Iridocorneal Endothelial Syndrome Masquerading as Iris Melanoma in 71 Cases

Abstract

Objective: To describe the clinical features of iridocorneal endothelial (ICE) syndrome in a group of patients referred because of suspected iris melanoma. Methods: In a noncomparative case series, we performed medical record review for clinical features of ICE syndrome in 71 patients. Results: At presentation, the median patient age was 54 years. All patients were referred for evaluation of a pigmented iris mass, suspected to be a melanoma. The iris color was blue or green in 51 (72%) and brown in 20 (28%). The mass proved to be a combination of iris stromal atrophy in 41 cases (58%) with exposure or loss of the underlying iris pigment epithelium; ectropion iridis in 24 (34%), imparting a disfigured iris with darkbrown color; iris nodules in 5 (7%); traction elevation with iris distortion from peripheral anterior synechia in 57 (80%); and corectopia in 53 (75%), a feature commonly found with iris melanoma. The mean extent of iris atrophy was 2 clock hours. Ectropion iridis was unidirectional in 10 and multidirectional in 14. Additional features of ICE included corneal endothelial guttata-like changes in 33 (46%), corneal edema in 7 (10%), iris pigment epithelial transillumination defects in 12 (17%), polycoria in 1 (1%), and secondary glaucoma with intraocular pressure higher than 22 mm Hg in 7 (10%). Conclusions: Iridocorneal endothelial syndrome can simulate iris melanoma. Features more suggestive of ICE syndrome include corneal endothelial guttata-like changes and edema, peripheral anterior synechia, multidirectional ectropion iridis, and iris atrophy.

The morphology of angle dysgenesis assessed by ultrasound biomicroscopy and its relationship with glaucoma severity and mutant genes in Axenfeld-Rieger syndrome

“…In this study, we considered 6 types of iridocorneal angle morphology of ARS. High insertion of the peripheral iris was observed in all eyes, which has also been reported by others as being a potential risk factor for glaucoma ( 5 , 21 ). Other abnormalities, including prominent Schwalbe’s line in type B, iris process in type C, trabecular-iris synechia in type D, and peripheral iridocorneal adhesion in type E, although different in morphology, all originate from the deposition and contraction of the basement membrane by the primordial endothelial layer.…”

Distinctive Findings in a Patient With Axenfeld–Rieger Syndrome Using High-Resolution AS-OCT

“…We propose that the membrane detected by AS-OCT in our case may represent this retained endothelial layer. Furthermore, the membrane was located near the pupil edge where the ectropion uveae was pronounced, which may support the theory that contraction of the abnormally retained primordial endothelial layer results in several of the clinical characteristics of Axenfeld–Rieger syndrome, such as pupil distortion and ectropion uveae 1…”

Chronic angle closure glaucoma with an atypical iris-pupillary membrane

“…On the other hand, iris melanoma was more often suspected in patients with iris nodules, corectopia, ectropion uveae, iris atrophy, and closed-angle glaucoma. 4 In this series, the tumour itself was often a solitary, nodular, pigmented mass inside the iris stroma with surrounding vascularization.…”