopathy of prematurity. APPLICATION TO CLINICAL PRACTICE When the retinal structure is normal but visual acuity is poor in infants with a history of severe retinopathy of prematurity, other diagnoses such as optic atrophy and cortical visual impairment could at least partially account for the discrepancy. TRIAL REGISTRATION clinicaltrials.gov Identifier: NCT00027222.

Objective To compare rates of visual field (VF) change in ocular hypertensive eyes with and without optic disc hemorrhage (DH). Methods Ocular Hypertension Treatment Study subjects (minimum 10 reliable VF tests, followed up ≥5 years) were included. Trend analyses of VF sequences over time of DH and non-DH eyes were assessed by regression of mean deviation (MDR) and pointwise linear regression (PLR). The main outcome measures were rates of VF change in DH and non-DH eyes. Results Two thousand six hundred seven eyes (1378 participants) were included. The mean (SD) number of VF tests per eye was 23.7 (4.9) spanning a mean (SD) of 12.2 (2.0) years. At least 1 DH was detected in 187 eyes (7.2%), of which 52 eyes had recurrent DH. Mean deviation rate of change was significantly worse in DH compared with non-DH eyes (mean [SD], −0.17 [0.27] vs −0.07 [0.19] dB/y; P Conclusions Eyes with DH had more rapid VF deterioration when assessed by global (MDR) or local (PLR) trend analysis than eyes without DH. Eyes with recurrent DH had similar rates of global VF change (MDR) when compared with eyes with a single DH but reached criteria for rapid PLR change more often. Intraocular pressure reduction in ocular hypertension reduces the risk of developing a DH. Ocular hypertensive eyes with DH should be monitored closely and may need more aggressive therapy. Trial Registration clinicaltrials.gov Identifier: NCT00000125

OBJECTIVES To investigate the importance of cerebral damage and retinopathy of prematurity (ROP) for visual impairment in preschool children born extremely premature and to determine the primary risk factor of the two. METHODS A clinical follow-up study of a Danish national cohort of children born extremely premature (gestational age,lt;28 weeks). The study sample consisted of 262 extremely preterm children born between February 13, 2004, and March 23, 2006, of whom 178 children (67.9%) participated. A matched control group consisted of 56 term-born children (gestational age, 37 tolt;42 weeks). All participants were identified through the National Birth Register and invited to participate in a clinical examination. The children were evaluated with regard to visual acuity, foveal sequelae, and maximum ROP stage and the presence of global developmental deficits (an indicator for cerebral damage) that was measured by the Ages and Stages Questionnaire. RESULTS Global developmental deficits and foveal sequelae occurred more often in extremely preterm children than in term-born control children and increased with ROP severity (χ2 test; P = .11 and P lt; .001, respectively). Global developmental deficits, moderate to severe foveal abnormality, and ROP treatment were independently associated with visual impairment (P lt; .05, for better and worse eyes). A stepwise multiple logistic regression for better-eye logarithmic visual acuities of 0.3 or greater (Snellen scale, ≤0.5) yielded an odds ratio of 8.7 (95% CI, 3.0-25.2; P lt; .001) for global developmental deficit and 6.3 (95% CI, 2.2-18.5; P lt; .001) for moderate to severe foveal sequelae. CONCLUSION Cerebral damage and ROP are independent risk factors for visual impairment in children born extremely premature, and cerebral damage may be the primary risk factor.

My husband, Carl B. Camras, MD (chairman of the Department of Ophthalmology and Visual Sciences, University of Nebraska Medical Center, Omaha), died at age 55 years in 2009. His dying wish was to be remembered for being the first to hypothesize that prostaglandins lower intraocular pressure and had potential as a medication to treat glaucoma. I reviewed the research he performed as an undergraduate at Yale University (New Haven, Connecticut), as a medical student at Columbia University (New York, New York), and on the faculty at Mount Sinai School of Medicine (New York, New York), which confirmed his hypothesis and led to the development of latanoprost. This article summarizes his contributions to glaucoma research, his role in the development of latanoprost, and the error of omission that prevented his recognition as its coinventor. Carl is best remembered as an ethical scientist, a gifted clinician, and a beloved teacher, who inspired the medical community and the next generation of ophthalmologists.

OBJECTIVE To review the effect of the fluocinolone acetonide implant in subjects with autosomal dominant neovascular inflammatory vitreoretinopathy (ADNIV), an inherited autoimmune uveitis. METHODS A retrospective case series was assembled from patients with ADNIV who received fluocinolone acetonide implants. Visual acuity and features of ADNIV, including inflammatory cells, neovascularization, fibrosis, and cystoid macular edema, were reviewed. RESULTS Nine eyes of 5 related patients with ADNIV with uncontrolled inflammation were reviewed. Follow-up ranged from 21.7 to 56.7 months. Visual acuity at implantation ranged from 20/40 to hand motion. Preoperatively, 8 eyes had vitreous cells (a ninth had diffuse vitreous hemorrhage). Eight eyes had cystoid macular edema, 7 had an epiretinal membrane, and 3 had retinal neovascularization. Following implantation, vitreous cells resolved in all eyes and neovascularization regressed or failed to develop. Central macular thickness improved in 4 eyes. During the postoperative course, however, visual acuity continued to deteriorate, with visual acuity at the most recent examination ranging from 20/60 to no light perception. There was also progressive intraocular fibrosis and phthisis in 1 case. Four eyes underwent cataract surgery. Six of the 7 eyes without previous glaucoma surgery had elevated intraocular pressure at some point, and 3 of these required glaucoma surgery. CONCLUSIONS The fluocinolone acetonide implant may inhibit specific features of ADNIV such as inflammatory cells and neovascularization but does not stabilize long-term vision, retinal thickening, or fibrosis. All eyes in this series required cataract extraction, and more than half required surgical intervention for glaucoma. Further studies may identify additional therapies and any benefit of earlier implantation.

