juvenile retinoschisis


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ju·ve·nile ret·i·nos·chi·sis

retinoschisis occurring before 10 years of age and within the nerve-fiber layer, with frequent macular involvement; at first, the inner wall is a translucent veillike membrane, but it becomes denser and may render the retina white; autosomal recessive inheritance. There is a form of this condition in middle age that is X linked [MIM*312700] and a rare autosomal dominant form [MIM*180270].

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'.
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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.
X-linked juvenile retinoschisis (XLRS) is a hereditary retinal dystrophy with a prevalence ranging between 1:5,000 and 1:25,000.
Positional cloning of the gene associated with X-linked juvenile retinoschisis.
X-linked juvenile retinoschisis was first described by Haas in 1898.
In X-linked juvenile retinoschisis there is a split between the nerve fibre and ganglion cell layers of the retina.
Macular retinoschisis may also form due to various reasons such as tractional causes, venous occlusive diseases, cystoid macular edema, and juvenile retinoschisis.

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