Delphi-Based Global Consensus on Fuchs Endothelial Corneal Dystrophy. An Endothelial Keratoplasty Learners Group Initiative.

PURPOSE: To identify areas of consensus among global experts for the management of Fuchs endothelial corneal dystrophy (FECD) in clinical practice, including its diagnosis, evaluation, decision-making principles with respect to intervention, and recommendations for performing cataract surgery in patients with FECD, including when to combine with keratoplasty.

DESIGN

Modified Delphi-based global consensus.

PARTICIPANTS

Thirty-seven ophthalmologists from around the world with significant expertise in the management and mechanisms of FECD.

METHODS

A series of consensus statements about FECD were developed from three iterative rounds of structured questions and statements posed to the panel of experts. Two rounds were asynchronous electronic questionnaires, and the third round was a live virtual meeting. Experts responded anonymously to statements assessing consensus and to open-ended questions that invited diverse input.

MAIN OUTCOME MEASURES: Consensus was defined as 70% agreement among experts.

RESULTS

Consensus was reached for 90 of 91 statements after three rounds. Experts agreed that FECD is defined by the presence of central or paracentral scattered or confluent guttae with or without edema. There was strong consensus that a chronic state of subclinical edema precedes the onset of clinically detectable edema that may or may not cause symptoms. With near-unanimous consensus, disease evaluation recommendations included assessing for findings that implicate the cornea as a source of decreased vision to separate it from the effect of comorbid conditions, as this would inform whether corneal intervention is appropriate. These findings include diurnal variation in vision, clinical or subclinical (tomographic) edema, and changes or differences in central corneal thickness. Based on current evidence, experts agreed that there are no effective medical therapies for FECD, and that Descemet membrane endothelial keratoplasty is the surgical treatment of choice when indicated.

CONCLUSIONS

The consensus statements provide current globally endorsed recommendations for the diagnosis and management of FECD. The guidelines are important and relevant for general ophthalmologists, who typically first diagnose and evaluate FECD, and for cornea specialists, by allowing them to benchmark their current practice patterns against expert recommendations. This could help improve patient outcomes and establish a framework adaptable to future advances and evolving technologies in the management of FECD.