Abstract

Background: Pellucid marginal degeneration (PMD) is an infrequent and non-inflammatory reduction of the corneal thickness near the inferior lateral region that produces astigmatism. Iridocorneal endothelial syndrome (ICE syndrome) is typically seen only in one eye and consists of unusual cell proliferation on the corneal endothelium, iris thinning, and glaucoma. It is uncommon for a patient to have both these diseases simultaneously.
Case Presentation: A 54-year-old lady presented with a progressive decrease in vision in her left eye. Her best-corrected visual acuity was 6/6 in the right eye and 6/60 in the left. On examination, there was nasal opacity of the cornea, iris-cornea touch, and extensive scar formation in the region where the cornea and iris are attached (peripheral anterior synechiae). Specular microscopy showed fewer numbers of endothelial cells (1146 cells per square millimeter). Topography revealed PMD with thinning of the cornea at 385 µm.
Conclusion: This was a very rare case of unidirectional association of PMD and ICE syndrome. Knowing about the possibility of association would help in making the right diagnosis and follow-ups, since both these conditions have the potential of making changes in the cornea and can cause secondary glaucoma.

Key words: Cornea, degeneration, iris Atrophy, pellucid corneal degeneration, iridocorneal endothelial syndrome, case report