Irido-corneal-endothelial syndrome: a confocal and anterior segment coherent tomography differential diagnosis

Essential iris atrophy represents one of the phenotypic expressions of irido-corneal-endothelial syndrome (ICE-S). Despite some typical clinical features, differential diagnosis of the endothelial alteration at early stage of the disease is not easy also with a careful slit lamp examination, with different overlapping clinical aspects. We report differential diagnosis by in vivo confocal microscopy and anterior segment optical coherent tomography, between ICE-syndrome related endotheliopathy in a 62 year male patient and other pathologies involving corneal endothelium with different therapeutic and prognostic implications: Posterior polymorphous corneal dystrophy, cornea guttata and Fuch’s endothelial dystrophy. Even if the ICS-Syndrome is generally unilateral and non familial, a bilateral occurrence may be possible. The disease may progress leading to secondary glaucoma and corneal decompensation. In this contest, early diagnosis becomes fundamental to monitor corneal endothelial and stromal changes, IOP variation and pupil distortion in order to establish a correct therapeutic strategy. Differential analysis with posterior polymorphous corneal dystrophy, cornea guttata and Fuch’s endothelial dystrophy is necessary, especially in bilateral disease due to different prognosis and management of these pathologies. Indeed in this clinical case, despite the apparent absence of stromal modifications at biomicroscopic examination, in vivo confocal microscopy allowed us to detect the presence of a stromal sub-clinical edema, explaining patients visual fluctuations with a consequent customized therapeutic strategy and prognostic evaluation.