Cogan-Reese syndrome


A very rare eye disorder where various eye anomalies may impair vision. Cogan-Reese Syndrome is an extremely rare, auto-immune syndrome, affecting vision and hearing. The vision component of this eye disorder is characterized by a matted or smudged appearance to the surface of the iris and development of small colored lumps on the iris. It is also characterized by the attachment of portions of the iris to the cornea and/or increased pressure in the eye (glaucoma). Secondary glaucoma caused by Cogan-Reese Syndrome may lead to vision loss. This disorder, which develops slowly over time, most frequently appears in young and middle-aged females and usually affects only one eye.


The patient with ICE syndrome is typically a younger female. It is most common in Caucasians, and there typically is no family history of this disease. It is most commonly a unilateral phenomenon, but bilateral cases have been documented.1It tends to manifest in early to middle adulthood.2 Common findings include a beaten bronze appearance to the corneal endothelium, corneal edema, iris atrophy and hole formation, corectopia, prominent iris nevus, and peripheral anterior synechiae with progressive angle closure and secondary closed-angle glaucoma. Vision may be unaffected or may be reduced due to endotheliopathy or glaucoma. The patient may occasionally complain of monocular diplopia secondary to an exposed area of full thickness iris atrophy creating another entrance for light to enter the eye (polycoria).


This, along with ensuing corneal edema, is a cause of vision reduction in these patients. The endothelium is most affected in essential iris atrophy. Some endo-thelial changes such as migration and reparative processes are identifiable, as is the presence of cell necrosis and chronic inflammation.