Description Keratoconus is a noninflammatory degenerative eye condition characterized by progressive steepening and thinning of the normally round cornea into a cone shape. These changes lead to decreased visual acuity (distorted or blurred vision) and sensitivity to light or glare. The condition typically presents itself during adolescence or early adulthood. Although keratoconus usually affects both eyes, it may occur in one eye first or be more severe in one eye than the other. Initial interventions generally include glasses or soft contact lenses, but as keratoconus progresses, an individual may require fitting with rigid gas permeable or other types of contact lenses. (Refer to Coverage Limitations section)

Treatments for keratoconus include, but may not be limited to:

Keratoconus Surgical Treatments

Effective Date: 05/25/2023
Revision Date: 05/25/2023
Review Date: 05/25/2023
Policy Number: HUM-0314-018

Conventional (epithelium-off, epi-off) corneal collagen cross-linking (CXL)

involves debriding the most superficial layer of the cornea (corneal epithelium) prior to the application of medicated drops containing riboflavin (vitamin B2) to the eye followed by exposure to ultraviolet (UV) light. The procedure strengthens the bonds between collagen fibers in the cornea, thereby improving its shape and halting the progression of keratoconus.

The proprietary iLink procedure consists of the US Food & Drug Administration (FDA)-approved medications Photrexa and Photrexa Viscous when used in conjunction with the UVA-emitting KXL system.

Intrastromal corneal ring segments (Intacs)

are clear, micro-thin ophthalmic medical devices designed to reduce or eliminate nearsightedness and astigmatism as well as avoid corneal transplantation. The rings are surgically inserted and rest between layers of tissue in the cornea, outside the central optical zone, flattening the curvature of the cornea to restore functional vision and contact lens tolerance. Intacs may be removed and replaced with ring segments of different thickness if the eyes change after the original surgery.

A deep anterior lamellar keratoplasty (DALK) procedure

retains approximately five percent of the original cornea and replaces the rest with donor tissue. The individual’s own endothelium (lining of the cornea’s inner surface) is retained, thereby reducing the risk of graft rejection.

Full thickness corneal transplantation (penetrating keratoplasty)

is the treatment of choice when keratoconus has progressed, and other interventions are no longer helpful or are not an option. During surgery, the diseased cornea is removed and replaced with a donor cornea. Prior to the availability of CXL and Intacs, keratoplasty was considered the standard treatment for keratoconus.

Other proposed treatments for keratoconus include, but may not be limited to:

• Accelerated corneal collagen cross-linking (CXL) is similar to conventional CXL but is performed by using a higher UV light dose and decreasing the total exposure time. (Refer to Coverage Limitations section)
• Combination therapy using Intacs to flatten the cornea and CXL to halt disease progression is under study. (Refer to Coverage Limitations section)

Refer to Medical and Pharmacy Coverage Policies to verify that this is the current version before utilizing.

Endothelial keratoplasty

refers to a partial thickness keratoplasty that removes the unhealthy endothelium and replaces it with donor tissue. (Refer to Coverage Limitations section)

Transepithelial (epithelium-on, epi-on) corneal collagen cross-linking (CXL)

is similar to conventional CXL but is performed without removing the most superficial layer of the cornea (corneal epithelium) prior to the administration of eye drops. (Refer to Coverage Limitations section)

Coverage Determination

Corneal Collagen Cross-Linking (CXL)

Humana members may be eligible under the Plan for conventional (epithelium-off, epi-off) CXL when the following criteria are met:

• Diagnosis of progressive keratoconus; AND
• Any one or more of the following vision changes within the preceding 12 months:
  • Increase of 1.00 diopter (D) or more in the steepest keratometry measurement; OR
  • Increase of mean keratometry of 0.7 D or more; OR
  • Thinning of corneal pachymetry of 10 microns or more; OR
  • Reduction in uncorrected visual acuity or best spectacle corrected visual acuity by more than one line;
• AND both of the following23:
  • Clear central cornea (without scarring or disease); AND
  • Corneal thickness of at least 400 microns at the time of treatment

