Clinical UM Guideline
|Subject:||Vertical Expandable Prosthetic Titanium Rib (VEPTR)|
|Guideline #:||CG-SURG-07||Current Effective Date:||08/10/2015|
|Status:||Revised||Last Review Date:||08/06/2015|
This document addresses the use of a vertical expandable prosthetic titanium rib (VEPTR) device as a treatment of severe progressive spinal deformities associated with thoracic insufficiency syndrome (TIS), which includes severe forms of scoliosis. TIS is defined as the inability of the thorax to support normal respiration or lung growth. The VEPTR device consists of a curved rod that conforms to the shape of the thoracic cage. This device, with clearance from the U.S. Food and Drug Administration (FDA), is surgically implanted and vertically affixed to two ribs above and below expansion thoracostomies, which are placed at the site of the vertebral deformity.
The implantation, expansion (lengthening), exchange (replacement), or conversion (rib to spine) of an FDA cleared vertical expandable prosthetic titanium rib (VEPTR) device is considered medically necessary in skeletally immature individuals for the treatment of severe, progressive spinal deformities and three-dimensional deformity of the thorax associated with, or at risk for, thoracic insufficiency syndrome (TIS) including severe progressive scoliosis for ANY of the following conditions (a thru d):
Not Medically Necessary:
Use of the vertical expandable prosthetic titanium rib (VEPTR) device is considered not medically necessary when the above criteria are not met and for all other indications.
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.
|21899||Unlisted procedure, neck or thorax [when specified as implantation of vertical expandable prosthetic titanium rib]|
|ICD-10 Diagnosis||[For dates of service on or after 10/01/2015]|
|ICD-9 Diagnosis||[For dates of service prior to 10/01/2015]|
Thoracic insufficiency syndrome (TIS) is a rare, life threatening condition that involves the inability of the thorax to support normal respiration and lung growth (Campbell, 2003). The condition results from serious congenital defects that affect 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, which may include Jeune's syndrome, achondroplasia, Jarcho-Levin syndrome, and Ellis-van Creveld syndrome. Children with TIS show visible signs of the disease in a variety of ways. The syndrome is frequently terminal without surgical treatment, and each afflicted child has the potential to develop pulmonary hypertension, respiratory failure, right ventricular failure, cor pulmonale, and death.
The VEPTR® Vertical Expandable Prosthetic Titanium Rib devices (DePuy Synthes® Spine, Raynham, MA) obtained U.S. Food and Drug Administration (FDA) clearance under a Humanitarian Device Exemption (HDE) in 2004. The data submitted to the FDA as part of the approval process consisted of a single-site feasibility study of 33 subjects and a prospective, multi-center, single treatment arm trial of 247 children, ranging from ages 6 months to 15 years. The children had various serious defects affecting their ribs or chest wall and their ability to breathe, including progressive scoliosis, flail chest syndrome, rib fusion and various hypoplastic thorax syndromes. The investigators reported study results for both trials that demonstrated probable benefit by enabling some of the children to breathe unassisted, or be less dependent on ventilators. Treatment-emergent events were reported for the entire length of follow-up for both trials, (with a mean follow-up for the feasibility study of 84.8 months and 22.6 months for the multi-center trial). A total of 29 (88%) of 33 subjects in the feasibility study reported a total of 182 adverse effects, while a total of 68 adverse effects were considered to be related to the device in 21 subjects (64%). A total of 11 adverse effects that were considered potentially life-threatening were reported for 2 subjects, and 13 adverse effects of fatal intensity were reported for 4 trial participants, none of which were considered to be device-related.
In the multi-center trial, a total of 119 (56%) of 214 subjects reported a total of 356 adverse effects, while a total of 141 adverse effects, considered to be related to the device, were reported in 71 subjects (33%). The investigators reported that the majority of adverse events were related to device migrations which occurred after the subjects had been implanted with the VEPTR for 2 or more years. A total of 12 deaths were reported, none of which were related to a problem with the device.
According to the original FDA approved product label, the VEPTR is indicated for:
The treatment of thoracic insufficiency syndrome in skeletally immature patients and it should not be used under the following conditions:
According to the FDA summary of safety and probable benefit data, during the course of these 14 years of study, 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 5 degrees or greater. Studies have typically involved expansion (lengthening) of the device at 4 to 6 month intervals depending on growth rates. In some cases, a device exchange was needed for growth, and device conversion from rib-rib to rib-spine was also reported. In one series, the mean number of lengthenings performed per individual was 3.5 ± 2.6 (Emans, 2005). The FDA concluded from the submitted trial results that the probable benefits associated with VEPTR implantation outweighed the risks for the high-risk population of skeletally immature individuals with TIS.
In 2014, the FDA Center for Devices and Radiological Health (CDRH) cleared the VEPTR®-VEPTR II™ device which was reclassified as a Class II device that is considered substantially equivalent to other similar predicate devices through the 510(k) approval process for the following indications:
For skeletally immature patients with severe, progressive spinal deformities and/or three dimensional deformity of the thorax associated with, or at risk of, Thoracic Insufficiency Syndrome (TIS). TIS is defined as the inability of the thorax to support normal respiration or lung growth. This would include patients with progressive congenital, neuromuscular, idiopathic, or syndromic scoliosis (FDA, 2014).
