Description:
The vertical expandable prosthetic titanium rib (VEPTR) 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 (TIS) to support thorax and lung development and in pediatric patients with scoliosis without TIS to slow or correct curve progression.

For individuals who have progressive TIS who receive VEPTR thoracoplasty, the evidence includes small case series. Relevant outcomes are symptoms, morbid events, functional outcomes and treatment-related mortality and morbidity. TIS occurs in a limited patient population. For example, the Boston center reported results on 31 children treated from 1999 to 2005. The natural history of progressive TIS is worsening pulmonary function and pulmonary insufficiency. Results from case series reported at different specialty centers have demonstrated improvement and/or stabilization in key measures with use of this device in progressive TIS. This improvement has been 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 very difficult in these patients, 1 study demonstrated an age-specific increase in forced vital capacity (FVC), and these series have 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 and/or improvement in these measures would be highly unlikely absent the intervention. Taken together, these outcome measures demonstrate the positive impact of this procedure. The evidence is sufficient to determine that the technology results in a meaningful improvement in the net health outcome. 

For individuals with early-onset scoliosis without TIS who receive VEPTR thoracoplasty, the evidence includes small case series. Relevant outcomes are symptoms, morbid events, functional outcomes and treatment-related mortality and morbidity. VEPTR is being evaluated for curves greater than 45° in infants and juveniles without thoracic insufficiency. Similar to TIS, very limited data are available on the use of the VEPTR for early-onset scoliosis without thoracic insufficiency. However, there is less certainty about disease progression, and, thus, in the risk-benefit tradeoff of VEPTR surgery. The evidence is insufficient to determine the effects of the technology on health outcomes.

Background 
Treatment of Thoracic Insufficiency Syndrome
While spinal fusion is an approach to treatment, it 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.  

Regulatory Status
A VEPTR initially received approval from FDA under a humanitarian device exemption for the treatment of TIS in skeletally immature patients.³ TIS is defined as the inability of the thorax to support normal respiration or lung growth. In 2014, FDA cleared the VEPTR 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 TIS. This would include patients with progressive congenital, neuromuscular, idiopathic or syndromic scoliosis. 

For the purpose of identifying potential TIS patients, categories in which TIS patients fall are as follows: 

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

FDA product code: MDI.

Related Policies
20183 Interventions for Progressive Scoliosis

Policy:
Use of the vertical expandable prosthetic titanium rib is considered MEDICALLY NECESSARY in the treatment of progressive thoracic insufficiency syndrome due to rib and/or chest wall defects in infants/children between 6 months of age and skeletal maturity.

Use of the vertical expandable prosthetic titanium rib for all other conditions, including, but not limited to, the treatment of scoliosis in patients without thoracic insufficiency, is investigational and/or unproven and therefore considered NOT MEDICALLY NECESSARY.

Policy Guidelines:
Due to complexity of thoracoplasty and the young age of the patient population undergoing such a procedure, implantation of the vertical expandable prosthetic titanium rib should be performed in specialized centers. Preoperative evaluation should require input from a pediatric orthopedist, a pulmonologist, and a thoracic surgeon. In addition, preoperative evaluation should require (when possible) a test for positive nutritional, cardiac, and pulmonary function.

Coding 
See the Codes table for details.

Benefit Application:
BlueCard^®/National Account Issues
State or federal mandates (e.g., FEP) may dictate that all FDA-approved devices may not be considered investigational and, thus, these devices may be assessed only on the basis of their medical necessity.

Rationale
Evidence reviews assess the clinical evidence to determine whether the use of a technology improves the net health outcome. Broadly defined, health outcomes are length of life, quality of life, and ability to function, including benefits and harms. Every clinical condition has specific outcomes that are important to patients and to managing the course of that condition. Validated outcome measures are necessary to ascertain whether a condition improves or worsens; and whether the magnitude of that change is clinically significant. The net health outcome is a balance of benefits and harms.

To assess whether the evidence is sufficient to draw conclusions about the net health outcome of a technology, 2 domains are examined: the relevance and the quality and credibility. To be relevant, studies must represent 1 or more intended clinical use of the technology in the intended population and compare an effective and appropriate alternative at a comparable intensity. For some conditions, the alternative will be supportive care or surveillance. The quality and credibility of the evidence depend on study design and conduct, minimizing bias and confounding that can generate incorrect findings. The randomized controlled trial (RCT) is preferred to assess efficacy; however, in some circumstances, nonrandomized studies may be adequate. Randomized controlled trials are rarely large enough or long enough to capture less common adverse events and long-term effects. Other types of studies can be used for these purposes and to assess generalizability to broader clinical populations and settings of clinical practice.

