Spermatic Corneal Degeneration

Abstract

Abstract: : Purpose: To report a distinctive but previously undescribed corneal degeneration most likely representing an atypical variant of band keratopathy. Methods: Observational case series. Results: Four patients demonstrating a bilateral inferocentral–central symmetric superficial opalescent corneal haze were identified. Randomly distributed within the haze matrix were variable numbers of small discreet chalk–white deposits morphologically resembling spermatozoa. In all cases, the sperm "heads" appeared to be located at the level of Bowman's membrane, with tails streaming posteriorly at various oblique angles. Two patients demonstrated additional scattered yellowish–white "mutton fat" deposits, again at the level of Bowman's membrane. Only one patient demonstrated 3 o'clock and 9 o'clock peripheral corneal calcific deposits characteristic of classic band keratopathy. Vision was significantly reduced (20/100) in only one of the eight eyes. Buccal mucosal swab DNA analysis performed on two patients was negative for mutations on the TGFB1 gene, excluding Reis–Buckler's, Granular, Avellino, or Lattice dystrophy as the diagnosis. Overall, the clinical presentation closely resembled the rare variant of band keratopathy termed Reticular Degeneration of Kobe, differing only in replacement of Kobe's net–like opacities with spermatozoa–shaped deposits. Conclusions: Like Reticular Degeneration of Kobe, Spermatic Corneal Degeneration appears to represent a rare but intriguing variant of band keratopathy, with distinctive spermatozoa–shaped corneal deposits embedded in an opalescent cloud.

Keywords: cornea: clinical science • degenerations/dystrophies • calcium