• Computerized Corneal Topography/Videokeratography (Policy #003 in the Medicine Section)
• Implantation of Intrastromal Corneal Ring Segments (Policy #058 in the Surgery Section)

• Corneal ectatic disorders (e.g., keratoconus, keratoglobus, pellucid marginal degeneration, Terrien marginal degeneration, Fuchs’ superficial marginal keratitis, post-surgical ectasia);
• Corneal scarring and/or vascularization;
• Irregular corneal astigmatism (e.g., after keratoplasty or other corneal surgery);
• Ocular surface disease (e.g., severe dry eye, persistent epithelial defects, neurotrophic keratopathy, exposure keratopathy, graft vs. host disease, sequelae of Stevens-Johnson syndrome, mucus membrane pemphigoid, post-ocular surface tumor excision, post-glaucoma filtering surgery) with pain and/or decreased visual acuity.

Medicare Coverage:
Medicare specifically excludes certain items and services from coverage, including eyeglasses and contact lenses. However, Medicare provides an exception for one pair of eyeglasses or contact lenses as a prosthetic device furnished after each cataract surgery with insertion of an IOL

Per CMS Medicare Benefit Policy Manual Chapter 15-Covered medical and other health Services Rev. 228, 10-13-16, prostheses replacing the lens of an eye include post-surgical lenses customarily used during convalescence from eye surgery in which the lens of the eye was removed. In addition, permanent lens are also covered when required by an individual lacking the organic lens of the eye because of surgical removal or congenital absence.

For beneficiaries who are aphakic (i.e., who have had a cataract removed but do not have an implanted intraocular lens (IOL) or who have congenital absence of the lens), the following lenses or combinations of lenses are covered when determined to be medically necessary:
1. Bifocal lenses in frames; or
2. Lenses in frames for far vision and lenses in frames for near vision; or
3. When a contact lens(es) for far vision is prescribed (including cases of binocular and monocular aphakia), payment will be made for the contact lens(es), and lens(es) in frames for near vision to be worn at the same time as the contact lens(es) and lenses in frames to be worn when the contacts have been removed.

For additional information and eligibility, refer to CMS Medicare Benefit Policy Manual Chapter 15 and LCD L33793.

CMS Medicare Benefit Policy Manual Chapter 15. Available to be accessed at CMS National Coverage Determinations (NCDs) Alphabetical Index search page: https://www.cms.gov/medicare-coverage-database/indexes/ncd-alphabetical-index.aspx.

Per LCD Refractive Lenses L33793, the PROSE® device is covered when used as a scleral cover shell for the following indications:
• Treatment of an eye rendered sightless and shrunken by inflammatory disease; or,
• Treatment of "dry eye" where the PROSE® device serves as a substitute for the function of the diseased lacrimal gland.

When the PROSE® device is used for any conditions other than those listed above, the device must be coded with HCPCS code V2531 (CONTACT LENS, SCLERAL, GAS PERMEABLE, PER LENS (FOR CONTACT LENS MODIFICATION)) and is subject to the Medicare refractive lens statutory coverage exclusion.

For additional information, refer to Local Coverage Determination (LCD): Refractive Lenses (L33793). LCD available to be accessed at Noridian Healthcare Services, LLC, (DME MAC), Local Coverage Determinations (LCDs) search page: https://www.cms.gov/medicare-coverage-database/indexes/lcd-list.aspx?Cntrctr=389&ContrVer=1&CntrctrSelected=389*1&s=38&DocType=All&bc=AggAAAAAAAAAAA%3d%3d&#ResultsAnchor.

Medicaid:
For members enrolled in Medicaid and NJ FamilyCare plans, Horizon BCBSNJ applies the above medical policy.

FIDE-SNP:
For members enrolled in a Fully Integrated Dual Eligible Special Needs Plan (FIDE-SNP): (1) to the extent the service is covered under the Medicare portion of the member’s benefit package, the above Medicare Coverage statement applies; and (2) to the extent the service is not covered under the Medicare portion of the member’s benefit package, the above Medicaid Coverage statement applies.

[RATIONALE: Searches of the PubMed database identified case series with gas permeable scleral contact lens. The largest series are described next.