Due to the rarity of TIS, there have been few published clinical trials of VEPTR device implantation with outcomes data. In 2007, Waldhausen and colleagues performed a retrospective chart review to analyze the cases of 22 children who received 36 VEPTR devices between October 2001 and December 2005; 2 of the implanted trial subjects had Jeune syndrome, 19 had scoliosis, and 1 had a chest wall resection for a tumor. The majority of trial participants had pulmonary restrictive disease, carbon dioxide retention or respiratory failure. A total of 11 had multiple fused ribs requiring an opening wedge thoracostomy. All but the most recently treated cases had undergone sequential VEPTR expansion; seven cases required revision for erosion through the bone or device dislodgment with three devices requiring removal. A total of five devices were outgrown and removed or replaced, and one eroded the soft tissue causing superficial infections that resolved with operative revision. The postoperative ventilation/perfusion scans showed the most improvement in the younger treated children with 2 of the 3 children with carbon dioxide retention pre-VEPTR showed reductions in carbon dioxide levels post-VEPTR. The authors concluded that the VEPTR may decrease carbon dioxide retention in some cases and may be most beneficial in younger children.
At the present time, there is little published evidence to demonstrate the safety and effectiveness of VEPTR therapy in the population of skeletally immature individuals with severe progressive scoliosis who are considered to have, or to be at risk for, TIS. The 2014 expansion of the FDA labeling for VEPTR was based on results of post marketing follow-up data from the original multi-center trial previously described. Additional trial results have consisted of small case series with outcomes data for 2 to 3 years that demonstrated some improvement in pulmonary function, thoracic height and the degree of Cobb angle deformity associated with severe scoliosis. Multiple additional expansion surgical procedures were necessary in the majority of cases to accommodate normal growth patterns, and high incidences of adverse events were seen in the reported outcomes. The investigators of these small studies concluded that the benefits outweighed the risks for the high-risk population with severe progressive scoliosis associated with TIS (Campbell, 2004; Farley, 2014; Flynn, 2013).
No definitive recommendations specific to VEPTR implantation have been issued by any of the specialty medical societies, to date. However, there is general consensus in the practice community that surgery is warranted to correct a spinal curvature with a Cobb angle greater than 45 degrees in skeletally immature individuals, in order to prevent further progression of the spinal curvature and the associated potential for pulmonary compromise.
Cobb angle: A measurement of the degree of spinal curvature. This is considered the standard measurement used in the practice community to quantify a scoliosis for the purpose of measuring curve progression over time. A curve is considered to be scoliosis at a Cobb angle of 10º or more. Any increase greater than 5º is considered a significant change indicative of curvature progression with scoliosis considered mild at 10º-24º, moderate at 25º-50º and severe at greater than 50º in skeletally mature individuals. Cobb angles greater than 45º are considered severe in skeletally immature persons.
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.
Scoliosis: A musculoskeletal condition that involves abnormal lateral curvature of the spine. There are several different types of scoliosis that affect children and adolescents. The most common type is considered idiopathic but additional types of scoliosis include congenital, neuromuscular and syndromic scoliosis.
Thoracic Insufficiency Syndrome (TIS): This rare condition is defined by the FDA as, "The inability of the thorax to support normal respiration or lung growth. This would include patients with progressive congenital, neuromuscular, idiopathic, or syndromic scoliosis" (FDA, 2014).
Peer Reviewed Publications:
Government Agency, Medical Society, and Other Authoritative Publications:
|Websites for Additional Information|
Thoracic Insufficiency Syndrome, TIS
Vertical Expandable Prosthetic Titanium Rib
The use of specific product names is illustrative only. It is not intended to be a recommendation of one product over another, and is not intended to represent a complete listing of all products available.
|Revised||08/06/2015||Medical Policy & Technology Assessment Committee (MPTAC) review. The medically necessary indications and criteria for the VEPTR device were expanded to include certain severe, progressive spinal deformities, associated with, or at risk for, TIS including scoliosis in skeletally immature individuals. The Discussion section, Definitions and References were updated.|
|Reviewed||08/14/2014||MPTAC review. Discussion and Reference sections updated.|
|Reviewed||08/08/2013||MPTAC review. Discussion and Reference sections updated.|
|Reviewed||08/09/2012||MPTAC review. Definition and Reference sections updated.|
|Reviewed||08/18/2011||MPTAC review. Discussion, Definition, Reference and Index sections updated.|
|Reviewed||08/19/2010||MPTAC review. Discussion and reference link updated.|
|Reviewed||08/27/2009||MPTAC review. Discussion and references updated. Definition section added.|
|Reviewed||08/28/2008||MPTAC review. Description, discussion and references updated.|
|Reviewed||08/23/2007||MPTAC review. Addition of Not Medically Necessary statement for clarification. References updated.|
|Revised||09/14/2006||MPTAC review. Updated language in criteria, description and discussion sections, references and coding. Added language about expansion, exchange and conversion procedures as medically necessary.|
|Revised||09/22/2005||MPTAC review. Revision based on Pre-merger Anthem and Pre-merger WellPoint Harmonization.|
|Pre-Merger Organizations||Last Review Date||Guideline Number||Title|
|WellPoint Health Networks, Inc.|
|3.07.20||Vertical Expandable Prosthetic Titanium Rib (VEPTR)|