Background: With a large portion of older adults living longer, the number of individuals diagnosed with low vision is increasing. The use of optical coherence tomography/scanning laser ophthalmoscope (OCT/SLO) to diagnose retinal disease has become common place in the last 10 years, yet currently there are no OCT/SLO databases for pathological vision. Our aim is to develop a clinical database of individuals who have drusen (i.e., lipid deposits found under the retina), or have been diagnosed with age-related macular degeneration (AMD), with information as to how the structure of the diseased retina changes over time, as well as measures of visual and cognitive functional performance.
Methods: Fundus photographs and retinal scans will be taken using the same model of optos OCT/SLO located in three test sites (MAB-Mackay Rehabilitation Centre, School of Optometry Clinic at the University of Montreal, and the Lighthouse Institute, New York, USA). For each individual entry in the database, demographic and diagnosis information will be available. All OCT/SLO images will be graded according to the Age-related Eye Disease Study standard, in addition to number and size of drusen, severity of geographic atrophy, severity of pigment mottling and presence of choroidal neovascularization. Retinal topography and Raster scans from the OCT/SLO will provide a cross-sectional look at affected retinas. Fixation stability will be recorded using the SLO function, and present four different tasks that are designed to reproduce typical tasks of daily vision, with each task lasting for 10 seconds. The tasks are cross fixation, face recognition, visual search, and reading. These tasks in addition to the retinal scans will be used to determine the eccentricity of a preferred retinal locus from the anatomical fovea, and can be used as an outcome measure for clinical interventions in visually impaired patients.
Results: The database will be available to professors training eye-care practitioners and rehabilitation specialists as a teaching tool. Students will be able to familiarize themselves with the retina and a variety of AMD-related pathologies before they start working with patients. The database will also be accessible by researchers interested in studying AMD from basic science to epidemiology, to investigate how drusen and AMD impact visual and cognitive functional performance.
Conclusions: The common infrastructure is easily accessible to all VHRN members on request. The database will also be accessible online in 2018 (see http://cvl.concordia.ca for more information).
Background: Epidermolysis bullosa (EB) is a heterogynous group of skin disorders characterized by formation of blisters and erosions of the skin in response to minor trauma. Subtypes include EB simplex (EBS), junctional EB (JEB), dystrophic form of EB (DEB) and finally Kindler syndrome (KS). In addition to dermal manifestation, patients can present with various ophthalmic pathologies.
Methods: We reviewed the pathobiology, epidemiology and management of ocular manifestations as well as current and future innovative therapies for EB.
Results: The severity and incidence of ocular involvement were the highest in the recessive DEB-generalized severe and JEB-generalized severe subtypes. Recurrent corneal erosions and blisters were the most common finding and seem to correlate with skin disease. Other manifestations include corneal scaring, blepharitis, ectropion, symblepharon, infantile cataracts, lacrimal duct obstruction as well as meibomian gland deficiency.
Conclusions: Ophthalmology consult as well as regular follow-up are essential in the multi-disciplinary approach of this disease. Indeed, parents’ and patients’ education as well as early diagnosis and treatment are crucial to prevent permanent and long-term visual disabilities.
Abstract: To describe the current aging population in China and globally, especially as it applies to age-related macular degeneration (AMD). To review the current standards of care for treating both wet (exudative) eAMD and dry (atrophic) aAMD. And to introduce a model for experimentation that is based on the Age-Related Eye Disease Study (AREDS) using eye bank tissue. A literature search that outlines current aging populations, standards of clinical treatment as defined by large, multicenter, randomized clinical trials that present level-I data with a low risk for bias. An experimental model system of AMD is presented that enables scientific analysis of AMD pathogenesis by applying grading criteria from the AREDS to human eye bank eyes. Analysis includes proteomic, cellular, and functional genomics. The standard of care for the treatment of eAMD is currently defined by the use of several anti-vascular endothelial growth (anti-VEGF) agents alone or in combination with photodynamic therapy. Monotherapy treatment intervals may be monthly, as needed, or by using a treat-and-extend (TAE) protocol. There are no proven therapies for aAMD. AMD that is phenotypically defined at AREDS level 3, should be managed with the use of anti-oxidant vitamins, lutein/zeaxanthin and zinc (AREDS-2 formulation). By understanding the multiple etiologies in the pathogenesis of AMD (i.e., oxidative stress, inflammation, and genetics), the use of human eye bank tissues graded according to the Minnesota Grading System (MGS) will enable future insights into the pathogenesis of AMD. Initial AMD management is with lifestyle modification such as avoiding smoking, eating a healthy diet and using appropriate vitamin supplements (AREDS-2). For eAMD, anti-VEGF therapies using either pro re nata (PRN) or TAE protocols are recommended, with photodynamic therapy in appropriate cases. New cellular information will direct future, potential therapies and these will originate from experimental models, such as the proposed eye bank model using the MGS, that leverages the prospective AREDS database.
