Case Reports Analyze Surgical Approaches for Fuchs’ Endothelial Dystrophy

TRUJILLO, PERU – APRIL 20: Dr Mark Mannis removes the rest of the donor rim after removing the cornea using a trephine tool during a transplant operation on Reis-Buckler syndrome patient Diana, aged 17, onboard the Orbis Flying Eye Hospital on April 20, 2018 in Trujillo, Peru. Reis-Buckler corneal dystrophy is a rare genetic condition which causes the Bowman’s layer of the cornea to disintegrate. Diana’s mother Rosa has the same syndrome and has struggled with sight loss since the age of nine. After suffering with the condition since the age of three, Diana was chosen for surgery during a programme run by Orbis, the ophthalmic training organisation. Founded in 1982 by ophthalmologist David Paton, Orbis trains eyecare teams across Africa, Asia and Latin America to improve the standard of eyecare in the region. As well as working in local hospitals, the charity also has a self-sufficient surgical unit on the Orbis Flying Eye Hospital, a converted McDonald-Douglas MD10 aircraft. (Photo by Leon Neal/Getty Images)
A series takes 3 patients with the corneal condition into account.

Transcorneal freezing does not affect ultrastructural composition of Descemet’s membrane in Fuchs’ endothelial corneal dystrophy but does not preclude subsequent Descemet membrane endothelial keratoplasty (DMEK), according to a correspondence published in Eye. 

Currently, no conclusive evidence suggests which surgical approach — removal of Descemet’s membrane without endothelial keratoplasty or removal of only the diseased corneal endothelial cells — is best when combined with Rho kinase (ROCK) inhibitor treatment to enhance the regeneration of corneal endothelial cells. 

Researchers reported their findings from 3 patients with moderate Fuchs’ endothelial corneal dystrophy using the Erbokryo® AE cryosurgical system (Erbe Elektromedizin). Erbokryo was applied at -60° Celsius for 15 seconds to the central 3 mm of the cornea to remove corneal endothelial cells, followed by ripasudil eye drops (Glantec® ophthalmic solution 0.4%; Kowa Company, Ltd.) 6 times daily for 1 week. 

The first patient, a 48-year-old woman) presented with corrected distance visual acuity (CDVA) of 0.3 logMAR, which increased to 0.1 logMAR after 1 month of cryotherapy/ripasudil treatment. At the 2-year posttreatment mark, CDVA dropped to 0.2 logMAR. Three and a half years after treatment, DMEK was performed. At the time of treatment, CDVA was 0.5 logMAR; this increased to 0.0 logMAR within 2 months of the DMEK.

The second patient, a 62-year-old woman, also presented with a CDVA of 0.3 logMAR, which remained the same at 1 month after treatment. DMEK was performed 14 months later, with a CDVA of 0.5 logMAR. Three months post-DMEK, CDVA increased to 0.1 logMAR. 

The third patient, a 64-year-old man, presented with a CDVA of 1.0, which improved to 0.2 logMAR after 6 months of treatment. At 18 months, CDVA dropped by 1 line due to herpes simplex keratitis, which prompted a penetrating keratoplasty with CDVA of 0.8 logMAR. 

Corneal tissue samples were obtained from patients 2 and 3 during surgery, and transmission electron microscopy studies were performed on these samples. Descemet’s membrane ultrastructure demonstrated alterations typical to Fuchs’ endothelial corneal dystrophy, but “no sequelae of transcorneal freezing and/or ripasudil” were noted. 

According to the researchers, these observations “suggest that transcorneal freezing does not affect the ultrastructural composition of Descemet’s membrane in Fuchs’ endothelial corneal dystrophy.” 

“The persistence of guttae may explain why this procedure yields improvement in CDVA only transiently, despite the application of ripasudil,” according to the publication. “However, the intervention does not preclude subsequent DMEK surgery with excellent visual outcomes. It may therefore provide a viable option in patients who benefit from postponing intraocular surgery.” 

Further studies are required to determine the mean duration of improved visual acuity as well as whether patients with endothelial disease “not characterized by corneal guttae may have a more sustained effect.” 


Menzel-Severing J, Schrader S, Schlötzer-Schrehardt U, Geerling G. Transcorneal freezing and topical Rho-kinase inhibitor treatment in Fuchs endothelial corneal dystrophy. Eye. Published online April 12, 2021. doi:10.1038/s41433-021-01520-2