The Ultimate Keratoconus Referral Guide

Keratoconus patients often end up in referral clinics, yet many of these patients start their eye care journey in retail-oriented practices. It often takes an astute eye doctor in one of these practices to suspect keratoconus. Today, it is more important than ever to diagnose keratoconus early and make sure these patients get the proper care. Not only can you intervene before further loss of vision—today, your license could depend on it.

In April of 2016, the FDA granted approval for a cross-linking system for progressive keratoconus—a UV light source and two riboflavin formulations by Avedro (since acquired by Glaukos). Cross-linking (CXL) seeks to slow or halt the progression of keratoconus. Because of the efficacy of CXL, a missed or delayed diagnosis of progressive keratoconus can result in preventable vision damages. Put bluntly, this means that if you do not make a prompt and proper referral for progressing keratoconus, you could get sued for malpractice.

Progression of keratoconus tends to occur in the adolescent years into the twenties. By the third to fourth decade of life, progression of keratoconus tends to self-arrest. Therefore, the greatest role for CXL is likely for keratoconus patients younger than age 30. In the same way that low-dose atropine therapy makes the most sense for a low myopic child and not the adult, the ideal time to arrest keratoconus is when it is mild and at the earliest sign of progression.

There is not much value to slow or halt keratoconus if it has advanced to displaying Munson’s sign, where the lower lid deflects over the grossly distorted corneal surface. Nor does it make sense to try arresting keratoconus when it has already run its natural course and has stabilized on its own later in life.

This is not a theoretical concern. It is a new and deep pitfall to dodge. I am the plaintiff’s expert witness in a medical malpractice suit where the defendant optometrist diagnosed keratoconus in a teenager but allegedly failed to order corneal topography and did not refer the patient for CXL when progression was evident. The teenager’s keratoconus drastically progressed and resulted in irreversible vision loss. The defendant could have avoided legal entanglement by referring the patient to a colleague with expertise in managing keratoconus. Expect more of these medicolegal claims to arise in the coming years.

For clinicians in retail settings, the amenities to manage keratoconus are not always available. These include corneal topography, diagnostic contact lens sets for keratoconus including hybrid and scleral lenses, pachymetry, anterior segment ocular coherence tomography, and genetic testing for keratoconus (AvaGen by Avellino). Furthermore, the exam time allocation in retail clinics is optimized for disposable contact lens and eyeglass workflow. This often does not lend itself well for scleral lens prescribing, training, and the necessary progress visits with lens exchanges.

While you can refer keratoconus patients directly to a corneal specialist for CXL and/or Intacs surgery, there are three weaknesses in this approach.

First, almost all keratoconus patients still need rigid contact lens optics for the best vision after corneal surgery. Keratoconus patients who start at the corneal specialist sometimes never receive contact lens treatment, particularly after surgery, and are left with subpar vision. This happens more with surgeons who do not work with a contact lens specialist.

Second, a small fragment of corneal surgeons promote CXL even when the benefits are questionable, breaking their fiduciary responsibility to the patient. For example, I have observed some of my older keratoconus patients with stable keratoconus sold on CXL. These keratoconus patients are best served by non-surgical treatment.

Third, CXL and/or Intacs can cause a keratoconus patient to lose eligibility for necessary contact lenses. As an example, one criterion under EyeMed Vision Care’s necessary contact lens authorization for “moderate/severe keratoconus” is a steep keratometry of 53.00D or greater. Without this stepped-up reimbursement, the economics do not allow to get the patient into scleral lenses. Therefore, if your keratoconus patient with EyeMed has CXL and ends up with steep keratometry values of 52.00 diopters but still needs scleral contact lenses to function, this patient will bear the full cost of these specialty custom lenses out-of-pocket each year.

Undergoing CXL may slow or halt progressing keratoconus, but in these unfortunate situations, it could impose a tyrannical and recurring out-of-pocket expense of thousands of dollars each year due to lost qualification for necessary contact lenses. Few corneal surgeons are familiar with this nuance. In fact, I have yet to find an informed consent form for CXL which discloses this possibility.

For all these reasons, there is good rationale to refer your keratoconus patients first to a contact lens specialist to evaluate for non-surgical vision restoration and coordinate any surgical consultation. If there is an indication for surgery, the contact lens practitioner will involve the expertise of a corneal surgeon either before or after contact lens prescribing.

