National Medical Policy

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1 National Medical Policy Subject: Policy Number: Growing Rods Spinal Surgery NMP354 Effective Date*: June 2007 Update: October 2015 This National Medical Policy is subject to the terms in the IMPORTANT NOTICE at the end of this document For Medicaid Plans: Please refer to the appropriate Medicaid Manuals for coverage guidelines prior to applying Health Net Medical Policies The Centers for Medicare & Medicaid Services (CMS) For Medicare Advantage members please refer to the following for coverage guidelines first: Use Source Reference/Website Link National Coverage Determination (NCD) National Coverage Manual Citation Local Coverage Determination (LCD)* Article (Local)* Other X None Use Health Net Policy Instructions Medicare NCDs and National Coverage Manuals apply to ALL Medicare members in ALL regions. Medicare LCDs and Articles apply to members in specific regions. To access your specific region, select the link provided under Reference/Website and follow the search instructions. Enter the topic and your specific state to find the coverage determinations for your region. *Note: Health Net must follow local coverage determinations (LCDs) of Medicare Administration Contractors (MACs) located outside their service area when those MACs have exclusive coverage of an item or service. (CMS Manual Chapter 4 Section 90.2) If more than one source is checked, you need to access all sources as, on occasion, an LCD or article contains additional coverage information than contained in the NCD or National Coverage Manual. Growing Rods Spinal Surgery Oct 15 1

2 If there is no NCD, National Coverage Manual or region specific LCD/Article, follow the Health Net Hierarchy of Medical Resources for guidance. Current Policy Statement Health Net, Inc. considers the use of traditional growing rods (e.g. Isola growing rods system, Expediun Growing Spine Systems, CD Horizon Growth Rod Conversion Set), or magnetically controlled growing rods (e.g., MAGEC Spinal Bracing and Distraction System), medically necessary for skeletally immature individuals with potential for additional spinal growth who are < 10 years of age and require surgical treatment to obtain and maintain correction of severe, progressive, life-threatening, early-onset spinal deformities, including early-onset scoliosis (e.g., e.g. Cobb angle of 30 degrees or more; thoracic spine height less than 22 cm), which are associated with or at risk of thoracic insufficiency syndrome, defined as the inability of the thorax to support normal respirations or lung growth. Codes Related To This Policy NOTE: The codes listed in this policy are for reference purposes only. Listing of a code in this policy does not imply that the service described by this code is a covered or noncovered health service. Coverage is determined by the benefit documents and medical necessity criteria. This list of codes may not be all inclusive. On October 1, 2015, the ICD-9 code sets used to report medical diagnoses and inpatient procedures will be replaced by ICD-10 code sets. Health Net National Medical Policies will now include the preliminary ICD-10 codes in preparation for this transition. Please note that these may not be the final versions of the codes and that will not be accepted for billing or payment purposes until the October 1, 2015 implementation date. ICD-9 Codes Spinal muscular atrophy Scoliosis (and kyphoscoliosis), idiopathic Resolving infantile idiopathic scoliosis Progressive infantile idiopathic scoliosis Curvature of spine (acquired) (idiopathic) NOS ICD-10 Codes G12-G12.9 M41-M41.9 M43.8X- M43.8X9 Spinal muscular atrophy and related syndromes Scoliosis Other specified deforming dorsopathies CPT Codes Unlisted procedures, spine HCPCS Codes NA Scientific Rationale Update October 2015 Growing Rods Spinal Surgery Oct 15 2

3 Early onset scoliosis (EOS) is a lateral curve of the spine that is diagnosed before the age of 10. There are several different types of EOS including infantile idiopathic scoliosis, juvenile idiopathic scoliosis and congenital scoliosis. There are other circumstances when the concerns unique to early onset scoliosis may also apply (neuromuscular scoliosis, syndromic scoliosis, thoracic insufficiency syndrome.) Traditional management of EOS included both nonoperative (e.g. casting, bracing) and operative techniques (e.g. insertion of growing rods, Vertical Expandable Prosthetic Titanium Rib). According to the Scoliosis Research Society (SRS), In the past, spinal fusions were the usual treatment for EOS. It is now understood that early fusion of the thoracic spine, will limit the growth of the lungs as well as the spine and can lead to severe respiratory problems. Growth friendly surgical procedures, that correct the deformity while avoiding long fusions of the spine, are being developed and refined. Current growing rod techniques combine the principle of fusionless surgery to control curve magnitude while achieving spinal growth using modern implant systems. Growth rods are currently the most commonly used distraction-based technique and which have the advantage of not interfering with the normal spinal growth and may even have a potential for growth stimulation beyond the normal growth rate. Growing rods (distractible spinal implants) were developed to address the limitations of spinal bracing or fusion for early onset scoliosis. Techniques of instrumentation include Harrington, Cotrel-Dubousset, or Luque rods. Growing rods are inserted across a segment of spinal deformity where no fusion is performed. Cranial and caudal anchoring foundations are made using hooks or pedicle screws. Each foundation is connected to a rod, and the rods are connected by cross-links. Distraction (lengthenings) of the growing rods is performed usually every six months in which the surgical incision site must be reopened for the distraction procedure. Once maximum spinal growth or skeletal maturity is reached definitive final fusion is performed. By this approach, growing rods could control progression of spinal deformity as well as gradually correct the deformity. Implant related complications are the most common complications in growth rod surgeries. These include rod fracture, anchor failure, or prominent implant, which can cause skin breakdown and even infection. Among the implant-related complications, rod fractures are the most common problem. Recently, as an alternative to avoid the limitations associated with the growing rod, an implant that allows less invasive distractions is proposed. A remotely distractable, magnetically controlled growing rod system has been developed to facilitate outpatient rod distractions, eliminate frequent surgeries under general anesthesia and reduce wound complications, and frequent hospitalization in young children (i.e., Ellipse MAGEC Spinal Bracing and Distraction System, Irvine CA.) The MAGEC system includes an adjustable growing rod that uses a magnetic, remote controlled technology to non-invasively lengthen the growing rods placed in skeletally immature patients with severe progressive spinal deformity, without requiring repeat surgical rod lengthening. Following an initial procedure to implant the MAGEC rods, the device can be distracted or retracted noninvasively during outpatient visits by using the MAGEC External Remote Controller (ERC). The technology is intended to be used in place of current growth rod systems that need repeated invasive surgical procedures. The MAGEC growth rods are usually removed and replaced by a spinal fixation system to fuse the spine when skeletal maturity is reached. Growing Rods Spinal Surgery Oct 15 3

