Long-term visual outcomes of children screened for retinopathy of prematurity with telemedicine in New Zealand.

CLINICAL RELEVANCE: Children with a history of regressed retinopathy of prematurity (ROP) are at increased risk of peripheral avascular retina. Wide-field digital retinal imaging and telemedicine is an effective tool for ROP screening. Ophthalmologists and Optometrists should have a high level of clinical suspicion for peripheral retinal changes in children screened for ROP. BACKGROUND: Retinopathy of prematurity, a vaso-proliferative disorder of the pre-term retina, is a preventable cause of childhood visual impairment. The Auckland Regional Telemedicine ROP (ART-ROP) network, established in 2006, utilises wide-field digital imaging and telemedicine to screen at-risk infants for ROP. This prospective observational study reports the long-term ocular outcomes of ART-ROP network infants. METHODS: A comprehensive paediatric eye examination including cycloplegic autorefraction and wide-field retinal imaging was completed on all participants. Participants had been screened for ROP by the ART-ROP network between May 2008 and October 2011. RESULTS: A total of 69 children, with a mean age of 5 to 8 years old were assessed and divided into two groups: those with or without a history of ROP, 44 and 25 children, respectively. Infants with a history of ROP had significantly lower gestational age (26.6 ± 1.9 vs. 29.1 ± 1.6 weeks, p < 0.001) and birth weight (937 ± 237 vs. 1177 ± 311 grams, p = 0.001). No significant differences were detected between the two groups for visual acuity (p = 0.596), stereopsis (p = 0.219), refractive error (p = 0.472), or strabismus. Clinically significant refractive error was noted in 10 participants; none with moderate or high myopia. Retinal imaging exposed asymptomatic, persistent, peripheral avascular retina in four children, all of whom had a history of regressed ROP. CONCLUSION: Visual and ocular outcomes did not vary based on history of ROP, with no participant having reduced vision as a result of undetected or untreated ROP. Further research is required into the long-term implication of persistent avascular retina in regressed ROP.

Eye Movement Abnormalities in Glaucoma Patients: A Review.

Glaucoma is a common condition that relies on careful clinical assessment to diagnose and determine disease progression. There is growing evidence that glaucoma is associated not only with loss of retinal ganglion cells but also with degeneration of cortical and subcortical brain structures associated with vision and eye movements. The effect of glaucoma pathophysiology on eye movements is not well understood. In this review, we examine the evidence surrounding altered eye movements in glaucoma patients compared to healthy controls, with a focus on quantitative eye tracking studies measuring saccades, fixation, and optokinetic nystagmus in a range of visual tasks. The evidence suggests that glaucoma patients have alterations in several eye movement domains. Patients exhibit longer saccade latencies, which worsen with increasing glaucoma severity. Other saccadic abnormalities include lower saccade amplitude and velocity, and difficulty inhibiting reflexive saccades. Fixation is pathologically altered in glaucoma with reduced stability. Optokinetic nystagmus measures have also been shown to be abnormal. Complex visual tasks (eg reading, driving, and navigating obstacles), integrate these eye movements and result in behavioral adaptations. The review concludes with a summary of the evidence and recommendations for future research in this emerging field.

Association of Nailfold Capillary Abnormalities With Primary Open-angle Glaucoma and Glaucomatous Visual Field Loss.

