P411 Acquired ectropion uveae within ICE syndrome Vujica

P411
Acquired ectropion uveae within ICE syndrome
Vujica Markovic1, Paraskeva Hentova-Sencanic1, Marija Bozic1, Dejan Rasic1, Ivan Marjanovic1, Vesna Babic2
1
Faculty of Medicine, University of Belgrade, University Eye Hospital - Glaucoma Department, Belgrade - Serbia
2
Clinic for eye diseases, KCS Serbia, Belgrade - Serbia
Purposes: To determine the frequency of the secondary glaucoma in eyes with ICE syndrome joined with
AEU and the effect of medical and surgical treatment, compared to cases of ICE syndrome without present
AEU. Acquired ectropion uveae (AEU) is an extremely rare disease, characterised by the ectropionated pigment
epithelium layer on the front surface of the iris from the pupillary ruff, the dysgenesis of the iridocorneal angle
and glaucoma. ICE syndrome includes a group of acquired diseases that affect younger people, it is always onesided and with significant participation of secondary glaucoma. Glaucoma is due to synechial angle closure
secondary to contraction of this abnormal tissue. It consists of three rare disorders:
1) Progressive iris atrophy
2) Chandler syndrome and
3) Cogan-Reese (iris nevus) syndrome, which could be manifested with or without present AEU.
Methods: Examination included biomicroscopic examination, specular microscopy, applanation tonometry,
indirect gonioscopy, computerized perimetry, ophthalmoscopy examination and HRT II, as well as pathohistological
sample examination of iridectomy after the completed TTR.
Results: During the five-year follow-up of 29 patients with ICE syndrome, 15 patients had no present AEU.
Secondary glaucoma in this group was found in 8 patients (53,33%). Glaucoma was compensated with medical
therapy in 4 patients, and 4 patients required surgical treatment. 14 patients with ICE syndrome had present
AEU and secondary glaucoma was found in all patients (100%). All patients required surgical treatment - TTR
combined with adjunctive antimetabolites.
Conclusion: AEU within ICE syndrome is a very rare iris malformation, with 100% presence of secondary
glaucoma. All patients required surgical treatment in controlling their IOP. Although AEU within ICE syndrome is
benign but progressive eye disease, it takes a high degree of caution to detect the associated glaucoma as soon
as possible, so that an irreversible optic disc neuropathy can be prevented with surgery.

Download Report