Corneal Treatments and Specialized Contact Lenses (Corneal remodeling, Corneal transplant, Corneal collagen crosslinking, Intrastomal Rings- INTACS, Keratoconus treatments, Keratoplasty, Scleral lenses) Medical Policy Service: Corneal Treatments and Specialized Contact Lenses (Corneal remodeling, Corneal transplant, Corneal collagen crosslinking, Intrastromal Rings- INTACS, Keratoconus treatments, Keratoplasty, Scleral lenses) PUM 250-0041-1709 Medical Policy Committee Approval 01/28/2021 Effective Date 02/01/2021 Prior Authorization Needed Yes Related Medical Policies: • Glaucoma Surgical Treatments • Non-Covered Services and Procedures Description: • Keratoconus is a degenerative non-inflammatory disorder of the cornea that results in progressive thinning and abnormal protrusion of the cornea. Distortion of the cornea results in decreased visual acuity (e.g. astigmatism and myopia). • Phototherapeutic Keratectomy (PTK) is performed to treat corneal scars, recurrent erosions, corneal dystrophies, and correct refractive errors caused by a diseased cornea such as keratoconus. PTK and corneal transplant are also performed to treat damage to the cornea caused by inflammatory, infectious, and traumatic conditions. • Corneal collagen cross-linking (CXL) is a procedure that uses UV light and a photosensitizer, such as riboflavin, to strengthen collagen bonds in the cornea to help restore the normal shape of the cornea, slow the process of keratoconus, and restore visual acuity. • Intrastromal corneal ring segments (INTACS) are prescription inserts that are fitted under the cornea to elevate the edge of the cornea to correct the corneal shape and improve visual acuity. • Scleral Shell lenses (also known as scleral lenses): Scleral Rigid Gas Permeable (RGP) lenses are very large (15 to 24 mm) lenses made to completely vault over the cornea. Scleral lenses also include prosthetic replacement of the ocular surface ecosystem (PROSE) treatment devices. These lenses are designed to extend beyond the cornea to rest on the sclera. They retain a layer of tears between the lens and the cornea. Page 1 of 5 • Keratoplasty (Corneal Transplant) is typically performed when glasses and contact lenses are no longer helpful to correct visual acuity. Keratoplasty can be performed in conjunction with cataract removal. Human cadaver corneal tissue is typically used for transplant. Artificial corneas (keratoprosthetics) have been developed and are proposed in cases of repeated failed cadaver transplants. Keratoprosthetics require review by the Health Plan’s Medical Director. Indications of Coverage: Note: Many health plans have specific coverage and exclusions for vision services, contact lenses, and implantable specialty lenses. When not specified by the health plan, scleral shell lenses are considered therapeutic contact lenses. I. Scleral Shell Contact Lenses: Scleral shell contact lenses and fittings are considered medically necessary when there is failure of topical medications, glasses, and contact lenses and at least one of the following: A. Corneal ectatic disorders (e.g., keratoconus, keratoglobus, pellucid marginal degeneration, Terrien’s marginal degeneration, Fuchs’ superficial marginal keratitis, post-surgical ectasia) B. Corneal scarring and/or vascularization C. Ocular surface disease (e.g., severe keratoconjunctivitis sicca/dry eye), persistent epithelial defects, neurotrophic keratopathy, exposure keratopathy, graft vs. host disease [GVHD], sequelae of Stevens Johnson syndrome, mucus membrane pemphigoid, post-ocular surface tumor excision, post-glaucoma filtering surgery) with pain and/or decreased visual acuity D. When prescribed to support orbital tissue (e.g. eye shrunken by inflammatory disease). Replacement lenses are considered medically necessary if there is a change in the eye condition (not including refractive changes). Note: Replacement of lenses that are lost, damaged, or requested solely due to refractive changes are typically not covered under the health plan. II. Corneal Transplant (Keratoplasty) using cadaver tissue is considered medically necessary for any of the following conditions in which standard conservative treatments (e.g. medications, glasses, contact lenses, and scleral shell lenses lenses) have failed or are contraindicated: A. To improve vision due to corneal opacity when best corrected vision still causes interference with activities of daily living (ADLs) B. To remove active corneal disease including keratoconus, corneal scaring with opacity, bullous keratopathy, Fuchs and other corneal dystrophies, and infectious / inflammatory keratitis after appropriate pharmacologic therapy (e.g. severe fungal, viral, bacterial or amoebic inflammation) C. To restore altered corneal structure or prevent loss