Latest Review Date: April 2024

Category: Surgery                                                                

POLICY:

Use of the vertical expandable prosthetic titanium rib may be considered medically necessary in the treatment of progressive thoracic insufficiency syndrome due to rib and/or chest wall defects in infants/children between six months of age and skeletal maturity (about age 14 for girls, 16 for boys).

Use of the vertical expandable prosthetic titanium rib for all other conditions, including but not limited to the treatment of scoliosis in individuals without thoracic insufficiency is considered investigational.

Note: Given the complexity of these procedures and patients, implantation of this device should be performed in specialized centers.  Preoperative evaluation requires input from a pediatric orthopedist, pulmonologist, and thoracic surgeon.  In addition, preoperative evaluation of nutritional, cardiac, and pulmonary function is required.

DESCRIPTION OF PROCEDURE OR SERVICE:

The vertical expandable prosthetic titanium rib is a curved rod placed vertically in the chest to help shape the thoracic cavity. It is being evaluated in skeletally immature patients with thoracic insufficiency syndrome to support thorax and lung development and in pediatric patients with scoliosis without thoracic insufficiency syndrome to slow or correct curve progression.

Progressive Thoracic Insufficiency Syndrome

Thoracic insufficiency syndrome (TIS) is the inability of the thorax to support normal respiration or lung growth. It results from serious defects affecting the ribs or chest wall (e.g. severe scoliosis with rib absence or rib fusion) and various hypoplastic thorax syndromes (e.g. Jeune’s Syndrome and Jarcho-Levin syndrome). Spine, lung, and chest growth are interdependent. While the coexistence of chest wall and spinal deformity is well documented, the effect on lung growth is not completely understood.

Progressive thoracic insufficiency syndrome includes respiratory insufficiency, loss of chest wall mobility, worsening 3-dimensional thoracic deformity, and/or worsening pulmonary function tests. As a child grows, progressive thoracic deformity and rotation toward concave side occurs with worsening respiratory compromise. This progression is often accompanied by a need for supplemental oxygen and can require mechanical ventilation.

Vertical Expandable Prosthetic Titanium Rib

While spinal fusion is an approach to treatment in individuals with thoracic insufficiency syndrome, or early-onset scoliosis without thoracic insufficiency syndrome, the procedure may not be successful and may limit growth (lengthening) of the spine.

The vertical expandable prosthetic titanium rib device is a curved rod placed vertically in the chest that helps to stabilize and shape the thoracic cavity. It is positioned either between ribs, or between the ribs and either the spine or pelvis. The vertical expandable prosthetic titanium rib may be described as “rib-based” growth-sparing instrumentation, which is compared with “spine-based” growing rods for Cobb angle correction. The vertical expandable prosthetic titanium rib device is designed to be expanded every 4 to 6 months as growth occurs and to be replaced if necessary. Some patients require multiple devices.

KEY POINTS:

The most recent review was performed through February 8, 2024.

Summary of Evidence

For individuals who have progressive thoracic insufficiency syndrome due to rib and/or chest wall defects in childhood who receive VEPTR thoracoplasty the evidence includes case series. Relevant outcomes include symptoms, morbid events, functional outcomes, treatment-related mortality, and treatment-related morbidity. Results from the series reported at different specialty centers demonstrate improvement and/or stabilization in key measures with use of this device in progressive thoracic insufficiency syndrome. This improvement is noted in measures related to thoracic structure (e.g., Cobb angle for those with scoliosis), growth of the thoracic spine and lung volumes, and stable or improved ventilatory status. While pulmonary function testing is difficult to track in patients, a study has demonstrated an age-specific increase in forced vital capacity (FVC); further still, that same study reported a final FVC in the range of 50% to 70% of predicted value. Given the usual disease course of worsening thoracic volume and ventilatory status, the stabilization/improvement in these measures would be highly unlikely in the absence of the intervention. Taken together, these outcomes demonstrate the positive impact of using the VEPT technology. The evidence is sufficient to determine that the technology results in an improvement in the net health outcome.

