Clinical Profile of Iridocorneal Endothelial Syndrome at a Tertiary Care Eye Centre

ABSTRACT

Irido-Corneal Endothelial (ICE) syndrome is an uncommon clinical entity which includes corneal endothelial damage, obstruction of anterior chamber angle, iris atrophy, and polycoria.ICE syndrome is a spectrum of clinical entities namely Progressive Iris Atrophy (PIA), Cogan Reese Syndrome (CRS), and Chandler Syndrome (CS). It is considered to be sporadic, usually unilateral and diagnosed in young adults, females and occasionally in children. Corneal decompensation and glaucoma are the commonest causes of vision loss in patients with ICE syndrome.Medical management of this condition is usually with topical antiglaucoma medication. In most cases medical therapy fails and requires Glaucoma Filtration surgery. Glaucoma drainage devices are the mainstay of treatment and many patients require keratoplasty. We wanted to study the clinical profile of ICE syndrome patients, its association with glaucoma in Indian population and compare the findings with those of previous studies. METHODSIn this retrospective study, data of 50 consecutive patients having ICE syndrome at a tertiary eye care centre was collected. RESULTSOut of 50 patients, 28 (56%) were females, aged 7 to 65 years (median - 40.72 years). 49 patients (98%) had unilateral presentation, and one had bilateral involvement (2%). Progressive Iris Atrophy (PIA) was seen in 20 (40%), Chandlers Syndrome (CS) in 20 (40%) and 10 eyes (20%) had Cogan-Reese Syndrome (CRS). 46 (92%) eyes had glaucoma. Mean Intra-Ocular Pressure (IOP) was 23.70 ± 11.7 mm of Hg. Mean AGM used was 2.44 ± 1.3. 44 (88%) eyes were diagnosed to have glaucoma at presentation, 1 (2%) eye was diagnosed with glaucoma during follow up. 20 (40%) eyes were managed medically for IOP control. 8 (16%) eyes had undergone previous glaucoma surgery. 20 (40%) eyes underwent glaucoma surgery for IOP control. 15 (30%) eyes had Trabeculectomy with Mitomycin C, 5 (10%) had Ahmed glaucoma valve. 4 (8%) eyes underwent diode cyclophotocoagulation. 6 (12%) eyes required more than 1 surgery for IOP control. 6 (12%) eyes underwent penetrating keratoplasty (PK) for corneal oedema. Mean period of follow up was 8.4 ± 6.4 months. There were no cases of sight threatening complications like suprachoroidal haemorrhage, blindness or endophthalmitis. CONCLUSIONSGlaucoma is strongly associated with ICE syndrome. ICE patients may require more than 1 surgery for IOP control. They are commonly associated with corneal complications which may require penetrating keratoplasty.