Difficulties in Treatment of Iridocorneal Endothelial Syndrome - Case Report

Abstract

The iridocorneal endothelial syndrome manifests in three clinical types: Chandler syndrome, progressive iris atrophy, and Cogan-Reese syndrome. It is caused by the presence of abnormal corneal endothelium on the iris stroma and anterior chamber angle leading to usually unilateral, progressive iris atrophy, glaucoma, and/or corneal edema. The etiology is yet unclear. It affects mostly young adults, mostly females. Management of iridocorneal endothelial syndrome is complex: conservative and surgical, depending on the stage of the disease and intensity of present symptoms. A 30-year-old female with a medical history of the iridocorneal endothelial syndrome was reported to the Ophthalmology Department for consultation. Slit-lamp examination revealed iris atrophy and superior-nasal corectopia in the left eye. On gonioscopy, the angle was wide open in the right eye, but there were iridocorneal adhesions and incomplete angle-closure in the left eye. The patient was provided with maximum local therapy consisting of three anti-glaucoma medications. On later check-ups, the patient presented corneal edema and increased intraocular pressure. She was qualified to ExPress mini shunt trabeculectomy with mitomycin C. Two years later, a patient came to the clinic because of increased values of intraocular pressure (up to 59 mmHg), slit-lamp examination showed that the ExPress implant was congested with fragments of the corneal endothelial cells. Thanks to YAG iridotomy the implant was recanalized.

Outcomes of Trabeculectomy and Ahmed Glaucoma Valve Implantation in Patients With Iridocorneal Endothelial Syndrome

“…11 There are also other case reports regarding the successful management of uncontrolled IOP in ICE syndrome by implantation of EX-PRESS mini-shunt with adjunctive MMC, even up to 2 years after surgery. 16,17 With regards to the role of minimally invasive glaucoma surgery methods in ICE syndrome, there are also a few case reports that claim good IOP control was achieved with XEN gel stent (AqueSys Inc.) implantation. 18,19 It is worth mentioning that in this small case series by Imamoglu et al, 11 keratoplasty was later performed in 3 out of these 5 cases that underwent AGV surgery.…”

“…The authors stated that the EX-PRESS mini-shunt appears to be safe for the management of ICE syndrome-related glaucoma and causes less postoperative inflammation than other drainage devices 11 . There are also other case reports regarding the successful management of uncontrolled IOP in ICE syndrome by implantation of EX-PRESS mini-shunt with adjunctive MMC, even up to 2 years after surgery 16,17 . With regards to the role of minimally invasive glaucoma surgery methods in ICE syndrome, there are also a few case reports that claim good IOP control was achieved with XEN gel stent (AqueSys Inc.) implantation 18,19 …”