This retrospective study published in the August issue of the Archives of Ophthalmology reports on the clinical features of 71 patients with a presumed diagnosis of iris melanoma who ultimately were found to have iridocorneal endothelial (ICE) syndrome. The authors' analysis confirms that ICE syndrome can indeed simulate iris melanoma. Their report also describes the differences between the two conditions.
Both conditions produce corectopia, ectropion iridis, iris distortion and glaucoma. Features more suggestive of ICE include corneal endothelial guttata-like changes, peripheral anterior synechia, iris atrophy and multidirectional corectopia/ ectropion iridis, which are rarely found with iris melanoma.
To conduct their study, they reviewed the records of all patients evaluated at the Ocular Oncology Service at Wills Eye Institute between July 1974 and August 2010 for codes for iris pseudomelanoma and iridocorneal endothelial syndrome. All 71 patients (71 eyes) they identified were referred for evaluation of a pigmented iris mass, suspected to be a melanoma.
The mass proved to be a combination of iris stromal atrophy in 41 cases (58 percent) with exposure or loss of the underlying iris pigment epithelium; ectropion iridis in 24 (34 percent), imparting a disfigured iris with dark-brown color; iris nodules in 5 (7 percent); traction elevation with iris distortion from peripheral anterior synechia in 57 (80 percent); and corectopia in 53 (75 percent), a feature commonly found with iris melanoma.
Additional features of ICE included corneal endothelial guttata-like changes in 33 (46 percent), corneal edema in seven (10 percent), iris pigment epithelial transillumination defects in 12 (17 percent), polycoria in one (1 percent) and secondary glaucoma with intraocular pressure higher than 22 mm Hg in seven (10 percent).
The authors conclude that additional endothelial imaging could assist in telling the difference between the benign condition of ICE syndrome and malignant iris melanoma. They say that differentiation of ICE syndrome from iris melanoma is important since the main therapeutic strategy for ICE syndrome-related glaucoma involves open surgical procedures, and these should be avoided in eyes with melanoma in order to prevent extraocular tumor spread.