Strategies lead to positive outcomes in tough cases
This article was reviewed by Ken K. Nischal, MD
Findings from studies investigating the efficacy and safety of corneal collagen crosslinking (CXL) for keratoconus in pediatric-age patients and developmentally delayed adults and the consequences of leaving their ocular disease untreated provide a solid base of evidence for offering CXL to these populations, according to Ken K. Nischal, MD.
“In the United States, CXL is only approved for the treatment of progressive keratoconus in patients from 14 to 65 years of age, and so it may not be offered to younger children even if indicated,” said Dr. Nischal, professor of ophthalmology and chief, Division of Pediatric Ophthalmology and Strabismus, University of Pittsburgh Medical Center, Pittsburgh, PA.
Keratoconous is not uncommon, with incidence rates reported up to 265 per 100,000 patients.
Unfortunately, disease onset tends to be in early adolescence, and these younger patients are at risk for rapid progression and a lifelong visual disability.
Dr. Nischal said his take on the issue is that technology should be for those who cannot advocate for themselves or who are the most vulnerable.
“Those individuals are the children and developmentally delayed adults,” he said, “In fact, there is mounting evidence that CXL should be offered to children at presentation rather than waiting for progression.”
Aggressive natural history
Several published studies demonstrate that keratoconus progresses at a rate that is much faster in children than in adults and that CXL arrests disease progression in pediatric patients, said Dr. Nischal.
Discussing some of these papers, he cited a study by Chatzis and Hafezi that reported a progression rate of 88% after one year of follow-up in a series of 59 eyes of 42 children awaiting CXL for keratoconus.1
Another study by Leon-Mesplie et al. showed that children who did not have CXL for keratoconus had a sevenfold increased risk of requiring a keratoplasty compared to adults.2
“If you do not want to do CXL in a child, you certainly do not want to have to perform penetrating keratoplasty considering the extra problems that it brings in terms of operative and postoperative issues,” Dr. Nischal said.
1. Chatzis N, Hafezi F. Progression of keratoconus and efficacy of pediatric corneal collagen cross-inking in children and adolescents. J Refract Surg. 2012’28:753-758.
2. Leoni-Mesplie S, Mortemousque B, Touboul D, et al. Scalability and severity of keratoconus in children. Am J Ophthalmol. 2012;154:56-62.
3. Padmanabhan P, Rachapalle Reddi S, Rajagopal R, et al. Corneal colleagen cross-linking for keratoconus in pediatric patients: log-term results. Cornea. 2017;36:138-143.
4. Mazzotta C, Traversi C, Baiocchi S, et al. Corneal collagen cross-linking with riboflavin and ultraviolet a light for pediatric keratoconus: ten-year results. Cornea. 2018;37:560-566.
5. Koppen C, Leysen I. Riboflavin/UVA cross-linking for keratoconus in Down syndrome. J Refract Surg. 2010;26:623-624.
6. S abti S, Tappeiner C, Frueh BE. Corneal cross-linking in a 4-year-old child with keratoconus and down syndrome. Cornea. 2015;34:1157-1160.
7. Lalgudi VG, Nischal KK. Pediatric corneal collagen cross-linking for keratoconus: not an experimental procedure. J AAPOS. 2019;23:63-65.