In this study we analyzed and objectified the characteristics of the Avellino corneal dystrophy patients considering disease severity using in vivo confocal microscopy (IVCM).

Each corneal layer of 36 eyes in 18 patients with Avellino corneal dystrophy was examined using IVCM (ConfoScan 4.0, NIDEK, Co. Ltd., Albignasego, Italy). Patients were classified into 3 groups based on disease severity (mild, moderate, or severe).

In the mild group, hyper-reflective granular deposits without dark shadows were observed in the anterior stroma. As the disease progressed, corneal deposits were also found at the posterior stroma and epithelium, and clusters of hyper-reflective corneal deposits resembling stromal opacity were noted. The range of corneal deposits measured using Z-scan optical pachymeter was 111.14 ± 30.95 um in the mild group, 157.47 ± 25.00 um in the moderate group, and 193.42 ± 52.23 um in the severe group ( p < 0.05).

The origin of the corneal deposits in Avellino corneal dystrophy may be related to corneal stromal cells and distributed from the corneal epithelial layer to the stromal layer. IVCM might be useful for the standardization of disease severity by digitalizing the range of deposits.