Interventions for Progressive Scoliosis

POLICY NUMBER

A.2.01.83

DESCRIPTION

Orthotic bracing attempts to slow spinal curve progression and reduce the need for fusion surgery in individuals with juvenile or adolescent idiopathic scoliosis who are at high-risk of progression. Vertebral body stapling and vertebral body tethering, both fusionless surgical procedures, have been evaluated to determine whether the procedures could be used as alternatives to traditional orthotic bracing. This policy does not address individuals who are not at high-risk of progression or conventional fusion surgery for scoliosis, such as individuals with Cobb angles measuring 45° or more.

Scoliosis

Scoliosis is an abnormal lateral and rotational curvature of the vertebral column. Adolescent idiopathic scoliosis is the most common form of idiopathic scoliosis, defined by the U.S. Preventive Services Task Force as “a lateral curvature of the spine with onset at ≥10 years of age, no underlying etiology, and risk for progression during puberty.” Progression of the curvature during periods of rapid growth can result in deformity, accompanied by cardiopulmonary complications. Diagnosis is made clinically and radiographically. The curve is measured by the Cobb angle, which is the angle formed between intersecting lines drawn perpendicular to the top of the vertebrae of the curve and the bottom vertebrae of the curve. Patients with adolescent idiopathic scoliosis are also assessed for skeletal maturity, using the Risser sign, which describes the level of ossification of the iliac apophysis.

The Risser sign measures remaining spinal growth by progressive anterolateral to posteromedial ossification. Risser sign ranges from 0 (no ossification) to 5 (full bony fusion of the apophysis). Immature patients will have 0% to 25% ossification (Risser grade 0 or 1), while 100% ossification (Risser grade 5) indicates maturity with no spinal growth remaining. Children may progress from a Risser grade 1 to grade 5 over a brief (eg, 2-year) period.

Males and females are equally affected by scoliosis, but curve progression is up to 10 times more common in females than males. Patients who are overweight or obese have a greater risk of presenting with larger Cobb angles and more advanced skeletal maturity, possibly due to delayed detection. A retrospective review of 341 patients with adolescent idiopathic scoliosis who underwent surgery at a single tertiary pediatric hospital between 2013 and 2018 found that the major curve magnitude at presentation was significantly higher in patients with public compared to private insurance (50.0° versus 45.1°; p=.0040 and in Black compared to White patients (51.8° versus 47.0°; p=.042). Additionally, the odds of having an initial major curve magnitude <40° within the range of nonoperative treatment were 67% lower among Black patients with public insurance compared to Black patients with private insurance (odds ratio [OR], 0.33; 95% CI, 0.13 to 0.83; p=.019).

Treatment

Treatment of scoliosis currently depends on 3 factors: the cause of the condition (idiopathic, congenital, or secondary), the severity of the condition (degrees of the curve), and the growth of the patient remaining at the time of presentation. Children who have vertebral curves measuring between 25° and 40° with at least 2 years of growth remaining are considered to be at high risk of curve progression. Genetic markers to evaluate the risk of progression are also being evaluated. Since severe deformity may lead to compromised respiratory function and is associated with back pain in adulthood, surgical intervention with spinal fusion is typically recommended for curves that progress to 45° or more.

Bracing

Bracing is used to reduce the need for spinal fusion by slowing or preventing further progression of the curve during rapid growth. Commonly used brace designs include the Milwaukee, Wilmington, Boston, Charleston, and Providence orthoses. The longest clinical experience is with the Milwaukee cervical-thoracic-lumbar-sacral orthosis. Thoracic-lumbar-sacral orthoses, such as the Wilmington and Boston braces, are intended to improve tolerability and compliance for extended (over 18-hour) wear and are composed of lighter weight plastics with a low profile (underarm) design. The design of the nighttime Charleston and Providence braces is based on the theory that increased corrective forces will reduce the needed wear time (i.e., daytime), thereby lessening social anxiety and improving compliance. The smart brace consists of a standard rigid brace with a microcomputer system, a force transducer, and an air-bladder control system to control the interface pressure. Braces that are more flexible than thoracic-lumbar-sacral orthoses or nighttime braces, such as the SpineCor® Scoliosis System, are also being evaluated. The SpineCor is composed of a thermoplastic pelvic base with stabilizing and corrective bands across the upper body.

Surgery

Fusionless surgical procedures, such as vertebral body stapling and vertebral body tethering, are being evaluated as alternatives to bracing. The goal of these procedures is to reduce the rate of spine growth unilaterally, thus allowing the other side of the spine to “catch up.” The mechanism of action is believed to be down-regulation of the growth plate on the convex (outer) side by compression and stimulation of growth on the endplate of the concave side by distraction. In the current stapling procedure, nickel-titanium alloy staples with shape memory are applied to the convex side of the curve. The shape memory allows the prongs to be straight when cooled and clamp down into the bone when the staple returns to body temperature. Anterolateral tethering uses polyethylene ligaments that are attached to the convex side of the vertebral bodies by pedicle screws or staples. The ligament can be tightened to provide greater tension than the staple. The optimum degree of tension is not known. The polyethylene ligaments are more flexible than staples and are predicted to allow more spinal mobility. The goal of a fusionless growth modulating procedure is to reduce the curve and prevent progression, maintain spine mobility following correction, and provide an effective treatment option for patients who are noncompliant or who have a large curve but substantial growth is remaining. Observational data suggest that overweight patients may be at higher risk for scoliosis progression after surgery.

