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Choroidal Caverns: A Previously Unreported Optical Coherence Tomography Finding in Best Vitelliform Dystrophy. 2018

Adriano Carnevali, and Riccardo Sacconi, and Eleonora Corbelli, and Lea Querques, and Francesco Bandello, and Giuseppe Querques

Best vitelliform macular dystrophy (VMD) is an autosomal dominant inherited dystrophy, most frequently caused by mutations in the BEST1 gene. The authors describe "choroidal caverns," a previously unreported optical coherence tomography finding in the choriocapillary and choroid of Best VMD. Histopathologic examination in eyes with Best VDM shows extensive deposition of lipofuscin in the retinal pigment epithelium (RPE) throughout the retina and accumulation of fibrillar material under the RPE and in the choroid. This supports the hypothesis that choroidal caverns could be a sign of the degenerative process involving the choroid in eyes with Best VMD. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:284-287.].

UI MeSH Term Description Entries
D008297 Male Males
D002829 Choroid The thin, highly vascular membrane covering most of the posterior of the eye between the RETINA and SCLERA. Choriocapillaris,Haller Layer,Haller's Layer,Sattler Layer,Sattler's Layer,Choroids
D005451 Fluorescein Angiography Visualization of a vascular system after intravenous injection of a fluorescein solution. The images may be photographed or televised. It is used especially in studying the retinal and uveal vasculature. Fluorescence Angiography,Fundus Fluorescence Photography,Angiography, Fluorescein,Angiography, Fluorescence,Fluorescence Photography, Fundus,Photography, Fundus Fluorescence
D005654 Fundus Oculi The concave interior of the eye, consisting of the retina, the choroid, the sclera, the optic disk, and blood vessels, seen by means of the ophthalmoscope. (Cline et al., Dictionary of Visual Science, 4th ed) Fundus of the Eye,Ocular Fundus,Fundus, Ocular
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D012171 Retinal Vessels The blood vessels which supply and drain the RETINA. Pecten Oculi,Retinal Vasculature,Retinal Blood Vessels,Retinal Blood Vessel,Retinal Vasculatures,Retinal Vessel,Vasculature, Retinal,Vessel, Retinal,Vessel, Retinal Blood
D055213 Retinal Pigment Epithelium The single layer of pigment-containing epithelial cells in the RETINA, situated closely to the tips (outer segments) of the RETINAL PHOTORECEPTOR CELLS. These epithelial cells are macroglia that perform essential functions for the photoreceptor cells, such as in nutrient transport, phagocytosis of the shed photoreceptor membranes, and ensuring retinal attachment. Epithelium, Retinal Pigment,Pigment Epithelium, Retinal
D057826 Vitelliform Macular Dystrophy Autosomal dominant hereditary maculopathy with childhood-onset accumulation of LIPOFUSION in RETINAL PIGMENT EPITHELIUM. Affected individuals develop progressive central acuity loss, and distorted vision (METAMORPHOPSIA). It is associated with mutations in bestrophin, a chloride channel. Adult-Onset Vitelliform Macular Dystrophy,Best Disease,Best Macular Dystrophy,Best Vitelliform Macular Dystrophy,Best's Disease,Foveomacular Dystrophy, Adult-Onset,Foveomacular Dystrophy, Adult-Onset, With Choroidal Neovascularization,Juvenile-Onset Vitelliform Macular Dystrophy,Macular Degeneration, Polymorphic Vitelline,Macular Dystrophy, Vitelliform,Macular Dystrophy, Vitelliform, Adult-Onset,Vitelliform Dystrophy,Vitelliform Macular Dystrophy Type 2,Vitelliform Macular Dystrophy, Adult-Onset,Vitelliform Macular Dystrophy, Early-Onset,Vitelliform Macular Dystrophy, Juvenile-Onset,Adult Onset Vitelliform Macular Dystrophy,Adult-Onset Foveomacular Dystrophies,Adult-Onset Foveomacular Dystrophy,Disease, Best,Disease, Best's,Dystrophies, Adult-Onset Foveomacular,Dystrophies, Vitelliform Macular,Dystrophy, Adult-Onset Foveomacular,Dystrophy, Best Macular,Dystrophy, Vitelliform Macular,Foveomacular Dystrophies, Adult-Onset,Foveomacular Dystrophy, Adult Onset,Juvenile Onset Vitelliform Macular Dystrophy,Macular Dystrophies, Vitelliform,Macular Dystrophy, Best,Vitelliform Macular Dystrophies,Vitelliform Macular Dystrophy, Adult Onset,Vitelliform Macular Dystrophy, Early Onset,Vitelliform Macular Dystrophy, Juvenile Onset
D041623 Tomography, Optical Coherence An imaging method using LASERS that is used for mapping subsurface structure. When a reflective site in the sample is at the same optical path length (coherence) as the reference mirror, the detector observes interference fringes. OCT Tomography,Optical Coherence Tomography,Coherence Tomography, Optical,Tomography, OCT

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