Archived Policies - Surgery


Vertical Expandable Prosthetic Titanium Rib (VEPTR) for Thoracic Insufficiency Syndrome (TIS)

Number:SUR705.025

Effective Date:01-01-2010

End Date:12-14-2013

Coverage:

This policy is no longer scheduled for routine literature review and update.

Implantation of the Vertical Expandable Prosthetic Titanium Rib (VEPTR) device may be considered medically necessary when provided according to the U.S. Food and Drug Administration’s (FDA) humanitarian device exemption labeled indications, for treatment of thoracic insufficiency syndrome (TIS) in skeletally immature patients. 

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.

The VEPTR device should not be used in patients with the following conditions:

  • Inadequate bone strength in the ribs or spine where the VEPTR device attaches,
  • Absence of proximal ribs for attachment of the VEPTR device,
  • Absent diaphragmatic function,
  • Inadequate soft issue for coverage of the VEPTR device,
  • Age below 6 months,
  • Age beyond skeletal maturity (about age 14 for girls and age 16 for boys),
  • Known allergy to any of the device materials, OR
  • Infection at the operative site.

Implantation of the VEPTR device for indications other than TIS is considered experimental, investigational and unproven.

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

Description:

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

Progressive thoracic insufficiency syndrome includes respiratory insufficiency, loss of chest wall mobility, worsening three-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.  While spinal fusion is one approach to treatment, it may not be successful and also may limit growth (lengthening) of the spine.

The VEPTR device is a curved rod placed vertically in the chest that helps to shape the thoracic cavity.  It is positioned either between ribs, or between the ribs and either the spine or the pelvis. The device is designed to be expanded every four to six months as growth occurs and also to be replaced if necessary.  Some patients require multiple devices.

A VEPTR has received approval from the FDA under a humanitarian device exemption (HDE).  The FDA review noted that the device is indicated for the treatment of TIS in skeletally immature patients.  This review also indicated that the device should not be used in patients younger than six months.

Rationale:

Information from the FDA web site reports results of an initial feasibility study involving 33 patients and a subsequent prospective study of 224 patients (214 with baseline data) at seven study sites.  Of these patients, 94 had rib fusion, 93 had hypoplastic thoracic syndrome, 46 had progressive scoliosis, and 14 had flail chest as a cause of their TIS.  Three- and five-year follow-up rates for the multicenter study were approximately 95%.  Of the 247 patients enrolled in either study, 12 patients died (4.8%) and two withdrew.  None of the deaths was determined by investigators to be device related.  Since standard pulmonary function testing was not possible for most of this population, an assisted ventilatory rating (AVR) was used to assess impact on respiratory status.  The AVR ranged from zero for unassisted breathing on room air to four for full-time ventilatory support.  In the multi-center prospective study, the AVR outcome improved or stabilized for 93% of the patients.  Data were not reported for the number of patients who were no longer ventilator dependent.

Several studies using this device have also been reported in the literature.  One study reported on 27 patients who had surgery for TIS and for whom at least two years of follow-up data were available; this series was based on 41 patients treated between 1990 and the acceptance of the paper.  Entry criteria for this study were acceptance by pediatric general surgeon, pediatric pulmonologist, and pediatric orthopedist; age six months to skeletal maturity; progressive thoracic insufficiency syndrome; more than 10% reduction in height of the concave hemithorax; and three or more anomalous vertebrae, with three or more fused ribs at the apex of the deformity.  Patients were followed up for an average of 3.2 (range 2-12) years.  Prior to surgery, the mean annual rate of progression was 15 degrees per year (range 2- 50).  Following surgery the Cobb angle (of scoliosis) improved from 74 degrees to a final value of 49 degrees.  Spine growth was at the 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 (PFTs).  Preoperatively, one patient required continuous positive airway pressure (CPAP) for ventilatory support; at final follow up, one patient required CPAP and one needed supplemental oxygen.

An additional study reported results on patients with TIS who underwent the procedure at Children’s Hospital in Boston from 1999 to 2005.  Thirty-one patients with fused ribs and TIS were treated; four 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).  A three-member team selected patients for surgery; cardiac function was also evaluated preoperatively.  Surgery was performed using the Campbell technique for VEPTR. Device lengthening was planned for every four to six 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, the spinal deformity was controlled and growth continued (1.2 cm/yr) in the thoracic spine during treatment at rates similar to normal children.  In this study the final FVC was 73.5% of predicted levels.  Pre-procedure, two patients were on ventilators and three patients required oxygen; at final follow-up, one patient required oxygen.  Lung volume (measured by CT scan in cubic centimeters) in the operated lung increased from 157 pre-operatively to 326 at the final follow-up visit.

