Analysis and documentation of progression of Fuchs corneal dystrophy with retroillumination photography

Purpose: Fuchs corneal dystrophy (FCD) is a degenerative disorder of the cornea that is characterized by the progressive accumulation of guttae, which are small excrescences of Descemet's membrane. We describe a method for documenting the location and number of guttae, and ask whether disease progression can be observed during relatively short periods. Methods: Patients with FCD were imaged by standard retroillumination photography with a slit lamp. Scanned photographs were analyzed by using NIH ImageJ software to determine the number of individual guttae and areas of confluence. Results: In 4 FCD patients, photographs taken 23 to 30 months apart revealed that, once formed, individual guttae and their relative positions persisted during this period. Very few guttae disappeared, and the emergence of many new guttae was observed. Determination of the area with confluent guttae was used to quantify disease stage. Conclusions: Computer-assisted analysis of retroillumination photographs is proposed as an effective way to document the number and distribution of individual guttae. Although the disease typically progresses slowly during decades, we have been able to detect the formation of new guttae within only 2 years. This rapid assessment of disease progression could be used to measure phenotypic differences between genetic subtypes of FCD. It also could provide important baseline information and methodology for clinical trials of therapeutic options, should these become available.