Abstract: Pediatric uveitis is an inflammatory ocular disease that can lead to sight-threatening complications. Pediatric patients have distinct challenges in the diagnosis and management of uveitis, secondary to difficulties in performing ophthalmic examinations in young children, delayed diagnosis due to lack of adherence with recommended screening schedules, medication side-effects, and increased burden of disease into adulthood. Measurement of outcomes in pediatric uveitis has traditionally relied upon the ophthalmic examination and general quality of life (QOL) measures. However, the ophthalmic examination does not take into account the impact of uveitis on a child’s QOL and general QOL measures do not adequately assess the specific effects of vision. Several vision-related quality of life (VR-QOL) instruments have been used to measure outcomes in both adults and children including: the National Eye Institute Visual Function Questionnaire (NEI VFQ-25), Vision-related Quality of Life of Children and Young People (VQoL_CYP), the Children’s Visual Function Questionnaire (CVFQ), and the Effect of Youngsters’ Eyesight on Quality of Life (EYE-Q). However, the NEI VFQ-25 is not a valid or applicable measure in children, and the VQoL_CYP and CVFQ are not uveitis specific and may not characterize disease specific burdens. The EYE-Q is the only uveitis-specific pediatric questionnaire that measures visual functioning and VR-QOL in 5–18 years old children and adolescents with uveitis. It has been shown to be a valid and reliable assessment tool in several cohorts of children with uveitis. A comprehensive assessment of the impact of uveitis on a child that includes a vision-specific measure, such as the EYE-Q, allows for better understanding of the true burden of uveitis in children. For this review, we describe traditional outcome measures in uveitis studies, general QOL measures and vision-specific measures in adults and in children.

Abstract: Hereditary, metabolic and toxic optic neuropathies cause bilateral, central vision loss and therefore can result in severe impairment in visual function. Accurate, early diagnosis is critical, as nutritional and toxic optic neuropathies may be reversible if identified early, and diagnosis of hereditary optic neuropathies can prevent unnecessary invasive workup, provide prognostic information, and allow for effective genetic counseling. Optical coherence tomography (OCT) is a valuable tool that aids in the diagnosis and prognostication of optic neuropathies as it allows for quantification of changes in the retinal ganglion cells (RGCs) and retinal nerve fiber layer (RNFL) over time. We review the characteristic clinical presentations of hereditary, metabolic and toxic optic neuropathies, with an emphasis on OCT findings.

Abstract: Optical coherence tomography (OCT) is a widely used non-invasive medical imaging technology that has revolutionized clinical care in ophthalmology. New developments, such as OCT angiography (OCTA) are expected to contribute even further to the widespread use of OCT-based imaging devices in the diagnosis and monitoring of patients with ophthalmic diseases. In recent years, many of the disadvantages such as limited field of view and imaging artefacts have been substantially reduced. Similar to the progress achieved in the assessment of retinal disorders, OCT is expected to change the approach to patients seen in the neuro-ophthalmology clinic. In this article, we review the technical features of OCT and OCT-based imaging techniques, highlighting the specific factors that should be taken into account when interpreting OCT in the field of neuro-ophthalmology.

Abstract: Effective and safe electrical stimulation of the retinal ganglion cells is at the heart of retinal prosthesis design. However, the effectiveness and safety demand of the electrical stimulation is often at odds against each other. Besides, the nerve fiber layer above retinal ganglion cells limits the spatial resolution of stimulation. Also, current retinal prosthesis still cannot selectively activate the ON or OFF visual pathways, thus cannot relay the correct luminance information to the brain. With decades of development, the stimulation protocol for retinal implants began to tackle these problems. We believe that a novel design of electrical stimulation scheme, combined with gene therapy technique, can improve the selectivity and spatial resolution of retinal implants and further lower the damage caused by electric stimulation.

Abstract: Focal intraretinal alterations have been studied to advance our understanding of the pathology of neurodegenerative diseases. The current literature involving focal alterations in the intraretinal layers was reviewed through PubMed using the search terms “focal alteration”, “region of interest”, “optical coherence tomography”, “glaucoma”, “multiple sclerosis”, “Alzheimer’s disease”, “Parkinson disease”, “neurodegenerative diseases” and other related items. It was found that focal alterations of intraretinal layers were different in various neurodegenerative diseases. The typical focal thinning might help differentiate various ocular and cerebral diseases, track disease progression, and evaluate the outcome of clinical trials. Advanced exploration of focal intraretinal alterations will help to further validate their clinical and research utility.

Abstract: Ischemic optic neuropathies are among the most common causes of sudden vision loss, especially in patients over age 50. The cause and prognosis of these disorders, and in particular non-arteritic anterior ischemic optic neuropathy, is poorly understood, and treatments remain poor in terms of restoring or preserving vision. Optical coherence tomography (OCT) and OCT angiography have allowed us to identify early and late structural changes in the optic nerve head and retina that may assist in predicting visual outcomes and may lead to greater understanding of pathogenesis and thus the development of effective medical interventions.

Abstract: Age-related macular degeneration (ARMD), one of the most common causes of blindness, should be considered more due to its exponential increase in the coming 20 years as a result of increasing the age of the population. Whereas more recent studies offered newer scaling systems for ARMD, traditionally it is classified as the early and late stages. The main injury in this disease occurred in retinal pigment epithelium (RPE) and the retina. RPE cells have a crucial role in hemostasis and supporting photoreceptors. In the early stages, damages to RPE are minimal and mainly no treatment is needed because most patients are asymptomatic. However, in the late stages, RPE impairment may lead to the invasion of choroidal vessels into the retina. Although anti-angiogenic agents can inhibit this abnormal growth of blood vessels, they cannot stop it completely, and finally, total loss of retinal cells may occur (geographical atrophy). Since this prevalent disease has not had any cure yet, the concept of substituting the RPE cells should be considered. Repairing the injury to central nervous system cells is almost impossible because the regenerative capacity of these cells is limited. Recently, the use of regenerative substitutes has been suggested to replace damaged tissues. Amniotic membrane (AM) has been raised as a suitable substitute for damaged RPE cells due to all of its unique properties: pluripotency, anti-angiogenic effect, and anti-inflammatory effect. Based on the few studies that have been published so far, it seems that the use of this membrane in the treatment of ARMD can be helpful, but more studies are needed.

Abstract: Age-related macular degeneration (AMD) remains a leading cause of severe visual impairment in developing countries. Although dry-type AMD and geographic atrophy (GA) are progressive conditions with the associated decrease of visual functions, no well-established treatment regimen was proposed for the disease. Wet-type AMD is effectively treated with intravitreal anti-angiogenic agents, but frequent injections are a major issue for the affected patients. Recent advances in AMD genetics have provided new insights into the pathogenesis and novel therapeutic targets of AMD, but the benefits of using genetic testing and genotype-based risk models for AMD development and progression still lacks evidence. Novel AMD treatments aim to increase the interval among intravitreal injections through new therapeutic agents and modern delivery devices. Simultaneously, gene therapy for dry and wet AMD is widely studied. Although gene therapy possesses a major superiority over other novel treatments regarding a persistent cure of disease, many challenges exist in the way of its broad impact on the ocular health of AMD patients.