Iridocorneal Endothelial Syndrome

Irido-corneo-endothelial syndrome is a rare condition that corresponds to a group of three clinical entities that respond to a single pathophysiology. It manifests in adults typically unilaterally. Glaucoma or corneal edema, which is difficult to treat and of varying success, is usually complicated. Irido-corneo-endothelial syndrome remains a severe condition for the prognosis of the affected eye.

S.Slama I.Abayahia S.Ghezali. H.Boudeja  M.Terahi  Nafissa Hamoud Hospital Algiers

Case report  of a 48 year old patient who consults for photophobia,night halos with blurred vision in the morningOS. 

The corrected distance visual acuity is 7/10 OD 6/10 OS.the anterior segment shows:OSdark brownish peduncle nodules distributed in temporal range of1h around6h associated with a heterogeneous hammered appearance of the corneal endothelium with a slightly deformed pupil without abnormalities OD;the ocular tone is12mmhg for the right eye and30mmhg for the left eye,an irido-corneal angle grade II of Shaffer with a pigmented trabéuclum on 360 OS,gradeVIof Shaffer OD,the fundus without particularity,a papillary excavation 0.3 for both eyes.Specular microscopy showing a clear asymmetry of cell density,she received a trabeculectomy.

In front of this clinical picture we retained the diagnosis of the entity of Cogan-Reese syndrome, (iris nævus ).This syndrome is evoked in women between 30 and 50 years, is the sum of three corneal pathological entities involving all three corneal endothelium. . The anomalies of irido-corneo-endothelial syndrome are endothelial, iridocornal and iridocornate angle.   In Cogan-Reese syndrome, the iridescent nodules dominate the clinical aspect. Their number increases over time. A heterogeneous aspect of the endothelial layer coexists. The endothelial progression and its production of a Descemet neomembrane induce the slow elevation of intraocular pressure (IOP) responsible for the chronic glaucoma.

Conclusions: Irido-corneo-endothelial syndrome remains a severe condition for the prognosis of the affected eye. The two major complications of ICE are glaucoma and corneal edema due to endothelial failure.  The management focuses on the treatment of hypertonia and corneal edema .Glaucoma associated with ICE syndrome is often difficult to manage and is usually treated with medications and/or filtering surgery. Glaucoma filtering surgery is usually successful when done early, but may fail due to endothelialization of the fistula by the abnormal corneal endothelium.