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DESCRIPTIONThoracic 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 such as severe scoliosis, rib fusion (which may accompany scoliosis), and various hypoplastic thorax syndromes such as 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 vertical expandable prosthetic titanium rib (VEPTR) is a curved rod placed horizontally in the chest that helps to shape the thoracic cavity. It is positioned either between ribs or between ribs and either spine or pelvis. The device is designed to be expanded every four to six month as growth occurs and also to be replaced if necessary. Some patients require multiple devices.
A VEPTR has received FDA approval under a humanitarian device exemption (HDE). The FDA review indicated that the device is indicated for the treatment of Thoracic Insufficiency Syndrome (TIS) in skeletally immature patients. This review also indicated the device should not be used at an age less than 6 months.
Use of the VEPTR in pediatric patients with scoliosis without TIS is an off-label indication.
POLICYUse 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 six 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 considered investigational.
POLICY EXCEPTIONSFederal Employee Program (FEP) may dictate that all FDA-approved devices, drugs or biologics may not be considered investigational and thus these devices may be assessed only on the basis of their medical necessity.
POLICY GUIDELINESSkeletal maturity occurs at about age 14 for girls and age 16 for boys.
Given the complexity of these procedures and patients, implantation of this device should be performed in specialized centers. Preoperative evaluation requires input from pediatric orthopaedists, pulmonologist, and thoracic surgeon. In addition, preoperative evaluation of nutritional, cardiac and pulmonary function (when possible) is required.
The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.
POLICY HISTORY3/27/2007: Policy added
7/25/2007: Reviewed and approved by the Medical Policy Advisory Committee (MPAC)
6/30/2008: Policy reviewed, no changes
12/30/1010: Policy reviewed; no changes.
12/01/2011: Policy reviewed; no changes.
07/12/2012: Policy description updated. Added the following policy statement: 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 considered investigational. Added FEP verbiage to the Policy Exception section.
08/14/2013: Policy reviewed; no changes.
SOURCE(S)Blue Cross and Blue Shield Assoication Policy # 7.01.110
CODE REFERENCEThis may not be a comprehensive list of procedure codes applicable to this policy.
The code(s) listed below are ONLY medically necessary if the procedure is performed according to the "Policy" section of this document.