Iridocorneal endothelial syndrome in a patient with keratoconus – a case report
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 pre...
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| Format: | Article |
| Language: | English |
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BMC
2019-11-01
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| Series: | BMC Ophthalmology |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s12886-019-1215-x |
| _version_ | 1819204571603402752 |
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| author | Michele De Maria Danilo Iannetta Antonio Moramarco Luigi Fontana |
| author_facet | Michele De Maria Danilo Iannetta Antonio Moramarco Luigi Fontana |
| author_sort | Michele De Maria |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-23T04:37:56Z |
| format | Article |
| id | doaj.art-84c1f7e1b5a14f36a014eb382a624255 |
| institution | Directory Open Access Journal |
| issn | 1471-2415 |
| language | English |
| last_indexed | 2024-12-23T04:37:56Z |
| publishDate | 2019-11-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Ophthalmology |
| spelling | doaj.art-84c1f7e1b5a14f36a014eb382a6242552022-12-21T17:59:51ZengBMCBMC Ophthalmology1471-24152019-11-011911610.1186/s12886-019-1215-xIridocorneal endothelial syndrome in a patient with keratoconus – a case reportMichele De Maria0Danilo Iannetta1Antonio Moramarco2Luigi Fontana3Ophthalmology Unit, AUSL-IRCCS of Reggio EmiliaOphthalmology Unit, AUSL-IRCCS of Reggio EmiliaOphthalmology Unit, AUSL-IRCCS of Reggio EmiliaOphthalmology Unit, AUSL-IRCCS of Reggio EmiliaAbstract 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.http://link.springer.com/article/10.1186/s12886-019-1215-xKeratoconusEssential iris atrophyIridocorneal endothelial syndromeKeratoplastyGlaucoma |
| spellingShingle | Michele De Maria Danilo Iannetta Antonio Moramarco Luigi Fontana Iridocorneal endothelial syndrome in a patient with keratoconus – a case report BMC Ophthalmology Keratoconus Essential iris atrophy Iridocorneal endothelial syndrome Keratoplasty Glaucoma |
| 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 | Keratoconus Essential iris atrophy Iridocorneal endothelial syndrome Keratoplasty Glaucoma |
| url | http://link.springer.com/article/10.1186/s12886-019-1215-x |
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