Outcomes of Corneal Collagen Crosslinking Under General Anesthesia in Down Syndrome

Down syndrome is one of the strongest risk factors of keratoconus. Corneal collagen crosslinking (CXL) is the standard-of-care treatment to reduce progression, but there is limited published literature about CXL in patients with Down syndrome. We sought to describe outcomes of epithelium-off CXL under general anesthesia in patients with Down syndrome and keratoconus.

We conducted a retrospective descriptive case series at a single tertiary pediatric hospital. Standard epithelium-off Dresden protocol CXL under general anesthesia was performed in all cases. Preoperative and postoperative corneal characteristics were obtained. All perioperative and postoperative complications were reported.

Between August 2020 and February 2024, CXL was performed on 34 eyes of 20 patients with Down syndrome. The mean age was 20.2 years at the time of crosslinking. The mean preoperative Kmax was 62.3 diopters (range 45.5-95.4), with a mean preoperative central corneal thickness of 466 μm (range 306-538). Visual acuity post procedure in patients seen 1 week postoperatively was stable to improved. One eye developed corneal edema that resolved within 3 weeks postoperatively, and another was found to have new apical scarring a year after the procedure. No other complications were noted.

Keratoconus was diagnosed at an advanced stage in most of the patients with Down syndrome in our cohort. Epithelium-off CXL under general anesthesia was well tolerated. Though follow-up was limited, our results suggest that CXL is feasible and effective in keratoconus associated with Down syndrome.