Clinical UM Guideline


Subject:Vertical Expandable Prosthetic Titanium Rib (VEPTR)
Guideline #:  CG-SURG-07Current Effective Date:  10/14/2014
Status:ReviewedLast Review Date:  08/14/2014

Description

This document addresses the use of a vertical expandable prosthetic titanium rib (VEPTR) as a treatment of thoracic insufficiency syndrome. The VEPTR consists of a curved rod that conforms to the shape of the thoracic cage and is vertically fixed to two ribs above and below expansion thoracostomies placed at the site of the vertebral abnormality.

Clinical Indications

Medically Necessary:

The implantation, expansion (lengthening), exchange (replacement), or conversion (rib to spine) of a vertical expandable prosthetic titanium rib is considered medically necessary as a treatment of thoracic insufficiency syndrome in children between 6 months of age and skeletal maturity (about age 14 for girls and 16 for boys).

Not Medically Necessary:

All other uses of the expandable prosthetic titanium rib are considered not medically necessary.

Coding

The following codes for treatments and procedures applicable to this document are included below for informational purposes. Inclusion or exclusion of a procedure, diagnosis or device code(s) does not constitute or imply member coverage or provider reimbursement policy. Please refer to the member's contract benefits in effect at the time of service to determine coverage or non-coverage of these services as it applies to an individual member.

CPT 
21899Unlisted procedure, neck or thorax [when specified as implantation of vertical expandable prosthetic titanium rib]
  
ICD-9 Diagnosis[For dates of service prior to 10/01/2015]
 All diagnoses
  
ICD-10 Diagnosis[For dates of service on or after 10/01/2015]
 All diagnoses
  
Discussion/General Information

Thoracic insufficiency syndrome is the inability of the thorax to support normal respiration or lung growth (Campbell, 2003). The condition results from serious defects affecting the ribs or chest wall including, but not limited to, flail chest syndrome, severe scoliosis, rib fusion (which may accompany scoliosis), and various hypoplastic thorax syndromes. In general, hypoplastic thorax syndromes may include Jeune's syndrome, achondroplasia, Jarcho-Levin syndrome, and Ellis-van Creveld syndrome. Children with thoracic insufficiency syndrome show visible signs of the disease in a variety of ways, but each child has the potential to develop pulmonary hypertension, respiratory failure, right ventricular failure, cor pulmonale, and death.

The VEPTR received U.S. Food and Drug Administration (FDA) approval under a humanitarian device exemption. The data submitted to the FDA as part of the approval process consisted of a case series of 247 children, ranging from ages six months to 15 years. The children had various serious defects affecting their ribs or chest wall and ability to breathe, including progressive scoliosis, flail chest syndrome, rib fusion and various hypoplastic thorax syndromes. The study demonstrated that the device was safe and showed probable benefit by enabling some of the children to breathe unassisted, or be less dependent on ventilators. A total of 12 deaths were reported, none of which were related to a problem with the device. According to the product label, the VEPTR is indicated for treatment of thoracic insufficiency syndrome in skeletally immature children and it should not be used under the following conditions:

According to the FDA summary of safety and probable benefit data, as the children experienced normal growth or as the spine and thorax required further correction, the device would require expansions or replacement of the components to increase the overall size of the device. As a guideline, children with scoliosis or flail chest syndrome were scheduled for expansion of the device when the Cobb angle increased by five degrees or greater. Studies have typically involved expansion (lengthening) of the device at four to six month intervals depending on growth rate. In some cases a device exchange is required for growth and occasionally device conversion from rib-rib to rib-spine is necessary. In one series, the mean number of lengthenings performed per individual was 3.5 plus or minus 2.6 (Emans, 2005).

