Meensmann corneal dystrophy; a clinic-pathologic, ultrastructural and confocal scan report

ABSTRACT

To report the microstructural features of Meesmann corneal dystrophy [MCD] in two patients. The first patient was a 10-year-old boy who presented with bilateral visual loss, diffuse corneal epithelial microcystic changes, high myopia and amblyopia. With a clinical impression of MCD, automated lamellar therapeutic keratoplasty was performed in his left eye. Histopathologic examination of the corneal button disclosed epithelail cell swelling and cyst-like intracytoplasmic inclusions. The cells contained moderate amounts of periodic acid-Schiff-positive and diastase-sensitive material [glycogen]. Transmission electron microscopy revealed numerous vacuoles and moderate number of electron-dense membrane-bound bodies in the cytoplasm, similar to lysosomes, some engulfed by the vacuoles. The second patient was a 17-year-old female with a clinical diagnosis of MCD and episodes of recurrent corneal erosion. On confocal scan examination of both corneas, hyporeflective rond-shaped areas measuring 6.8 to 41.4 micro m were seen within the superficial epithelium together with irregular and ill-defined high-contrast areas in the sub-basal epithelial region. The subepithelial nervous plexus was not visible due to regional hyperreflectivity. This case report further adds to the microstructural features of Meesmann corneal dystrophy and suggests confocal scan as a non-invasive method for delineating the microstructural appearance of this rare dystrophy