retinoschisis


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retinoschisis

 [ret″ĭ-nos´kĭ-sis]
splitting of the retina, occurring in the nerve fiber layer (in juvenile form), or in the external plexiform layer (in adult form).

ret·i·nos·chi·sis

(ret'i-nos'ki-sis),
Degenerative splitting of the retina, with cyst formation between the two layers.
[retino- + G. schisis, division]

ret·i·nos·chi·sis

(ret'i-nos'ki-sis)
Degenerative splitting of the retina, with cyst formation between the two layers.
[retino- + G. schisis, division]

retinoschisis

Local splitting of the retina into two layers with an intervening space.

retinoschisis 

A vitreoretinal degeneration characterized by splitting of the retina into two layers. It occurs either as a hereditary disease or as an acquired condition (70% of these patients are hyperopic). The X-linked hereditary condition (called juvenile retinoschisis) affects only males and usually involves the macula with loss of central vision. The congenital condition is characterized by a splitting of the nerve fibre layer from the retina whereas the acquired form, which is the most common, results in a splitting at the outer plexiform layer. The latter usually begins in the temporal periphery appearing as a coalescence of microcystoid degenerations with a smooth transparent elevation and associated with an absolute scotoma. The condition may spread to involve the entire peripheral fundus. Holes in the two layers are common and are a sign of progression. The inner layer contains blood vessels and sometimes has small whitish flakes on it, which are called 'snowflakes'.
References in periodicals archive ?
On histopathologic examination, marked outer retinal atrophy and retinoschisis were identified as abnormal ophthalmic findings in both eyes.
(4,12) It is typically characterized by hyperpigmented RPE clumps that form along the retinal vascular arcades, areas of chorioretinal atrophy, cystoid or schisis-like changes in the fovea, central or peripheral retinoschisis, vitreous degeneration, and cataract.
For peripheral retinoschisis, a wide-field SD-OCT imaging technique or ERG may be good auxiliary methods to monitor progress.
Other applications of OCT in ophthalmology include retinal pigment epithelial detachment, epiretinal membranes (ERM)/ Macular pucker (cellophane maculopathy), vitreomacular traction, rhegmatogenous retinal detachment, macular pseudoholes, lamellar holes, full thickness holes, retinoschisis, age related macular degeneration, choroidal neovascular membranes, retinal angiomatosis, and disciform scars / subretinal fibrosis, etc.
When Jonathan had a routine eye test after applying for a motorbike licence he was referred to hospital where doctors told him he had a rare genetic condition known as X-linked retinoschisis and would go blind.
After the match, Kavanagh not only reiterated that the players were behind their boss but also revealed how Lawrence helped him when his sister Julie developed a serious eye condition called retinoschisis.
(29) This is of particular concern as eyes with increased axial length and deformation of the posterior segment can lead to several lesions including myopic choroidal neovascularisation, lacquer cracks, chorioretinal atrophy, posterior staphyloma and macular retinoschisis. (29,30) Macular neovascularisation has poor prognosis and often leads to severe loss of central vision.
Applied Genetic Technologies (AGTC) announced the completion of enrollment in a clinical study of the company's gene therapy product candidate, in collaboration with Biogen (BIIB), for the treatment of x-linked retinoschisis. This multicenter study is designed to evaluate the collaboration's AAV vector expressing retinoschisin in patients with XLRS caused by mutations in the RS1 gene.
Other ocular findings include topographic corneal steepening and irregular astigmatism [33], absent or rudimentary foveal avascular zone [34], optic disc drusen, retinoschisis and foveoschisis and retinitis pigmentosa (RP) [35, 36], crowded optic disk, chorioretinal folds, and retinal cysts [37], central retinal vein occlusion [38], increased subfoveal choroidal thickness [39], and abnormalities in the retinal layers' thickness and distribution [40, 41] (Figure 1).
evaluated the SD-OCT findings of three cases of X-linked juvenile retinoschisis (XLRS).
Moreover, one retinal specialist carefully reviewed these scans for abnormalities such as vitreoretinal tractions, retinoschisis, and lamellar macular holes.
Diseases covered include age-related macular degeneration, Leber congenital amaurosis, Stargardt disease, X-linked juvenile retinoschisis, retinal degeneration related to Usher syndrome and retinitis pigmentosa.