Abstract
PurposeTo report the demographic profile, clinical features, and prevalence of glaucoma and its management in patients with Iridocorneal endothelial (ICE) syndrome.MethodsRetrospective review of 203 consecutive subjects with ICE syndrome at a tertiary eye care centre between January 1988 and June 2013.ResultsICE syndrome was present in 223 eyes of 203 subjects, 124 (61%) were female and 79 (39%) were male. The median age at presentation was 43 years (1st (Q1) and 3rd (Q3) quartile; 34, 51 years). ICE syndrome was unilateral in 183 (90%) subjects, and bilateral in 20 (10%) subjects. The most common clinical variant was progressive iris atrophy (PIA, 115; 52% eyes), followed by Chandler syndrome (CS, 87; 39% eyes) and Cogan-Reese syndrome (CRS, 21; 9% eyes). Glaucoma was found in 156 eyes (70%) at presentation and the median (Q1, Q3) intraocular pressure in eyes with glaucoma was 24 (16, 38) mm Hg. Seven eyes developed glaucoma during the follow-up period, increasing the percentage of eyes with glaucoma to 73%. Intraocular pressure was managed medically in 81 eyes (50%) and the other 82 eyes (50%) required surgical intervention. Corneal edema was present in 124 eyes (56%) of which, 32 eyes (14%) required keratoplasty.ConclusionsIn our study on ICE syndrome in Indian population, the presentation was predominantly uniocular and more common in middle aged women. Progressive iris atrophy was the most common clinical variant. ICE syndrome was associated with glaucoma in over 70% of the eyes and half of the eyes had corneal edema.
Highlights
The iridocorneal endothelial (ICE) syndrome is a disease spectrum which includes Chandler syndrome (CS), Progressive iris atrophy (PIA) and Cogan-Reese syndrome (CRS)
ICE syndrome was associated with glaucoma in over 70% of the eyes and half of the eyes had corneal edema
The ICE syndrome is a progressive disease of the corneal endothelium and is associated with variable features like corneal edema, hammered silver appearance of the posterior corneal surface, iris abnormalities ranging from corectopia to polycoria or iris nodules, broad based peripheral anterior synechiae (PAS) and secondary glaucoma [3]
Summary
The iridocorneal endothelial (ICE) syndrome is a disease spectrum which includes Chandler syndrome (CS), Progressive iris atrophy (PIA) and Cogan-Reese syndrome (CRS). The ICE syndrome is a progressive disease of the corneal endothelium and is associated with variable features like corneal edema, hammered silver appearance of the posterior corneal surface, iris abnormalities ranging from corectopia to polycoria or iris nodules, broad based peripheral anterior synechiae (PAS) and secondary glaucoma [3]. It typically presents as a unilateral condition, but bilateral or subclinical abnormalities in the corneal endothelium of the fellow eye are not uncommon [4, 5]. It usually manifests in the early to middle adulthood and is seen more frequently in females [3, 6]
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