1,721,093 research outputs found

    Case Report: Multiple Retinal Astrocytic Hamartomas in Congenital Disorder of Glycosylation-Ia

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    Congenital disorder of glycosylation-Ia (CDG-Ia) is a rare autosomal recessive genetic disorder, characterized by systemic and ophthalmological abnormalities. Here, we report multiple retinal astrocytic hamartomas as a new retinal finding in an adolescent affected by congenital disorder of CDG-Ia. A 15-year-old boy affected by CDG-Ia underwent full ophthalmic examination, full field electroretinography (ERG) evaluation and retinal multimodal imaging, including: fundus photography, spectral domain optical coherence tomography (SD-OCT) and blue fundus autofluorescence (FAF). Blue FAF showed multiple papillary and iuxtapapillary bilateral hyper-FAF lesions, corresponding to hyperreflective thickening of the retinal nerve fiber layer, with internal optical empty spaces and posterior dense optical shadowing at SD-OCT. These imaging findings were consistent with retinal astrocytic hamartomas. Scotopic ERG response was significantly reduced in both eyes. Macular edema and absence of the retinal outer segments layer were also detectable. Retinal multi-modal imaging provides additional insights about retinal involvement of patients affected by CDG-Ia. In particular, this case shows the presence of multiple retinal astrocytic hamartomas

    Mikroperimetrie

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    Core Messages: Age related macular degeneration causis visual functions changes mainly quantified by visual acuity. Exact quantification of fundus-related retinal sensitivity and retinal fixation caracteristics are essential in AMD eyes. These parameters can not be obtained by measuring visual acuity. Microperimetry (fundus perimetry) allows a precise quantification of retinal sensitivity and fixation in macular disorders, and it allows accurate and reproducible follow-up examination. In both early and advanced AMD, microperumetry shows that retinal sensitivity changes are related to macular alterations detected by advanced macular imaging techniques (OCT, fundus autofluorescence). Moreover, microperimetry may detect macular derangement more precociously than other retinal imaging tecniques. Microperimetry is complementary to imaging in the diagnosis and follow-up of treated and untreated, providing more visual function information compared to visual acuity

    High myopia and choroidal neovascularization: microperimetry guided selection for photocoagulation

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    Purpose. To demonstrate that a precise identification of the center of fixation in patients with high myopia and choroidal neovascularization (CNV) can lead to a better selection of those eyes eligible for photocoagulation. Methods. Sixty-one eyes of 59 patients with high myopia and CNV were submitted to a fluorescein and indocyanine green angiography in order to identify the neovascular membrane and the foveal avascular zone (FAZ). The patients were also examined by fundus pe- rimetry using a scanning laser ophthalmoscope (SLO) to obtain an objective documentation of the center of fixation The angiography and the fundus perimetry with the fixation area were then aligned and superimposed. The relative distances among CNV, FAZ and the center of fixation were eventually calculated. Results. The distance between the CNV boundaries and the center of fixation was less than 200 μ in 45 eyes (74%), while in the remaining 16 eyes ranged between 200 μ and 750 μ (mean 380 μ). The center of fixation was within the FAZ in 38 eyes (62%), while in 23 eyes (38%) it could be identified out the edge of the FAZ at a mean distance of 320 μ. Conclusions. Due to the heavy changes of the posterior retina in high myopia, the conventional techniques of identification of the fixation area do not lead to satisfactory results. The fundus perimetry with the SLO demonstrates that in more than one-third of these patients the anatomic fovea and the functional fovea do not correspond. Furthermore, most cases of CNV lay at a short distance from the center of fixation which means that laser photocoagulation of the choroi- dal membrane could dramatically affect the visual acuity. None.,....

    Microperimetry in age: related macular degeneration

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    Abstract Age-related macular degeneration (AMD) is one of the major causes of visual loss and legal blindness in people over 55. Visual function tests are the cornerstone of visual function investigation and any therapeutic approach to AMD implies, as primary endpoint, the maintenance or improvement of visual function. The progression of visual impairment and the quantification of final residual visual function are currently determined by means of visual acuity quantification. The quantification of high-contrast visual acuity though has many drawbacks and cannot be considered a complete functional examination. Microperimetry is a non-invasive method used to analyse fixation and central visual field defects in a topographic related manner. The introduction of mesopic and more recently scotopic microperimetry, in research and clinical practice of macular disorders, now allows us to better investigate macular function as it strictly relates to macular morphology. We therefore can monitor the functional natural history and quantify the beneficial or detrimental effects of different therapies. The application of microperimetry in clinical studies has provided interesting diagnostic and prognostic information on functional macular changes in AMD patients. The present review brings new updates on the correlation between macular changes, mainly described with optical coherence tomography, and microperimetry changes in patients with AMD
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