4 Per the FDA approval (Sep 2014), the Ellipse MAGEC Spinal Bracing and Distraction System is intended for skeletally immature patients < 10 years of age with severe progressive spinal deformities (e.g. Cobb angle of 30 degrees or more; thoracic spine height less than 22 cm) associated with or at risk of Thoracic Insufficiency Syndrome (TIS). TIS is defined as the inability of the thorax to support normal respirations or lung growth. The FDA notes The MAGEC system is substantially equivalence to the pre-amendment Harrington Rod. The system is based on similar indications for use, technological characteristics, and on pre-clinical testing and clinical data. Per the FDA approval, "The safety and probable benefit of the MAGEC System was evaluated outside the United States in a retrospective clinical study for children who had either a primary or revision spinal bracing procedure using the MAGEC System. In assessing probable benefit, the endpoints chosen in the study included Cobb angle correction in the coronal plane, thoracic spine height increase, improvement in space available for lung (SAL), coronal and sagittal balance, reduction in the number of subsequent surgical procedures, and weight gain. The results of the clinical study showed the MAGEC System provides the benefits of spinal deformity correction and continued growth, similar to that for traditional growing rods, without the need for regular surgical lengthening procedures in these children. As with traditional growing rods, the MAGEC System provides direct bracing to the spine. This bracing provides for correction and maintenance of the scoliotic curve as defined by the Cobb Angle. In addition, a return to a more normal symmetry of the thoracic cavity is provided as demonstrated by the space available for lung (SAL). While implantation of the MAGEC System shares many of the same risks and hazards associated to those of traditional growing rods, the MAGEC System offers the benefit of non-invasive adjustment to lengthen the implanted rod without the need to perform another surgery. The ability of the device to be adjusted non-invasively in length provides the ability of the spine to continue growing in these subjects and for the Thoracic Spine Height to increase with this growth." It did not state how many were included in the retrospective clinical study. According to a NICE technology guidance, The MAGEC system for spinal Lengthening in children with scoliosis (June 2014): The case for adopting the MAGEC system for spinal lengthening in children with scoliosis is supported by the evidence. Using the MAGEC system would avoid repeated surgical procedures for growth rod lengthening. This could reduce complications and have other physical and psychological benefits for affected children and their families. The MAGEC system should be considered for use in children with scoliosis aged 2 years and over who need surgery to correct their spinal curvature, for example when conservative methods such as bracing or casting have failed. Traditional Growing Rods Akgül et al (2014) sought to determine the safety and effectiveness of the single and dual growing rod techniques and which technique was the most effective in the management of EOS respectively. From 2003 to 2009, 23 patients underwent single (15) or dual (8) growing rod procedures using a pedicle screw construct and tandem connectors. The etiology of the patients' spinal deformities were as follows; infantile, juvenile idiopathic, congenital and neuromuscular. Clinical evaluation included age, sex, diagnosis, follow-up, number and frequency of lengthenings, and complications. Radiographic evaluation included measured changes in Cobb angle, kyphosis, lordosis, frontal and sagittal balance. Overall 46 lengthening procedures were performed, the average number of lengthening procedures being 2.1 +/ per patient. The average time between two lengthening procedures was 13 (2-28) Growing Rods Spinal Surgery Oct 15 4

5 months. Average follow-up time was / months. The mean coronal Cobb angle was improved from / to / Statistically, at the final follow-up, early postoperative measurements in the coronal plane were better in the dual growing rod group than in the single rod group. Nine patients underwent fusion surgery. Their mean age was 11 (10-14) years, with a follow-up of 34.6 (14-54) months. The mean Cobb angle before fusion was 58.7 (40-75 ). There were 0.9 complications per patient in all groups, 0.38 in the dual rod and 1.2 in the single rod group, respectively. The authors concluded dual growing rods result in better deformity correction and stability of correction with an acceptable complication rate Liang et al (2015) sought to identify risk factors for postoperative complications associated with growing rod surgery for EOS. A total of 55 consecutive patients underwent growing rod surgery for EOS were examined from database. Data included age at initial surgeries, sex, diagnosis, body mass index (BMI), duration of follow-up, initial and final measure of major curve, T2-5, T5-12, T10-L2, and T12-S1 kyphosis angles, levels and type of instrumentation, total number of surgeries, number of rods inserted, number of lengthenings, lengthening intervals and rod location were studied. Risk factors for postoperative complications were analyzed using binomial multiple logistic regression analysis. Postoperative complications were associated with 37 of 272 procedures (14%) and affected 23 patients (42%). Complications included 25 implant-related failures (66%), 4 alignment complications (11%), 4 infections (11%), 1 neurological impairment (3%), 3 respiratory problems, 2 gastrointestinal problems, 1 urinary problem, and 1 dural tear. The most frequent implant-related failure was dislodged implant (76%) and 92% of the dislodgements occurred at the proximal foundation. Binomial multiple logistic regression analysis demonstrated that curve magnitude in last follow-up (OR: 1.042; P=0.036), duration between growing-rod lengthening procedures (OR: 1.121; P=0.003) and duration of follow-up (OR: 1.079; P=0.001) maintained its significance in predicting likelihood of postoperative complications. The authors concluded the occurrence of postoperative complications in growing rod surgery for EOS is most likely multifactorial and is related to curve magnitude in last follow-up and duration between growing-rod lengthening procedures. Sánchez Márquez et al (2013) sought to determine the efficacy of growing rods in the treatment of early onset scoliosis. A total of 32 patients were treated using fusion techniques that included double growing rods and Vertical Expandable Prosthetic Titanium Ribs (VEPTR), in our Early Onset Scoliosis Centre between 2004 and After analyzing the clinical histories and x-rays, 20 patients were included due to meeting the inclusion criteria. All patients had previously received conservative treatment with cranial traction and a series of plasters/corsets. The deformity was analyzed before and after the initial surgery, and in successive tightenings, using the x-rays of the coronal and sagittal planes by means of the Cobb angle, as well as the longitudinal and coronal growth of the thorax, and the growth of the spinal column. A series of 188 x-rays of 53 patients with cystic fibrosis were studied in order to perform a comparative analysis with the patients with early-onset scoliosis. There was significant improvement in the angle (Cobb and kyphosis) and linear parameters (T1-S1 distance, T1-T12 distance, and coronal width of the thorax) after the initial surgery, but the successive tightenings had a minimal beneficial effect, losing effectiveness over a period of time. The patients with early-onset scoliosis showed a lower growth of the thorax compared to the patients with cystic fibrosis. The authors concluded treatment of early-onset scoliosis with expandable devices is mainly beneficial with the initial procedure and the first tightenings, but shows a loss of efficacy over a period time. Growing Rods Spinal Surgery Oct 15 5

6 Caniklioglu et al (2012) sought to investigate the effects of growing rod treatment on the clinical and radiographic outcome and respiratory function of young children with scoliosis. Data from 25 patients (24 females, 1 male) who underwent surgical treatment with growing rods for scoliosis between 1997 and 2007 were evaluated retrospectively. Dual growing rods were used in 16 patients and single growing rods in 9. Patients' average age was 7.38 ± 3.8 years at the initial surgery. Cobb angle, T1-S1 length, and instrumentation length were measured radiographically. Respiratory functions were evaluated at the final follow-up. Patients received an average of 4.2 lengthening treatments over an average period of 44.9 months. Cobb angles improved from 56.7 to 25.1 after final fusion. T1-S1 length increased from 27.2 ± 3.4 to 34.9 ± 3.6 cm after the initial surgery and 38.6 ± 3.7 cm post final fusion. Average growth was 1 ± 0.4 cm per year. Mean values of respiratory parameters at the last follow-up were FVC: 83.5 ± 3.5, FEV: 84.8 ± 5.3, and FVC/FEV1: 1 ± Twelve patients experienced complications, of which eight were instrument-related and four medical. The authors concluded the growing rod technique is effective in the treatment of spinal deformity in young scoliosis patients and appropriate for improving both spinal column height and pulmonary function. Zhao et al (2012) investigated the initial efficacy of single and dual growing rods in treatment of EOS. A retrospective study of 25 early onset scoliosis cases treated with growing rod technique between November 2002 and May 2010 was performed, including six cases in the single growing rod group and 19 cases in the dual growing rod group. Operation time, intra-operative bleeding, correction rate, changes in C7- S1 distance, and incidence of complications of the first operation were compared for the two techniques. The average post-operative follow-up duration was 31.9 months. There was no statistical difference observed between operation time, intraoperative bleeding, and complication incidence between the single and dual growing rod groups. In addition, no statistical difference was observed in the pre-operative coronal Cobb's angle (P > 0.05), or in the pre-operative sagittal Cobb's angle between both groups (P > 0.05). The correction rate of the dual growing rod group was significantly superior to that of the single growing rod group in the coronal plane (P < 0.01), but not in the sagittal plane (P > 0.05). The C7-S1 distance in the dual growing rod group was significantly larger than that in the single growing rod group (P < 0.05). The authors concluded the growing rod technique is an effective option for surgical treatment of EOS. The dual growing rod technique shows relative superiority in the correction outcome as compared to the single growing rod technique. Bess et al (2010) sought to evaluate the clinical and radiographic complications associated with growing-rod treatment. Data from the multicenter Growing Spine Study Group database were evaluated. Inclusion criteria were growing-rod treatment for early-onset scoliosis and a minimum of two years of follow-up. Patients were divided into treatment groups according to rod type (single or dual) and rod location (subcutaneous or submuscular). Complications were categorized as wound, implant, alignment, and general (surgical or medical). Surgical procedures were classified as planned and unplanned. Between 1987 and 2005, 140 patients met the inclusion criteria and underwent a total of 897 growing-rod procedures. The mean age at the initial surgery was six years, and the mean duration of follow-up was five years. Eighty-one (58%) of the 140 patients had a minimum of one complication. Nineteen (27%) of the seventy-one patients with a single rod had unplanned procedures because of implant complications, compared with seven (10%) of the sixty-nine patients with dual rods (p 0.05). Thirteen (26%) of the fifty-one patients with Growing Rods Spinal Surgery Oct 15 6

