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A.9.03.28
Corneal collagen cross-linking is a photochemical procedure approved by the U.S. Food and Drug Administration for the treatment of progressive keratoconus and corneal ectasia following refractive surgery. Keratoconus is a dystrophy of the cornea characterized by progressive deformation (steepening) of the cornea while corneal ectasia is keratoconus that occurs following refractive surgery. Both conditions can lead to functional loss of vision and need for corneal transplantation.
Keratoconus and Ectasia
Keratoconus is a bilateral dystrophy characterized by progressive ectasia (paracentral steepening and stromal thinning) that impairs visual acuity. While frequently diagnosed at a young age, the progression of keratoconus is variable. Results from a longitudinal study of over 900 patients with keratoconus showed that there was a decrease of 2 high- and 4 low-contrast letters in best-corrected visual acuity over 7 years follow-up. About 1 in 5 patients showed a decrease of 10 or more letters in high-contrast visual acuity and one-third of patients showed a decrease of 10 or more letters in low-contrast visual acuity.
Ectasia (also known as keratectasia, iatrogenic keratoconus, or secondary keratoconus) is a serious long-term complication of laser in situ keratomileusis (LASIK) surgery and photorefractive keratectomy. It is similar to keratoconus, but occurs postoperatively and primarily affects older populations. It may result from unrecognized preoperative keratoconus or, less frequently, from the surgery itself. Similar to keratoconus, it is characterized by progressive thinning and steepening of the cornea, resulting in corneal optical irregularities and loss of visual acuity.
Treatment
The initial treatment for keratoconus often consists of hard contact lenses. A variety of keratorefractive procedures have also been attempted, broadly divided into subtractive and additive techniques. Subtractive techniques include photorefractive keratectomy or laser in situ keratomileuses, although generally, results of these techniques have been poor. Implantation of intrastromal corneal ring segments is an additive technique in which the implants are intended to reinforce the cornea, prevent further deterioration, and potentially obviate the need for penetrating keratoplasty. Penetrating keratoplasty (i.e., corneal grafting) is the last line of treatment. About 20% of patients with keratoconus will require corneal transplantation. All of these treatments attempt to improve the refractive errors, but are not disease-modifying.
Treatment options for ectasia include intraocular pressure-lowering drugs and intracorneal ring segments. Frequently, penetrating keratoplasty is required.
None of the currently available treatment options for keratoconus and corneal ectasia halt the progression of the disease, and corneal transplantation is the only option available when functional vision can no longer be achieved.
Corneal collagen cross-linking has the potential to slow the progression of the disease. It is performed with the photosensitizer riboflavin (vitamin B2) and ultraviolet A irradiation. There are 2 protocols for corneal collagen cross-linking:
Epithelium-off corneal collagen cross-linking (also known as “epi-off”): In this method, about 8 mm of the central corneal epithelium is removed under topical anesthesia to allow better diffusion of the photosensitizer riboflavin into the stroma. Following de-epithelialization, a solution with riboflavin is applied to the cornea (every 1-3 minutes for 30 minutes) until the stroma is completely penetrated. The cornea is then irradiated for 30 minutes with ultraviolet A 370 nm, a maximal wavelength for absorption by riboflavin, while the riboflavin continues to be applied. The interaction of riboflavin and ultraviolet A causes the formation of reactive oxygen species, leading to additional covalent bonds (cross-linking) between collagen molecules, resulting in stiffening of the cornea. Theoretically, by using a homogeneous light source and absorption by riboflavin, the structures beyond a 400-mm thick stroma (endothelium, anterior chamber, iris, lens, retina) are not exposed to an ultraviolet dose that is above the cytotoxic threshold.
Epithelium-on corneal collagen cross-linking (also known as “epi-on” or transepithelial): In this method, the corneal epithelial surface is left intact (or may be partially disrupted) and a longer riboflavin loading time is needed.
Currently, the only corneal collagen cross-linking treatment approved by the U.S. Food and Drug Administration (FDA) is the epithelium-off method. There are no FDA-approved corneal collagen cross-linking treatments using the epithelium-on method. Corneal collagen cross-linking is being evaluated primarily for corneal stabilization in patients with progressive corneal thinning, such as keratoconus and corneal ectasia following refractive surgery. Corneal collagen cross-linking may also have anti-edematous and antimicrobial properties.
In 2016, riboflavin 5'-phosphate in 20% dextran ophthalmic solution (Photrexa Viscous™; Avedro) and riboflavin 5'-phosphate ophthalmic solution (Photrexa™; Avedro) were approved by the FDA for use with the KXL System in corneal collagen cross-linking for the treatment of progressive keratoconus and corneal ectasia after refractive surgery.
Related policies are –
Corneal collagen cross-linking using riboflavin and ultraviolet A may be considered medically necessary as a treatment of progressive keratoconus or corneal ectasia resulting from refractive surgery in individuals who have failed conservative treatment (eg, spectacle correction, rigid contact lens).
Corneal collagen cross-linking using riboflavin and ultraviolet A is considered investigational for all other indications.
None
The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.