Boston Ocular Surface Prosthesis
A retrospective analysis of 875 eyes (538 patients) fitted with a Boston scleral lens was reported in 2005 by Rosenthal (founder and president of the nonprofit Boston Foundation for Sight) and Croteau.(1) Rigid gas-permeable corneal contact lenses either were not tolerated or were contraindicated in all eyes. Patients who failed a trial period were not fitted and were excluded from this study. Follow-up ranged from 2 months to 18 years. Of 501 eyes that were fitted primarily to improve vision, 262 had corneal ectasia, and 130 eyes were fitted due to inadequate best corrected visual acuity (BCVA) after penetrating keratoplasty. The primary indication was to maintain the integrity of the corneal epithelium in 374 eyes with severe ocular surface disease including corneal stem-cell disorders (Stevens-Johnson syndrome, corneal ectasia, chemical, ocular cicatricial pemphigoid, aniridia), neurotrophic corneas (congenital corneal anesthesia, acquired cranial nerve V paresis, after acoustic neuroma surgery, after trigeminal ganglionectomy, after herpes simplex keratitis, after herpes zoster keratitis), and severe dry eye syndrome (graft vs host disease (GVHD), Sjögren syndrome, corneal ectasia, rheumatoid arthritis, radiation), dermatological-associated disorders, exposure, and corneal neuropathic pain. Scleral lenses were found to improve vision, promote healing of persistent epithelial defect, and in patients with dry eye syndrome, reduce ocular pain and disabling photophobia. Attenuation of symptoms was insufficient to continue wearing the prosthesis in eyes with neuropathic pain and in eyes with corneal edema before fitting.

In 2010, Stason et al reported use of the Boston Ocular Surface Prosthesis in a series of 101 patients with corneal disease who had not responded satisfactorily to conventional treatments and were seen at a tertiary care clinic.(2) The fitting procedure was not completed or was deferred in 21 patients; 80 patients were fitted with a prosthesis in one or both eyes. Of those fitted with a prosthesis, the principal eye diagnosis was corneal ectasia or irregular astigmatism in 42 patients and ocular surface disease (eg, dry eye syndrome, chronic GVHD) in 38 patients. Sixteen patients had undergone a previous corneal transplantation, and 3 had undergone laser in situ keratomileusis (LASIK). About half were experiencing photophobia and one-third reported eye pain at baseline. At 6-month follow-up after fitting, BCVA improved by a change in mean logarithm of the angle of resolution (logMAR) of -0.39 with a change of - 0.54 logMAR units in patients with ectasia or astigmatism and -0.22 logMAR in patients with ocular surface disease. For all 141 fitted eyes, 27% had no significant change in vision, 35% gained 1 line, 23% gained 2 lines, and 14% gained 3 lines or more. Mean composite visual functioning scores on the Visual Functioning Questionnaire (VFQ) increased from 57.0 to 77.8 for patients who received a prosthesis (measured in 69/80 patients) and were not significantly improved in patients (measured in 12/21 patients) who did not (from 65.1 to 69.3). There was significant improvement in all of the vision-related subscales on the VFQ, which included the categories of vision, activities, and ocular pain (from 49.9 at baseline to 72.8 with a prosthesis). Lower baseline VFQ scores were strong predictors of subsequent improvement in visual functioning. The report concluded that controlled clinical studies will be needed to confirm the effectiveness of the Boston Ocular Surface Prosthesis and to compare it with corneal transplantation, tarsorrhaphy, or other techniques in patients with advanced ectasia or ocular surface disease.

Baran et al from the Boston Foundation for Sight reported 6-month outcomes from prosthetic replacement of the ocular surface ecosystem (PROSE) treatment in a series of 59 patients with corneal ectasia.(3) The primary diagnosis was keratoconus in 83% of patients (98 eyes). Fifteen patients (21 eyes) had previously undergone penetrating keratoplasty. Sixteen of the 118 eyes were considered non-candidates because conventional correction was adequate. No devices were dispensed in another 13 eyes due to little improvement in vision during the 6-hour trial period (n=12) or low endothelial cell count (n=1). There was significant improvement in visual acuity; of 102 candidate eyes, 95 (93.1%) achieved visual acuity of 20/40 or better. At mean 9-month follow-up, the sclera contact lens was being worn in 88% of the 89 eyes that had a satisfactory fit. For patients still wearing a device at follow-up, the National Eye Institute Visual Function Questionnaire score improved by 27.6 points on a 100-point scale. Reasons for not wearing the device included discomfort (n=4), lack of motivation to follow the insertion and removal regimen (n=2), and limited improvement in visual acuity (n=1).