7 subcutaneous rod placement had wound complications compared with nine of the eighty-eight patients (10%) with submuscular rod placement (p 0.05). The patients with subcutaneous dual rods had more wound complications, more prominent implants, and more unplanned surgical procedures than did those with submuscular dual rods (p 0.05). The risk of complications occurring during the treatment period decreased by 13% for each year of increased patient age at the initiation of treatment. The complication risk increased by 24% for each additional surgical procedure performed. The authors concluded regardless of treatment modality, the management of early-onset scoliosis is prolonged; therefore, complications are frequent and should be expected. Complications can be reduced by delaying initial implantation of the growing rods if possible, using dual rods, and limiting the number of lengthening procedures. Submuscular placement reduces wound and implant-prominence complications and reduces the number of unplanned operations. Magnetically Controlled Growing Rods LaRosa et al (2015) presented a series of 10 patients with EOS managed with magnetically controlled growing rod (MCGR) (Ellipse MAGEC System). The authors implanted MCGR in 10 patients affected by EOS. Scoliosis and kyphosis angles, T1- T12 and T1-S1 length were evaluated preoperatively, postoperatively, and at the last follow-up. A visual analogue scale score was used to evaluate pain during outpatient rod distraction procedures. The mean follow-up is 27 months. All patients attended distractions of the magnetic rod through an external remote control every 3 months. The mean predicted distraction was 3 mm at each lengthening session. The mean Cobb angle value was 64.7±17.4 degrees (range, 45 to 100 degrees) preoperatively and 28.5±13.9 degrees (range, 15 to 59 degrees) at the latest follow-up. The mean T1-S1 length value was 27.1±5.4 cm (range, 16 to 34.8 cm) preoperatively and 32.8±4 cm (range, 26.5 to 39 cm) at the latest follow-up. The mean T1-T12 length value was 16.2±2.7 cm (range, 10 to 19 cm) preoperatively and 20.6±2.9 cm (range, 15.5 to 23.5 cm) at the latest follow-up. The average monthly T1-T12 height increase was 0.8 mm, whereas the average monthly T1-S1 increase was 0.9 mm. Two patients experienced a rod breakage and 1 patient had a pull-out of the apical hooks. The authors concluded although implant-related complications could occur, as in all EOS growing rods procedures, MCGR can be effectively used in patients with EOS. This spinal instrumentation can overcome many of the complications related with the traditional growing rods implants. This procedure can be effectively used in outpatient settings, minimizing surgical scarring, surgical site infection, and psychological distress due to multiple surgeries needed in the traditional growing rods system, improving quality of life, and saving health care costs. Akbarnia et al (2014) compared the effectiveness of MCGR versus traditional growing rods (TGR) for the treatment of early-onset scoliosis. Magnetically controlled growing rod patients were selected based on the following criteria: aged less than 10 years, major curve greater than 30, thoracic height less than 22 cm, no previous spine surgery, and minimum 2-year follow-up. A total of 17 MCGR patients met the inclusion criteria, 12 of whom had complete data available for analysis. Each MCGR patient was matched with a TGR patient by etiology, gender, single versus dual rods, preoperative age, and preoperative major curve. Magnetically controlled growing rod patients had a mean age of 6.8 years and mean follow-up of 2.5 years. Mean followup was greater for TGR patients by 1.6 years. Major curve correction was similar between MCGR and TGR patients throughout treatment. The MCGR patients experienced an average of 8.1 mm/year increase in T1 S1 during the lengthening period, compared with 9.7 mm/year for TGR patients (p =.73). There was a mean Growing Rods Spinal Surgery Oct 15 7

8 increase in T1 T12 of 1.5 mm/year for MCGR patients and 2.3 mm/year for TGR patients (p =.83). The TGR patients had 73 open surgeries, 56 of which were lengthenings. The MCGR patients had 16 open surgeries and 137 noninvasive lengthenings. Three TGR patients underwent 5 unplanned revision surgeries whereas 3 MCGR patients underwent 4 unplanned revisions. The authors concluded major curve correction was similar between MCGR and TGR patients throughout treatment. Annual T1 S1 and T1 12 growth was also similar between groups. The MCGR patients had 57 fewer surgical procedures than TGR patients. Incidence of unplanned surgical revisions as a result of complications was similar between groups. Per the authors the retrospective study was limited by the following: The study included patients from multiple centers; therefore, the variability in surgical technique, postoperative care, and lengthening regimen introduced several confounding variables for which the researchers did not account in their analysis. Patients were not matched by curve pattern or levels of instrumentation, and these were not factored into the radiographic analysis. In addition, the MCGR patient cohort was not a consecutive series because some patients were eliminated for lack of follow-up or did not have complete medical records. Furthermore, depending on when treatment is initiated, growing rod surgery typically requires several years of lengthenings until patients reach skeletal maturity. Therefore, there may be clinically relevant differences between the groups that may not be evident within the 2-year follow-up period. Additional follow-up until skeletal maturity will be required to truly determine whether differences exist between these 2 surgical techniques. If the authors had been able to account for these factors in their analysis, it is feasible to consider that the results of this study would have been significantly different. With regard to the patient with the 36 preoperative major curve, although the patient would generally be considered a candidate for continued nonsurgical therapy, the treating physician and parents of the child both thought MCGR surgery was the best treatment option in this particular situation. Dannawi et al (2013) described the outcomes and complications of using a noninvasive MCGR in children with EOS. Lengthening was performed on an outpatient basis using an external remote control with the patient awake. Between November 2009 and March 2011, 34 children with a mean age of eight years (5 to 12) underwent treatment. The mean length of follow-up was 15 months (12 to 18). In total, 22 children were treated with dual rod constructs and 12 with a single rod. The mean number of distractions per patient was 4.8 (3 to 6). The mean pre-operative Cobb angle was 69 (46 to 108 ); this was corrected to a mean 47 (28 to 91 ) post-operatively. The mean Cobb angle at final review was 41 (27 to 86 ). The mean pre-operative distance from T1 to S1 was 304 mm (243 to 380) and increased to 335 mm (253 to 400) in the immediate post-operative period. At final review the mean distance from T1 to S1 had increased to 348 mm (260 to 420).Two patients developed a superficial wound infection and a further two patients in the single rod group developed a loss of distraction. In the dual rod group, one patient had pull-out of a hook and one developed prominent metalwork. Two patients had a rod breakage; one patient in the single rod group and one patient in the dual rod group. The authors concluded the early results show that the MCGR is safe and effective in the treatment of progressive early-onset scoliosis with the avoidance of repeated surgical lengthenings. Akbarnia et al (2013) report the preliminary results of MCGR technique in children with progressive EOS in a prospective multicenter nonrandomized study of clinical and radiographical data. The study included patients who underwent MCGR surgery and at least 3 distractions. Distractions were performed in clinic without Growing Rods Spinal Surgery Oct 15 8