Objective To determine the long-term safety of pars plana vitrectomy (PPV) in eyes with plaque-irradiated posterior uveal melanoma. Methods In this retrospective case series, patients with plaque-irradiated posterior uveal melanoma subsequently underwent PPV for vitreous hemorrhage. The main outcome measures are the rates of intraocular melanoma dissemination, extrascleral extension of melanoma, local melanoma recurrence, and systemic melanoma metastasis after PPV. Results Forty-seven eyes of 47 patients underwent PPV for vitreous hemorrhage after iodine 125–labeled plaque radiotherapy for choroidal melanoma. The mean interval between the onset of vitreous hemorrhage and PPV was 13 (median, 10; range, 0-52) months. The mean time from PPV to last follow-up was 5 (range, 0.5-16) years. There were no cases of intraocular melanoma dissemination or extrascleral extension of melanoma. One patient (2%) developed local choroidal melanoma recurrence (2 years after PPV and 5 years after initial plaque radiotherapy) and was successfully managed with transpupillary thermotherapy. Systemic melanoma metastasis occurred in 4 patients (9%) during a mean interval of 5 years after plaque radiotherapy. During follow-up, 43 patients (91%) were alive without systemic metastasis and 4 patients (9%) were alive with metastasis. Conclusion Management of vitreous hemorrhage by PPV in eyes with previously irradiated uveal melanoma appears to be safe and without increased risk for intraocular, local, orbital, or systemic dissemination of the tumor.

Objective: To report the percentage of new cases of myopia in 4927 children aged 5 to 16 years who participated in the Collaborative Longitudinal Evaluation of Ethnicity and Refractive Error Study between 1989 and 2009. Design: A multicenter, longitudinal, observational, volunteer study of refractive error and ocular development in children from 5 racial/ethnic groups in which the participants were children who were not myopic (right eye cycloplegic autorefraction of less myopia/more hyperopia than �0.75 diopters [D] in both principal meridians) at study entry. A new case was a diagnosis of myopia (right eye cycloplegic autorefraction of �0.75 D or more myopia in both principal meridians) after study entry. Results: Of the 4556 children entering the study who were not myopic, 749 (16.4%) received a diagnosis of myopia after study entry. Among these 749 children, the ages of the participants at diagnosis varied from 7 to 16 years, with the largest number diagnosed at age 11 years (136 participants [18.2%]). New cases of myopia occurred in 27.3% of Asians, 21.4% of Hispanics, 14.5% of Native Americans, 13.9% of African Americans, and 11% of whites. Female participants had more new cases than did male participants (18.5% vs 14.5%). Normal-birthweight children had more new cases than did low-birthweight children (16.9% vs 15.5%). Conclusions: Sixteen percent of children enrolled in the Collaborative Longitudinal Evaluation of Ethnicity and Refractive Error Study developed myopia during their school-aged years. The percentage increased yearly until age 11 years, after which it decreased. New cases of myopia varied by ethnic/racial group.

To evaluate the effectiveness of low-vision rehabilitation in 2 cohorts derived from the Veterans Affairs Low-Vision Intervention Trial.In a prospective study, we observed 44 participants randomly assigned to outpatient low-vision rehabilitation who did not receive additional treatment after the trial ended at 4-month follow-up and 56 participants randomly assigned to the waiting-list control group and thereafter to standard therapy. The outcome measures included visual ability domains (reading, mobility, visual information processing, and visual motor skills) and overall visual ability estimated from difficulty ratings using the 48-item Veterans Affairs Low-Vision Visual Functioning Questionnaire. Mean visual ability scores for the treatment and control groups were compared at baseline, 4 months, and 1 year. A mixed-effects model was used to test treatment effects between groups over time. Differences in visual ability mean scores from baseline to 1 year were compared between the 2 groups. Within-group changes in visual ability were compared from baseline to 1 year, from baseline to 4 months, and from 4 months to 1 year.At baseline, there were no significant differences in mean visual ability scores between groups. From baseline to 4 months, the treatment effects for all visual ability domains and overall visual ability increased to a maximum in the treatment group (P.001), whereas the mean scores (except visual motor skills) decreased in the control group (P.01). From 4 months to 1 year, the differences became smaller. There was a loss of visual ability in reading and visual information processing (but not in visual motor skills, mobility, or overall visual ability) in the treatment group and a gain in all visual ability measures in the control group. Interactions of treatment and follow-up time in the mixed models showed the trend of treatment effects significantly changed over time from baseline to 1 year (P.001) for all visual ability domains and overall visual ability. Both groups demonstrated improvement in visual ability from baseline to 1 year (P.001) (except for mobility in the control group). Overall visual ability (but not other visual ability domains) improved more in the treatment group than in the control group (P= .01).Visual ability improved significantly in both groups from baseline to 1 year. The Low-Vision Intervention Trial treatment effect is robust and well maintained for patients with macular diseases.