Thoracic Insufficiency Syndrome
Clinical Context and Therapy Purpose
Thoracic insufficiency syndrome is the inability of the thorax to support normal respiration or lung growth.² The condition 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 syndrome, Jarcho-Levin syndrome). Spine, chest, and lung growth are interdependent.³ While the coexistence of chest wall and spinal deformity is well-documented, this 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 the concave side occurs with worsening respiratory compromise. This progression is often accompanied by a need for supplemental oxygen and can require mechanical ventilation.

The purpose of the vertical expandable prosthetic titanium rib in patients who have progressive thoracic insufficiency syndrome is to provide a treatment option that is an alternative to or an improvement on existing therapies.

The question addressed in this evidence review is: Does the vertical expandable prosthetic titanium rib improve net health outcomes in patients who have progressive thoracic insufficiency syndrome?

The following PICO was used to select literature to inform this review.

Populations
The relevant population of interest is children who have progressive thoracic insufficiency syndrome.

Interventions
The therapy being considered is the vertical expandable prosthetic titanium rib. 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 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.

Comparators
For progressive thoracic insufficiency syndrome, relevant comparators include respiratory supportive care.

Outcomes
The general outcomes of interest are symptoms, morbid events, functional outcomes, treatment-related mortality, and treatment-related morbidity. Based upon existing literature, follow-up of 2 to 5 years is recommended.

Study Selection Criteria
Methodologically credible studies were selected using the following principles:

1. To assess efficacy outcomes, comparative controlled prospective trials were sought, with a preference for RCTs.
2. In the absence of such trials, comparative observational studies were sought, with a preference for prospective studies.
3. To assess long-term outcomes and adverse events, single-arm studies that capture longer periods of follow-up and/or larger populations were sought.
4. Studies with duplicative or overlapping populations were excluded.

Review of Evidence
Uncontrolled Studies
Thoracic insufficiency occurs in a limited patient population, and the literature on the use of the vertical expandable prosthetic titanium rib consists mostly of case series from single institutions (some series are from specialized pediatric centers); no comparative trials have been identified.

Data submitted to the U.S. Food and Drug Administration (FDA) on thoracic insufficiency syndrome include an initial feasibility study involving 33 patients and a subsequent prospective study of 224 patients (214 with baseline data) at 7 study sites.¹ Of these, 94 had rib fusion, 93 had hypoplastic thoracic syndrome, 46 had progressive scoliosis, and 14 had flail chest as a cause of their thoracic insufficiency syndrome. Three- and 5-year follow-up rates for the multicenter study were approximately 95%. Of the 247 patients enrolled in either study, 12 (4.8%) patients died, and 2 withdrew. None of the deaths, as determined by investigators, were related to the vertical expandable prosthetic titanium rib. Because standard pulmonary function testing was not possible for most of this population, an assisted ventilatory rating was used to assess impact on respiratory status. The assisted ventilatory rating ranged from 0 (unassisted breathing on room air) to 4 (full-time ventilatory support). In the multicenter prospective study, the assisted ventilatory rating outcome improved or stabilized for 93% of the patients. Data were not reported for the number of patients who were no longer dependent on a ventilator.

Campbell (2004), who developed the vertical expandable prosthetic titanium rib, and colleagues reported on 27 patients who had surgery for thoracic insufficiency syndrome and at least 2 years of follow-up data. This series was based on 41 patients treated between 1990 and the study reporting.⁴ Entry criteria for this study were acceptance by a pediatric general surgeon, pediatric pulmonologist, and pediatric orthopedist; age 6 months to skeletal maturity; progressive thoracic insufficiency syndrome; more than 10% reduction in height of the concave hemithorax; and 3 or more anomalous vertebrae, with 3 or more fused ribs at the apex of the deformity. Patients were followed for an average of 3.2 years (range, 2 – 12 years). Before surgery, the mean annual rate of progression was 15° per year (range, 2° – 50° per year). Following surgery, the Cobb angle (of scoliosis) improved from 74° to a final value of 49°. Spine growth was at a rate of 0.8 cm per year. (Normal spinal growth is 0.6 cm/year for ages 5 – 10 years.) The final forced vital capacity (FVC) was 49% of predicted value in the 19 children who could complete pulmonary function tests. Preoperatively, 1 patient required continuous positive airway pressure, and another needed supplemental oxygen for ventilatory support at final follow-up.