9 anesthesia/analgesics. T1-T12 and T1-S1 heights and the distraction distance inside the actuator were measured after lengthening. Fourteen patients (7 girls, 7 boys) with a mean age of 8 years, 10 months (3 yr, 6 mo to 12 yr, 7 mo) had 14 index surgical procedures. Of the 14, 5 had single-rod (SR) surgery and 9 had dual-rod (DR) surgery, with overall 68 distractions. Diagnoses were idiopathic (N = 5), neuromuscular (N = 4), congenital (N = 2), syndromic (N = 2), and neurofibromatosis (N = 1). Mean follow-up was 10 months ( ). The Cobb angle changed from 60 to 34 after initial surgery and 31 at latest follow-up. During distraction period, T1-T12 height increased by 7.6 mm for SR (1.09 mm/mo) and mm for DR (1.97 mm/mo). T1-S1 height gain was 9.1 mm for SR (1.27 mm/mo) and 20.3 mm for DR (3.09 mm/mo). Complications included superficial infection in 1 SR, prominent implant in 1 DR, and minimal loss of initial distraction in 3 SR after index. Partial distraction loss observed after 14 of the 68 distractions (1 DR and 13 SR) but regained in subsequent distractions. There was no neurological deficit or implant failure. The authors concluded preliminary results indicated MCGR was safe and provided adequate distraction similar to standard GR. DR achieved better initial curve correction and greater spinal height during distraction compared with SR. No major complications were observed during the follow-up. Cheung et al (2012) assessed the effectiveness and safety of MCGR for non-invasive outpatient distractions. Five patients were implanted with MCGR, two of whom have now reached 24 months' follow-up. Each patient underwent monthly outpatient distractions. We used radiography to measure the magnitude of the spinal curvature, rod distraction length, and spinal length. The authors assessed clinical outcome by measuring the degree of pain, function, mental health, satisfaction with treatment, and procedure-related complications. In the two patients with 24 months' follow-up, the mean degree of scoliosis, measured by Cobb angle, was 67 (SD 10 ) before implantation and 29 (4 ) at 24 months. Length of the instrumented segment of the spine increased by a mean of 1 9 mm (0 4 mm) with each distraction. Mean predicted versus actual rod distraction lengths were 2 3 mm (1 2 mm) versus 1 4 mm (0 7 mm) for patient 1, and 2 0 mm (0 2 mm) and 2 1 mm (0 7 mm) versus 1 9 mm (0 6 mm) and 1 7 mm (0 8 mm) for patient 2's right and left rods, respectively. Throughout follow-up, both patients had no pain, had good functional outcome, and were satisfied with the procedure. No MCGR-related complications were noted. The authors concluded the MCGR procedure can be safely and effectively used in outpatient settings, and minimizes surgical scarring and psychological distress, improves quality of life, and is more cost-effective than is the traditional growing rod procedure. The technique could be used for non-invasive correction of abnormalities in other disorders. A clinical trial (NCT ) sponsored by Ellipse, Study of the Surgical Treatment of Early Onset Scoliosis Using a Non-invasive Growing Rod is ongoing but no longer recruiting participants. Scientific Rationale Update May 2015 Shah et al (2014) reported the effect of repeated growing rod (GR) lengthenings on the sagittal and pelvic profile in patients with early-onset scoliosis in a retrospective case series. The authors retrospectively reviewed data from a multicenter earlyonset scoliosis database. Forty-three patients who were able to walk with minimum 2-year follow-up who underwent single- or dual-gr surgery were included for review. Mean number of lengthenings was 6.4 (range, 3-16). Mean preoperative age was 5.6 years (standard deviation, 2.4 yr), and mean follow-up was 3.5 years. Maximum thoracic kyphosis (TK), lumbar lordosis (LL), and sagittal balance were assessed Growing Rods Spinal Surgery Oct 15 9

10 preoperatively, after index surgery, and at the latest follow-up. There was a significant decrease both in TK and LL after index surgery, which then increased during the lengthening period. There was a significant increase in both proximal junctional kyphosis and distal junctional angle. Pelvic parameters (pelvic tilt, pelvic incidence, sacral slope) were unchanged during the treatment period. Significant improvement was observed in sagittal balance. There was a correlation between the change in TK and change in LL. The authors concluded TK decreased after index surgery and increased between the index surgery and the latest follow-up, which was accompanied by an increase in LL. All-screw proximal constructs had mean 9 more proximal junctional kyphosis than all-hook proximal constructs. An increase in proximal junctional kyphosis and distal junctional angle was found during the treatment period. Although there was an independent effect of number of lengthenings on TK, there was no significant detrimental effect on other sagittal spinopelvic parameters. GRs had a positive effect on sagittal vertical axis, which returned patients to a more neutral alignment through the course of treatment. Kabirian et al (2014) reported deep surgical site infection may change the course of growing-rod treatment of early-onset scoliosis. Our goal was to assess the effect of this complication on subsequent treatment. A multicenter international database was retrospectively reviewed; 379 patients treated with growing-rod surgery and followed for a minimum of two years were identified. Deep surgical site infection was defined as any infection requiring surgical intervention. Forty-two patients (11.1%; twenty-five males and seventeen females) developed at least one deep surgical site infection. The mean age at the initial growing-rod surgery was 6.3 years (range, 0.6 to 13.2 years) and the mean duration of follow-up was 5.3 years (range, 2.2 to 14.3 years). The mean interval between the initial surgery and the first deep surgical site infection was 2.8 years (range, 0.02 to 7.9 years). Ten (2.6%) of the 379 patients developed deep surgical site infection before the first lengthening. Twenty-nine patients (7.7%) developed the infection during the course of the lengthening procedures, and three patients (0.8%) developed it after final fusion surgery. Thirty (13.6%) of 221 patients with stainless-steel implants had at least one deep surgical site infection compared with twelve (8%) of 150 patients with titanium implants (p < 0.05). (The remaining patients were treated with chromium-cobalt implants.) Twenty-two (52.4%) of the forty-two patients with deep surgical site infection underwent implant removal, which was complete in thirteen and partial in nine. Growing-rod treatment was terminated in two patients with partial removal and six patients with complete removal. An increased risk of deep surgical site infection was associated with stainless-steel implants (odds ratio [OR] = 5.7), non-ambulatory status (OR = 2.9), and the number of revisions before the development of deep surgical site infection (OR = 3.3). Neuromuscular etiology and non-ambulatory status increased the possibility of implant removal to treat infection (p < 0.05). The reviewers concluded the prevalence of deep surgical site infection associated with growing-rod surgery is higher than that associated with standard pediatric spinal fusion (historical data). Non-ambulatory status, more revisions, and stainless-steel implants increased the risk of deep surgical site infection. After eight surgical procedures, the risk of deep surgical site infection increased to approximately 50%. When patients have implant removal, efforts should be made to retain one longitudinal implant to continue treatment. Scientific Rationale Update May 2014 Kamaci et al (2014) reported treating progressive early-onset idiopathic scoliosis is challenging. Surgical treatment is indicated in patients whose curves progress despite nonsurgical treatment. Dual growing rod (DGR) technique allows control of Growing Rods Spinal Surgery Oct 15 10