A study conducted at Children’s Hospital in Pittsburgh reported on follow-up of ten patients with thoracic insufficiency with follow-up as long as 33 months.  Using a special portable pulmonary function testing device, the study reported on lung function in ten children who had placement of VEPTR.  In this population, the median age was 4.3 years (range 1.8–9.8 years) at first test, and patients were followed an average of 22 months (range 7–33 months).  At baseline, FVC showed a moderate-to-severe decrease (69% of predicted), indicating the presence of significant restrictive lung defect.  FVC 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 up with body growth.

The complications that occur with this device need to be considered by practitioners and families as they are discussing this procedure.  About 25% of patients will experience device migration, including rib erosion.  However, there does not seem to be significant long-term consequences from this.  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 four patients (15%) in the study published by Campbell.

Summary

No comparative trials have described the use of this device.  Thoracic insufficiency 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 worsening pulmonary insufficiency.

Results from the series reported at different specialty centers demonstrate improvement and/or stabilization in key measures with use of this device in progressive TIS.  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 very difficult in these patients, one study does demonstrate an age-specific increase in FVC and the studies report a final FVC in the range of 50%–70% of predicted value.

Given the usual disease course of worsening thoracic volume and ventilatory status, the stabilization or improvement in these measures would be highly unlikely in the absence of the intervention.  Taken together, these various outcome measures demonstrate the positive impact of this procedure.

A search and review of scientific literature conducted through July 2007 did not identify any published peer-reviewed literature that would alter the indications and/or limitations noted in the above discussion.  Therefore, the coverage position of this medical policy remains unchanged.

2009 Update

A search of peer-reviewed literature through October 2009 identified no new clinical trial publications or any additional information that would change the coverage position of this medical policy.

Contract:

Each benefit plan, summary plan description or contract defines which services are covered, which services are excluded, and which services are subject to dollar caps or other limitations, conditions or exclusions. Members and their providers have the responsibility for consulting the member's benefit plan, summary plan description or contract to determine if there are any exclusions or other benefit limitations applicable to this service or supply. If there is a discrepancy between a Medical Policy and a member's benefit plan, summary plan description or contract, the benefit plan, summary plan description or contract will govern.

Coding:

None


Medicare Coverage:

The information contained in this section is for informational purposes only.  HCSC makes no representation as to the accuracy of this information.  It is not to be used for claims adjudication for HCSC Plans.

The Centers for Medicare and Medicaid Services (CMS) does not have a national Medicare coverage position.  Coverage may be subject to local carrier discretion.

A national coverage position for Medicare may have been developed or changed since this medical policy document was written.  See Medicare's National Coverage at <http://www.cms.hhs.gov>.

References:

Hell, A.K, Hefti, F., et al.  Treatment of congenital scoliosis with the vertical expandable prosthetic titanium rib implant.  Orthopade (2004 August) 33(8):911-8.

U.S. Food and Drug Administration.  CDRH Consumer Information – New Humanitarian Device Approval – Vertical Expandable Prosthetic Titanium Rib (VEPTR) – H030009. (Accessed on 2004 August 24) Available at <http://www.fda.gov>.

U. S. Food and Drug Administration.  Summary of Safety and Probable Benefit Data – Vertical Expandable Prosthetic Titanium Rib (VEPTR).  (Accessed on 2004 August 24) Available at <http://www.fda.gov>.

Campbell, R.M., Mayes, T.C., et al.  The characteristics of thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis.  Journal of Bone and Joint Surgery (2003):85-A (3):399-408.

Campbell, R.M., Smith MD, et al.  The effects of opening wedge thoracostomy on thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis.  Journal of Bone Joint Surgery (2004) 86-A(8):1659-74.

Emans,  J.B., Caubet, J.F., 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 (2005): 30(17 supplement):S58-68.

Motoyama, E.K., Deeney, V.F., et al.  Effects on lung function of multiple expansion thoracoplasty in children with thoracic insufficiency syndrome: a longitudinal study.  Spine (2006) 31(3):284-90.

Thompson, G.H., Akbarnia, B.A., et al.  Growing rod techniques in early onset scoliosis.  Journal of Pediatric Orthopedics (2007 April-May) 27(3):354-61.

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 2007; 42(1):76-80.

Vertical Expandable Prosthetic Titanium Rib.  Chicago Illinois:  Blue Cross Blue Shield Association Medical Policy Reference Manual (2008 June) Surgery 7.01.110.

Policy History:

1/1/2010          Revised/Updated Entire Document.  No change in conditional coverage criteria.  This policy is no longer scheduled for routine literature review and update.

9/1/2007          Revised/Updated Entire Document

8/1/2005          New Medical Document

Archived Document(s):

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