Jacobs and Rosenthal published patient-reported outcomes from 33 consecutive patients with severe dry eye from chronic GVHD who were fitted with the Boston scleral lens.(4) All patients had been previously treated with various conventional therapies including punctal occlusion, topical cyclosporine, topical and systemic steroids, and partial tarsorrhaphy. The questionnaire results were obtained between 1 week and greater than 2 years after the lenses were dispensed. All but 1 patient reported reduction in eye pain, with 27 patients (82%) reporting that pain was moderately to greatly reduced. Photophobia was resolved or greatly improved in 20 patients (62%). Ninety-one percent of patients reported moderate to great improvement in quality of life, with 20 of 24 patients (83%) reporting moderate to outstanding improvement in driving and 25 of 28 patients (89%) reporting moderate to outstanding improvement in reading. Two patients (6%) reported that they were not wearing their lenses on a regular basis. One had discontinued because of no improvement while the other discontinued wear because of improvement in symptoms over the prior 4 months.

Jupiter Scleral Lens
In 2000, Jupiter and Katz reported the management of irregular astigmatism in 48 eyes (29 patients) with rigid gas-permeable contact lenses.(5) The corneal diagnosis included keratoconus, postkeratoplasty, pellucid marginal degeneration, interstitial keratitis, traumatic scarring, trachoma, rosacea keratitis, keratoglobus, Terrien degeneration, measles keratitis, postlamellar keratectomy, microbial keratitis, herpes simplex keratitis, postcataract surgery astigmatism, postepikeratophakia, post raidal keratotomy, and Wegener granulomatosis. In this study, nearly one-third of the patients with irregular astigmatism had BCVA of 20/25 or better with spectacles. Patients with 20/40 spectacle visual acuity achieved a 2-line average improvement, patients with 20/50 to 20/200 achieved a 4-line average improvement, and patients with 20/400 achieved a 6-line average improvement with the scleral lens.

Pecego et al reported a series of 63 patients (107 eyes) who were fitted with the Jupiter sclera lens.(6) The most common primary diagnoses included keratoconus (42% of eyes), postkeratoplasty astigmatism (30%), and pellucid marginal degeneration (7%). Patients gained a mean of 3.5 lines of vision compared with previous contact lens or glasses correction. A mean of 3.2 lenses per eye were needed to obtain the ideal sclera lens, with a mean number of return to clinic visits of 6.2 over a period of 3 to 17 months. After at least 3 months of wear, 78% of patients reported the lenses to be comfortable, with wear discontinued in 25 eyes (23%).

Schornack and Patel described use of the Jupiter scleral lens in a retrospective review of patients with keratoconus in 2010.(7) Of 209 patients evaluated for possible scleral lens wear, 52 eyes of 32 patients (15%) had keratoconus and were included in the report. The primary reason for scleral lens evaluation was contact lens intolerance. At the time of presentation, 16 patients were wearing spectacle correction, 8 were wearing corneal rigid gas-permeable lenses, 1 was wearing hydrogel toric lenses, 3 were wearing piggyback systems, and 4 were wearing no correction. Successive diagnostic lens were placed until a lens was applied that had complete limbal and corneal clearance and had a fluid reservoir depth between 0.15 and 0.4 mm. At follow-up visits, revised lenses were ordered as needed to achieve optimal vision, comfort, and fit. The authors noted that at the time of publication, no specific fitting guidelines for scleral lenses have been validated or published. After the initial consultation, 12 patients (20 eyes) chose not to proceed with the fitting process primarily due to a lack of visual benefit compared with habitual correction. Nineteen patients (30 eyes) were fit with Jupiter lens in an average of 2.8 visits (range, 2-4) with an average of 1.5 lenses (range, 1-3). Standard lens designs were prescribed for 23 eyes (77%), and 7 eyes required a custom design to optimize the scleral lens fit. With an average follow-up of 22.5 months (range, 5-34 months), the median BCVA improved in these eyes from 20/40 at baseline to 20/20 with the scleral lens, with an average improvement of 2.9 lines. A 2014 report by the same authors described the successful fitting of the Jupiter scleral lens in 188 eyes of 115 patients.(8) The most common indications were undifferentiated ocular surface disease, exposure keratopathy, and neurotrophic keratopathy. The goals of treatment, achieved in all but 2 patients, were improved ocular comfort, ocular surface protection, and resolution of epitheliopathy. Visual acuity improved from 20/42 to 20/26. Of the patients with at least 12 months of follow-up, 63% reported continued lens wear and 37% had discontinued lens wear.

