COMPARISON OF SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY SCAN PATTERNS AND CLINICAL REVIEW STRATEGIES IN NEOVASCULAR AGE-RELATED MACULAR DEGENERATION.

PURPOSE: To compare various spectral domain optical coherence tomography scan patterns and review strategies to identify an optimal imaging workflow for neovascular age-related macular degeneration. METHODS: A retrospective consecutive case series was performed in eyes after anti-vascular endothelial growth factor therapy for neovascular age-related macular degeneration with concurrent spectral domain optical coherence tomography imaging (Zeiss Cirrus), including horizontal/vertical five-line rasters, and macular cube analysis. For each scan pattern, a single report was independently reviewed in a masked fashion within the clinical image review software, whereas the cube was reviewed line-by-line in the reader software for the presence of fluid. RESULTS: One hundred and fifty-six reports and 39 cube scans of 39 patients were included. Among all spectral domain optical coherence tomography scans, 64% (25/39) had definitive fluid and 95% (37/39) had possible fluid. Sensitivities for definite fluid detection for horizontal, combined horizontal/vertical, and horizontal/vertical/map reviews were 68%, 76%, and 88%, respectively. When assessing for possible fluid, sensitivities for the detection for horizontal, combined horizontal/vertical, and horizontal/vertical/map reviews were 76%, 92%, and 97%, respectively. Line-by-line review of the cube scan had a sensitivity for definite and possible fluid detection of 96% and 86%, respectively. CONCLUSION: Optimizing both clinical accuracy and workflow are important factors in managing neovascular age-related macular degeneration. A zero-tolerance strategy with vertical/horizontal raster scans and thickness maps was comparable with line-by-line review of the cube to detect possible fluid.

Bevacizumab therapy and multimodal ultrawide-field imaging in immunogammopathy maculopathy secondary to Waldenström's macroglobulinemia.

Waldenström's macroglobulinemia (WM) is associated with retinal findings of hyperviscosity, such as venous dilation, and findings of immunogammopathy maculopathy, such as serous macular detachment. This report describes a case of bilateral serous macular detachment with intraretinal schisis-like fluid in a patient with WM. Enhanced depth imaging optical coherence tomography revealed a thickened choroid with hyperreflective accumulations in the retinal pigment epithelium layer. Ultrawide-field fundus autofluorescence demonstrated a central area of hyperautofluorescence corresponding to the area of serous macular detachment. Ultrawide-field fluorescein angiography was characteristically silent. Intravitreal bevacizumab therapy resulted in significant reduction in intraretinal fluid but minimal change in subretinal fluid. Long-term follow-up demonstrated alterations in retinal architecture and improved serous detachments.

Retinal morphology of patients with achromatopsia during early childhood: implications for gene therapy.

IMPORTANCE: While older children and adults with achromatopsia have been studied, less is known of young children with achromatopsia. OBJECTIVES: To characterize the macular and foveal architecture of patients with achromatopsia during early childhood with handheld spectral-domain optical coherence tomographic imaging and to make phenotype-genotype correlations. DESIGN, SETTING, AND PARTICIPANTS: Comparative case series of 9 patients with achromatopsia and 9 age-matched control participants at a tertiary ophthalmology referral center. MAIN OUTCOMES AND MEASURES: Patients underwent complete ocular examination, full-field electroretinography, handheld spectral-domain optical coherence tomographic imaging, and screening for genetic mutations. RESULTS: The mean (SD) age of the patients with achromatopsia was 4.2 (2.4) years, and the mean (SD) age of the control participants was 4.0 (2.1) years. Cone-driven responses to photopic single-flash or 30-Hz stimuli were nonrecordable in 7 patients and severely attenuated in 2. Rod-driven responses to dim scotopic single-flash stimuli were normal in 7 patients and mildly subnormal in 2. Six patients (67%) had foveal ellipsoid zone disruption, of which 1 had a hyporeflective zone. Four patients (44%) had foveal hypoplasia. The average total retinal thicknesses of the macula and fovea in the patients with achromatopsia were 14% and 17% thinner than in the control participants (P < .001 and P = .001), which was mostly due to the outer retina that was 18% and 26% thinner than in control participants (both P < .001), respectively. Genetic testing revealed a common homozygous mutation in CNGB3 in 5 patients with complete achromatopsia and heterozygous mutations in CNGA3 in 2 patients with incomplete achromatopsia. The youngest and worst-affected patient harbored compound heterozygous mutations in CNGB3 and a single mutation in CNGA3. CONCLUSIONS AND RELEVANCE: In early childhood, there is a spectrum of foveal pathology that is milder than reported in older individuals with achromatopsia, which suggests the need for early therapeutic intervention. Neither age alone nor genotype alone predicts the degree of photoreceptor loss or preservation. Thus, in anticipation of future gene therapy trials in humans, we propose that handheld spectral-domain optical coherence tomography is an important tool for the early assessment and stratification of macular architecture in young children with achromatopsia.

Animal models of age related macular degeneration.

Age related macular degeneration (AMD) is the leading cause of vision loss of those over the age of 65 in the industrialized world. The prevalence and need to develop effective treatments for AMD has lead to the development of multiple animal models. AMD is a complex and heterogeneous disease that involves the interaction of both genetic and environmental factors with the unique anatomy of the human macula. Models in mice, rats, rabbits, pigs and non-human primates have recreated many of the histological features of AMD and provided much insight into the underlying pathological mechanisms of this disease. In spite of the large number of models developed, no one model yet recapitulates all of the features of human AMD. However, these models have helped reveal the roles of chronic oxidative damage, inflammation and immune dysregulation, and lipid metabolism in the development of AMD. Models for induced choroidal neovascularization have served as the backbone for testing new therapies. This article will review the diversity of animal models that exist for AMD as well as their strengths and limitations.

Intravitreal anakinra inhibits choroidal neovascular membrane growth in a rat model.

PURPOSE: To determine the effect of intravitreal anakinra on choroidal neovascularization (CNV) in an animal model. METHODS: Laser photocoagulation used to rupture Bruch's membrane; animals then assigned to groups based on varying frequency of intravitreal anakinra injections. At day 30, digital angiography was used to measure the CNV area. RESULT: Intravitreal anakinra dose-dependently inhibited CNV growth. Injections given one time resulted in a 26% reduction in CNV; in the once weekly group, a 41% reduction was seen; in the twice-weekly group, a 62% reduction was seen (all p < .001). CONCLUSION: Suppressing IL-1 function may provide another therapeutic avenue in exudative AMD.