Flynn et al. (2013), reported an average 40.7 month follow-up (range, 25-78 months) in 24 children with nonsyndromic congenital scoliosis.⁵ Twenty-three (95.8%) children had associated rib fusions, and the average age at surgery was 3.3 years (range, 0.7-12.5 years). With a mean of 5 expansion surgeries per patient (range, 1 – 10), the mean Cobb angle improved by 8.9° and mean thoracic height improved by 3.41 cm. Eight (33%) patients had a total of 16 adverse events, all of which required surgery.

Gadepalli et al. (2011) examined growth and pulmonary function in 26 children who received a vertical expandable prosthetic titanium rib between 2006 and 2010.⁶ In this case series, the children underwent 29 insertions and 57 expansions, with an average of 3 surgeries per child. Each procedure required an average 0.97 days in the intensive care unit and 4.41 days in the hospital. The mean Cobb angle improved by 29%, from 64.7° preoperatively to 46.1° postoperatively. Lung volumes measured by yearly thoracic computed tomography scans were similar when corrected for age. Pulmonary function tests were performed every 6 months in patients (n = 12) who were not ventilator-dependent and could cooperate with the procedure. Pulmonary function tests showed no significant change from baseline to follow-up in percent predicted values for forced expiratory volume in 1 second (54.6 L vs. 51.8 L), FVC (58.1 L vs. 55.9 L), or residual volume (145.3 L vs. 105.6 L). Reoperation was required for 14 complications, 4 for chest tube placement (pneumothorax), 1 for seroma drainage, 6 for hardware removal (for infection), and 3 for hardware repositioning (for dislodgement). Another 22 complications were treated nonoperatively.

Emans et al. (2005) reported results for patients with thoracic insufficiency syndrome who underwent the procedure at a single children’s hospital from 1999 to 2005.⁷ Thirty-one patients with fused ribs and thoracic insufficiency syndrome were treated; 4 patients had prior spinal arthrodesis with continued progression of deformity. Before surgery, all patients showed progressive spinal deformity, progressive chest deformity, or progressive hemithoracic constriction. The mean age was 4.2 years, and mean follow-up was 2.6 years (range, 0.5 – 5.4 years). A 3-member team selected patients for surgery, and cardiac function was evaluated preoperatively. Lengthening of the vertical expandable prosthetic titanium rib was planned for every 4 to 6 months but often was longer due to intercurrent illness or difficulty with travel. The mean number of device lengthenings was 3.5 (range, 0 – 10). Six patients had device exchanges for growth. In 30 patients, spinal deformity was controlled, and growth continued (1.2 cm/y) in the thoracic spine during treatment at rates similar to normal children. In this study, final FVC was 73.5% of predicted levels. Prior to the procedure, 2 patients were on ventilators and 3 patients required oxygen. At final follow-up, 1 patient required oxygen. Lung volume (measured by computed tomography scan) in the operated lung increased from 157 cm3 preoperatively to 326 cm³ at the final follow-up visit.

Motoyama et al. (2006) reported on 10 patients with thoracic insufficiency syndrome.⁸ Using a special portable pulmonary function test device, they evaluated lung function in 10 children who had a vertical expandable prosthetic titanium rib. The median age was 4.3 years (range, 1.8 – 9.8 years) at first test, and patients were followed for an average of 22 months (range, 7 – 33 months). At baseline, FVC showed moderate-to-severe decrease (69% of predicted), indicating the presence of significant restrictive lung defect. Forced vital capacity increased significantly over time, with an average rate of 26.8% per year, similar to that of healthy children of comparative ages. In terms of percent predicted values, FVC did not change significantly between the baseline and last test (70.3%), indicating that, in most children studied, lung growth kept pace with body growth.

Waldhausen et al. (2007) published a series of 22 patients.⁹ Seven (19%) of the 36 vertical expandable prosthetic titanium rib units placed required revision and 10 of 22 children reported better activity levels while 2 of 22 children reported better respiratory function.