For individuals with early-onset scoliosis without thoracic insufficiency syndrome who receive vertical expandable prosthetic titanium rib thoracoplasty, the evidence includes a non-randomized controlled study, an uncontrolled cohort study, and a case series. Relevant outcomes include symptoms, morbid events, functional outcomes, treatment-related mortality, and treatment-related morbidity. The VEPTR is also being evaluated for curves greater than 45° in infants and juveniles without thoracic insufficiency. The vertical expandable prosthetic titanium rib is being evaluated for curves greater than 45° in infants and juveniles without thoracic insufficiency. Similar to thoracic insufficiency syndrome, limited data are available on the use of the vertical expandable prosthetic titanium rib for early-onset scoliosis without thoracic insufficiency. Additionally, little is known about the disease progression of early onset scoliosis, and therefore little is known regarding the risk-benefit trade-off of the vertical expandable prosthetic titanium rib surgery. The evidence is insufficient to determine that the technology results in an improvement in the net health outcome.

Practice Guidelines and Position Statements

No guidelines or statements were identified.

U.S. Preventive Services Task Force Recommendations

Not Applicable.

KEY WORDS:

Vertical expandable prosthetic titanium rib (VEPTR), thoracic insufficiency syndrome, Jeune's syndrome, achondroplasia, Jarcho-Levin syndrome, Ellis van Creveld syndrome, hypoplastic thorax syndrome

APPROVED BY GOVERNING BODIES:

The VEPTR™ (DePuy Synthes Spine, Raynham, MA) was initially cleared (in 2004) for marketing by the U.S. Food and Drug Administration (FDA) through a humanitarian device exemption for the treatment of thoracic insufficiency syndrome in skeletally immature patients. In 2014, the VEPTR/VEPTR II™ was cleared for marketing by the FDA through the 510(k) process. The VEPTR/VEPTR II device is indicated for skeletally immature patients with severe, progressive spinal deformities and/or 3-dimensional deformity of the thorax associated with or at risk of thoracic insufficiency syndrome. This would include patients with progressive congenital, neuromuscular, idiopathic, or syndromic scoliosis.

To identify potential patients with thoracic insufficiency syndrome , the following categories are used:

• Flail chest syndrome;
• Rib fusion and scoliosis; and
• Hypoplastic thorax syndrome, including:
  • Jeune's syndrome,
  • Achondroplasia,
  • Jarcho-Levin syndrome, and
  • Ellis van Creveld syndrome

BENEFIT APPLICATION:

Coverage is subject to member’s specific benefits. Group-specific policy will supersede this policy when applicable.

ITS: Home Policy provisions apply

FEP contracts: Special benefit consideration may apply. Refer to member’s benefit plan. 

CURRENT CODING:

CPT Codes:

REFERENCES:

