Descemet's Membrane Endothelial Keratoplasty In An Eye With Iridocorneal Endothelial Syndrome And Rare Association Of Keratoconus

To present a case of a rare association of iridocorneal endothelial (ICE) syndrome and keratoconus, whose first presentation was decompensated cornea.

This report stems from a real case encountered at Queen’s Hospital, NHS in East London, UK.

A 60-year-old male, with a previously unremarkable eye history, presented with a progressive, painless decline in vision over four years in his left eye. Best corrected visual acuity (BCVA) was 1.1 LogMar, revealing stromal oedema with a central thickness of 765 microns. Ocular inflammation and scarring were absent, and the fellow eye revealed no evidence of any stromal dystrophy. Initiating with hypertonic saline and systemic aciclovir yielded no improvement. Subsequently, an iridocorneal contact in the far-periphery of the inferno-temporal cornea was observed. Uveal malignancies were excluded through oncology consultation.

With suspected ICE syndrome, a combination of cataract surgery and a 7.5 mm Descemet's membrane endothelial keratoplasty (DMEK) was performed. An aqueous tap ruled out viral endotheliitis. Histological examination of the Descemet membrane confirmed ICE syndrome. Two months post-op, BCVA improved to 0.4 LogMar despite a clear cornea, normal optic disc, and macular OCT. 

Corneal Pentacam unveiled the rare coexistence of keratoconus with ICE syndrome, maximum keratometry of 54.9 D, thinnest pachymetry of 482u and astigmatism of 7.8 D in the left eye and normal findings in the right eye. Ten months post-op, the graft remains transparent, and the patient, content with vision, declines a rigid contact lens trial.

This case underscores the exceptional association of keratoconus with ICE syndrome. Descemet's membrane endothelial keratoplasty demonstrated efficacy in resolving corneal oedema and achieving substantial visual improvement in corneal decompensation despite the coexistence of keratoconus.