Successful CXL on patients with Down Syndrome depends on patient cooperation and proper patient selection.
Last year, an article in International Ophthalmology reported on results of crosslinking (CXL) in this special population. The authors, who are based in the Netherlands, wrote, “Although the prevalence of keratoconus in patients with Down syndrome (DS) is much higher than in the general keratoconus population, the percentage of CXL treatments in our keratoconus centre is much lower in this group.” Despite the belief that CXL is a promising treatment for this population, the number of procedures actually performed is few. This observation would probably hold true in most locations performing CXL today.
Although the prevalence of keratoconus in patients with Down Syndrome is much higher than in the general keratoconus population, the percentage of CXL treatments is much lower.”
– N. Soeters et al
They developed a clinical decision tool that they use to assess patient compliance based on three periods of care. In the pre-operative assessment, the doctors were looking to see if the patient could make eye contact and communicate verbally. The doctors were also looking for spectacle tolerance and if slit-lamp and topographic map examinations could be performed.
The second domain assessed the ability of the patient to undergo the procedure: are there abrupt movements? Can eye drops be tolerated? Can eyelid touch be tolerated? Can the patient fixate on a light for five minutes while lying down?
The after-care assessment estimated post-procedure compliance: can the patient follow instructions and refrain from eye rubbing? And, finally, are the parents or caregivers competent and supportive of the treatment? With positive responses to this checklist, the doctors felt confident going forward with local anesthesia (eyedrops) rather than the more complex general anesthesia when performing CXL.
Dr. Ann Ostrovsky, MD, clinical assistant professor of ophthalmology at New York University Medical Center has experience crosslinking patients with developmental disabilities including patients with DS. She notes that:
Crosslinking, like any surgical procedure that requires patient cooperation, can be challenging. In order to avoid general anesthesia, we want to create a comfortable environment for the child to maximize cooperation.”
She suggests outfitting the treatment room in a child-friendly way, including “toys, posters of child-friendly themes and children’s music”. Ostrovsky also encourages parents to stay in the room with their children for the duration of the procedure.
Individuals with DS do no often complain about their vision, despite having a much higher incidence of keratoconus. It is often left to family members and others to notice vision problems and seek referrals. This can lead to a much more advanced case of KC when it is finally treated.
Dr. Marjan Farhad, MD, associate clinical professor of ophthalmology at UCI’s Gavin Herbert Eye Institute points out the problem with delayed treatments. “It is preferable to avoid corneal transplantation as it carries multiple risks including infection, rejection, and requires lifelong care. As such, CXL is essential in cases where even the slightest progression is seen. We need to stop the progression and avoid transplantation” for these patients.
Resource:
1. Soeters N, et al, Performing corneal crosslinking under local anaesthesia in patients with Down syndrome, Int Ophthalmol 2017 Apr 19. doi: 10.1007/s10792-017-0535-1