Procornea Scleral Lens
Visser et al reported a prospective study of the indications and clinical performance of the Procornea lens in 2007.(9) All of the 178 patients (284 eyes) included in the study had been referred to the tertiary clinic for one of a variety of corneal conditions that had not responded to other contact lenses or therapeutic management. Patients with either fit or early wearing failure were excluded from the study. About half of the patients (50.4%) were diagnosed with keratoconus and 19.7% were postpenetrating keratoplasty. Other forms of irregular corneal surface included eyes with scars related to herpes simplex keratitis (n=8), other forms of keratitis (n=2), trauma (n=5), irradiation (n=3), pellucid marginal degeneration (n=7), pterygium (n=2), and macula corneae (n=1). There were 4 types of corneal dystrophy: map-dot-fingerprint (n=5), Fuchs endothelial (n=2), Reis-Bucklers (n=2), and lattice (n=1). Primary keratitis sicca was diagnosed in 4 eyes, neurotrophic keratitis in 7, ocular cicatricial pemphigoid in 2 eyes, and Sjögren syndrome in 2 eyes. The primary indication was for visual correction in 249 (87.7%) eyes. Median visual acuity was 20/100 without a scleral lens and 20/28 with the lens.

Clinical Input Received From Physician Specialty Societies and Academic Medical Centers
While the various physician specialty societies and academic medical centers may collaborate with and make recommendations during this process through the provision of appropriate reviewers, input received does not represent an endorsement or position statement by the physician specialty societies or academic medical centers, unless otherwise noted.

In response to requests, input was received from 1 physician specialty society and 3 academic medical centers while this policy was under review in 2011. The input supported the medical necessity of the rigid gas permeable scleral contact lens for corneal ectatic disorders, corneal scarring and/or vascularization, irregular corneal astigmatism, and ocular surface disease with pain and/or decreased visual acuity in cases that had failures of all other available treatments (ie, topical medications or standard contact lens fittings). One reviewer commented that the prosthesis can help to avoid potentially blinding complications with ocular surface disease and that the alternative for patients with keratoconus and other forms of irregular astigmatism would be cornea transplant surgery, which involves a lifetime of close medical monitoring and significant risk.

Summary of Evidence
Gas permeable scleral contact lenses, which are also known as ocular surface prostheses, are formed with an elevated chamber over the cornea and a haptic base over the sclera. Scleral contact lenses are being evaluated in patients with corneal disease, including keratoconus, Stevens Johnson syndrome, chronic ocular graft-versus-host disease (GVHD), and in patients with reduced visual acuity after penetrating keratoplasty or other types of eye surgery. The literature on gas permeable scleral contact lenses consists of a number of large case series that enrolled more than 100 patients. The largest series was a retrospective review of more than 538 patients with more than 40 different clinical indications who were fitted with the Boston Ocular Surface Prosthesis. These case series report an improvement in health outcomes in patients who have failed all other available treatments. These uncontrolled studies are suggestive of benefit, but the lack of controlled trials precludes a definite conclusion on treatment benefit.

Clinical input was obtained and supports the medical necessity of the gas permeable scleral contact lens in cases of corneal ectatic disorders, corneal scarring and/or vascularization, irregular corneal astigmatism, and ocular surface disease with pain and/or decreased visual acuity when all other available treatments have failed. For patients with ocular surface diseases who have not responded adequately to topical medications, there is a lack of alternative treatments. For patients with corneal ectatic disorders and irregular astigmatism who have failed standard contact lens, the alternative of corneal transplant surgery is associated with risks. Therefore, the gas permeable scleral contact lens is considered medically necessary in these patient populations.

Practice Guidelines and Position Statements
The American Academy of Ophthalmology published a July 2012 guide to diagnosis and treatment of corneal ulcers.(10) Scleral contact lenses were mentioned as a treatment option for improving vision without surgery after a bacterial infection has resolved and the ulcer has scarred over. There is no discussion on the efficacy of scleral contact lenses.