PRECIS: Nailfold capillary abnormalities are associated with primary open-angle glaucoma (POAG) and increased severity of global and central glaucomatous visual field (VF) loss. PURPOSE: The purpose of this study was to investigate whether nailfold capillary abnormalities are associated with POAG and the severity of glaucomatous VF loss. MATERIALS AND METHODS: A cross-sectional study of 83 POAG cases and 40 controls was conducted. Nailfold capillaroscopy images were assessed by masked graders for dilated capillaries >50 µm, crossed capillaries, tortuous capillaries, hemorrhages, avascular zones >100 µm, capillary density, and capillary distribution. VF loss in glaucoma cases was quantified using mean deviation and mean central pattern standard deviation (PSD) from the worst-affected eye. RESULTS: Logistic regression analyses of cases and controls showed that avascular zones [odds ratio (OR)=1.24; 95% confidence interval (CI): 1.06, 1.47; P=0.005], capillary density (OR=0.63; 95% CI: 0.46, 0.83; P<0.001), and capillary distribution (OR=7.88; 95% 95% CI: 2.53, 28.40; P=0.001) were associated with POAG. Simple linear regression analysis of cases only showed that nailfold hemorrhages were associated with mean deviation (ß=-5.10; 95% CI: -9.20, -1.01; P=0.015) and mean central PSD (ß=-4.37; 95% CI: -8.18, -0.57; P=0.025), and this remained significant in the multiple linear regressions. After controlling for demographic and clinical factors, avascular zones were associated with both mean deviation (ß=-0.76; 95% CI: -1.48, -0.04; P=0.040) and mean central PSD (ß=-0.78; 95% CI: -1.45, -0.10; P=0.024), whereas capillary distribution was only associated with mean deviation (ß=-4.67; 95% CI: -7.92, -1.43; P=0.017). CONCLUSION: Nailfold capillary abnormalities are associated with POAG as well as increased global and central vision loss.

Multimodal Retinal Image Analysis via Deep Learning for the Diagnosis of Intermediate Dry Age-Related Macular Degeneration: A Feasibility Study.

RESULTS: The CNN trained using OCT alone showed a diagnostic accuracy of 94%, whilst the OCT-A trained CNN resulted in an accuracy of 91%. When multiple modalities were combined, the CNN accuracy increased to 96% in the AMD cohort. CONCLUSIONS: Here we demonstrate that superior diagnostic accuracy can be achieved when deep learning is combined with multimodal image analysis.

Quantification of Optical Coherence Tomography Angiography in Age and Age-Related Macular Degeneration Using Vessel Density Analysis.

PURPOSE: The aim of this study was to determine whether vessel density (VD) as measured by optical coherence tomography (OCT) angiography provided insights into retinal and choriocapillaris vascular changes with aging and intermediate dry age-related macular degeneration (AMD). DESIGN: Non-randomized observational study. METHODS: Seventy-five participants were recruited into 3 cohorts: young healthy group, old healthy, and those at high-risk for exudative AMD. Raw OCT and OCT angiography data from TOPCON DRI OCT Triton were exported using Topcon IMAGENET 6.0 software, and 3D datasets were analysed to determine retinal thickness and VD. RESULTS: Central macular thickness measurements revealed a trend of overall retinal thinning with increasing age. VD through the full thickness of the retina was highest in Early Treatment Diabetic Retinopathy Study (ETDRS) sector 4 (the inferior macula) in all the cohorts. Mean VD was significantly higher in the deep capillary plexus than the superficial capillary plexus in all ETDRS sectors in all cohorts, but there was no significant difference noted between groups. Choriocapillaris VD was significantly lower in all ETDRS sectors in the AMD group compared with the young healthy and the old healthy groups. CONCLUSIONS: Retinal VD maps, derived from the retinal plexi, are not reliable biomarkers for assessing the aging macular. Our nonproprietary analysis of the vascular density of the choriocapillaris revealed a significant drop off of VD with age and disease, but further work is required to corroborate this finding. If repeatable, choriocapillaris VD may provide a noninvasive biomarker of healthy aging and disease.

Gender differences in Australasian ophthalmologists' experiences of the workplace.