11 the curve while permitting continued spine growth and pulmonary development. Correction in coronal and sagittal planes with this technique has demonstrated both clinically and radiologically in previous studies. It is shown that apical vertebra rotation (AVR) increases with single-rod instrumentation technique. The effect of DGR technique on AVR has not been investigated. The aim of the study was to assess the impact of DGR instrumentation technique on the apical AVR. The study included 12 patients with early-onset idiopathic scoliosis treated with DGR technique. Mean follow-up was 74 months. Vertebral rotation angle in the apex of the curve in preindex surgery was measured with Perdriolle and Stokes' method. As pedicle shadows were masked by rods postoperatively, vertebral rotation angle of same levels in final computed tomography scans was measured using Aaro and Dahlborn's method. Standing anterior-posterior and lateral x-rays were measured for assesing Cobb angle, thoracic kyphosis, lumbar lordosis in coronal and sagittal planes preoperatively, postoperatively, and at the time of final follow-up. Mean age at the time of growing rod instrumentation was 69 (36 to 108) months. Mean follow-up was 77 (57 to 91) months. The mean preoperative AVR angle was 27 (18 to 38) degrees and decreased to 18 (4 to 35) degrees at the time of final follow-up. The difference between preoperative and final follow-up AVR was found to be significant (P=0.003). Preoperative mean Cobb angles were found to be 63.8 (40 to 98) degrees, 25 (10 to 46) degrees (60%) (P<0.001) after index surgery and at the time of final follow-up 20 (7 to 42) degrees (66%) (P<0.001). The mean thoracic kyphosis and lumbar lordosis angles were found to be 46 (20 to 90) and 34 (16 to 80) degrees at preoperative stage; 25 (12 to 50) and 22 (8 to 35) degrees at immediate postoperative stage; and 38 (16 to 83) and 37 (16 to 60) degrees at the time of final follow-up. Investigators concluded the study proves that the DGR technique has no negative effect on transverse plane deformities. When compared with preoperative values, correction of the AVR during the treatment period suggests that DGR is effective in controlling the coronal and sagittal planes along with transverse plane deformities. Further studies are needed to prove that DGR treatment definitely prevents progression of AVR. Flynn et al (2013) searched a multicenter early-onset-scoliosis database to identify patients who had undergone treatment with growing rods and either had had a final operative procedure or were still being treated with the growing rods after reaching skeletal maturity (defined as fourteen years of age or older). Clinical, radiographic, and operative data were analyzed. Ninety-nine patients met the inclusion criteria, and ninety-two (93%) of them had had a final operative procedure. The remaining seven patients (7%) were older than fourteen years but had not undergone a final procedure. Of the ninety-two patients who had a final procedure, seventy-nine (86%) had an instrumented fusion, nine (10%) had growing-rod exchanges and fusion in situ, three (3%) had the growing rods left in place and fusion in situ, and one (1%) had only growing-rod removal. The mean age (and standard deviation) at the final fusion was 12.4 ± 1.9 years. In forty-four (55%) of eighty patients for whom the information was available, the number of vertebral levels fused was the same as the number of vertebral levels spanned by the growing rods. The percent correction of the curve after final fusion was none or minimal ( 20 %) in eleven (18%) of the sixty-two patients for whom sufficient-quality radiographs were available, moderate (21% to 50%) in thirty (48%), and substantial ( 51 %) in nine (15%); the curve had worsened in twelve patients (19%). The mean duration of growing-rod treatment was 5.0 ± 2.6 years. Of fifty-eight operative reports made at final fusion that contained comments on spinal flexibility, eleven (19%) described the spine as being mobile, eleven (19%) described decreased flexibility, and thirty-six (62%) described the spine as being completely stiff. At final fusion, twenty-two Growing Rods Spinal Surgery Oct 15 11

12 patients (24%) had osteotomies and seven patients (8%) had a thoracoplasty. Authors concluded most patients underwent growing-rod removal and final instrumented fusion. The final fusion often included the same levels spanned by the growing rods and usually achieved <50% additional correction of the deformity remaining at the end of the growing-rod management. Latalski et al (2013) presented a surgical method of treating early onset scoliosis which consists in a single-stage insertion of special implants that enable three-plane correction of spinal deformities, allowing the spine to continue growing, does not require multi-stage surgical distractions, and ends after the growth period with a conventional spinal fusion. The results of this pilot study were obtained in a homogeneous group of patients treated identically by insertion of original implants guiding spinal growth. The study involved 15 females and 2 males aged between 5 and 13 years (mean age: 9.8 years). All children in the study group had single-curve thoracic scoliosis. The duration of follow-up was between 6 and 40 months (mean duration: 18 months). The efficacy of the guided-growth implant treatment was assessed based on standard radiographs by evaluating the angle of the curvature, T1-S1 length, and apical vertebral rotation (AVR) preoperatively, postoperatively, and in long-term follow-up. After surgery the scoliosis improved significantly in the range of 51% to 80% (mean improvement: 65%). The degree of the correction depended directly on the initial angle of curvature, which ranged from 56 to 95 (mean angle of curvature: 67 ). During the entire follow-up period, twelve patients did not show any loss of correction, or the loss was within the bounds of measurement error. Because of a growth spurt, two female patients had to have the rods replaced with longer ones, since there was a risk that they might slide out of the farthest lower screws. In three patients further spontaneous improvement occurred during the follow-up period. Apical vertebral derotation was achieved during the surgery in all patients, and it was maintained throughout the follow-up period. All patients showed an increase in spinal length in the range of 7 to 40 mm (mean increase: 1mm/month). Investigators concluded the surgical method described provided for very good correction in the first stage of treatment. The maintenance of the correction does not require the use of corrective braces or any indirect multistage surgical procedures and the probability of complications during the insertion of the implants is not higher than that seen with conventional multi-stage treatment. Scientific Rationale Update May 2013 Watanabe et al. (2013) completed a retrospective, multicenter study, to identify risk factors for postoperative complications associated with growing-rod surgery (GR) for early-onset scoliosis (EOS). Results and complications of GR for EOS have not been adequately studied. The authors evaluated clinical and radiographic results from 88 EOS patients who underwent GR in 12 spine centers in Japan. The mean age at the time of initial surgery was 6.5±2.2 years (range, years) and the mean follow-up period was 3.9±2.6 years (range, years). Risk factors for postoperative complications were analyzed using binomial multiple logistic regression analysis. The authors considered the potential factors of gender, age, number of rodlengthening procedures, whether a pedicle-screw foundation was used, the uppermost level of the proximal foundation and lowermost level of the distal foundation, Cobb angles of the proximal thoracic, main thoracic, and lumbar curves, and the kyphosis angles in the proximal, main thoracic, thoracolumbar, and lumbar spine. Kaplan-Meier analysis was used to determine the complication-free survival rate of GR as a function of the number of surgical procedures. Complications affected 50 of the patients (57%) and were associated with 119 of 538 surgical procedures, with 86 implant-related failures (72%), 19 infections (16%), 3 neurological Growing Rods Spinal Surgery Oct 15 12

13 impairments (3%), and 11 other complications. The most frequent implant-related failure was dislodged implant (71%) and 95% of the dislodgements occurred at the proximal foundation. Kaplan-Meier analysis demonstrated a linear decrease in complication-free rates as the number of rod-lengthening procedures increased. Binomial multiple logistic regression analysis found the following significant independent risk factors: 6 or more rod-lengthening procedures (odds ratio [OR], 6.534), an increase of every 20 in the proximal thoracic Cobb angle (OR, 3.091), and an increase of every 25 in the lumbar lordosis angle (OR, 2.607) in the preoperative condition. Increases in the upper thoracic scoliotic curve, thoracic kyphosis, and number of rod-lengthening procedures are positively associated with an increased risk of complications after GR for EOS. Scientific Rationale Update May 2012 Sankar et al (2011) evaluated the effect of repeated surgical lengthenings and time on spinal growth and Cobb angle in children with early onset scoliosis and dual growing rods. Medical records from five different centers were reviewed to identify children treated with dual growing rods for early onset scoliosis who had a minimum of 2-year follow-up and at least three lengthening procedures. Initial radiographs, postimplantation radiographs, and radiographs from before and after each lengthening were measured for T1-S1 distance and Cobb angle. Linear regression and analysis of variance were used for statistical analysis. Thirty-eight patients from five centers met the inclusion criteria. The average age of patients was 5.7 years (range years); mean follow-up was 3.3 years (range 2-7 years). The average interval between lengthenings was 6.8 months. Cobb angle decreased from a mean value of 74 preoperatively to 36 after the primary implantation and did not change significantly with repeated lengthenings (P = 0.96). After initial implantation, the average annual T1-S1 gain was 1.76 ± 0.71 cm/year. The T1-S1 gain after a given lengthening, however, decreased significantly with repeated lengthenings (P = 0.007). When the effect of time was considered, there was also a significant decrease in T1-S1 gain over time (P = 0.014). Investigators concluded there seems to be a "law of diminishing returns" with repeated lengthenings of dual growing rods. Repeated lengthenings still result in a net T1-S1 increase; however, this gain tends to decrease with each subsequent lengthening and over time. This phenomenon may be due to autofusion of the spine from prolonged immobilization by a rigid device. McElroy et al (2011) evaluated structural effectiveness, complications, and length of hospital stay associated with growing rods (GRs) for scoliosis in spinal muscular atrophy (SMA) and to compare values with those of infantile and juvenile idiopathic scoliosis (IIS/JIS). The reviewers searched a multicenter database and found 15 patients with SMA and scoliosis treated with GRs for 54 ± 33 months. Radiographic measurements, complications, and hospital stay durations were compared with those of 80 GR patients with IIS/JIS observed for 43 ± 31 months. Measures of rib collapse, including T6:T10 mean rib-vertebral angle and T6:T12 thoracic width, were compared. Student t test was used to compare SMA and IIS/JIS values (significance level, P = 0.05). Primary radiographic measurements in patients with SMA improved from preoperative to latest follow-up as follows: curve, 89 ± 19 to 55 ± 17 ; pelvic obliquity, 31 ± 14 to 11 ± 10 ; space-available-for-lung ratio, 0.86 ± 0.15 to 0.94 ± 0.21; and T1-S1 length grew 8.7 ± 3.2 cm. Rib collapse continued despite GR treatment in SMA but not in IIS/JIS. Hospital stays were longer for SMA than for IIS/JIS for lengthening procedures (P = 0.01) and trended to be longer for initial surgery (P = 0.08) and final fusion (P = 0.06). Patients with SMA and IIS/JIS experienced, respectively, 0.5 and 1.1 major complications per patient (P = 0.02). Growing Rods Spinal Surgery Oct 15 13

