Abstract
Case A 60-year-old male presented with gradual, painless deterioration of vision in his left eye over four years. His past ophthalmic history was clear with no record of trauma, surgery, infection or inflammation. His left eye’s best corrected visual acuity (BCVA) was 1.1 LogMar, and the cornea showed stromal oedema and a central thickness of 765 microns. There was no evidence of ocular inflammation or corneal scar in the affected eye, and the fellow eye looked pristine. Hypertonic saline and empiric systemic aciclovir were started, which made no significant improvement. However, an area of raised, mildly pigmented iris with iridocorneal contact was noticed in the far-periphery of the inferotemporal cornea. Oncology consultation excluded uveal malignancies. With the probable diagnosis of ICE syndrome, combined cataract surgery and 7.5 mm endothelial keratoplasty (DMEK) was performed with an aqueous tap to exclude HSV/VZV/CMV endotheliitis. The aqueous tap result was negative for any viral infection, and the Descemet membrane histology confirmed the diagnosis of ICE syndrome. Two months post-op, the patient was happy with the BCVA of 0.54 LogMar. The cornea looked clear with no scar or residual oedema, the IOP was within normal limits, the disc OCT was normal, and the macular OCT showed minimal epiretinal membrane with no traction, which did not justify his vision. Corneal Pentacam showed the rare association of keratoconus with ICE syndrome in his left eye. 10 months post-op, the graft looks clear, and he is happy with his vision and not keen on a rigid contact lens trial.
