Iridocorneal Endothelial Syndrome: Case Report of Essential Progressive Iris Atrophy

Med Arch. 2022 Jun;76(3):224-228. doi: 10.5455/medarh.2022.76.224-228.


Background: Iridocorneal endothelial (ICE) syndrome is a group of ophthalmic disorders, first reported by Eagle and Yanoff in 1979, a disease characterized by abnormalities of the iris and the corneal endothelium, and mainly occurs unilaterally in young and middle-aged women, with no family history. ICE syndrome comprises a spectrum of three clinical variants: Progressive essential iris atrophy (corectopia, iris atrophy or iris hole), Chandler syndrome (corneal oedema with mild to absent iris change), and Cogan - Reese syndrome (nodular pigmented lesion of the iris).

Objective: We are presenting this case because of its rarity, diagnostic intricacy and therapeutic challenge.

Case report: We report in this study a case of Essential Progressive Iris Atrophy, an Iridocorneal Endothelial Syndrome variant in a 40 years old patient, female, complaining about the shape of the pupil in the left eye, as well as photophobia in the same side.In the first evaluation, we observed visual acuity of 1.0 in both eyes.Intraocular pressure was 14 mm Hg in the right eye and 12 mm Hg in the left eye. On the biomicroscope, we had a proper right eye finding, on the left eye Iris atrophy with deformity in the direction from 12 to 6 hours. We performed gonioscopy, an ultra sound (UBM), spectral microscopy, pachymetry, OCT and Octopus perimetry.

Conclusion: We confirmed the diagnosis of essential iris atrophy based on the clinical findings, and in abnormalities in complementary exams. Nowadays, the patient is being followed in the Ophthalmology department at JZU Brcko District Bosnia and Herzegovina.

Keywords: Corneal diseases; Endothelial corneal; Glaucoma; Iridocorneal endothelial syndrome.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Atrophy / pathology
  • Endothelium, Corneal / pathology
  • Female
  • Humans
  • Intraocular Pressure
  • Iridocorneal Endothelial Syndrome* / diagnosis
  • Iridocorneal Endothelial Syndrome* / pathology
  • Iris* / pathology