14 The reviewers concluded GRs improve trunk height and the space-available-for-lung ratio while controlling curve and pelvic obliquity in young patients with SMA with severe scoliosis, but they do not halt rib collapse. For patients with SMA, hospital stays were longer than those for patients with IIS/JIS, whereas the rate of major complications was lower. Yang et al (2011) performed a review of a prospectively collected growing rod database to define risk factors for and characterize the nature of growing rod fractures. Records of 327 patients in a prospectively collected growing rod database were studied. Risk factors studied were studied as patient-related and rod-related. Multivariate analysis was performed. Eighty-six rod fractures occurred in 49 patients (49 of 327, 15%). Sixteen patients had repeat fractures with eight patients having more than two fractures (maximum six). The most common fracture locations were above or below the tandem connectors (34 of 86) and near the thoracolumbar junction (35 of 86). Other locations were adjacent to anchors (12 of 86) and crosslinks (2 of 86). Syndromic diagnoses had the highest rate of fracture; significantly greater than neuromuscular diagnoses (14% vs. 2%, P = 0.01). Patients who were ambulatory had a higher fracture rate (21% vs. 8.7%, P = 0.01). Single rods had a higher fracture rate than dual rods (36% vs. 11%, P < 0.001). Repeat fracture was also more common in patients with single rods (13% vs. 2%, P = ). In dualrod constructs, the incidence of both rods breaking at the same time was 26% (7 of 27). Stainless steel rods had a higher fracture rate than titanium rods (29% vs. 18%, P = 0.02). The nonfracture group had larger diameter rods than the fracture group (P = 0.01). The fracture group had shorter tandem connectors than the nonfracture group (P < 0.001). Neither the size of preoperative scoliosis (P = 0.2) nor kyphosis (P = 0.4) was a risk factor for fracture. Length of instrumentation (P = 0.9), anchor type (P = 0.6), and pelvic fixation (P = 0.38) had no significant effect on fracture rates. Eight wound complications were reported, including three cases of skin breakdown at the rod fracture site. The reviewers concluded risk factors for rod fractures include prior fracture, single rods, stainless steel rods, small diameter rods, proximity to tandem connectors, short tandem connectors, and preoperative ambulation. Repeat fractures are common, especially with single rods. Rod replacement, with larger diameter rods if appropriate, may be a preferred strategy over connecting the broken rods as fractures signal fatigue of the rod. Farooq et al (2010) performed a retrospective clinical and radiologic review of consecutive series of patients treated with single submuscular growing rods from a single center with a minimum of 2-year follow-up. Between 1999 and 2007, 88 patients underwent the insertion of a single, submuscular growing-rod construct for scoliosis. A clinical and radiologic review of these 88 consecutive patients with a minimum of 2-year follow-up was conducted. Diagnoses include idiopathic, neuromuscular, syndromic, and congenital. Data include Cobb angle measurements, T1-S1 heights, number, and frequency of lengthening as well as complications. The patients underwent single submuscular growing-rod insertion at an average age of 7.0 years. The mean follow-up period was 42 months. Twenty-eight patients had a simultaneous apical fusion. Growing-rod lengthening was performed on an average at 9-month intervals. The average initial Cobb angle was 73 (range: ) and improved to 44 (range: 9-90) at final follow-up. T1-S1 height gain was 3.37 cm; this translates to 1.04 cm growth/yr. No significant difference was noted between those who had undergone apical fusion and those without. Complications noted in this series include 8 incidences of superficial infection and 3 of deep infection, proximal junctional kyphosis in 2 patients requiring early fusion, 31 rod fractures, 10 cases of proximal anchor failure, and 6 distal anchor failures. Thirty patients within study group have reached definitive fusion. Growing Rods Spinal Surgery Oct 15 14

15 Scientific Rationale Updated June 2010 Congenital scoliosis has the potential for severe spinal deformity and thoracic insufficiency syndrome (TIS). Conventional fusion treatments in children tend to shorten the spine further exacerbating trunk shortening and TIS. In the surgical treatment of congenital spinal deformities in young children, while reconstructing the spinal deformity, one should simultaneously pursue preserving the growth potential of the vertebrae, improving the volume, symmetry, and functions of the thorax, and protecting this improvement during the growth. The treatment of severe, early onset scoliosis is challenging. The goals of surgery include curve correction, and delay spinal fusion while permitting spinal growth. Currently, there are three systems being proposed for the surgical treatment of severe early-onset scoliosis (EOS): single growing rod, dual growing rods, and the vertical expandable titanium prosthetic rib implant (VEPTR). The (VEPTR), which is not considered a true growing rod system, is proposed to be more effective in the treatment of congenital scoliosis with fused ribs. This is especially beneficial for the youngest patients, whose thoracic insufficiency compromises their ability to breathe and applies significant cardiac pressure. The vertical expandable titanium prosthetic rib implant pushes the ribs apart on the concave side of the curve and may be especially useful. In addition, the VEPTR construct provides the benefit of straightening the spine in all three dimensions while allowing the spine to grow. The other alternative in the growing child is a single or dual growing rod system, which avoids fusing the entire curve, but requires biannual surgery through limited incisions to lengthen the rods and spine. Traditionally, only one growth rod was inserted into young patients with scoliosis, however, researchers are now proposing that dual growing rods are a better approach for correcting spinal deformity and allowing improved growth of the spine. Although the growing rods are novel and promising, these treatments are only suitable for growing patients. All three systems have been proposed for the surgical treatment of severe earlyonset scoliosis and they all have a moderate complication rate, especially rod breakage. Complication rates with VEPTR are usually addressed during routine lengthening procedures. There have been some small retroreview studies (Thompson et al. 2005; Akbarnia et al. 2008; Sponseller 2009 et al.), and reviews by (Yazici et al and Wick et al. 2009), regarding growing rods. Although there was greater growth and correction achieved in the growing rods that were lengthened more frequently, complications were noted (i.e. iliac screws had a higher rate of breakage; complications were noted within the treatment period, postfinal, and during and after treatment). There was a Clinical Trial on Shilla Growth Permitting Spinal Instrumentation System for Treatment of Scoliosis in the Immature Spine that had started in 2007, but was suspended in The Institutional Review Board (IRB) has requested additional information from the Investigational Device Exemption (IDE). The objective of this study was to retrospectively and prospectively review patients who have undergone this technique looking at age of the patient, magnitude of the curve preoperatively, postoperatively and over time, diagnosis, pulmonary function, surgical procedures, complications, and spinal growth. The hypothesis is that Shilla growth permitting Growing Rods Spinal Surgery Oct 15 15