Waldhausen and colleagues (2007) performed a retrospective chart review and analyzed the cases of 22 children who received 36 VEPTR devices between October 2001 and December 2005. Two children had Jeune syndrome, 19 had scoliosis, and one had a chest wall resection for tumor. Most had pulmonary restrictive disease, carbon dioxide retention or respiratory failure. Eleven had multiple fused ribs requiring opening wedge thoracostomy. All but the most recently treated cases had undergone sequential VEPTR expansion. Seven VEPTR devices required revision for erosion through the bone or dislodgment and three were removed. Five devices were outgrown and removed or replaced and one eroded soft tissue causing superficial infection that resolved with operative revision. Postoperative ventilation/perfusion scans improved most in younger children. Two of the three children with carbon dioxide retention pre-VEPTR had carbon dioxide reduction post-VEPTR. The authors concluded that the VEPTR may decrease carbon dioxide retention in some cases and may be most beneficial in younger children.

Definitions

Cobb angle: A measurement of the degree of spinal curvature.

Ellis-van Creveld syndrome: Autosomal recessive genetic disorder characterized by skeletal dysplasia.

Jarcho-Levin syndrome: Heritable axial skeleton growth disorder associated with malformation of the vertebral column and ribs.

Jeune syndrome: Congenital dwarfism associated with asphyxiating thoracic dystrophy.

References

Peer Reviewed Publications:

  1. Campbell RM Jr. VEPTR: past experience and the future of VEPTR principles. Eur Spine J. 2013; 22 Suppl 2:S106-S117.
  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. 2003; 85-A(3):399-408.
  3. 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. 2005; 30(17 Suppl):S58-68.
  4. Hell AK, Campbell RM, Hefti F. The vertical expandable prosthetic titanium rib implant for the treatment of thoracic insufficiency syndrome associated with congenital and neuromuscular scoliosis in young children. J Pediatr Orthop B. 2005; 14(4):287-293.
  5. Maruyama T, Takeshita K. Surgical treatment of scoliosis: a review of techniques currently applied. Scoliosis. 2008; 3:6.
  6. 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. 2006; 31(3):284-290.
  7. 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.

Government Agency, Medical Society, and Other Authoritative Publications:

  1. U.S. Food and Drug Administration. Vertical Expandable Prosthetic Titanium Rib (VEPTR). Approval Order, Summary of Safety and Probable Benefit, Professional and Patient Labeling, and Other Consumer Information. Humanitarian Device Exemption No. H030009. Rockville, MD: FDA. Updated September 30, 2004. Available at: http://www.accessdata.fda.gov/scripts/cdrh/cfdocs/cftopic/pma/pma.cfm?num=H030009. Accessed on June 26, 2014.
Index

Congenital Scoliosis
Thoracic Insufficiency Syndrome
VEPTR
Vertical Expandable Prosthetic Titanium Rib

History
StatusDateAction
Reviewed08/14/2014Medical Policy & Technology Assessment Committee (MPTAC) review. Discussion and Reference sections updated.
Reviewed08/08/2013MPTAC review. Discussion and Reference sections updated.
Reviewed08/09/2012MPTAC review. Definition and Reference sections updated.
Reviewed08/18/2011MPTAC review. Discussion, Definition, Reference and Index sections updated.
Reviewed08/19/2010MPTAC review. Discussion and reference link updated.
Reviewed08/27/2009MPTAC review. Discussion and references updated. Definition section added.
Reviewed08/28/2008MPTAC review. Description, discussion and references updated.
Reviewed08/23/2007MPTAC review. Addition of Not Medically Necessary statement for clarification. References updated. 
Revised09/14/2006MPTAC review. Updated language in criteria, description and discussion sections, references and coding. Added language about expansion, exchange and conversion procedures as medically necessary.
Revised09/22/2005MPTAC review. Revision based on Pre-merger Anthem and Pre-merger WellPoint Harmonization.
Pre-Merger OrganizationsLast Review
Date
Guideline
Number
Title
Anthem, Inc.  No document
WellPoint Health Networks, Inc.09/23/20043.07.20Vertical Expandable Prosthetic Titanium Rib (VEPTR)