Research Recommendations

The Scoliosis Research Society provided evidence-based recommendations in 2005, which were updated in 2015, for bracing studies to standardize inclusion criteria, methodologies, and outcome measures to facilitate comparison of brace trials. Janicki and colleagues reported the first study to use the Scoliosis Research Society criteria. It concluded that a brace should prevent progression in 70% of patients to be considered effective. The Scoliosis Research Society evidence review and recommendations may also aid in the evaluation of fusionless surgical treatments for scoliosis progression in children.

The Scoliosis Research Society review of the natural history of scoliosis indicated that skeletally immature patients and patients with larger curves (between 20° and 29°) are significantly more likely to have more than 5° curve progression. Brace treatment for idiopathic scoliosis is usually recommended for juveniles and adolescents with curves measuring between 25° and 40° who have not completed spinal growth, with maturity defined as Risser grade 4, or at least 2 years after menarche for girls. Bracing may also be recommended for curves greater than 20° in a patient who has a rapidly progressing curve with more than 2 years of growth remaining.

Success from brace treatment is most frequently defined as progression of less than 5° before skeletal maturity, although alternative definitions may include progression of less than 10° before skeletal maturity or preventing the curve from reaching the threshold for surgical intervention. Surgery is usually recommended when the curve magnitude exceeds 45° to 50° (before or at skeletal maturity), although many patients will not undergo surgery at this point. Based on this information, Scoliosis Research Society provided the following recommendations for brace studies on adolescent idiopathic scoliosis:

• “Optimal inclusion criteria for brace studies consist of: age is 10 years or older when the brace is prescribed, Risser [grade] 0 to 2, curve 25° to 40°, and no prior treatment.”

• Outcomes of brace effectiveness should include all of the following:

  • “The percentage of patients with 5° or less curve progression and the percentage of patients who have 6° or more progression at skeletal maturity.”

    • The number of patients at the start and end of treatment exceeding 10°, 30°, and 50° Cobb angles, as these risk thresholds have potential health consequences in adulthood, such as back pain and curve progression.

    • “A minimum of 2-year follow-up beyond skeletal maturity for each patient who was ‘successfully’ treated with a brace to determine the percentage who subsequently required or had surgery recommended. The surgical indications must be documented.”

  • Clinically significant outcomes such as aesthetics, deformity progression, disability, pain, and quality of life.

• “Skeletal maturity should be considered achieved when <1 cm change in standing height has occurred on measurements made on 2 consecutive visits 6 months apart…. when Risser 4 is present and, in females, when the patient is 2 years after menarche.”

• “All patients, regardless of subjective reports of compliance, should be included in the results. This process makes ‘intent to treat’ analysis possible.... An ‘efficacy analysis’ … should also be considered.”

Some braces used to treat scoliosis are considered class I devices by the U.S. Food and Drug Administration (FDA) and are exempt from 510(k) requirements. Examples include the Boston scoliosis brace [Boston Orthotics & Prosthetics] and the SpineCor Scoliosis System. This classification does not require submission of clinical data regarding efficacy but only notification prior to marketing.

Staples, using a shape memory nickel-titanium alloy, have been cleared for marketing by the FDA through the 510(k) process for various bone fixation indications. For example, nitinol staples (Sofamor Danek) are indicated for fixation with spinal systems. Other memory shape staples cleared for marketing by the FDA through the 510(k) process for bone fixation include the OSStaple™ (BioMedical Enterprises) and the reVERTO™ Dynamic Compression Device. Vertebral body stapling in scoliosis is considered off-label use. FDA product code: JDR

A vertebral body tethering device (The Tether™; Zimmer Biomet Spine) received an FDA Humanitarian Device Exemption (HDE) (H190005, product code QHP) on 6/4/2019. The FDA HDE states that this device is indicated for "skeletally immature patients that require surgical treatment to obtain and maintain correction of progressive idiopathic scoliosis, with a major Cobb angle of 30 to 65 degrees whose osseous structure is dimensionally adequate to accommodate screw fixation, as determined by radiographic imaging. Patients should have failed bracing and/or be intolerant to brace wear." The REFLECT™ Scoliosis Correction System (Globus Medical), another vertebral tethering system, was granted HDE by the FDA on 5/15/2023 and intended for use in the same population as The Tether.

Several of the FDA cleared devices are described in the table below.

