scholarly journals Unilateral Retinal Pigment Epithelium Dysgenesis: Long-Term Natural Evolution and Multimodal Imaging

2019 ◽  
Vol 6 (1) ◽  
pp. 74-78 ◽  
Author(s):  
Paraskevi Riga ◽  
Anna Dastiridou ◽  
Despoina Tzetzi ◽  
Sofia Androudi ◽  
Periklis Brazitikos
Keyword(s):  
Retinal Pigment Epithelium ◽  
Multimodal Imaging ◽  
Vascular Tissue ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Early Adulthood ◽  
Retinal Disease ◽  
Severe Visual Loss ◽  
Retinal Pigment Epithelium Atrophy

Purpose: To present the natural course and describe the characteristic findings of a case of unilateral retinal pigment epithelium dysgenesis (URPED) and highlight the optical coherence tomography angiography characteristics of this rare fundus pattern. Methods: Case report. Results: A 52-year-old male was referred 8 years ago to our clinic due to a distinctive unilateral lesion in his left fundus, of which he was aware from early adulthood. Clinical evaluation revealed an irregularly shaped patch of retinal pigment epithelium atrophy surrounding his left optic nerve, with a characteristic scalloped border and with severe distortion of the overlying retinal and vascular tissue. Retinal pigment epithelial hyperplasia was noted in the margin as well as in distinct lacunae clustered around the periphery of the lesion. Right fundus examination was normal. The patient was diagnosed with URPED and was followed annually with multimodal imaging ever since. No choroidal neovascularization or other complication was noted. However, the lesion appeared to slowly but steadily grow to eventually affect the foveal region causing severe visual loss. Best corrected visual acuity was 20/40 at baseline and 20/200 at last follow-up 8 years after the original diagnosis. Conclusion: URPED is a rare retinal disease with unique clinical characteristics that can progress relentlessly even in the absence of secondary complications.

scholarly journals Loss of melanoregulin (MREG) enhances cathepsin-D secretion by the retinal pigment epithelium

2013 ◽  
Vol 30 (3) ◽  
pp. 55-64 ◽  
Author(s):  
LAURA S. FROST ◽  
VANDA S. LOPES ◽  
FRANK P. STEFANO ◽  
ALVINA BRAGIN ◽  
DAVID S. WILLIAMS ◽  
...  
Keyword(s):  
Retinal Pigment Epithelium ◽  
Cathepsin D ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Retinal Disease ◽  
Protective Role ◽  
Phagocytic Cells ◽  
Phagocytic Capacity ◽  
Photoreceptor Outer Segments ◽  
Age Dependent

AbstractCathepsin-D (Cat-D) is a major proteolytic enzyme in phagocytic cells. In the retinal pigment epithelium (RPE), it is responsible for the daily degradation of photoreceptor outer segments (POSs) to maintain retinal homeostasis. Melanoregulin (MREG)-mediated loss of phagocytic capacity has been linked to diminished intracellular Cat-D activity. Here, we demonstrate that loss of MREG enhances the secretion of intermediate Cat-D (48 kDa), resulting in a net enhancement of extracellular Cat-D activity. These results suggest that MREG is required to maintain Cat-D homeostasis in the RPE and likely plays a protective role in retinal health. In this regard, in the Mregdsu/dsu mouse, we observe increased basal laminin. Loss of the Mregdsu allele is not lethal and therefore leads to slow age-dependent changes in the RPE. Thus, we propose that this model will allow us to study potential dysregulatory functions of Cat-D in retinal disease.

scholarly journals New antioxidant SkQ1 is an effective protector of rat eye retinal pigment epithelium and choroid under conditions of long-term organotypic cultivation

2012 ◽  
Vol 01 (02) ◽  
pp. 31-37 ◽  
Author(s):  
E. N. Grigoryan ◽  
Y. P. Novikova ◽  
O. S. Gancharova ◽  
O.V. Kilina ◽  
P. P. Philippov
Keyword(s):  
Retinal Pigment Epithelium ◽  
Pigment Epithelium ◽  
Retinal Pigment

Long-term treatment with anti-VEGF does not induce cell aging in primary retinal pigment epithelium

2018 ◽  
Vol 171 ◽  
pp. 1-11 ◽  
Author(s):  
Johann Schottler ◽  
Niklas Randoll ◽  
Ralph Lucius ◽  
Amke Caliebe ◽  
Johann Roider ◽  
...  
Keyword(s):  
Retinal Pigment Epithelium ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Term Treatment ◽  
Cell Aging ◽  
Anti Vegf ◽  
Long Term Treatment

Multimodal imaging evaluation of combined hamartoma of the retina and retinal pigment epithelium

2019 ◽  
Vol 30 (3) ◽  
pp. 595-599 ◽  
Author(s):  
Andrea Scupola ◽  
Gabriela Grimaldi ◽  
Maria G Sammarco ◽  
Paola Sasso ◽  
Michele Marullo ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Retinal Pigment Epithelium ◽  
Multimodal Imaging ◽  
Optical Coherence Tomography Angiography ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Case Series ◽  
Variable Degree ◽  
Optical Coherence ◽  
Imaging Evaluation

Purpose: Combined hamartoma of the retina and retinal pigment epithelium is a rare benign tumor characterized by a variable combination of glial, vascular, and pigmented components. The purpose of our study was to analyze the features of combined hamartoma of the retina and retinal pigment epithelium on optical coherence tomography angiography. Methods: Small case series of two cases of combined hamartoma of the retina and retinal pigment epithelium with macular and optic nerve involvement, evaluated with multimodal imaging including optical coherence tomography, fluorescein angiography, and optical coherence tomography angiography. Results: On optical coherence tomography, combined hamartoma of the retina and retinal pigment epithelium is characterized by disruption of the inner neurosensory retina and a variable degree of involvement of the external retina. Optical coherence tomography angiography showed diffuse alterations of the retinal vessels of the superficial and deeper layers, extended to the peripapillary area. Vessel abnormalities included increased tortuosity and caliber of vessels, vascular traction, and vessel stretching within the lesion. Conclusion: Optical coherence tomography angiography allows in-depth multilayer analysis of tumor vascular network, highlighting the fine abnormalities of retinal vasculature characteristic of combined hamartoma of the retina and retinal pigment epithelium.

scholarly journals LONG-TERM SD-OCT/SLO IMAGING OF NEURORETINA AND RETINAL PIGMENT EPITHELIUM AFTER SUBTHRESHOLD INFRARED LASER TREATMENT OF DRUSEN

Retina ◽  
2011 ◽  
Vol 31 (2) ◽  
pp. 235-242 ◽  
Author(s):  
Francesca Mojana ◽  
Manpreet Brar ◽  
Lingyun Cheng ◽  
Dirk-Uwe G Bartsch ◽  
William R Freeman
Keyword(s):  
Retinal Pigment Epithelium ◽  
Laser Treatment ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Infrared Laser ◽  
Sd Oct
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