The Dark Choroid in Posterior Retinal Dystrophies
References (13)
- et al.
Fundus flavimaculatus: clinical, functional and histopathologic observations
Am J Ophthalmol.
(1967) Le signe du silence choroïdien dans les dégénérescences tapéto-rétiniennes centrales examinées sous fluorescéine
Bul Soc Ophtalmol Fr
(1971)- et al.
Le signe du silence choroïdien dans les dégénérescences tapéto-rétiniennes posterieures
Doc Ophthalmol Proc Ser
(1976) - et al.
Leakage from retinal capillaries in hereditary dystrophies
Doc Ophthalmol Proc Ser
(1976) Flecked retinal diseases
Fundus fluorescein angiography in fundus flavimaculatus and Stargardt's disease
Acta Ophthalmol
(1979)
Cited by (20)
Bilateral visual loss, behavioral changes, and overlooking in a young child with stargardt disease: Neurodiagnostic considerations
2022, American Journal of Ophthalmology Case ReportsCitation Excerpt :These deposits disrupt the interdigitation and ellipsoid zones adjacent to the external limiting membrane, leading to thinning of the outer nuclear layer.12 Fluorescein angiography shows absence of choroidal fluorescence (termed a dark or silent choroid) in the majority of cases.14 Mucus membrane biopsy to look for intracellular inclusions, or peripheral blood smear to look for vacuolated lymphocytes were once standard confirmatory tests for Batten disease but have now been superseded by genetic testing.
Retinal flecks, dots and crystals
2016, Taylor and Hoyt's Pediatric Ophthalmology and Strabismus, Fifth EditionFlecked retina disorders
2012, Pediatric Ophthalmology and Strabismus: Expert Consult - Online and PrintPeripapillary Dark Choroid Ring as a Helpful Diagnostic Sign in Advanced Stargardt Disease
2010, American Journal of OphthalmologyCitation Excerpt :The presence of a dark ring was not related to the presence of diffuse posterior pole retinal atrophy, because both groups of patients had a similar prevalence of diffuse retinal atrophy (8.7% in patients with the dark ring sign, 5.2% in patients without the dark ring sign; P = .4462). The existence of peripapillary sparing in Stargardt disease has been observed by previous authors,9,11,12,14 and recent investigation concerning the peripapillary-sparing phenomenon in patients with ABCA4 disease has been conducted using autofluorescence imaging, which showed normal-appearing autofluorescence in the peripapillary region at all disease stages.18 Cideciyan and associates showed that lipofuscin accumulation is the first pathophysiologic process to manifest in Stargardt disease,4 but it is unclear why the peripapillary area remains a focus for lipofuscin pathologic features.
Progressive Cone and Cone-Rod Dystrophies: Phenotypes and Underlying Molecular Genetic Basis
2006, Survey of OphthalmologyCitation Excerpt :Peripheral RPE atrophy, retinal pigmentation, arteriolar attenuation, and optic disc pallor can be seen in the late stages of the disease process (Fig. 3). The dark choroid sign may be seen on fluorescein angiography.196 A tapetal-like sheen that may change in appearance on dark adaptation (Mizuo–Nakamura phenomenon) has been described in association with X-linked (XL) CORD.72
Indocyanine green angiography in Stargardt's flavimaculatus
1995, American Journal of Ophthalmology