In vivo confocal microscopy in primary congenital glaucoma with megalocornea

To report the microscopic findings of congenital glaucoma-related megalocornea using in vivo confocal microscopy.

Two consecutive adult patients presenting the typical features of glaucomatous megalocornea underwent a complete ophthalmologic examination. The first patient presented with progressive glaucoma with bilateral megalocornea. The second patient's left eye was affected by megalocornea without actual evidence of glaucoma, whereas the right eye was healthy. Both patients were examined using a new-generation scanning slit corneal confocal microscope.

In both patients, confocal microscopy revealed a mild reduction of keratocyte density in the mid and rear stroma, a particular abnormal "clew-shaped" morphology of stromal nerves, and the presence of discontinuous hyperreflective structures overhanging the endothelial layer at the level of the Descemet membrane. The endothelium showed severe polymegethism, pleomorphism, and a markedly decreased cell density, and focal cellular lesions were noted.

Confocal microscopy is a diagnostic tool used to evaluate microscopic aspects of Haab striae and endothelial morphologic changes in glaucomatous megalocornea. Unsuspected alterations, such as nerves abnormalities and focal endothelial tractions by scar tissue, were observed.