Fovea centralis, located at the center of the macula, is packed with cone photoreceptors and is responsible for central visual acuity. Isolated foveal photoreceptor disruption may occur in a variety of hereditary, degenerative, traumatic, and toxic chorioretinal diseases. These have been known previously by multiple synonyms including macular microhole, foveal spot, and outer foveal microdefects. A common clinical feature underlying these conditions is the presence of apparently normal fovea or subtle hypopigmented lesion at the foveal or juxtafoveal area. A detailed history along with high-resolution optical coherence tomography is often helpful to derive a conclusive diagnosis in majority of these cases. Focal photoreceptor disruption usually involves loss or rarefaction of ellipsoid/interdigitation zone, either in isolation or associated with external limiting membrane or retinal pigment epithelium disruption in the fovea. Vitreomacular interface (VMI) disorders including vitreomacular traction, posterior vitreous detachment, epiretinal membrane, and impending macular hole possibly remain the most common cause. Retinal dystrophies such as cone dystrophy, occult macular dystrophy, and achromatopsia may present with diminution of vision and normal appearing fundus in a younger age group. Other causes include photic retinopathy (e.g., from a history of sun gazing or laser pointer exposure), blunt trauma, drug exposure (e.g., poppers maculopathy or tamoxifen retinopathy), and acute retinal pigment epitheliopathy (ARPE). Visual prognosis depends on the underlying etiology with complete recovery common in the subset of patients with VMI, and ARPE, whereas persistent outer retinal defects are the rule in other conditions. We discuss the differential diagnoses that lead to isolated foveal photoreceptor defects. Identifying and understanding the underlying disease processes that cause foveal photoreceptor disruption may help predict visual prognosis.
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http://dx.doi.org/10.1016/j.survophthal.2023.03.003 | DOI Listing |
Invest Ophthalmol Vis Sci
December 2024
Scheie Eye Institute, Department of Ophthalmology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States.
Purpose: The purpose of this study was to determine the natural history of the photoreceptor disease in a large group of pediatric patients with RHD12-associated Leber congenital amaurosis (RDH12-LCA), to estimate the changes expected over the duration of a clinical trial, and to define the relationship between the photoreceptor loss and visual dysfunction.
Methods: Forty-six patients representing 36 families were included. The great majority of patients were under the age of 18 years.
Invest Ophthalmol Vis Sci
December 2024
UCL Institute of Ophthalmology, University College London, London, England, United Kingdom.
Purpose: Although it is well known that photoreceptor damage and color vision loss occur in patients with diabetic retinopathy (DR), the relationship between structural and functional changes in diabetes mellitus (DM) remains unclear. Using highly sensitive measures of photoreceptor structure and function, we aim to determine whether early loss of color sensitivity in DM is also accompanied by decreased cone density.
Methods: Monocular data from 26 patients with DM and 25 healthy controls were examined to assess cone photoreceptor metrics, using confocal adaptive optics scanning light ophthalmoscopy, and red/green (RG) and yellow/blue (YB) color vision thresholds, using the Colour Assessment and Diagnosis test.
Invest Ophthalmol Vis Sci
December 2024
Department of Ophthalmology & Vision Science, School of Medicine, University of California Davis, Sacramento, California, United States.
Invest Ophthalmol Vis Sci
December 2024
Scheie Eye Institute, University of Pennsylvania, Perelman School of Medicine, Philadelphia, Pennsylvania, United States.
Purpose: To evaluate the effect on outer retina integrity of foveal sparing internal limiting membrane (ILM) peeling compared to standard ILM peeling on proliferative diabetic retinopathy (PDR) eyes affected by diabetic macular edema (DME) and epiretinal membrane (ERM).
Materials And Methods: Eyes diagnosed with PDR, DME and ERM eligible for vitrectomy were prospectively recruited and randomly assigned to either foveal sparing (FS) group and no foveal sparing (nFS) group. Ellipsoid zone (EZ) lesion size, EZ reflectivity and external limiting membrane (ELM) integrity and angular sign of Henle Fiber Layer Hyperreflectivity (ASHH) were assessed preoperatively and 1 year postoperatively.
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