16 spinal instrumentation coupled with a surgical technique of aggressive correction of the apex of the scoliotic curve will allow for natural growth of the spine in a guided fashion with a limited number of future surgeries required. Traditional "growing rod" constructs of spinal instrumentation to treat severe scoliosis in young children require a return to the operating room every six to nine months until skeletal maturity. The Shilla system allows for more spinal growth with fewer surgical procedures necessary for lengthenings. This is a major advantage over existing growth permitting systems and allows surgery to be performed at younger ages with better deformity correction without concerns of repeated surgeries. ClinicalTrials.gov Identifier was NCT Yang et al. (2010) completed a case-based survey regarding growing rod use preferences with early onset scoliosis, which were completed by an international group of surgeons. Two hundred and sixty-five growing rod patients treated over 4.7+/-2.1 years in the Growing Spine Study Group database, who were analyzed to characterize actual practice and compare it with the survey results. All patients had at least 2 years of treatment. There was correlation (P=0.04, r=0.58) between increasing curve size and choice of growing rods over nonoperative treatment, ribbased distraction (vertically expandable prosthetic titanium rib), growth guidance (i.e. Shilla growing rods were used), and primary fusion. In practice, growing rods were used for most types of early onset spine deformity. Most surgeons stated that their indication for growing rod treatment was a curve over 60 degrees (10/13) in a patient younger than 8 to 10 years (14/17). In practice, mean curve at rod insertion was 73+/-20 degrees and age was 6.0+/-2.5 years. Other factors favoring growing rods included curve rigidity (8/17), brace intolerance (6/17) and syndromic diagnoses (2/17). In the database, idiopathic scoliosis represented <50% of diagnoses. The most common preferred surgical lengthening interval was 6 months. In the database, the number of growing rod insertions per year (P=0.02, r=0.96) and percentage of surgeons using dual rods over single rods (P=0.065, r=0.93) increased over time. Insertion age (P=0.075, r=-0.87) and lengthening interval (P=0.006, r=-0.69) decreased as time progressed. The most common stated indication on the survey for final fusion was skeletal maturity (13/17), and 7/13 surgeons used a Risser score of 3 or more. Indications to stop lengthening included complications such as infection or implant failure (14/17), curves progressing past 90 degrees (8/17), and failure to distract (6/13). The most common method of final fusion was replacement of implants with more intermediate anchors. Significant practice variation exists in growing rod treatment, but there is some consensus on indications for surgery including curve size, diagnosis and age, and lengthening intervals and final fusion methods. Mean curve size and lengthening interval are greater in practice than in surgeons' stated aims. In principle and in practice, most growing rods are used for curves over 60 degrees in patients under 10, in all diagnoses. Although growing rods are proposed as a form of growth guidance for patients with early onset scoliosis, no studies exist to characterize their use among a large group of surgeons. The information noted in this 2010 survey may form a starting point as practice variation is studied. In summary, although in small retroreviews and author s reviews, growing rods are deemed to be a reliable method in the treatment of congenital spine deformity of young children who meet a specific criteria, there continues to be a lack of larger, well-designed, controlled, randomized or comparison trials, that demonstrate the long-term safety and effectiveness of this procedure. There are few outcome studies in the published peer-reviewed medical literature to support the efficacy of growing rods in the long-term. Growing Rods Spinal Surgery Oct 15 16

17 Scientific Rationale Updated June 2008 The surgical treatment of severe early-onset scoliosis (EOS) is controversial. Obtaining and maintaining deformity correction, achieving adequate spinal growth, allowing lung development, and the high complication rate make surgical treatment very challenging. Growing rods are the most common method of management, however they have a moderate complication rate, especially rod breakage. The ability to control progressive spinal deformity and children using fusionless spinal instrumentation systems remains a significant challenge for the future. Current methods are fraught with an unacceptable rate of complications, most notably loss of fixation, implant breakage, and device migration. There is a significant need for selfexpanding implants and better methods for fixation to the spine and chest wall, obviating the need for multiple surgeries for expansion of growing instrumentation. Scientific Rationale - Initial The "growing rod" instrumentation is performed without fusion to preserve spinal growth. A hook is implanted at each end of the concave side of the deformity and then linked by a rod that is tunneled subcutaneously, rather than below the muscle. Growing rods control the deformity and gradually straighten the spine while enabling it to grow as a result of periodic surgeries in which doctors lengthen the rods over several years. This type of procedure is considered a more conservative surgical treatment that has been recommended for various types of scoliosis. Initially, the more conservative medical treatments would be initiated. After failing the conservative medical treatments, conservative surgical treatments are attempted if possible. These could include limited fusion (surgery just at the apex of the curvature), a "growing rod" (a rod with cables around the spine but no fusion, with the idea that the spine can grow along the rod like a trolley), or the vertical expandable prosthetic titanium rib (VEPTR) placed vertically between the ribs to keep the chest wall expanded. None of these options are perfect, and each has its own difficulties. As such, they should be discussed at length with the physician relying both on the literature and the doctor s and family s own judgment and experience. The FDA site notes device breakage on 11/2/2005 of the Depuy Acromed spine inc. lengthening growing spinal rod. In this situation, the pt had the device for three months, when the rod fractured. Surgery was performed to remove the broken rod and replace it about four months later. The FDA has still not approved this device and this has been noted as recently as April 27, Scoliosis Scoliosis is a progressive disease causing curvature of the spine, out of the normal plane. A normal spine is straight, or has a curve less than 10 degrees. Mild curvature is from 10 to 20 degrees. Moderate curvature is 20 to 50 degrees. Severe spinal curvature is measured at over 50 degrees. Idiopathic scoliosis is the most common form of spinal curvature. It usually starts in children in the pre-pubescent years. (e.g. Ages ten to twelve are the most common.) The condition can start in younger children and sometimes in early teens. Adult scoliosis usually occurs in older adults, and is most common past middle age. Congenital scoliosis is a birth defect that starts when the child is a fetus. Growing Rods Spinal Surgery Oct 15 17

18 The spinal curvature of scoliosis or kyphosis may cause some difficulties with already compromised pulmonary function. Since the curve is relatively flexible, this can often be compensated by adjusting seating or external support. The child may lean to the side, requiring support with the arms, which can lead to decreased freedom for the upper extremities for function or other daily activities. In attempting to treat the scoliosis, conservative or non-operative methods, such as a brace or a body jacket, could help support the spine. These will almost certainly not prevent development nor halt the progression of the deformity, but will lend support to the trunk and spine of the child to better perform activities of daily living. Care must be taken in judging the tolerance of these measures, including the tendency toward skin breakdown. The goal of this conservative non-surgical treatment will delay the progression of the curvature, allowing the child to grow more. The literature supports the use of a brace after a curve reaches about 20 degrees, but the brace may be helpful for these same aspects well beyond that. Again, one has to watch for the child s tolerance of the brace, which may become somewhat worse as muscular weakness worsens. In summary, bracing or special seating is really used to improve the functional performance of the children, and not to prevent long-term development of scoliosis. In 2004, the U.S. Preventive Services Task Force (USPSTF) has developed recommendations regarding the screening and treatment of idiopathic scoliosis. They include all of the following: 1. The USPSTF recommends against the routine screening of asymptomatic adolescents for idiopathic scoliosis. a. The USPSTF did not find good evidence that screening asymptomatic adolescents detects idiopathic scoliosis at an earlier stage than detection without screening. The accuracy of the most common screening test the forward bending test with or without a scoliometer in identifying adolescents with idiopathic scoliosis is variable, and there is evidence of poor follow-up of adolescents with idiopathic scoliosis who are identified in community screening programs. 2. The USPSTF found fair evidence that treatment of idiopathic scoliosis during adolescence leads to health benefits (decreased pain and disability) in only a small proportion of people. Most cases detected through screening will not progress to a clinically significant form of scoliosis. Scoliosis needing aggressive treatment, such as surgery, is likely to be detected without screening. 3. The USPSTF found fair evidence that treatment of adolescents with idiopathic scoliosis detected through screening leads to moderate harms, including unnecessary brace wear and unnecessary referral for specialty care. As a result, the USPSTF concluded that the harms of screening adolescents for idiopathic scoliosis exceed the potential benefits Growing Rods Spinal Surgery Oct 15 18

