Elevating optometric keratoconus management

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Professional practice guidelines can help to establish a level understanding for how a particular condition should be diagnosed and managed. Formal guidelines bridge the gap between different generations of optometrists, establishing evidence-based standards for all of us, whether we have been in practice for 2 years or 2 decades. And for primary eye care doctors like me, they can be a very helpful reminder. In any given day, I see patients ranging in age from 5 to 85 years, and I deal with conditions ranging from amblyopia to age-related macular degeneration. There are a lot of things on my radar, so I actively use guidelines from the American Optometric Association (AOA) to develop processes and protocols that keep my offices up to date, especially for conditions we might not see as often.

Unfortunately, we don’t currently have any professional society guidelines in optometry for the diagnosis and management of keratoconus (KC).

Ophthalmology guidelines

The American Academy of Ophthalmology (AAO) is in the process of updating its preferred practice pattern (PPP) on corneal ectasia, which covers KC. It was last updated in 2018, recognizing that corneal cross-linking, at that time, had been recently approved in the US. Among other changes, the 2018 PPP was updated to clearly state, “Once progression is observed, early detection and prompt treatment with corneal cross-linking can reduce or stop keratoconus progression and preserve good visual acuity with eyeglasses and/or contact lenses.”¹ It also recommends follow-up every 3 to 6 months (or even more frequently for younger patients) to look for progression once KC has been diagnosed.¹

Medicolegally, the PPP guidelines can influence what courts consider to be the local standard of care. However, “The PPP is probably not the best document to guide primary care providers’ referrals, because the diagnostic criteria it discusses tend to be from tests such as tomography that are not widely available in primary care offices,” says Daniel Choi, MD, a Honolulu, Hawaii–based corneal surgeon and member of the AAO PPP Cornea and External Disease Panel.

The new ectasia PPP, which was released in February 2024,² places greater emphasis on identifying patients in the early or suspected stages of KC, according to Choi. “It is unfortunate that we still see so many patients presenting with significant vision loss that could have been prevented with earlier cross-linking,” he says. “Optometrists are the ones who can really identify these patients early in the course of the disease, so it is very important that we work together to spread awareness and advocate for our patients.”

Optometric initiatives

There have been some attempts at optometric guidelines on KC, including one spearheaded by the AAO’s Section on Cornea, Contact Lenses, and Refractive Technologies. “We are in the very early stages of developing suggested guidelines for both KC and myopia management,” says section chair Louise A. Sclafani, OD, FAAO, FSLS. Like Choi, she agrees that optometrists are the gatekeepers. “We see these cases much earlier than our ophthalmology colleagues, and so we have the opportunity and the responsibility to direct patients to practitioners who can perform cross-linking and/or fit them with advanced contact lens designs,” Sclafani says. She believes that guidelines would help primary eye care doctors educate patients and families: “It really helps to have a reputable and independent source validating that the treatment you are recommending is widely accepted,” she says.

Sclafani notes that there is always some tension between the gold standard in diagnosis vs what most would consider to be reasonable practice. “Optometrists don’t need to have every diagnostic tool at our disposal, but we do need to know when to refer a patient for further testing—and feel confident that patients will return back to our practices. There are so many opportunities for shared care and comanagement even within our field of optometry,” she says.

The International Keratoconus Academy (IKA) of Eye Care Professionals has long worked to promote awareness of KC and develop a knowledge base on its diagnosis and management. Recently, the IKA developed and completed a prospective observational study on patients 3 to 18 years old to explore the prevalence of KC in the pediatric population. It highlights how systematic screening with advanced diagnostics using tomography could improve the diagnosis of KC. The study, which has been submitted for publication, evaluated a pediatric population in Chicago, Illinois; the data showed a higher prevalence in this population than what has previously been reported in the general US population.³ These findings, from a subset of the general population in the US, underscore the importance of implementing a routine screening policy in your practice.

“I’d like to see every child be screened for keratoconus by age 12, and in an ideal world, that evaluation would include tomography,” says Andrew Morgenstern, OD, FAAO, FNAP, an author on the paper and member of the IKA executive board. “We now know that KC commonly begins to show clinical signs in the pediatric years, yet the comprehensive pediatric eye exam often does not include screening for KC even with classical equipment such as a corneal topographer or retinoscope,” he says. “In a pediatric eye exam, we always perform tests to evaluate IOP, but it almost never yields a case of pediatric glaucoma. Here, we have a more prevalent disease in the population that is not being routinely screened for.”

In fact, it was a lawsuit regarding a missed diagnosis of glaucoma that originally prompted guidelines to include glaucoma screening across all ages.⁴ When it comes to KC, we know that in practice most doctors are not routinely screening for it; many don’t have topography or tomography and may not be aware of the signs and symptoms to look out for with basic equipment.

