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

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 PRESENTATIO...

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Autores principales: De Maria, Michele, Iannetta, Danilo, Moramarco, Antonio, Fontana, Luigi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6849214/
https://www.ncbi.nlm.nih.gov/pubmed/31711443
http://dx.doi.org/10.1186/s12886-019-1215-x
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author De Maria, Michele
Iannetta, Danilo
Moramarco, Antonio
Fontana, Luigi
author_facet De Maria, Michele
Iannetta, Danilo
Moramarco, Antonio
Fontana, Luigi
author_sort De Maria, Michele
collection PubMed
description 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.
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spelling pubmed-68492142019-11-15 Iridocorneal endothelial syndrome in a patient with keratoconus – a case report De Maria, Michele Iannetta, Danilo Moramarco, Antonio Fontana, Luigi BMC Ophthalmol Case Report 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. BioMed Central 2019-11-11 /pmc/articles/PMC6849214/ /pubmed/31711443 http://dx.doi.org/10.1186/s12886-019-1215-x Text en © The Author(s). 2019 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
De Maria, Michele
Iannetta, Danilo
Moramarco, Antonio
Fontana, Luigi
Iridocorneal endothelial syndrome in a patient with keratoconus – a case report
title Iridocorneal endothelial syndrome in a patient with keratoconus – a case report
title_full Iridocorneal endothelial syndrome in a patient with keratoconus – a case report
title_fullStr Iridocorneal endothelial syndrome in a patient with keratoconus – a case report
title_full_unstemmed Iridocorneal endothelial syndrome in a patient with keratoconus – a case report
title_short Iridocorneal endothelial syndrome in a patient with keratoconus – a case report
title_sort iridocorneal endothelial syndrome in a patient with keratoconus – a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6849214/
https://www.ncbi.nlm.nih.gov/pubmed/31711443
http://dx.doi.org/10.1186/s12886-019-1215-x
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