Other series have discussed weight gain after use of vertical expandable prosthetic titanium rib in thoracic insufficiency syndrome¹⁰ or early changes in pulmonary function.¹¹

Section Summary: Thoracic Insufficiency Syndrome
The evidence evaluating use of vertical expandable prosthetic titanium rib thoracoplasty to treat children with progressive thoracic insufficiency syndrome due to rib and/or chest wall defects consists of case series. Results from the case series reported by different specialty centers have demonstrated improvement and/or stabilization in key measures with use of the vertical expandable prosthetic titanium rib in progressive thoracic insufficiency syndrome. This improvement has been 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 suffering with thoracic insufficiency syndrome, a study has demonstrated an age-specific increase in 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 and/or improvement in the clinical measures outlined above would be highly unlikely if not for the intervention. Taken together, these outcomes demonstrate the positive impact of using the vertical expandable prosthetic titanium rib technology.

Scoliosis Without Thoracic Insufficiency Syndrome
Clinical Context and Therapy Purpose
The purpose of the vertical expandable prosthetic titanium rib in patients who have early-onset scoliosis without thoracic insufficiency syndrome is to provide a treatment option that is an alternative to or an improvement on existing therapies.

The question addressed in this evidence review is: Does vertical expandable prosthetic titanium rib improve net health outcomes in patients who have scoliosis without thoracic insufficiency syndrome?

The following PICO was used to select literature to inform this review.

Populations
The relevant population of interest is young children with early-onset scoliosis without thoracic insufficiency syndrome.

Interventions
The therapy being considered is the vertical expandable prosthetic titanium rib. 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 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.

Comparators
For early onset scoliosis, relevant comparators include spinal fusion and bracing.

Outcomes
The general outcomes of interest are symptoms, morbid events, functional outcomes, treatment-related mortality, and treatment-related morbidity. Based upon the limited literature available on vertical expandable prosthetic titanium rib for scoliosis without thoracic insufficiency syndrome, follow-up of at least 4 years is recommended.

Study Selection Criteria
Methodologically credible studies were selected using the following principles:

1. To assess efficacy outcomes, comparative controlled prospective trials were sought, with a preference for RCTs.
2. In the absence of such trials, comparative observational studies were sought, with a preference for prospective studies.
3. To assess long-term outcomes and adverse events, single-arm studies that capture longer periods of follow-up and/or larger populations were sought.
4. Studies with duplicative or overlapping populations were excluded.

Review of Evidence
Non-randomized Controlled Studies
Farley et al. (2014) used data from a prospective registry to compare treatment of congenital scoliosis using vertical expandable prosthetic titanium rib (n = 22) with treatment using spinal fusion (n = 27) and observation (n = 184).¹² Function, pain, and mental health status were measured with the 22-item Scoliosis Research Society questionnaire. Compared with the observation group, the vertical expandable prosthetic titanium rib group had higher total and image scores at the second and third visits and higher function scores at the third and fourth visits. Interpretation of this study is limited due to confounding factors, including age at treatment, unknown comorbidities, and the rationale for treatment selection.

Uncontrolled Studies
An uncontrolled cohort study conducted by El-Hawary et al. (2017) enrolled 63 children (mean age 6.1 years) with early-onset scoliosis measuring more than 45 degrees (mean 72 degrees) and no rib abnormalities or thoracic dysplasia.¹³ Outcomes of interest were change in major and secondary scoliosis curves and spinal growth, based on change in coronal spine height and sagittal spine length. After 2.2 years follow-up, the mean major scoliosis curve was reduced from 72 to 57 degrees (p < 0.0001), while the secondary scoliosis curve was reduced from 42.8 to 39.6 degrees (p = 0.009). Results were similar for the change from baseline in coronal spine height (p < 0.0001) and sagittal spine length (p < 0.0001). Seventy-nine percent (42/65) of patients were deemed to have treatment success, based on a composite outcome that included controlling the major scoliosis curve and improving the coronal spine height. Nearly half of the patients (49%; 31/65) had an adverse event associated with vertical expandable prosthetic titanium rib surgery, including 15 instances of device migration.

