BLOG: For young patients with keratoconus, urgency is warranted
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Key takeaways:
- Keratoconus can progress more rapidly in pediatric patients.
- Referrals, patient education, treatment scheduling and ongoing follow-up are part of the keratoconus process.
There has been a sea change in the care of patients with keratoconus in the U.S. since the FDA approval of iLink corneal collagen cross-linking in 2016.
Ophthalmologists have shifted from monitoring patients with vision correction alone to more definitive treatment as early as possible. Recently, the American Academy of Ophthalmology updated its Preferred Practice Pattern on corneal ectasia to emphasize that cross-linking is the recommended treatment for progressive keratoconus. Early diagnosis and treatment of pediatric patients are particularly important because keratoconus in this population can progress more rapidly than in older patients.
I find that in young patients who have high keratometry values, specifically maximum keratometry, in the setting of vision loss uncorrectable with glasses, keratoconus should be strongly suspected. I consider these patients to be high risk for further disease progression and future vision loss and would advocate for treatment as quickly as possible. iLink (Glaukos) is indicated for patients between the ages of 14 and 65 years due to the age of the patients originally enrolled in the pivotal FDA study. However, many have been able to successfully obtain insurance coverage for patients even younger than this, at times requiring a peer-to-peer call to explain the medical necessity of cross-linking. Fortunately, many insurance companies are not pushing back on pediatric cross-linking, likely as they recognize its cost-effectiveness over a potential corneal transplant surgery.
We have put in place processes to maintain a sense of urgency in referrals, patient education, treatment scheduling and ongoing follow-up.
Thanks to significant outreach efforts by universities, private practices, professional societies and Glaukos, most optometrists now recognize the importance of ordering imaging or making a referral when there is any suspicion of keratoconus. Warning signs include frequently changing refractions, an inability to correct vision with glasses or standard contact lenses, and progressive or unusual myopic astigmatism in a young patient. The combination of corneal topography and Scheimpflug tomography makes early diagnosis much more reliable, so there is no reason to delay imaging even when the clues are subtle.
Next, it’s a matter of conveying to the patient and their family the urgency of a procedural intervention. We are fortunate to have a lot of good printed and video resources available to explain the natural history of keratoconus progression. I emphasize that the goal of cross-linking is to preserve their vision so it doesn’t get worse, and I share that we have been successful in doing so for many other children and families. In addition, it is very important to talk about behavioral changes (no eye rubbing) and mitigation strategies, such as making sure that allergies are being appropriately managed with systemic antihistamines and topical antihistamine/mast cell stabilizers.
Once the family is on board with treatment, it is important to make sure that treatment can be provided in a timely manner — ideally, within 6 weeks of diagnosis for patients younger than 18 years.
I have designated spots every week in my clinic for keratoconus patients. At UCSF, we’re fortunate to have multiple doctors who are comfortable treating pediatric patients; in a smaller practice, one may need to build in that redundancy with colleagues at other practices who can treat young patients during the surgeon’s absences or at times when the schedule is full.
Finally, it is important to monitor young patients after cross-linking. Although there is a report in the literature of 10-year outcomes after cross-linking in the U.S., long-term follow-up of young patients is still limited, so I follow patients who are younger than 15 years old every 6 months after cross-linking to make sure that there’s no postoperative progression. Although I haven’t seen any progression yet, it makes sense to me to stratify follow-up protocols by age and disease severity.
References:
- Buzzonetti L, et al. Cornea. 2020;doi:10.1097/ICO.0000000000002420.
- Greenstein SA, et al. Eye Contact Lens. 2023;doi:10.1097/ICL.0000000000001018.
- Jhanji V, et al. Ophthalmology. 2024;doi:10.1016/j.ophtha.2023.12.038.
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