Scoliosis Bracing Devices Cleared by the U.S. Food and Drug Administration

Related policies -

• Vertebral Stapling and Endoscopic Surgery in the Treatment of Scoliosis

• Vertical Expandable Prosthetic Titanium Rib

POLICY

A rigid cervical-thoracic-lumbar-sacral or thoracic-lumbar-sacral orthosis may be considered medically necessary for the treatment of scoliosis in juvenile and adolescent individuals at high risk of progression that meets the following criteria:

• Idiopathic spinal curve angle between 25° and 40°; AND

• Spinal growth has not been completed (Risser grade 0-3; no more than 1 year post-menarche in females)

OR

• Idiopathic spinal curve angle greater than 20°; AND

• There is a documented increase in the curve angle; AND

• At least 2 years of growth remain (Risser grade 0 or 1; pre-menarche in females)

Use of an orthosis for the treatment of scoliosis that does not meet the criteria above is considered investigational.

Vertebral body staplingand vertebral body tethering for the treatment of scoliosis are considered investigational.

POLICY EXCEPTIONS

Federal Employee Program (FEP) may dictate that all FDA-approved devices, drugs or biologics may not be considered investigational and thus these devices may be assessed only on the basis of their medical necessity.

POLICY GUIDELINES

The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.

This policy does not address conventional surgery for scoliosis in individuals with curve angles measuring 45° or more. Brace treatment for idiopathic scoliosis is usually recommended for juveniles and adolescents with curves measuring between 25° and 40° who have not completed spinal growth, with maturity defined as Risser grade 4, or 2 years post-menarche for girls. Bracing may also be recommended for curves over 20° in an individual who has a rapidly progressing curve with more than 2 years of growth remaining.

• A rigid cervical-thoracic-lumbar-sacral orthosis is primarily prescribed for individuals with thoracic apices above T7 for control of upper thoracic sagittal deformities and other spinal deformities not amenable to treatment with lower-profile designs.

• A low profile, rigid thoracic-lumbar-sacral orthosis worn full-time (18-23 hours per day) through skeletal maturity is used for most idiopathic curve patterns with a thoracic curve apex at or below T7 (most idiopathic curves).

• Nighttime bracing systems are more effective in individuals with isolated flexible thoracolumbar and lumbar curves than in double curves; they may also be indicated in individuals who are noncompliant with a full-time wear program, individuals in whom other types of orthotic management have failed, and individuals nearing skeletal maturity who may not require full-time wear.

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.

POLICY HISTORY

07/22/2010: Approved by Medical Policy Advisory Committee.

07/29/2011: Policy reviewed; no changes.

07/12/2012: Removed the following policy statement as it is addressed in the Vertical Expandable Prosthetic Titanium Rib policy: Use of the vertical expandable titanium prosthetic rib (with or without expansion thoracoplasty) for the treatment of scoliosis in patients without thoracic insufficiency is considered investigational. Other policy statements unchanged.

08/09/2013: Policy reviewed; no changes.

07/09/2014: Policy reviewed; description updated. Policy statement unchanged.

08/28/2015: Medical policy revised to add ICD-10 codes. Removed ICD-9 diagnosis code 737.32 from the Covered Codes table in the Code Reference section.

09/17/2015: Policy description updated to include vertebral body tethering as a fusionless surgical procedure. Policy statement revised to add vertebral body tethering as investigational for the treatment of scoliosis. Policy Guidelines section updated to add medically necessary and investigative definitions. Investigational Codes table updated to revise the code description for CPT code 22899 to include vertebral body tethering.

06/01/2016: Policy number A.2.01.83 added.

12/02/2016: Policy description updated regarding fusionless surgical procedures. Policy statements unchanged.

08/30/2017: Policy description updated. Policy statements unchanged.

04/27/2018: Policy description updated regarding adolescent idiopathic scoliosis and research recommendations. Policy statement updated to state that a "rigid" cervical-thoracic-lumbar-sacral or thoracic-lumbar-sacral orthosis may be considered medically necessary for the treatment of scoliosis in juvenile and adolescent patients at high risk of progression that meets certain criteria.

05/07/2019: Policy description updated regarding devices. Policy statements unchanged.

05/15/2020: Policy description updated regarding devices. Policy statements unchanged.

06/11/2021: Policy reviewed. Policy statements unchanged. Policy Guidelines updated to change "Nervous/Mental Conditions" to "Mental Health Disorders" and "Medically Necessary" to "medical necessity."

06/26/2021: Code Reference section updated to add new CPT codes 0656T and 0657T, effective 07/01/2021.

05/24/2022: Policy description updated regarding scoliosis and devices. Policy statements unchanged.

05/08/2023: Policy description updated regarding scoliosis. Policy statement updated to change "patients" to "individuals."

12/21/2023: Code Reference section updated to add new 2024 CPT codes 22836, 22837, 22838, and 0790T. Revised the code description for CPT codes 0656T and 0657T, effective 01/01/2024.

05/07/2024: Policy description updated regarding devices. Policy statements unchanged. Policy Guidelines updated to change "patients" to "individuals."

05/06/2025: Policy description and Policy Guidelines updated with minor changes. Policy statements unchanged.

SOURCE(S)

Blue Cross Blue Shield Association policy # 2.01.83

CODE REFERENCE

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.

Covered Codes

Investigational Codes

CPT copyright American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association.