of the globe after puncture injury D. Failure or rejection of previous corneal transplant Page 2 of 5 III. Intrastromal corneal ring segments, ICRS (INTACS) are considered medically necessary under the FDA approved humanitarian option when all of the following are met: A. Corneal transplantation is the only remaining option to improve the individual’s functional vision B. Progressive deterioration in vision, such that the individual can no longer achieve adequate functional vision on a daily basis with their contact lenses or glasses C. Individual is age 21 years or older D. Clear central corneas (no scarring or disease) E. Corneal thickness of 450 microns or greater at the proposed incision site IV. Conventional (epithelium-off) Corneal Collagen Cross-Linking (C-CXL) is considered medically necessary as a one-time treatment when all of the following are met: A. Diagnosis of keratoconus with progressively worsening vision or progressive corneal ectasia (that is not the result of previous LASIK procedure/refractive eye surgery) B. Individual is at least 14 years of age C. Corrected vision with eyeglasses or contact lenses is not adequate (is worse than 20/20). D. Central corneas have no scarring or disease E. Corneal thickness of at least 400 microns (verified with pachymetry) F. Procedure is performed using a U.S. Food and Drug Administration (FDA)-approved device, drug solution, and protocol. (At the time of this policy printing, only the Avedro KXL System® used with riboflavin solution Photrexa® or Photrexa Viscous® [following the Dresden protocol that was used in the clinical trials] is FDA-approved in the United States). Limitations of Coverage: A. Review health plan and endorsements for exclusions and prior authorization or benefit requirements. B. If used for a condition/diagnosis other than is listed in the Indications of Coverage, it will be denied as experimental, investigational, and unproven to affect health outcomes. C. If used for a condition/diagnosis that is listed in the Indications of Coverage, but the criteria are not met, it will be denied as not medically necessary. D. Transepithelial corneal collagen cross-linking (T-CXL), partial epithelium-off corneal cross-linking (P-CXL), and any collagen cross-linking procedure other than conventional epithelium-off (C-CXL) will be denied as experimental, investigational, and unproven to affect health outcomes. E. Accelerated corneal collagen cross-linking (A-CXL) will be denied as experimental, investigational, and unproven to affect health outcomes. Page 3 of 5 F. Topography-guided corneal collagen cross-linking (TG-CXL) will be denied as experimental, investigational, and unproven to affect health outcomes. G. Intrastromal corneal ring segments (INTACS) are considered experimental, investigational, and unproven to affect health outcomes unless all criteria in the Indications of Coverage are met. H. Corneal Hysteresis Measurement to diagnose or monitor keratoconus will be denied as experimental, investigational, and unproven to affect health outcomes. See also: Non-covered Services and Procedures medical policy. I. Corneal reconstruction using Amniotic Membrane Transplantation (AMT) will be denied as experimental, investigational, and unproven to affect health outcomes. J. Keratoplasty performed solely for correction of refractive errors or astigmatism will be denied as experimental, investigational, and unproven to affect health outcomes. K. Conventional corneal collagen cross-linking for the treatment of LASIK-related ectasia will be denied as experimental, investigational and unproven to affect health outcomes. L. More than one conventional (epithelium-off) corneal collagen cross-linking (C-CXL) procedure per eye per individual’s lifetime will be denied as experimental, investigational, and unproven to affect health outcomes. M. Conventional corneal collagen cross-linking used in conjunction with other treatments (e.g., CXL-plus, intrastromal corneal ring segments [INTACS], PRK, phakic intra-ocular lens implantation) will be denied as experimental, investigational, and unproven to affect health outcomes. N. Conventional corneal collagen cross-linking performed with a device, drug solution, or protocol that is not U.S. Food and Drug Administration (FDA)-approved for the procedure will be denied as experimental, investigational, and unproven to affect health outcomes. O. Use of BostonSight Prosthetic Replacement of the Ocular Surface Ecosystem (PROSE) for the treatment of dry eye disease (DED) will be denied as experimental, investigational, and unproven to affect health outcomes. P. Use of keratoprosthetics requires review by the Health Plan’s Medical Director. Documentation Required: • Office notes including history, physical, ophthalmic medication