A syndrome of iris nodules, ectopic descemet’s membrane, and unilateral glaucoma

Abstract

In this report we wish to describe the clinical and histopathologic abnormalities in two patients who presented with unilateral glaucoma and pedunculated nodules, thought to be melanomas, arising in a sector of the iris. Pathologically both eyes showed nevus-like clusters on the iris covered by a hyaline layer (ectopic Descemet's membrane) extending from the posterior surface of the cornea. To our knowledge this syndrome has been referred to only once previously and that in a paper describing 'pseudomelanomas' of the iris by KLIEN (1941) whose second case was similar to our two. All three patients were adult women in whom the condition occurred idiopathically and unilaterally.We have no explanation for the pathogenesis of this syndrome but its recognition appears to be important since the pathologic abnormalities suggest a benign process while the clinical impression in our two cases indicated malignant melanomas requiring enucleation. KLIEN'S (1941) patient had an enucleation for advanced glaucoma and corneal ulceration. CASE REPORTSCase 1 S.S. (MEEI 144-75-80) Fifty-one year old woman who had noted a "film over the left eye" for one month. She denied any previous trouble with her eyes but had not had prior ophthalmic examination. When examined by an optometrist several years previously she had not been told of any unusual ocular abnormality. (The optometrist's records had been destroyed in the meantime.)Examination showed a normal right eye with an acuity of 20120 and an applanation tension of 16 mm.Hg. The left eye had an acuity of 20125 and a

Don’t it make my blue eyes brown: heterochromia and other abnormalities of the iris

“…133 A diffuse iris naevus may also be encountered in Cogan-Reese syndrome. This condition first described in 1969, 282 is a variant of the iridocorneal endothelial syndrome, and is characterised by unilateral glaucoma; abnormalities of the corneal endothelium; and iris changes, including, corectopia, ectropion uvea, pigmented nodules, and iris atrophy. 283 Iris naevi are generally indolent lesions, which remain asymptomatic throughout life.…”

Iridocorneal Endothelial Syndrome: Case Report of Chandler’s Variant

“…In 1956, Chandler (3) described a rare unilateral condition characterized by iris atrophy, corneal edema and glaucoma called Chandler's Syndrome, which was subsequently classified as a subtype of ICE syndrome. In turn, in 1976, Cogan and Reese (4) reported a similar condition associated to iris nodules, which they called Cogan-Reese Syndrome. Later studies showed that the three clinical conditions presented similar clinical condition and story, and therefore were classified as the spectra of the same syndrome: ICE syndrome, or endothelial iridocorneal syndrome.…”

Iridocorneo-endotheliales Syndrom (ICE-S): Klassifikation, Klinik und Diagnostik

“…Cogan-ReeseSyndrom [5] subsumiert. Der allen Erkrankungen zugrunde liegende gleiche Pathomechanismus erklärt einerseits fließende Übergänge, zum anderen auch nicht immer eindeutig voneinander unterscheidbare und zuordnungsfähige Krankheitsbilder.…”