Cross-linking may prevent keratoconus progression in patients with intellectual disability
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Corneal cross-linking is the best noninvasive step to protect patients with intellectual disability and keratoconus from vision loss, according to one specialist.
“Keratoconus is strikingly prevalent in the intellectual disability (ID) population,” Kirk Stephenson, MD, said at the virtual European Society of Cataract and Refractive Surgeons winter meeting. “In this population, it is difficult to modify behavioral risk factors such as eye rubbing, and vision loss contributes to the progression of intellectual disability and dementia.”
Ireland is the nation with the highest rate of Down syndrome in Europe, accounting for 26.3 live births in 10,000 as compared with 9.9 in Europe overall. In a study carried out at the Royal Victoria Eye and Ear Hospital in Dublin, a search of operating theater and clinic electronic records identified eligible patients among those who underwent CXL under general anesthesia as well as patients with ID followed in keratoconus clinics.
Twenty-four patients with ID were identified, two-thirds were male, and mean age was 32 years, ranging between 19 and 43 years. ID type was Down syndrome (66.6%), autism (25%) and other (8.3%). Mean follow-up was 21 months.
“Keratoconus was diagnosed in 98% of eyes, with 45.8% having untreatable advanced disease, 33.3% bilateral. CXL was performed in the 39.6% of eyes (48 eyes of 19 patients) that were amenable to this treatment. One-fifth of patients had bilateral cross-linking,” Stephenson said.
A small percentage of eyes with advanced disease (6.3%) had corneal transplantation.
CXL was performed under general anesthesia using an epi-off accelerated protocol with 10 minutes riboflavin saturation, 4 minutes UVA irradiation and total energy of 30 mJ/cm².
No general anesthesia-related adverse events were reported despite the high prevalence of congenital heart disease in the Down syndrome population. There were no severe ocular adverse events, with two cases of delayed re-epithelialization. Visual acuity was significantly better in the CXL group (logMAR 0.58) than the advanced group (logMAR 1.21).
“Patients with ID are rarely suitable for corneal transplantation, and CXL is a safe and effective intervention to prevent corneal blindness in this already socially restricted group,” Stephenson said. “Our next step is to assess patients in the pediatric and teenage population in Down syndrome clinics. We are proposing an algorithm for investigation and treatment of keratoconus and also advocate early screening to diagnose it while noninvasive treatment is an option.”
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Stephenson K, et al. The management of keratoconus in an Irish intellectual disability population. Presented at: European Society of Cataract and Refractive Surgeons winter meeting; Feb. 19-21, 2021 (virtual meeting).
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