Light for Sight 21 initiative important in treating keratoconus in children with Down syndrome
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The last decade revolutionized how we look at and treat keratoconus. Improvements in surgical techniques such as deep anterior lamellar keratoplasty resulted in better long-term visual outcomes. But the one thing that has significantly made an impact since its initiation is the use of collagen cross-linking as a treatment modality to stabilize keratoconus at an earlier stage of the disease process, such that the quality of vision can be better preserved and the probable need for keratoplasty can be reduced.
Down syndrome is one of the most common chromosomal abnormalities that can have an increased incidence of keratoconus. The importance of the Light for Sight 21 initiative to increase awareness about the presence of keratoconus in Down syndrome patients, possibly resulting in earlier diagnosis and treatment, cannot be overemphasized. These children have various intellectual disabilities and developmental delays, and good visual acuity goes a long way in improving their quality of vision and hence their quality of life.
Because most of these patients are cared for by general ophthalmologists, making them aware about the various ophthalmic associations of Down syndrome and an earlier diagnosis with a prompt referral to a cornea specialist might help in diagnosis of keratoconus in the milder form. Treatment of keratoconus at that stage might help in preserving better spectacle vision, with a lower need for using contact lenses for better vision in these patients, who might have issues using contact lenses.
The idea of working with the Down syndrome society is probably the right way forward to evaluate these patients in a comprehensive manner, especially from the second decade onward when the incidence of keratoconus increases.
The classical technique of cross-linking is epithelium off, and based on the level of patient cooperation, the procedure can be done under topical anesthesia with or without sedation or general anesthesia. In select cases, an epithelium-on technique or partial removal of the epithelium can be attempted. In patients with advanced disease and corneas thinner than 400 µm, the use of hypo-osmolar riboflavin solution and contact lens-assisted cross-linking are options to safely perform cross-linking.
Because these patients have variable levels of intellectual disability, it would be ideal to have their check-ups done in such a way that they do not have to travel a long distance. Working with a cornea specialist nearby who has the required facility to help in diagnosis and treating these patients seems to be a logical answer to the problem.
Even though results with penetrating keratoplasty and deep anterior lamellar keratoplasty are good, the risk of graft dehiscence after trauma is a real concern, especially in this group of patients, and every effort should be made to maintain or improve their visual acuity without the need for keratoplasty.
The data collected by collating all the results of treatment of patients might help us better understand the disease and set up better protocols to tackle the problem.
The efforts by the Hafezis and their group in initiating this movement to target this group of patients need to be applauded.
Disclosure: No products or companies that would require financial disclosure are mentioned in this article.