Especially in females, bilateral involvement and FSHD should be ruled out.Ī montage of fundus image in a case of Coat's disease showing central and peripheral exudation. Other genes which have been implicated include:Ĭoats like response has been noted in cases in patients with Turner Syndrome (XO), Senior Loken Syndrome, retinitis pigmentosa (both syndromic and isolated), facioscapulohumeral dystrophy (FSHD), and Linear Scleroderma/ Parry Romberg Syndrome. No genetic basis has been found but there has been described chromosomal instability in chromosomes 3 and 13. These abnormal vessels are leaky and there is exudation in various degrees. The usual location of these vessels is within the temporal retina. The disease is primarily due to aneurysms and telangiectatic vessels. Type 1 idiopathic macular telegiectasia is now considered the same disease as Coats disease. Lebers miliary aneurysm is considered to be a milder variant of the Coats disease, which usually does not have massive subretinal exudation, hemorrhage or exudative retinal detachment.
It’s more frequent in males (at least 3:1) than females, and in patients younger than 8 years old, even though it has been observed in infants as well as older patients. 2.4.3 Shields' classification (usually followed) Ĭoats disease is usually characterized by unilateral (95%), progressive development of abnormal vessels in the retina of the affected individuals.
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