IMPORTANCE: Gender differences were identified in experiences of the workplace and family responsibilities amongst Australian and New Zealand ophthalmologists. BACKGROUND: To survey ophthalmologists regarding their balance of career, family and workplace experiences and to identify gender differences. DESIGN: Online questionnaire sent to 1000 randomly selected Royal Australian and New Zealand College of Ophthalmologists (RANZCO) Fellows in 2017. PARTICIPANTS: The response rate was 28% (n = 282) with 192 males. METHODS: Confidential questionnaire. MAIN OUTCOME MEASURES: Questionnaire responses. RESULTS: Gender differences were noted in working hours (59% of males worked greater than 40 hours a week vs 26% of females, P < 0.001) and frequency of private practice work (mean of 6.6 half-day sessions per week for men vs 4.9 sessions for women, P < 0.001). Female ophthalmologists reported additional obstacles to career advancement including difficulty receiving mentorship (57% vs 40%, P = 0.027), travel difficulties due to family responsibilities (59% vs 34%, P < 0.001) and rigid timelines for promotion/tenure (38% vs 19%, P = 0.005). Female ophthalmologists delayed child-bearing, with 59% becoming parents after fellowship training. Women spent more time child-rearing (67% vs 8% of men cared for children >20 hours per week, P < 0.001). Female ophthalmologists were more likely to report experiencing discrimination (31% vs 8% of men, P < 0.001). CONCLUSIONS AND RELEVANCE: Female ophthalmologists worked fewer hours, mainly in the private sector, to fulfil their greater family commitments. Female ophthalmologists reported additional obstacles to career advancement and were more likely to report experiencing discrimination in the workplace.

Corneal nerve microstructure in Parkinson's disease.

Ocular surface changes and blink abnormalities are well-established in Parkinson's disease. Blink rate may be influenced by corneal sub-basal nerve density, however, this relationship has not yet been investigated in Parkinson's disease. This case-control study examined the ocular surface in patients with moderately severe Parkinson's disease, including confocal microscopy of the cornea. Fifteen patients with moderately severe Parkinson's disease (modified Hoehn and Yahr grade 3 or 4) and fifteen control participants were recruited. Ophthalmic assessment included slit-lamp examination, blink rate assessment, central corneal aesthesiometry and in vivo corneal confocal microscopy. The effect of disease laterality was also investigated. Of the 15 patients with Parkinson's disease, ten were male and the mean age was 65.5±8.6years. The corneal sub-basal nerve plexus density was markedly reduced in patients with Parkinson's disease (7.56±2.4mm/mm2) compared with controls (15.91±2.6mm/mm2) (p<0.0001). Corneal sensitivity did not differ significantly between the patients with Parkinson's disease (0.79±1.2mBAR) and the control group (0.26±0.35mBAR), p=0.12. Sub-basal nerve density was not significantly different between the eye ipsilateral to the side of the body with most-severe motor symptoms, and the contralateral eye. There was a significant positive correlation between ACE-R scores and sub-basal corneal nerve density (R2=0.66, p=0.02). This is the first study to report a significant reduction in corneal sub-basal nerve density in Parkinson's disease and demonstrate an association with cognitive dysfunction. These results provide further evidence to support the involvement of the peripheral nervous system in Parkinson's disease, previously thought to be a central nervous system disorder.

Optical coherence tomography findings in a patient with type 1 sialidosis.

Type 1 sialidosis is a metabolic storage disorder characterised by the accumulation of sialylated oligosaccharides. The condition is also known as macular cherry-red spot and myoclonus syndrome due to the characteristic macular appearance in affected individuals. This case outlines the presentation of a patient with type 1 sialidosis, including ophthalmological assessment with retinal photography and spectral domain optical coherence tomography (OCT). OCT scans showed thickening of the perimacular and peripapillary retinal nerve fibre layer, thought to be due to the abnormal accumulation of metabolic products. The cherry-red spot appearance is due to the normal appearing macula being seen in contrast to the abnormally pale surrounding, thickened retinal nerve fibre layer.

Optical coherence tomography findings in Huntington's disease: a potential biomarker of disease progression.

