To report an unusual presentation of Fuch's endothelial dystrophy (FED) and its management. A 53-year-old male patient presented with unilateral progressive painless diminution of vision. Best-corrected visual acuity of the right and left eyes were 20/20 and 20/400, respectively. Slit lamp examination of the right eye was unremarkable, on left eye examination there was corneal edema, shallow anterior chamber, areas of 360 degrees iridocorneal touch with few synechiae on gonioscopy and age-related nuclear sclerosis. Pachymetry and specular microscopy imaging were performed before and after the surgical procedure. Ultrasound biomicroscopy (UBM) was performed preoperatively to rule out the presence of any angle anomalies. Combined procedure of Phacoemulsification with intraocular lens implantation and Descemet's membrane endothelial keratoplasty (DMEK) with peripheral iridectomy were performed. At 6-weeks follow-up, best-corrected visual acuity improved from 20/400 to 20/20. Slit lamp examination of the left eye showed clear DMEK graft with patent inferior peripheral iridectomy. Specular microscopy showed a cell count of 1761 cells/mm2. In a patient presenting with unilateral corneal edema, shallow anterior chamber depth, and iridocorneal adhesions, one needs to rule out the diagnosis of atypical variant of FED.
Keywords: Atypical Fuch's endothelial dystrophy; DMEK.