1. Campbell RM, Jr. VEPTR: past experience and the future of VEPTR principles. Eur Spine J. Mar 2013; 22 Suppl 2: S106-17.
2. Campbell RM, Jr., Smith MD, Mayes TC et al. The characteristics of thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis. J Bone Joint Surg Am.Mar 2003; 85-A(3):399-408.
3. Campbell RM Jr., Smith MD, Mayes TC, et al. The effect of opening wedge thoracostomy on thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis. J Bone Joint Surg Am. Aug 2004; 86(8): 1659-74.
4. El-Hawary R, Kadhim M, Vitale M, et al. VEPTR Implantation to Treat Children With Early-Onset Scoliosis Without Rib Abnormalities: Early Results From a Prospective Multicenter Study. J Pediatr Orthop. Dec 2017; 37(8): e599-e605.
5. El-Hawary R, Morash K, Kadhim M, et al. VEPTR Treatment of Early Onset Scoliosis in Children Without Rib Abnormalities: Long-term Results of a Prospective, Multicenter Study. J Pediatr Orthop. Jul 2020; 40(6): e406-e412.
6. Emans JF, Caubet JF, Ordonez CL, et al. The treatment of spine and chest wall deformities with fused ribs by expansion thoracostomy and insertion of vertical expandable prosthetic titanium rib: Growth of thoracic spine and improvement of lung volumes. Spine (Phila Pa 1976). Sep 01 2005; 30(17 Suppl): S58-S68.
7. Farley FA, Li Y, Jong N, et al. Congenital scoliosis SRS-22 outcomes in children treated with observation, surgery, and VEPTR. Spine (Phila PA 1976) Oct 15 2014; 39(22):1868-74.
8. Flynn JM, Emans JB, Smith JT et al. VEPTR to treat nonsyndromic congenital scoliosis: a multicenter, mid-term follow-up study. J Pediatr Orthop. 2013; 33(7):679-84.
9. Food and Drug Administration. Vertical Expandable Prosthetic Titanium Rib (VEPTR). 2004; www.accessdata.fda.gov/cdrh_docs/pdf14/k142587.pdf.
10. Gadepalli SK, Hirschl RB, Tsai WC et al. Vertical expandable prosthetic titanium rib device insertion: does it improve pulmonary function? J Pediatr Surg. Jan 2011; 46(1):77-80.
11. IOM (Institute of Medicine). 2011. Clinical practice guidelines we can trust. Washington, DC: The National Academies Press.
12. Mayer OH, Redding G. Early changes in pulmonary function after vertical expandable prosthetic titanium rib insertion in children with thoracic insufficiency syndrome. J Pediatr Orthop. 2009; 29(1):35-8.
13. Motoyama EK, Deeney VF, Fine FG, et al. Effects on lung function of multiple expansion thoracoplasty in children with thoracic insufficiency syndrome: A longitudinal study. Spine (Phila Pa 1976). Feb 01 2006; 31(3): 284-90.
14. Skaggs DL, Sankar WN, Albrektson J, et al. Weight gain following vertical expandable prosthetic titanium ribs surgery in children with thoracic insufficiency syndrome. Spine. (Phila Pa 1976). Nov 1 2009; 34(23):2530-3.
15. Waldhausen JH, Redding G, White K, et al. Complications in using the vertical expandable prosthetic titanium rib (VEPTR) in children. J Pediatr Surg. Nov 2016; 51(11):1747-1750.
16. Waldhausen JH, Redding G, White K, et al. Complications in using the vertical expandable prosthetic titanium rib (VEPTR) in children. J Pediatr Surg. Nov 2016; 51(11): 1747-1750. 
17. White KK, Song KM, Frost N et al. VEPTR™ growing rods for early-onset neuromuscular scoliosis: feasible and effective. Clin Orthop Relat Res May 2011; 469(5):1335-41.

POLICY HISTORY:

Medical Policy Group, January 2007 (2)

Medical Policy Administration Committee, February 2007

Available for comment February 10-March 26, 2007

Medical Policy Group, January 2009 (1)

Medical Policy Group, June 2011; Updated Key Points and References

Medical Policy Group, October 2011 (1) Update to Key Points and References; no change in policy statement.

Medical Policy Panel, May 2012

Medical Policy Group, August 2012 (2):  Policy updated with literature search through March 2012; policy statement on use of vertical expandable prosthetic titanium rib for patients without thoracic insufficiency is investigational.  Key Points and References updated to support policy statement. 

Medical Policy Administration Committee, September 2012

Available for comments September 18 through November 1, 2012

Medical Policy Group, June 2013 (2): 2013 Updates to Description, Key Points, and References

Medical Policy Panel, May 2014

Medical Policy Group, May 2014 (4): Updated Key Points and References. No changes to the policy at this time.

Medical Policy Panel, May 2015

Medical Policy Group, June 2015 (2): 2015 Updates to Key Points, Approved by Governing Bodies, and References; no change to policy statement.

Medical Policy Group, June 2015 (2): updated policy statement to include “progressive thoracic insufficiency syndrome due to rib and/or chest wall defects;” no change to intent of policy statement.

