Bilateral Iridocorneal Endothelial Syndrome

Objectives@#We presented a case of bilateral iridocorneal endothelial (ICE) syndrome with secondary glaucoma and discussed its clinical presentation and management.@*Study design@#This is a case report.@*Results@#A 12-year old female consulted for a one-year history of progressive blurring of vision in both eyes associated with abnormal pupils and occasional eye pain. Polycoria, shallow anterior chambers, increased intraocular pressures (IOP), areas of closed angles on gonioscopy, and increased cup-to-disc ratio in both eyes indicative of ICE syndrome were present. Management included anti-glaucoma medications and implantation of glaucoma drainage devices (GDD).@*Conclusion@#Early detection with regular follow-ups leading to prompt management of the ICE syndrome are necessary. IOP control may be challenging due to the nature of the disease and may require multiple surgeries including GDD implantation to achieve successful outcomes. Early use of GDD may be beneficial for pediatric patients with ICE and glaucoma, and may require additional surgeries to adequately manage the IOP.

Progress of iridocorneal endothelial syndrome / 国际眼科杂志(Guoji Yanke Zazhi)

@#Iridocorneal endothelial syndrome(ICES)is a rare ocular disease characterized by abnormal structure and proliferation of the corneal endothelium, the anterior chamber angle, and the iris. Common clinical features include corneal edema, secondary glaucoma and iris atrophy. ICES often occurs in young women, and most of them are monocular. Its pathogenesis is still unclear, the symptoms are various, and the blindness rate is high. The disease is difficult to diagnose, and there is no ideal treatment. The purpose of this article is to review the literature on the characteristics, diagnosis and treatment of ICES in order to help the diagnosis and treatment of the disease.

Progressive Iris Atrophy

Progressive iris atrophy is a disease characterized by marked iris atrophy, iris hole, corneal endotherial abnormality, perripheral anterior synechia, and glaucoma. It is considered as a variant of iridocorneal endotherial syndrome with Chandler's syndrome and Cogan-Reese syndrome. We have experianced a 34 year old male patient complaning visual blurring in his left eye. At examination, we found characteristics of progressive iris atrophy. After filerring surgery, associated glaucoma was well controlled. Then we report a case with review of the literatures.

A Case of Cogan-Reese Syndrome

Cogan-Reese syndrome is a disease characterized by glaucoma in an eye with peripheral anterior synechia, multiple pigmented iris nodules, and ectopic Descemet's membranes. Cogan-Reese syndrome is a variant of iridocorneal endothelial syndrome and shares common properties whcih are corneal endothelial degeneration, iris atrophy and secondary glaucoma, with progressive iris atrophy and Chandler's syndrome. We have experienced a 43-year-old female patient complaining of visual dimness in the left eye. On examination, we found characteristics of Cogan-Reese syndrome which were corneal endothelial pleomorphism and cell loss, corneal edema, iris atrophy and multiple pigmented nodules on the surface of the iris, peripheral anterior synechia, and increased intraocular pressure in the left eye. We report a case of Cogan-Reese syndrome which has not been reported in Korea.