title: Diagnosis and Management of Iridocorneal Endothelial Syndrome.
abstract: The iridocorneal endothelial (ICE) syndrome is a rare ocular disorder that includes a group of conditions characterized by structural and proliferative abnormalities of the corneal endothelium, the anterior chamber angle, and the iris. Common clinical features include corneal edema, secondary glaucoma, iris atrophy, and pupillary anomalies, ranging from distortion to polycoria. The main subtypes of this syndrome are the progressive iris atrophy, the Cogan-Reese syndrome, and the Chandler syndrome. ICE syndrome is usually diagnosed in women in the adult age. Clinical history and complete eye examination including tonometry and gonioscopy are necessary to reach a diagnosis. Imaging techniques, such as in vivo confocal microscopy and ultrasound biomicroscopy, are used to confirm the diagnosis by revealing the presence of "ICE-cells" on the corneal endothelium and the structural changes of the anterior chamber angle. An early diagnosis is helpful to better manage the most challenging complications such as secondary glaucoma and corneal edema. Treatment of ICE-related glaucoma often requires glaucoma filtering surgery with antifibrotic agents and the use of glaucoma drainage implants should be considered early in the management of these patients. Visual impairment and pain associated with corneal edema can be successfully managed with endothelial keratoplasty.
authors.affiliation: Cornea and Ocular Surface Unit, Ospedale San Raffaele IRCCS, Via Olgettina 60, 20132 Milan, Italy.
authors.forename: Marta
authors.initials: M
authors.lastname: Sacchetti
authors.affiliation: Department of Biology, College of Science and Technology, Temple University, 1900 N. 12 Street, Philadelphia, PA 19122, USA.
authors.forename: Flavio
authors.initials: F
authors.lastname: Mantelli
authors.affiliation: Department of Sense Organs, Sapienza University, Viale del Policlinico 155, 00186 Rome, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Marenco
authors.affiliation: Department of Ophthalmology, Campus Bio-Medico University, Via Alvaro del Portillo 200, 00128 Rome, Italy.
authors.forename: Ilaria
authors.initials: I
authors.lastname: Macchi
authors.affiliation: Cornea and Ocular Surface Unit, Ospedale San Raffaele IRCCS, Via Olgettina 60, 20132 Milan, Italy.
authors.forename: Oriella
authors.initials: O
authors.lastname: Ambrosio
authors.affiliation: Cornea and Ocular Surface Unit, Ospedale San Raffaele IRCCS, Via Olgettina 60, 20132 Milan, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Rama
mesh_terms: D016039:Corneal Transplantation / Q000379:methods*; D003937:Diagnosis, Differential; D019317:Evidence-Based Medicine; D005260:Female; D006068:Gonioscopy / Q000379:methods*; D006801:Humans; D057129:Iridocorneal Endothelial Syndrome / Q000175:diagnosis* / Q000628:therapy*; D008297:Male; D008487:Medical History Taking / Q000379:methods; D033401:Microscopy, Acoustic / Q000379:methods; D018613:Microscopy, Confocal / Q000379:methods; D009887:Ophthalmoscopy / Q000379:methods*; D014065:Tonometry, Ocular / Q000379:methods*; D016896:Treatment Outcome