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Progressive bifocal chorioretinal atrophy, also known for its abbreviations PBCRA or CRAPB, [1] is a rare, slowly progressive, autosomal dominant syndrome characterized by relatively large-sized atrophic hole-shaped lesions in the macular and nasal retina, myopia, low visual acuity, and nystagmus.
Presumed ocular histoplasmosis syndrome (POHS) is a syndrome affecting the eye, which is characterized by peripheral atrophic chorioretinal scars, atrophy or scarring adjacent to the optic disc and maculopathy. The loss of vision in POHS is caused by choroidal neovascularization.
The retinal circulation, on the other hand, derives its circulation from the central retinal artery, also a branch of the ophthalmic artery, but passing in conjunction with the optic nerve. [3] They branch in a segmental distribution to end arterioles and not anastomoses. This is clinically significant for diseases affecting choroidal blood supply.
The definition of the classes and Frisén scale used for optic disc edema diagnosis are listed below: Class 0 < 0.50 diopter cycloplegic refractive change; No evidence of optic-disc edema, nerve sheath distention, choroidal folds, globe flattening, scotoma or cotton-wool spots compared to baseline; Class 1 Repeat OCT and visual acuity in 6 weeks
The optic nerve can be damaged when exposed to direct or indirect injury. Direct optic nerve injuries are caused by trauma to the head or orbit that crosses normal tissue planes and disrupts the anatomy and function of the optic nerve; e.g., a bullet or forceps that physically injures the optic nerve.
Dominant optic atrophy was first described clinically by Batten in 1896 and named Kjer’s optic neuropathy in 1959 after Danish ophthalmologist Poul Kjer, who studied 19 families with the disease. [3] Although dominant optic atrophy is the most common autosomally inherited optic neuropathy (i.e., disease of the optic nerves), it is often ...
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The optic disc may appear mildly hyperemic with small splinter hemorrhages on or around the disc, or may appear nearly normal. Optic atrophy typically develops later and may appear mild. In later stages the optic atrophy can become severe, which indicates less opportunity for recovery. [3]