Previous reports of ocular abnormalities in Huntington's disease (HD) have detailed eye movement disorders. The objective of this case-control study was to investigate optic nerve and macular morphology in HD using optical coherence tomography (OCT). A total of 26 HD patients and 29 controls underwent a thorough ophthalmic examination including spectral domain OCT scans of the macula and peripapillary retinal nerve fibre layer (RNFL). Genetic testing results, disease duration, HD disease burden scores and Unified HD Rating Scale motor scores were acquired for HD patients. Temporal RNFL thickness was significantly reduced in the HD group (62.3 vs. 69.8 µm, p = 0.005), and there was a significant negative correlation between temporal RNFL thickness and disease duration (R (2) = -0.51, p = 0.04). Average peripapillary RNFL thickness was not significantly different between the HD and control groups. There was a significant negative correlation between macular volume and disease duration (R (2) = -0.71, p = 0.002), and motor scores (R (2) = -0.56, p = 0.01). Colour vision was significantly poorer in the HD group. Temporal RNFL is preferentially thinned in HD patients, possibly implicating mitochondrial dysfunction as the temporal RNFL is reduced in the patients with some mitochondrial disorders, including Leber's hereditary optic neuropathy. The correlation between the decrease in macular volume and temporal RNFL, and increasing disease severity suggests that OCT may be a useful biomarker for disease progression in HD. Larger, longitudinal studies are required.

Ophthalmic manifestations of inherited neurodegenerative disorders.

Ophthalmic findings are common features of neurodegenerative disorders and, in addition to being clinically important, have emerged as potentially useful biomarkers of disease progression in several conditions. Clinically, these visual system abnormalities can be a clue to diagnosis, as well as being a prominent cause of disability in affected patients. In this Review, we describe the various afferent visual system and other ophthalmic features of inherited neurodegenerative disorders, including the muscular dystrophies, Friedreich ataxia, the spinocerebellar ataxias, hereditary spastic paraplegia, Charcot-Marie-Tooth disease, and other conditions. We focus on the expanding role of optical coherence tomography in diagnostic imaging of the retina and optic nerve head, and the possible use of ophthalmic findings as biomarkers of disease severity in hereditary neurodegenerative disorders. In addition, we discuss the ophthalmic manifestations and treatment implications of mitochondrial dysfunction, which is a feature of many inherited neurodegenerative diseases.

Epiretinal membrane: a treatable cause of visual disability in myotonic dystrophy type 1.

A wide range of ocular abnormalities have been documented to occur in patients with myotonic dystrophy type 1. The objectives of this study were to investigate the macular and optic nerve morphology using optical coherence tomography in patients with myotonic dystrophy type 1. A total of 30 myotonic dystrophy type 1 patients and 28 controls were recruited for participation. All participants underwent a thorough ophthalmologic examination, including spectral-domain optical coherence tomography of the macula and retinal nerve fibre layer. Images were reviewed by a retinal specialist ophthalmologist, masked to the diagnosis of the participants. Average macular thickness was significantly greater in the myotonic dystrophy group compared to controls [327.3 µm vs. 308.5 µm (p < 0.001)]. Macular thickness was significantly greater (p < 0.005) in five of the nine macular regions. The increase in macular thickness was due to the increased prevalence of epiretinal membranes in the myotonic dystrophy patient group (p = 0.0002): 48.2 % of myotonic dystrophy patient eyes had evidence of epiretinal membrane, compared with 12.5 % of control eyes. Examination revealed that 56.7 % of myotonic dystrophy patients had an epiretinal membrane in at least one eye. Visual acuity was reduced due to the presence of epiretinal membrane in six patient eyes and none of the control eyes. The presence of an epiretinal membrane was significantly correlated with increasing age in the patient group. We report an increased prevalence of epiretinal membrane in the myotonic dystrophy type 1 group. This may be a previously under-recognised form of visual impairment in this group. Epiretinal membranes can be treated surgically. We suggest that, in addition to a comprehensive clinical examination, optical coherence tomography examination is implemented as part of an ophthalmological assessment for the myotonic dystrophy type 1 patient with reduced visual acuity.