Purpose: The purpose of this study was to report the progression of the iridocorneal endothelial syndrome during a 5-year period in a patient with the essential iris atrophy variant using confocal microscopy and ultrabiomicroscopy (UBM).
Methods: A 47-year-old woman was referred to us in January 2002 with suspected iridocorneal endothelial syndrome. Examination of both eyes included visual acuity, biomicroscopy, intraocular pressure measurement, gonioscopy, visual field test, and UBM. Afterward, she was examined yearly and confocal microscopy performed.
Results: Visual acuity was 20/20 in both eyes. Examination of the right eye revealed endothelial degeneration, peripheral anterior synechiae, and iris atrophy around the pupil that appeared displaced to the temporal side. Biomicroscopy of the left eye was unremarkable. Posterior segment was normal in both eyes. UBM of the right eye showed thickening of the iris adherent to the corneal endothelium. Diagnosis of essential iris atrophy was confirmed. One year later, the right eye showed increasing iridoendothelial adherences confirmed by UBM. Confocal microscopy revealed endothelial cell abnormalities and groups of endothelial "epithelium-like" cells with hyperreflective nuclei. In April 2007, the pupil appeared more temporally dislocated. UBM showed more extended synechiae. Confocal microscopy displayed hyporeflective opacities in the endothelial layer and many grouped keratocytic clusters in the posterior stroma. No alteration of the intraocular pressure and visual field was found during the follow-up.
Conclusions: UBM and confocal microscopy permit observation of any corneal and iridocorneal angle changes and evaluation of essential iris atrophy progression.
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