Rev Bras Oftalmol.2026;85:e0018

Cogan-Reese syndrome and iridocorneal endothelial membrane syndrome: case reports and differential diagnosis

Caio Henrique Peres , Marina Siqueira , Nicoli Lopes de , Isabella Passarelli Giabardo , Marcello Novoa Colombo , Priscilla Fernandes

DOI: 10.37039/1982.8551.20260018

ABSTRACT

This article is aimed at presenting the clinical features and management of two distinct subtypes of Iridocorneal Endothelial Syndrome (ICE), highlighting the importance of differential diagnosis and individualized treatment. It shows two case reports of female patients with progressive visual loss. Ophthalmologic examination was performed, including biomicroscopy, gonioscopy, and specular microscopy. Treatment was defined according to clinical need. The first patient, diagnosed with Cogan-Reese Syndrome, presented with pigmented nodules on the iris, uveal ectropion, and posterior synechiae, being treated with hypotensive eye drops. The second patient, consistent with Progressive Essential Iris Atrophy, exhibited iris atrophy, polycoria, and corneal edema, requiring surgery with a drainage tube implant for intraocular pressure control. Specular microscopy revealed pleomorphism and polymegathism in both cases, indicating endothelial dysfunction. The unilateral presentation and predominance in women support the epidemiology described in the literature. The differential diagnosis includes conditions such as pigment dispersion syndrome, iris melanoma, and iris nevi. Early diagnosis, correct subtype classification, and individualized therapeutic approaches are essential to prevent irreversible glaucomatous damage and preserve visual function.

Cogan-Reese syndrome and iridocorneal endothelial membrane syndrome: case reports and differential diagnosis

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