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Published in: BMC Ophthalmology 1/2019

Open Access 01-12-2019 | Glaucoma | Case report

Iridocorneal endothelial syndrome in a patient with keratoconus – a case report

Authors: Michele De Maria, Danilo Iannetta, Antonio Moramarco, Luigi Fontana

Published in: BMC Ophthalmology | Issue 1/2019

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Abstract

Background

To describe a case of a rare association of bilateral keratoconus and unilateral essential iris atrophy and to conduct a literature review of the current strategies of treatment of the corneal disease and glaucoma in patients with Iridocorneal Endothelial Syndrome (ICE).

Case presentation

We report a rare association of bilateral keratoconus and unilateral essential iris atrophy in a 38-year-old man. Diagnosis of bilateral keratoconus was confirmed by corneal topography. Slit-lamp examination showed extensive iris atrophy with corectopia and policoria in one eye. Corneal specular microscopy revealed an abnormal endothelium morphology in the same eye with extensive peripheral anterior synechiae and closure of the drainage angle at gonioscopy. Intraocular pressure was 26 mmHg, despite maximal topical therapy. Optic disc examination showed severe glaucomatous cupping. Surgery by glaucoma drainage device implantation was performed.

Conclusion

Essential iris atrophy is a rare clinical variant of ICE syndrome characterized by profound anatomical alterations of the anterior segment associated with corneal edema and secondary glaucoma. In these patients, selective keratoplasties have replaced penetrating keratoplasty to treat corneal decompensation and glaucoma drainage devices are preferred to conventional trabeculectomy for the treatment of secondary glaucoma.
Literature
8.
go back to reference Levy SG, McCartney ACE, Baghai MH, Barrett MC, Moss J. Pathology of the iridocorneal-endothelial syndrome: the ICE-cell. Investig Ophthalmol Vis Sci. 1995;36(13):2592–601. Levy SG, McCartney ACE, Baghai MH, Barrett MC, Moss J. Pathology of the iridocorneal-endothelial syndrome: the ICE-cell. Investig Ophthalmol Vis Sci. 1995;36(13):2592–601.
39.
go back to reference Saleem AA, Ali M, Akhtar F. Iridocorneal endothelial syndrome. J Coll Physicians Surg Pak. 2014;24(Suppl 2):S112–4 doi: 05.2014/JCPSP.S112S114.PubMed Saleem AA, Ali M, Akhtar F. Iridocorneal endothelial syndrome. J Coll Physicians Surg Pak. 2014;24(Suppl 2):S112–4 doi: 05.2014/JCPSP.S112S114.PubMed
Metadata
Title
Iridocorneal endothelial syndrome in a patient with keratoconus – a case report
Authors
Michele De Maria
Danilo Iannetta
Antonio Moramarco
Luigi Fontana
Publication date
01-12-2019
Publisher
BioMed Central
Published in
BMC Ophthalmology / Issue 1/2019
Electronic ISSN: 1471-2415
DOI
https://doi.org/10.1186/s12886-019-1215-x

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