Longer follow-up of the cohort was subsequently reported by El-Hawary et al. (2020).¹⁴ Data were available for 59 patients (mean age 6.1 years) at a mean 6.9 years follow-up. At follow-up, the vertical expandable prosthetic titanium rib was in place in 24 patients. Among the other patients, 3 had the prosthetic rib removed, 11 converted to other devices, and 13 had undergone definitive fusion. Two patients had died and 6 were lost to follow-up. At final follow-up, the mean major scoliosis curve was 61 degrees (p < 0.001 vs. baseline), while secondary scoliosis curve regressed to nearly baseline (42 degrees; p = 0.54 vs. baseline). Coronal spine height (p < 0.001) and sagittal spine length (p < 0.001) remained significantly improved from baseline. Results were similar in a subset of 29 patients that had the vertical expandable prosthetic titanium rib in place for over 5 years. At 5-years follow-up, there were 24 instances of device migration and 1 device-related Grade 3 adverse event occurred; 2 deaths were deemed not treatment-related.

Case Series
A case series conducted by White et al. (2011) reported on the off-label use of spine-to-spine vertical expandable prosthetic titanium rib to treat spinal deformity in 14 children without chest wall abnormalities.¹⁵ The indications for the dual spine-to-spine rods were absence of a primary chest wall deformity, progression of spinal deformity to a Cobb angle of greater than 50°, and migration of a previously placed proximal rib anchor or a prior non-vertical expandable prosthetic titanium rib growing rod to the point of loss of stable fixation. At final follow-up (24 – 48 months), there was an improvement in the Cobb angle from 74° to 57°, an increase in T1-S1 height from 260 to 296 mm, and no significant change in kyphosis. Complications occurred in 6 (43%) of 14 patients and included 3 rod fractures in 2 patients, 3 superficial infections, and 1 case of prominent hardware that threatened skin integrity. As noted by the authors, while results were similar to those obtained with other growing rods, “the high complication rates, need for multiple procedures in growing children, and small relative gains in radiographic parameters still challenge proof of the efficacy of all such treatment methods.”

Section Summary: Scoliosis Without Thoracic Insufficiency Syndrome
The evidence evaluating use of vertical expandable prosthetic titanium rib thoracoplasty to treat young children with early-onset scoliosis without thoracic insufficiency syndrome consists of a non-randomized controlled study, an uncontrolled cohort study, and a case series. 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.

Adverse Events
Complications that occur with vertical expandable prosthetic titanium rib need to be considered by practitioners and families when discussing this procedure. The FDA review and the articles by Campbell et al. (2004) and Emans et al. (2005) have informed the summary on complications arising from vertical expandable prosthetic titanium rib.^1,4,7 Up to 25% of patients may experience device migration, including rib erosion. Approximately 10% of patients had infection-related complications. Brachial plexus injury or thoracic outlet syndrome occurred in 1% to 7% of these series. Skin sloughing was reported in 4 (15%) patients in the study by Campbell. Waldhausen et al. (2016), in a single-center series, reported on device-related complications in 22 of 65 patients treated for thoracic insufficiency syndrome over a 13-year period.¹⁶

Summary of Evidence
For individuals who have progressive thoracic insufficiency syndrome due to rib and/or chest wall defects in childhood who receive vertical expandable prosthetic titanium rib thoracoplasty, the evidence includes case series. Relevant outcomes are symptoms, morbid events, functional outcomes, and treatment-related mortality and morbidity. Results from case series reported at different specialty centers have demonstrated improvement and/or stabilization in key measures with use of the vertical expandable prosthetic titanium rib in progressive thoracic insufficiency syndrome. This improvement has been 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 suffering with thoracic insufficiency syndrome, a study has demonstrated an age-specific increase in 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 and/or improvement in the clinical measures outlined above would be highly unlikely if not for the intervention. Taken together, these outcomes demonstrate the positive impact of using the vertical expandable prosthetic titanium rib 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 are symptoms, morbid events, functional outcomes, and treatment-related mortality and morbidity. 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.

The purpose of the following information is to provide reference material. Inclusion does not imply endorsement or alignment with the evidence review conclusions.

Practice Guidelines and Position Statements
Guidelines or position statements will be considered for inclusion in Supplemental Information if they were issued by, or jointly by, a U.S. professional society, an international society with U.S. representation, or National Institute for Health and Care Excellence (NICE). Priority will be given to guidelines that are informed by a systematic review, include strength of evidence ratings, and include a description of management of conflict of interest.