Medical Policy Group, January 2016 (2): added code 22899 to coding section.

Medical Policy Panel, November 2016

Medical Policy Group, November 2016 (7): 2016 Updated Key Points and References. No change in Policy Statement.

Medical Policy Panel, August 2017

Medical Policy Group, September 2017 (7): 2017 Update - No new literature to review or add. No change in Policy Statement.

Medical Policy Panel, April 2018

Medical Policy Group, April 2018 (7): 2018 Update to Key Points. No new literature to review or add. No change in Policy Statement.

Medical Policy Panel, April 2019

Medical Policy Group, April 2019 (7): 2019 Minor update to Key Points. No new literature to review or add. No change in Policy Statement.

Medical Policy Panel, April 2020

Medical Policy Group, April 2020 (7): Updates to Key Points and References. No change to policy statements.

Medical Policy Panel, April 2021

Medical Policy Group, April 2021 (7): Updates to Key Points and References. Policy statement updated to remove “not medically necessary,” no change to policy intent.

Medical Policy Panel, April 2022

Medical Policy Group, April 2022 (7): Updates to Key Points and References. No change to policy intent.

Medical Policy Panel, April 2023

Medical Policy Group, April 2023 (7): Updates to Description, Key Points, Approved by Governing Bodies, Benefit Application and References.  Replaced the word “patients” with the word “individuals” in Policy Statement. No change to policy intent.

Medical Policy Panel, April 2024

Medical Policy Group, April 2024 (7): Updates to Key Points, Approved by Governing Bodies, and References. Added Key Words: “thoracic insufficiency syndrome, Jeune's syndrome, Achondroplasia, Jarcho-Levin syndrome, Ellis van Creveld syndrome, Hypoplastic thorax syndrome.” No change to Policy Statement.

This medical policy is not an authorization, certification, explanation of benefits, or a contract. Eligibility and benefits are determined on a case-by-case basis according to the terms of the member’s plan in effect as of the date services are rendered. All medical policies are based on (i) research of current medical literature and (ii) review of common medical practices in the treatment and diagnosis of disease as of the date hereof. Physicians and other providers are solely responsible for all aspects of medical care and treatment, including the type, quality, and levels of care and treatment.

This policy is intended to be used for adjudication of claims (including pre-admission certification, pre-determinations, and pre-procedure review) in Blue Cross and Blue Shield’s administration of plan contracts.

The plan does not approve or deny procedures, services, testing, or equipment for our members. Our decisions concern coverage only. The decision of whether or not to have a certain test, treatment or procedure is one made between the physician and his/her patient. The plan administers benefits based on the member’s contract and corporate medical policies. Physicians should always exercise their best medical judgment in providing the care they feel is most appropriate for their patients. Needed care should not be delayed or refused because of a coverage determination.

As a general rule, benefits are payable under health plans only in cases of medical necessity and only if services or supplies are not investigational, provided the customer group contracts have such coverage.

The following Association Technology Evaluation Criteria must be met for a service/supply to be considered for coverage:

1. The technology must have final approval from the appropriate government regulatory bodies;

2. The scientific evidence must permit conclusions concerning the effect of the technology on health outcomes;

3. The technology must improve the net health outcome;

4. The technology must be as beneficial as any established alternatives;

5. The improvement must be attainable outside the investigational setting.

Medical Necessity means that health care services (e.g., procedures, treatments, supplies, devices, equipment, facilities or drugs) that a physician, exercising prudent clinical judgment, would provide to a patient for the purpose of preventing, evaluating, diagnosing or treating an illness, injury or disease or its symptoms, and that are:

1. In accordance with generally accepted standards of medical practice; and

2. Clinically appropriate in terms of type, frequency, extent, site and duration and considered effective for the patient’s illness, injury or disease; and

3. Not primarily for the convenience of the patient, physician or other health care provider; and

4. Not more costly than an alternative service or sequence of services at least as likely to produce equivalent therapeutic or diagnostic results as to the diagnosis or treatment of that patient’s illness, injury or disease.