Morgenstern, who also serves as director of the AOA Clinical Resources Group, charged with developing evidence-based clinical practice guidelines for the AOA, says that evaluating young people for KC as part of a comprehensive eye examination is important for 2 reasons. “First, early detection can lead to earlier treatment, but even if we aren’t ready to recommend treatment yet, kids with risk factors should be followed closely because there can be really dramatic and rapid changes from the onset of puberty through early adulthood,” he says. The IKA’s efforts will likely continue to focus on diagnosis. “Guidelines for treatment are challenging because there is great variability in the presentation of KC across different stages, age, corneal thickness, curvature, and refractive error,” says Morgenstern. “However, US practitioners should, at minimum, follow the guidance of the FDA clinical trials and approvals.”

Medicolegal risk

Speaking as both a doctor and a lawyer who specializes in health care law, I’d like to see optometry develop clear professional standards around caring for patients with KC.

Many optometrists practicing today were taught in school to fit patients with KC in gas-permeable lenses and then just monitor the patient until they needed a corneal transplant. With the availability of iLink cross-linking (Glaukos), a treatment that can slow or halt KC progression, that guidance no longer serves the patient in many cases. Of course, doctors always have to weigh potential risks and benefits in making decisions about the appropriate care path for their patients, but my concern is that if a doctor relies on contact lenses and monitoring alone, just because that is “how it has always been done,” without seeking a cross-linking consultation, they may be held liable if the patient progresses and loses vision. In my opinion, just claiming that you didn’t know is no longer defensible. The reality is that from a medicolegal perspective, we all have a responsibility to keep up with changes in the field and to see that patients get the best care today.

Any guidelines should address these 3 elements that have the potential to pose a legal risk that optometrists face: failure to detect,⁵ failure to refer,⁶ and negligent referral.

• Detection of risk factors and corneal or vision changes consistent with KC is within the capability of any optometrist, using common tools such as the slit lamp, keratometer, and retinoscope, as well as through patient history. If you don’t have topography or tomography to confirm the diagnosis, then you have to send the patient out to someone who does, whether that is a corneal specialist or an optometrist with a more medically oriented practice.
• Referral for evaluation and possible treatment of progressive KC is necessary, even if the patient’s vision can be managed with contact lenses or hasn’t been affected yet, which ultimately is the goal. A key point here is that just because you don’t provide a particular test or service the patient needs doesn’t mean that you’re not obligated to make an educated referral to someone who does. If a patient is a new patient to me and I don’t know if they have progressed or not, for example, I would rather have in my chart that I sent them for an evaluation.
• We have a responsibility to refer the patient for evidence-based care. In the US, there is only one FDA-approved cross-linking treatment: iLink with the KXL system (Glaukos), riboflavin 5’-phosphate ophthalmic solution (Photrexa; Glaukos), and riboflavin 5’-phosphate in 20% dextran ophthalmic solution (Photrexa Viscous; Glaukos). This drug/device combination is also the only one that can be reimbursed by insurance as a covered procedure. Referring a patient to physicians who perform treatment with unapproved drugs or devices, unless they are part of a registered clinical trial that not only has investigational review board oversight but also explicitly has investigational new drug/device exemption approval from the FDA, exposes the referring optometrist to medicolegal risk.

In my opinion, new guidelines based on current research would go a long way toward helping busy optometrists ensure that they are doing the best thing for their patients with KC, a disease for which standards of care have evolved rapidly, while protecting themselves medicolegally in the process. Until then, we must follow the science and do what’s right for our patients and our practice; it is incumbent on all optometrists to detect and refer KC earlier and to ensure an evidence-based referral.

References:

1. Garcia-Ferrer FJ, Akpek EK, Amescua G, et al; American Academy of Ophthalmology Preferred Practice Pattern Cornea and External Disease Panel. Corneal Ectasia Preferred Practice Pattern. Ophthalmology. 2019;126(1):170-215. doi:10.1016/j.ophtha.2018.10.021

2. Corneal Ectasia Preferred Practice Pattern. American Academy of Ophthalmology. February 2024. Accessed May 14, 2024. https://www.aao.org/education/preferred-practice-pattern/corneal-ectasia-ppp-2023

3. Block SS, Harthan J, Zhuang X, et al. Prevalence of abnormal corneas in the United States based on Scheimpflug tomography analytics of a pediatric population. Invest Ophthalmol Vis Sci. 2022;63:2415.

4. Helling v Carey, 83 Wn.2d 514,519 P.2d 981,67 ALR.3d 175 (Wash 1974).

5. Duszak RS, Duszak R Jr. Malpractice payments by optometrists: an analysis of the national practitioner databank over 18 years. Optometry. 2011;82(1):32-37. doi:10.1016/j.optm.2010.05.009

6. Medical liability: missed follow-ups a potent trigger of lawsuits. American Medical News. July 15, 2013. Accessed October 9, 2023. https://amednews.com/article/20130715/profession/130719980/2/