Iridocorneal endothelial syndrome in a patient with keratoconus - A case report

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

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Article number221
JournalBMC Ophthalmology
Volume19
Issue number1
DOIs
Publication statusPublished - Nov 11 2019

Keywords

  • Essential iris atrophy
  • Glaucoma
  • Iridocorneal endothelial syndrome
  • Keratoconus
  • Keratoplasty

ASJC Scopus subject areas

  • Ophthalmology

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