PubMed · 12843902
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E Frau. 2003. [Choroidal melanomas].. https://pubmed.ncbi.nlm.nih.gov/12843902/
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Fibulin-5 is an extracellular matrix glycoprotein that participates in elastogenesis. Mutations in the gene for fibulin-5 have been found to be associated with age-related macular degeneration. Little is known, however, about the expression of this gene in normal eyes or eyes with age-related macular degeneration. In this study, we evaluated the expression of the fibulin-5 protein in human donor eyes and localized this protein to Bruch's membrane and the intercapillary pillars of the choriocapillaris in normal eyes. In eyes with age-related macular degeneration, fibulin-5 was localized to pathologic basal deposits beneath the retinal pigment epithelium as well as some small drusen. These results suggest that fibulin-5 may promote extracellular deposit formation in macular degeneration.
Age-related macular degeneration (AMD) is the leading cause of blindness in the western world. Over the last decade, there have been significant advances in the management of exudative AMD with the introduction of anti-VEGF drugs; however, many patients with exudative AMD continue to lose vision and there are no effective treatments for advanced exudative AMD or geographic atrophy. Initial attempts at macular reconstruction using cellular transplantation have not been effective in reversing vision loss. Herein we discuss the current status of surgical attempts to reconstruct damaged subretinal anatomy in advanced AMD. We reinforce the concept of maculoplasty for advanced AMD, which is defined as reconstruction of macular anatomy in patients with advanced vision loss. Successful maculoplasty is a three-step process that includes replacing or repairing damaged cells (using transplantation, translocation or stimulation of autologous cell proliferation); immune suppression (if allografts are used to replace damaged cells); and reconstruction or replacement of Bruch's membrane (to restore the integrity of the substrate for proper cell attachment). In the current article we will review the rationale for maculoplasty in advanced AMD, and discuss the results of initial clinical attempts at macular reconstruction. We will then discuss the role of Bruch's membrane damage in limiting transplant survival and visual recovery, and discuss the effects of age-related changes within human Bruch's membrane on the initial attachment and subsequent proliferation of transplanted cells. We will discuss attempts to repair Bruch's membrane by coating with extracellular matrix ligands, anatomic reconstitution of the inner collagen layer, and the effects of Bruch's membrane reconstruction of ultrastuctural anatomy and subsequent cell behavior. Lastly, we will emphasize the importance of continued efforts required for successful maculoplasty.
PURPOSE: To further define the origin of the "outer red line" (ORL) present in retinal optical coherence tomograms. DESIGN: Laboratory investigation. METHODS: Eleven formalin-fixed caps of seven human eyes previously enucleated for choroidal melanoma at the Wilmer Eye Institute were studied. The ORL evaluation consisted of sequential, surgical elimination of the retinal pigment epithelium, Bruch's membrane (BM), and choroid in separate, but adjacent, areas. Optical coherence tomography (OCT) images through each of these areas were correlated with corresponding histologic sections. The relative contribution of the retinal pigment epithelium, BM, and choroid to the ORL in retinal OCT3 images was evaluated. RESULTS: In the specimens examined, the ORL correlated anatomically with a highly reflective optical surface at the level of the outer retina, and did not appear to represent a specific anatomic layer. The reflecting layers appeared to be composed of the retinal pigment epithelium, BM, and inner choroid. The relative contribution of BM together with the inner choroid to the ORL appeared greater than that of the retinal pigment epithelium. CONCLUSIONS: This study suggests that the ORL present in retinal OCT3 images and generally associated with the retinal pigment epithelium/BM/inner choroidal complex appears to indicate a highly reflective optical surface which, when eye layers are normally configured, represents this complex of anatomic structures with a predominant contribution from BM and inner choroid. The findings of this study suggest a potential role for OCT in the eye pathology laboratory.