Collaborative Case #001: To Treat or Not to Treat...

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Case Presentation

A 48-year-old female presented with reduced BCVA OU. She reported wearing glasses for many years and had no history of contact lens wear. Her slit-lamp examination was completely normal except for mild punctate staining inferiorly OU. She had a clear lens and normal optic nerve and retinal examination. Her eye pressure was normal. UCVA was 20/200 OU. The refraction was -4.50 +2.50 x 110 OD = 20/30 OD and -4.00 +2.00 x 071 = 20/30 OS.

Answer: E is correct. All of the tests above are reasonable in the setting of a patient with reduced BCVA and a normal slit-lamp examination. Answers A-C above were all normal. The abnormal findings were found on topography.

Topography reveals:

Topography (Pentacam) reveals keratoconus OU. Corneal thickness is 444 µm OD and 460 µm OS. KMax is 52.8 OD and 52.0 OS.

One of the most important interventions for patients with keratoconus is to educate them about the effect of eye rubbing. Eye rubbing can weaken the cornea and advance the progression of keratoconus. On the other hand, there is no evidence that the use of rigid gas permeable contact lenses slow the rate of progression. Additionally, because the patient is relatively happy with her vision in spectacles, there is no reason to perform Intacs Corneal Implants (CorneaGen), as Intacs do not reduce the risk for progression of keratoconus. Because she does not correct to 20/20, scleral lenses are a potential option for this patient.

The main controversy is whether a 48-year-old patient with keratoconus will remain stable, or whether her condition will progress. In this case, the patient was advised that progression was possible. The patient elected to hold off on corneal collagen crosslinking (CXL), and return in 1 year for repeat topography.

Fast-forward 12 years

The patient skipped her 1-year appointment and returned 12 years after the initial visit. The patient is now age 60.

Answer: Topography was determined to have progressed significantly over the past 12 years.

The patient denied eye rubbing. She was evaluated and found to have progressed not only on topography, but also on refraction. In the more than the 12 years since her last visit, she developed additional myopia and astigmatism OU. Her refraction was now -5.50 +3.50 x 105 OD, -5.50 +4.00 x 050 OS. Her BCVA worsened to 20/40 OU. She also reported that she is now experiencing significant ghosting when looking in the distance with either eye. The slit-lamp examination remained normal. Pentacam Difference maps demonstrate that the cornea has progressed significantly OU over the 12 years.

Important Takeaways

Keratoconus is a progressive disease, and patients of any age can progress. Although the risk for keratoconus progression is lower in patients over 40 years of age compared to teenagers, progression can still occur. Patients over 40 years of age can therefore consider CXL to prevent further progression. iLink CXL (Glaukos) is effective at stopping progression of keratoconus in all ages, and many patients undergoing iLink CXL will have improvement of their corneal shape over the following months and/or years.

If patients elect to delay crosslinking, patients over 40 years of age can return for topographies every 6 to 12 months to monitor for progression. One of the main challenges with diagnosing keratoconus early is that the slit-lamp examination is typically normal. The classic findings on slit-lamp examination in patents, such as Vogt’s striae (fine vertical lines in the deep stroma) or a Fleischer ring (iron line in the deep layers of the epithelium that forms a ring under the protrusion of the cone) are not typically visible in early keratoconus. So, clinicians need to be suspicious and order topography when patients have worsening refractions (developing more myopia and astigmatism) and/or develop loss of BCVA.

William B. Trattler, MD

Director of Cornea, Center for Excellence in Eye Care, Miami

wtrattler@gmail.com

Financial disclosures: Consultant (Glaukos, CXL Ophthalmics, Oculus Optikgeräte)

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ABOUT THIS SERIES

On the path from initial diagnosis to severe disease management, patients may encounter a number of eye care providers. The Collaborative Care Case Series was developed, with guidance from William Trattler, MD, and Diana Shechtman, OD, FAAO, to challenge clinicians' understanding of diagnostic and treatment conventions and advance knowledge of all eye care providers along the continuum.

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