Meesmann Corneal Dystrophy; a Clinico-Pathologic, Ultrastructural and Confocal Scan Report

PURPOSE: To report the microstructural features of Meesmann corneal dystrophy (MCD) in two patients. CASE REPORT: 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 epithelial 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 numbers 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 round-shaped areas measuring 6.8 to 41.4 μm 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. CONCLUSION: 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.