ICE Syndrome

Iridocorneal endothelial (ICE) syndrome comprises a spectrum of three clinical variations: Chandler syndrome, progressive iris atrophy and Cogan-Reese syndrome. Although initially described separately and with distinct clinical manifestations, these variants are linked by a fundamental defect of the corneal endothelium as shown by histopathologic and ultrastructural studies. This acquired disorder is usually unilateral and affects predominantly women in early to middle adulthood, although bilateral cases and occurrence in a child1 have been reported.

Visual loss associated with ICE syndrome usually is secondary to either corneal decompensation or refractory glaucoma, which frequently requires surgical intervention that has variable success rates.