The American Academy of Ophthalmology has not set forth definitive criteria defining progressive keratoconus, but has suggested that signs of progression include changes in refraction, visual acuity and corneal shape ( https://eyewiki.aao.org/Corneal_Collagen_Cross-Linking ). In the trials leading to FDA approval of corneal collagen cross-linking, progressive keratoconus or corneal ectasia is defined as one or more of the following:
An increase of 1 diopter (D) in the steepest keratometry value
An increase of 1 D in regular astigmatism evaluated by subjective manifest refraction
A myopic shift (decrease in the spherical equivalent) of 0.50 D on subjective manifest refraction
A decrease ≥0.1 mm in the back optical zone radius in rigid contact lens wearers where other information was not available.
Medically Necessary is defined as those services, treatments, procedures, equipment, drugs, devices, items or supplies furnished by a covered Provider that are required to identify or treat a Member's illness, injury or Mental Health Disorders, and which Company determines are covered under this Benefit Plan based on the criteria as follows in A through D:
A. consistent with the symptoms or diagnosis and treatment of the Member's condition, illness, or injury; and
B. appropriate with regard to standards of good medical practice; and
C. not solely for the convenience of the Member, his or her Provider; and
D. the most appropriate supply or level of care which can safely be provided to Member. When applied to the care of an Inpatient, it further means that services for the Member's medical symptoms or conditions require that the services cannot be safely provided to the Member as an Outpatient.
For the definition of medical necessity, “standards of good medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. BCBSMS makes no payment for services, treatments, procedures, equipment, drugs, devices, items or supplies which are not documented to be Medically Necessary. The fact that a Physician or other Provider has prescribed, ordered, recommended, or approved a service or supply does not in itself, make it Medically Necessary.
Investigative is defined as the use of any treatment procedure, facility, equipment, drug, device, or supply not yet recognized as a generally accepted standard of good medical practice for the treatment of the condition being treated and; therefore, is not considered medically necessary. For the definition of Investigative, “generally accepted standards of medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. In order for equipment, devices, drugs or supplies [i.e, technologies], to be considered not investigative, the technology must have final approval from the appropriate governmental bodies, and scientific evidence must permit conclusions concerning the effect of the technology on health outcomes, and the technology must improve the net health outcome, and the technology must be as beneficial as any established alternative and the improvement must be attainable outside the testing/investigational setting.
07/19/2012: Approved by Medical Policy Advisory Committee.
05/08/2013: Policy reviewed; no changes.
07/03/2014: Policy reviewed; description updated regarding devices. Added Corneal Topography/Computer-Assisted Photokeratoscopy as a related policy. Policy statement unchanged.
07/13/2015: Code Reference section updated for ICD-10.
10/30/2015: Policy description updated. Policy statement unchanged. Investigative definition updated in policy guidelines section.
05/27/2016: Policy number A.9.03.28 added.
07/11/2016: Policy description updated regarding devices. Policy statement unchanged. Code Reference section updated to add CPT code 0402T.
09/15/2017: Policy description updated regarding treatment of keratoconus and corneal ectasia. Added policy statement that corneal collagen cross-linking using riboflavin and ultraviolet A may be considered medically necessary as a treatment of progressive keratoconus or corneal ectasia after refractive surgery in patients who have failed conservative treatment (eg spectacle correction, rigid contact lens). Investigational statement revised to state that corneal collagen cross-linking using riboflavin and ultraviolet A is considered investigational for all other indications. Policy Guidelines updated to define progressive keratoconus and corneal ectasia. Added medically necessary definition. Code Reference section updated to change investigational codes table to medically necessary codes table. Added ICD-10 code ranges H18.601 - H18.629 and H18.711 - H18.719.
04/16/2018: Policy reviewed; no changes.
12/19/2018: Code Reference section updated to add new HCPCS code J2787, effective 01/01/2019.
04/11/2019: Policy reviewed; no changes.
07/01/2019: Code Reference section updated to revise code description for CPT code 0402T.
04/20/2020: Policy reviewed; no changes.
06/09/2021: Policy reviewed. Policy statements unchanged. Policy Guidelines updated to change "Nervous/Mental Conditions" to "Mental Health Disorders" and "Medically Necessary" to "medical necessity."
05/23/2022: Policy description updated regarding a study of patients with keratoconus. Medically necessary policy statement updated for clarification to change "after refractive surgery" to "resulting from refractory surgery." Policy Guidelines updated regarding the American Academy of Ophthalmology.
06/28/2022: Code Reference section updated to revise code description for CPT code 0402T, effective 07/01/2022.
04/20/2023: Policy reviewed. Policy statement updated to change "patients" to "individuals."
04/25/2024: Policy reviewed; no changes.
04/23/2025: Policy reviewed; no changes.
Blue Cross Blue Shield Association policy # 9.03.28
This may not be a comprehensive list of procedure codes applicable to this policy.
The code(s) listed below are ONLY medically necessary if the procedure is performed according to the "Policy" section of this document.
Code Number | Description |
CPT-4 | |
0402T | Collagen cross-linking of cornea, including removal of the corneal epithelium when performed, and intraoperative pachymetry, when performed |
66999 | Unlisted procedure, anterior segment of eye |
HCPCS | |
J2787 | Riboflavin 5'-phosphate, ophthalmic solution, up to 3 mL |
ICD-10 Procedure | |
ICD-10 Diagnosis | |
H18.601 - H18.629 | Keratoconus code range |
H18.711 - H18.719 | Corneal ectasia code range |
CPT copyright American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association.