U.S. Preventive Services Task Force Recommendations
Use of scleral contact lenses is not a preventive service.]
________________________________________________________________________________________

Horizon BCBSNJ Medical Policy Development Process:

This Horizon BCBSNJ Medical Policy (the “Medical Policy”) has been developed by Horizon BCBSNJ’s Medical Policy Committee (the “Committee”) consistent with generally accepted standards of medical practice, and reflects Horizon BCBSNJ’s view of the subject health care services, supplies or procedures, and in what circumstances they are deemed to be medically necessary or experimental/ investigational in nature. This Medical Policy also considers whether and to what degree the subject health care services, supplies or procedures are clinically appropriate, in terms of type, frequency, extent, site and duration and if they are considered effective for the illnesses, injuries or diseases discussed. Where relevant, this Medical Policy considers whether the subject health care services, supplies or procedures are being requested primarily for the convenience of the covered person or the health care provider. It may also consider whether the services, supplies or procedures are more costly than an alternative service or sequence of services, supplies or procedures that are at least as likely to produce equivalent therapeutic or diagnostic results as to the diagnosis or treatment of the relevant illness, injury or disease. In reaching its conclusion regarding what it considers to be the generally accepted standards of medical practice, the Committee reviews and considers the following: all credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, physician and health care provider specialty society recommendations, the views of physicians and health care providers practicing in relevant clinical areas (including, but not limited to, the prevailing opinion within the appropriate specialty) and any other relevant factor as determined by applicable State and Federal laws and regulations.

___________________________________________________________________________________________________________________________

Index:
Gas Permeable Scleral Contact Lens
Scleral Contact Lens
Boston Ocular Surface Prosthesis
Prosthetic Replacement of the Ocular Surface Ecosystem (PROSE)
PROSE (Prosthetic Replacement of the Ocular Surface Ecosystem)
Jupiter Scleral Lens
Procornea Scleral Lens
Ocular Surface Prostheses

References:
1. Rosenthal P, Croteau A. Fluid-ventilated, gas-permeable scleral contact lens is an effective option for managing severe ocular surface disease and many corneal disorders that would otherwise require penetrating keratoplasty. Eye Contact Lens. May 2005;31(3):130-134. PMID 15894881

2. Stason WB, Razavi M, Jacobs DS, et al. Clinical benefits of the Boston Ocular Surface Prosthesis. Am J Ophthalmol. Jan 2010;149(1):54-61. PMID 19878920

3. Baran I, Bradley JA, Alipour F, et al. PROSE treatment of corneal ectasia. Cont Lens Anterior Eye. May 24 2012;35(5):222-227. PMID 22633003

4. Jacobs DS, Rosenthal P. Boston scleral lens prosthetic device for treatment of severe dry eye in chronic graft-versus-host disease. Cornea. Dec 2007;26(10):1195-1199. PMID 18043175

5. Jupiter DG, Katz HR. Management of irregular astigmatism with rigid gas permeable contact lenses. CLAO J. Jan 2000;26(1):14-17. PMID 10656303

6. Pecego M, Barnett M, Mannis MJ, et al. Jupiter Scleral Lenses: the UC Davis Eye Center experience. Eye Contact Lens. May 2012;38(3):179-182. PMID 22543730

7. Schornack MM, Patel SV. Scleral lenses in the management of keratoconus. Eye Contact Lens. Jan 2010;36(1):39-44. PMID 20009945

8. Schornack MM, Pyle J, Patel SV. Scleral lenses in the management of ocular surface disease. Ophthalmology. Jul 2014;121(7):1398-1405. PMID 24630687

9. Visser ES, Visser R, van Lier HJ, et al. Modern scleral lenses part I: clinical features. Eye Contact Lens. Jan 2007;33(1):13-20. PMID 17224674

10. American Academy of Ophthalmology (AAO). Confronting corneal ulcers. 2012; http://www.aao.org/publications/eyenet/201207/feature.cfm. Last accessed June, 2014.

11. Katzman LR, Jeng BH. Management strategies for persistent epithelial defects of the cornea. Saudi J Ophthalmol. 2014 Jul;28(3):168-72.

12. Marsack JD, Ravikumar A, Nguyen C, et al. Wavefront-guided scleral lens correction in keratocouns. Optom Vis Sci. 2014 Oct;91(10):1221-30.

13. Lunardi LH, Arroyo D, Andrade Sobrinho MV, et al. Descriptive analysis of the type and design of contact lenses fitted according to keratoconus severity and morphology. Arg Bras Oftalmol 2016 Apr;79(2):82-4.

14. UpToDate. Dry eyes. Literature review current through July 2016. Topic last updated December 22, 2015.

15. UpToDate. Treatment of dry eye in Sjogren's syndrome. Literature review current through July 2016. Topic last updated October 5, 2015..

16. UpToDate. Overview of contact lenses. Literature review current through June 2016. Topic last updated September 11, 2015.

17. Lipson MJ. Overview of Contact Lenses. In: UpToDate, Trobe J, Libman H (Eds), UpToDate, Waltham, MA. (Accessed June 12, 2017.)

92313
92317
92325

S0515
V2531