Descemet's membrane endothelial keratoplasty in an eye with iridocorneal endothelial syndrome and rare association of corneal ectasia.

Abstract

We report the first case of concurrent iridocorneal endothelial (ICE) syndrome and keratoconus, treated successfully with Descemet's membrane endothelial keratoplasty (DMEK). A 60-year-old male presented with gradual visual deterioration in his left eye over 4 years. Best corrected visual acuity was 1.1 LogMar, with corneal stromal oedema. Hypertonic saline and systemic acyclovir provided no improvement. Further examination revealed peripheral anterior synechiae and possible ICE syndrome. Combined cataract surgery and adapted DMEK were performed, using right eye data for intraocular lens calculation. Postoperative histopathology confirmed ICE syndrome. Two months postoperatively, vision improved to 0.54 LogMar, with normal intraocular pressure and optical coherence tomography. Ten months later, unaided visual acuity reached 0.4 LogMar, with no significant changes observed in regular follow-ups. The patient remains satisfied with his vision. This case highlights the rare association of keratoconus with Chandler Syndrome and the first report of such a case where DMEK was used as management. The diagnosis of ICE syndrome complicates treatment, however, despite the challenges, DMEK demonstrated promising results for ICE-related corneal oedema in a patient with concurrent keratoconus, offering improved visual acuity and no complications.