No guidelines or statements were identified.

U.S. Preventive Services Task Force Recommendations
Not applicable

Ongoing and Unpublished Clinical Trials
Some currently ongoing and unpublished trials that might influence this review are listed in Table 1.

Table 1. Summary of Key Trials

NCT No.	Trial Name	Planned Enrollment	Completion Date
Ongoing
NCT01672749	Evaluation of a Growth Guiding Construct vs. Standard Dual Growing Rods and vertical expandable prosthetic titanium rib (VEPTR) for the Treatment of Early Onset Scoliosis Patients: A Prospective Multi-center Cohort Study With a Matched Historical Control	51	Apr 2027
Completed
NCT02241954	Vertical Expandable Prosthetic Titanium Rib (VEPTR) for Thoracic Insufficiency Syndrome	7 (actual)	Feb 2020

NCT: national clinical trial.
^a Denotes industry-sponsored or cosponsored trial.  

References: 

1. Food and Drug Administration. Vertical Expandable Prosthetic Titanium Rib (VEPTR). 2004; https://www.accessdata.fda.gov/cdrh_docs/pdf14/k142587.pdf. Accessed March 11, 2022.
2. Campbell RM, 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(3): 399-408. PMID 12637423
3. Campbell RM. VEPTR: past experience and the future of VEPTR principles. Eur Spine J. Mar 2013; 22 Suppl 2: S106-17. PMID 23354777
4. Campbell RM, 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. PMID 15292413
5. Flynn JM, Emans JB, Smith JT, et al. VEPTR to treat nonsyndromic congenital scoliosis: a multicenter, mid-term follow-up study. J Pediatr Orthop. Oct-Nov 2013; 33(7): 679-84. PMID 23812154
6. 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. PMID 21238644
7. Emans JB, 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): S58-68. PMID 16138067
8. Motoyama EK, Deeney VF, Fine GF, 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. PMID 16449900
9. Waldhausen JH, Redding GJ, Song KM. Vertical expandable prosthetic titanium rib for thoracic insufficiency syndrome: a new method to treat an old problem. J Pediatr Surg. Jan 2007; 42(1): 76-80. PMID 17208544
10. 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 01 2009; 34(23): 2530-3. PMID 19927103
11. 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. Jan-Feb 2009; 29(1): 35-8. PMID 19098643
12. 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. PMID 25099323
13. 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. PMID 28141685
14. 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. PMID 32501900
15. 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. PMID 21213088
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. PMID 27397045

Coding Section 

Codes	Number	Description
CPT	22899	Unlisted procedure code, spine -no specific code
ICD-10-CM	M41.00-M41.9	Scoliosis code range
	Q76.3	Congenital scoliosis due to congenital bony malformation
	Q76.6	Other congenital malformations of ribs (includes congenital absence of rib and congenital fusion of ribs)
	Q77.2	Osteochondrodysplasia with defects of growth of tubular bones and spine; short rib syndrome (includes Asphyxiating thoracic dysplasia [Jeune])
	Q87.2	Congenital malformation syndromes predominantly involving limbs (includes VATER syndrome)
ICD-10-PCS		There is no specific ICD-10-PCS code for insertion of this device but the following codes might be used
	0PR10JZ, 0PR20JZ,0PR13JZ, 0PR23JZ,0PR14JZ, 0PR24JZ	Surgery, upper bones, replacement, rib (right or left), synthetic substitute; open, percutaneous and percutaneous endoscopic approach codes
Type of Service	Surgery
Place of Service	Outpatient/Inpatient

Procedure and diagnosis codes on Medical Policy documents are included only as a general reference tool for each policy. They may not be all-inclusive.

This medical policy was developed through consideration of peer-reviewed medical literature generally recognized by the relevant medical community, U.S. FDA approval status, nationally accepted standards of medical practice and accepted standards of medical practice in this community, Blue Cross Blue Shield Association technology assessment program (TEC) and other nonaffiliated technology evaluation centers, reference to federal regulations, other plan medical policies, and accredited national guidelines.

"Current Procedural Terminology © American Medical Association. All Rights Reserved" 

History From 2024 Forward     

01012024 NEW POLICY

06/21/2024 Annual review, no change to policy intent