Intrastromal Corneal Ring Segments (Intacs)

Humana members may be eligible under the Plan for Intacs when the following criteria are met:

• Diagnosis of keratoconus with progressive deterioration of vision; AND
• Inadequate vision correction with eyeglasses or contacts; AND
• Corneal transplantation is the ONLY alternative to Intacs for improving vision; AND
• 21 years of age or older; AND
• Clear central cornea; AND
• Corneal thickness of 450 microns or more at the proposed incision site

Deep Anterior Lamellar Keratoplasty (DALK)

Humana members may be eligible under the Plan for deep anterior lamellar keratoplasty (DALK) for keratoconus when the following criteria are met:

• Absence of contraindications; AND
• Disease involving the anterior 95% of corneal thickness with normal endothelium; AND
• Other treatment options (eg, conventional CXL, Intacs) have failed or are not indicated

Full Thickness Corneal Transplantation (Penetrating Keratoplasty)

Humana members may be eligible under the Plan for full thickness corneal transplantation (penetrating keratoplasty) for a diagnosis of keratoconus when other treatment options (conventional CXL, Intacs, DALK) have failed or are not indicated.

Coverage Limitations

Humana members may NOT be eligible under the Plan for conventional (epithelium-off, epi-off) CXL, Intacs or full thickness corneal transplantation (penetrating keratoplasty) for any indications other than those listed above. All other indications are considered not medically necessary as defined in the member’s individual certificate.

Please refer to the member’s individual certificate for the specific definition.

Humana members may NOT be eligible under the Plan for deep anterior lamellar keratoplasty (DALK) for any indications other than those listed above, or in the presence of the following contraindications:

• Conditions relating to endothelial dysfunction or loss (eg, pseudophakic bullous keratopathy, iridocorneal endothelial syndrome); OR
• Posterior corneal dystrophies (eg, Fuchs’ corneal endothelial dystrophy, posterior polymorphous corneal dystrophy, congenital hereditary endothelial dystrophy)

This is considered experimental/investigational as it is not identified as widely used and generally accepted for any other proposed use as reported in nationally recognized peer-reviewed medical literature published in the English language.

Humana members may NOT be eligible under the Plan for the following procedures for keratoconus:

• Any CXL method other than conventional (epithelium-off, epi-off) (eg, accelerated protocol, transepithelial [epithelium-on, epi-on]); OR
• CXL in conjunction with Intacs; OR
• Endothelial keratoplasty

These are considered experimental/investigational as they are not identified as widely used and generally accepted for the proposed use as reported in nationally recognized peer-reviewed medical literature published in the English language.

Keratoconus Surgical Treatments

Effective Date: 05/25/2023
Revision Date: 05/25/2023
Review Date: 05/25/2023
Policy Number: HUM-0314-018
Page: 6 of 10

Humana's documents are updated regularly online. When printed, the version of this document becomes uncontrolled. Do not rely on printed copies for the most up-to-date version. Refer to Medical and Pharmacy Coverage Policies to verify that this is the current version before utilizing.

Humana members may NOT be eligible under the plan for:

• Correction of refractive errors; OR
• Fitting or purchase of contact lenses or eyeglasses; OR
• Refractive surgeries; OR
• Treatment for complications of noncovered elective procedures (corneal ectasia post-LASIK surgery)

These are generally excluded by certificate. In the absence of a certificate exclusion, it would be considered not medically necessary as defined in the member’s individual certificate.

Please refer to the member’s individual certificate for the specific definition.

Additional information about keratoconus or other visual problems may be found from the following websites:

Background

• American Academy of Ophthalmology
• National Eye Institute
• National Keratoconus Foundation
• National Library of Medicine

Medical Alternatives

Physician consultation is advised to make an informed decision based on an individual’s health needs.

Any CPT, HCPCS or ICD codes listed on this medical coverage policy are for informational purposes only. Do not rely on the accuracy and inclusion of specific codes. Inclusion of a code does not guarantee coverage and or reimbursement for a service or procedure.