Abstract: Autoimmune retinopathy (AIR) refers to both paraneoplastic and non-paraneoplastic forms of a rare, acquired retinal degeneration thought to be mediated by the production of antiretinal antibodies. However, the mechanisms underlying AIR pathogenesis are incompletely understood, and it remains a diagnosis of exclusion given the lack of definitive testing as well as its protean clinical presentation. This review summarizes the current literature on the epidemiology, diagnosis, and management of AIR, with a focus on non-paraneoplastic disease and the potential role of immunomodulatory therapy. A recent expert consensus statement on diagnosis and management of non-paraneoplastic AIR served as a framework for interpreting the limited data available, a process that was complicated by the small sample sizes, heterogeneity, and retrospective nature of these studies. Additional work is needed to characterize AIR patients on the basis of cytokine and immunogenetic profiling; to establish the pathogenicity of antiretinal antibodies; and to standardize treatment regimens as well as assessment of clinical outcomes.
Background and Objective: Intraocular lymphoma (IOL) is a heterogenous category of rare malignancies that are often misdiagnosed and underrecognized. The rarity of IOL impedes clinical research and contributes to difficulty in standardizing its management. In this article we review the existing scientific literature to identify the current diagnostic tools and discuss comprehensive management of various categories of IOL. Our objective is to increase disease recognition of IOL as a whole and explore updated management options for each subtype.
Methods: PubMed and Embase were searched for publications using the terms ‘intraocular lymphoma’, ‘vitreoretinal lymphoma’, ‘uveal lymphoma’, ‘iris lymphoma’, ‘choroidal lymphoma’ and ‘ciliary body lymphoma’ published from 1990 to June 2021. Inclusion criteria were English language articles. Exclusion criteria were non-English language articles, case reports and animal studies.
Key Content and Findings: IOL often presents in middle-aged and older patients with symptoms of floaters and vision changes, but a broad array of clinical signs and symptoms are possible depending upon subtype. IOL can be subdivided by location of involvement into vitreoretinal and uveal lymphoma. These subtypes express key differences in their pathophysiology, clinical presentation, histology, prognosis, and treatment. Primary vitreoretinal lymphomas (PVRL) generally originate from B-lymphocytes and are associated with central nervous system (CNS) lymphoma. Ophthalmic findings include retinal pigment epithelium changes with yellow subretinal deposits known as “leopard spotting.” Primary uveal lymphomas generally originate from low-grade B-lymphocytes invading the choroid and carry an improved prognosis compared to vitreoretinal lymphomas. Funduscopic findings of primary uveal lymphoma include yellow to pink-yellow choroidal swelling with infiltrative subconjunctival “salmon-patch” lesions. Diagnosis for IOL is often delayed due to insidious onset, low prevalence, and tendency to mimic diseases such as uveitis. Diagnosis may be challenging, often relying on biopsy with specialized laboratory testing for confirmation of IOL. Optimal treatment regimens are currently debated among experts. Management of IOL is best coordinated in association with neuro-oncology clinicians due to the tendency for intracranial involvement.
Conclusions: IOL represents a group of multiple malignancies with distinct clinicopathologic features. Future outlook for treatment and prognosis of IOL is likely to improve with less invasive molecular diagnostic techniques and increased awareness. Clinicians should be circumspect in all patients with possible IOL and promptly refer to oncologic specialists for rapid evaluation and treatment.
