Histopathological and in vivo Confocal Microscopic Analysis of Pre–Descemet’s Membrane Dystrophy With Unusual "Gelatinous Droplike" Appearance

Abstract

Abstract: : Purpose: To report histopathological and in vivo confocal microscopic findings of pre–Descemet’s membrane dystrophy with unusual "gelatinous droplike" appearance. Methods:A 54–year–old woman was referred to our hospital with a complaint of progressing bilateral visual disturbance. Her visual acuity was 20/50 OD, 20/150 OS. She has been noted asymptomatic corneal abnormality since childhood. Family history was not contributable except that her dead father had poor visual acuity. We analyzed her cornea with slit–lamp biomicroscopy, scanning–slit corneal topography, and confocal microscopy. The corneal tissue after penetrating keratoplasty was processed for histopathological examinations. Results:Slit–lamp biomicroscopy showed bilateral numerous "gelatinous droplike" white patches in the deep stromal layer just anterior to the endothelium. Scanning–slit corneal topography revealed bullous changes of the stroma, with 680 µm of total corneal thickness at the central cornea. By in vivo confocal microscopy, round bright structures approximately 100 to 200 µm in diameter were observed at the deep stroma adjacent to the endothelium. The endothelial cell counts were within normal range (mean 2133 cells/mm²);however pleomorphism and polymegathism were noted. There are no intra– or extra– cellular granular deposits in the keratocytes. Histopathological examinations showed edematous swollen corneal stroma and degenerated corneal endothelial cells. Eosinophilic and PAS–positive focal linear deposits just anterior to the Descemet’s membrane were seen, and the Descement’s membrane itself was abnormally thickened with some corneal guttatae containing granular deposits. Conclusions:Histopathological examinations suggested pre–Descemet’s membrane corneal dystrophy. However, slit–lamp biomicroscopy and confocal microscopy showed peculiar unreported images, indicating that this case might be a new subtype of pre–Descemet’s membrane dystrophy.

Keywords: microscopy: confocal/tunneling • pathology: human • cornea: clinical science