It should go without saying: recommend a contact lens specialist who has a track record of bringing desirable outcomes. If the patient has a good experience, that reflects favorably on you. Yet the opposite is also true. Therefore, ask your colleagues who they trust.

Your selected practitioner should demonstrate commitment to these complex cases. Some indicators are inclusion on the National Keratoconus Foundation’s Doctor Locator, Scleral Lens Education Society fellowship status, and availability of advanced technology in the practice including corneal topography, anterior segment ocular coherence tomography (OCT), and pachymetry.

Other nice-to-have indicators include a history of presenting continuing education on keratoconus, writing in trade publications on keratoconus, consulting and performing clinical evaluations for industry including scleral lens manufacturers, and in-office capability of prescribing custom-level scleral lenses using profilometry or an eye surface impression (e.g., EyePrintPRO).

It helps if the practice you refer to accepts major vision plans such as Vision Service Plan and EyeMed Vision Care, since that will minimize cost to your patients. Online ratings and reviews of the practice and doctor (as imperfect as they are) can provide insight on the level of customer service your patient may receive.

Finally, the doctor you refer to should send you correspondence regarding their findings and treatment and freely communicate with you. Not only does this satisfy your curiosity of what happened to your patient and broadens your clinical knowledge, it also provides you with protective documentation against future medicolegal allegations of failure to refer.

When making the referral, record in your exam record the reason for your referral, the name of the doctor you would like the patient to see, and the timeline when the patient should be seen by. Let the patient know why you are making this referral. As an example, when I refer patients out to the retina or glaucoma specialist, I might say, “For any friend, family member or patient with your condition, I would like a specialist to evaluate this within 4 weeks. The care you need is outside of my expertise, and I recommend Dr. Smith who has the qualifications and I know will take great care of you.” I have my staff help schedule the patient while also providing relevant clinical data to the specialist’s office. Remember that these referrals constitute treatment under the HIPAA Privacy Rule. In other words, disclosure of the patient’s protected health information (PHI) for treatment and coordination of care does not require obtaining the patient’s signed medical release.

Convey to your keratoconus patient that at their referral appointment, their doctor will review their situation and options, and go over proposed treatment. Patients need to know that contact lens prescribing is not always initiated at that visit due to preparatory work. This is analogous to sending a patient with cataracts for cataract surgery where the initial appointment is to plan the course of treatment ahead.

The initial visit with the contact lens specialist is an opportunity for the keratoconus patient to have their diagnosis confirmed, learn about the severity, prognosis, and treatment options (e.g., corneal GP, hybrid contacts, scleral GP, Intacs, CXL, etc.), review anticipated out-of-pocket costs, and understand the time needed to achieve a desirable outcome. For patients with a necessary contact lens authorization, clinical data collected at the initial visit is submitted for approval. This initial visit can also bring much-needed peace of mind for the newly diagnosed keratoconus patients since they frequently have many accumulated questions. Finally, it helps the specialist and patient develop rapport and a common understanding before moving ahead.

In the past two decades, few eye conditions have experienced such compelling advances as keratoconus. CXL and scleral contact lenses have transformed this former clinical obstacle into an opportunity. Yet understandably, not all optometrists are in practice settings amenable to providing care for keratoconus. It is also completely understandable that not all optometrists are comfortable or have the background to provide the needed care. In these cases, it is wise to refer to an appropriate specialist. Your patient will thank you for it, and it also ensures that you meet the community standard of care.

I would be happy to help your keratoconus patients!

My highest clinical calling and passion is providing care for keratoconus. More than 125 eye care practitioners have recommended and entrusted me to their keratoconus patients. While each case of keratoconus is unique, my team and I frequently bring compelling and desirable outcomes to these patients, allowing them to live more enjoyable, productive lives.

After your keratoconus patient is evaluated at ReVision Optometry, I send out correspondence summarizing what was done and found. This documentation is appropriate to include in the patient’s medical file. It can minimize your exposure to potential claims of failure to refer or negligent referral.

Referring doctors wishing to learn the art and science of scleral lens prescribing are welcome to observe their patient’s examination, diagnostic contact lens fitting, and related progress visits.

To learn more about referring your keratoconus patient to ReVision Optometry, please visit our referral page.