19 Spinal Muscular Atrophy Spinal muscular atrophy (SMA) disorders are characterized by degeneration of the anterior horn cells in the spinal cord and motor nuclei in the lower brainstem. These diseases are classified as types 1, 2, or 3 depending upon the age of onset and clinical course. SMA type 1, also known as infantile spinal muscular atrophy or Werdnig-Hoffmann disease, is the most common and severe type. SMA type 1 typically presents in the neonatal period, with symptoms progressing rapidly; severe symmetric flaccid paralysis and inability to sit unsupported could be noted. The majority of these children have previously died before one year of age from respiratory failure. However, as a result of advances in the care of chronic respiratory insufficiency, long term survivors have now been reported. Type 2 (intermediate form) and type 3 (Kugelberg-Welander disease) have a later onset and a less severe course. SMA 2 presents between three and fifteen months of age, whereas SMA 3, the least severe, typically presents with signs of weakness at or after twelve months of age and progresses to a chronic course. Patients with all forms of SMA have diffuse symmetric proximal muscle weakness that is greater in the lower than upper limbs and absent or markedly decreased deep tendon reflexes. All SMA types are associated with a restrictive, progressive respiratory insufficiency, particularly SMA 1. The intercostal muscles typically are more affected than is the diaphragm, resulting in paradoxical breathing (inspiratory efforts cause the rib cage to move inward and the abdomen to move outward) and the development of a characteristic bell-shaped chest deformity. A very high percentage of people with spinal muscular atrophy develop scoliosis, most likely because of a lack of muscular support of the bones of the spinal column or muscular imbalance. The bones, discs and ligaments of the spinal column only have so much rigidity and are meant to be quite pliable. Because of this, when they lack normal muscular support, there s a tendency for them to bend further; either to the side, forward or backward. In spinal muscular atrophy, as the muscular weakness progresses along with growth, there is a tendency for the curvature to develop and, over time, for it to become more severe. Treatment for SMA is supportive and directed at providing nutrition and respiratory support as needed. Physical therapy may be helpful. Spinal bracing may be used to delay the development of progressive scoliosis that is caused by muscle weakness. However, spinal bracing applied to patients with SMA types 1 or 2 while in the sitting position significantly reduces expiratory tidal volume, and thus it should be used cautiously. In addition, the use of long-term ventilator support in patients with SMA 1 is controversial. In children who develop spinal muscular atrophy at a very young age, the curvature will almost always occur by the age of ten. Scoliosis can start as early as eight or nine months of age, but in most studies it was identified between four and six years of age. (In addition, most lung growth also occurs during 4-6 yrs of life). It appears that the longer and later that you watch for curvature, the more often it will be identified. The occurrence ranges from 57 to 95 percent in the orthopedic literature. Whether scoliosis is inevitable over time is uncertain, but it seems probable. There appears to be a relationship between the residual motor function and the development of scoliosis. If left untreated, the scoliosis will most often be progressive. Growing Rods Spinal Surgery Oct 15 19

20 The need and the timing of an operation for scoliosis in spinal muscular atrophy is somewhat controversial. Because almost all cases of spinal deformity in spinal muscular atrophy will progress, despite conservative measures, and the progression of this leads to poorer sitting posture with pulmonary and functional limitations increasing, the early treatment of the spine with fusion is often considered necessary. Some physicians feel that spinal fusion will affect only quality of life and therefore remains a choice. Certainly, if one has chosen to proceed with surgical intervention "at some point", it remains optimal to proceed while the spine is still flexible, while pulmonary function is still adequate, but once sufficient height has been gained. Often times, these three criteria do not occur at the same time. The overall goals of treatment in patients with SMA and scoliosis are to try to maintain a straight spine over a level pelvis to promote maximum comfort, positioning, and ease of care. The short-term goals are to slow progression of the disease, to delay definitive treatment for as long as possible, to try to maximize lung growth and trunk height, and to monitor curve magnitude and flexibility. Surgical control without fusion could be obtained in a number of different ways, including the any of the following: Soft spinal orthosis; or Casting methods (e.g. soft, firm or rigid); or Growing Rod; or Vertical expandable prosthetic titanium rib (VEPTR). Studies on the Growing Rod Results with this single-rod distraction technique can be unpredictable, and there are implant related complications, such as rod breakage and hook dislocation. In an attempt to improve on these results, Akbarnia et al (2003), developed a dual-rod technique. In this method, hooks are placed on both sides of the spine in "claw" patterns over 2 to 3 spinal levels to avoid hook crowding. Pedicle screws can be included in the lower formation and appear to add significantly to the stability of the construct. Rods are inserted subcutaneously on both sides of the spine and are joined together on each side with extended tandem connectors. Initial results from a series of 23 patients, presented at the 2003 meeting of the Scoliosis Research Society, suggested that the dual-rod technique has increased implant stability and fewer complications compared with single-rod systems. This procedure remains investigational, however, because of the lack of well-designed, randomized, controlled clinical trials with adequate follow-up. Akbarnia et al (2004) Further data on 28 patients, presented at the 2004 meeting of the Scoliosis Research Society, compared 3 methods; single rod with fusion (5 patients), single rod without fusion (16 patients), and dual rod (7 patients), concluded that both growing rod methods were effective in controlling severe spinal deformities in young children, but the dual rods offered better initial curve correction as well as maintenance of correction with less implant-related complications. Data from large-scale, well designed controlled or comparison trials are still needed to establish patient selection criteria and demonstrate the safety and efficacy of growing rods. Growing Rods Spinal Surgery Oct 15 20

21 Akbarnia et al (2005) reviewed 38 patients with progressive early onset scoliosis who had primary dual rod constructs utilizing connectors for periodic lengthenings. A two year follow up was completed on 23 patients, with 5-7 years noted as the average surgical age. The average number of lengthenings was 6. The mean scoliosis angle improved from 81 to 38 post-op and 40 at follow-up. T1-S1 length increased from 23.3 cm to 28.6 cm after first surgery (elongation) and to 32.3 cm at follow-up. In addition to initial elongation averaging 5.3 cm, the length increase over the follow-up period was an average of 0.82 cm per year. Seven patients had their final definitive fusion. For these patients a total length of 14 cm was achieved. There were 2 implant related complications. This is the first report of patients treated surgically with dual growing rods with a minimum of 2 years follow-up. The authors presented this report at an annual meeting of the American Academy or Orthopedic Surgeons; they believe that the dual rod technique is safe and effective, provides superior stability, reduces complications and allows continued spinal growth in this challenging patient population. Since only a few, small studies on the growing rods have been completed, this continues to be investigational. Although promising, the technologies regarding the 'Growing Rods' are not yet widely available. Initial recipients of growing rods are just getting old enough to be sure that lengthening the implants twice a year really worked. Studies tracking 275 children for two years or longer suggest the rods straighten as well as fusion but allow the spine and rib cage to grow. The current dilemma is how to lengthen the rods noninvasively. In one closely watched attempt, Arkansas doctors are testing self-lengthening screws to hold the rods in place. Several groups have reported very exciting advances in the field of deformity spine surgery. Updated findings concerning the successful implementation of growing rods have revived this technique as a viable option for preserving near normal growth of the spine. New techniques and instrumentation at the disposal of spine surgeons allow the treatment of this challenging patient population to approach the goals of deformity correction and maintenance with preservation of potential growth. Preliminary outcomes from the different techniques are promising, but further investigation, including long-term follow-up, is necessary. In summary, the growing rod, especially with the dual rod technique, has been shown in small studies to suggest that the rods assist in straightening the spine as well as a fusion, in addition to allowing the spine and ribs to grow. However, device breakage has occurred, resulting in repeat operations. In addition, the 'growing rod' is not FDA approved at the current time. Therefore, more research, and larger, randomized, peer-reviewed studies are necessary, to demonstrate the safety and efficacy of this procedure in the long term. Comparison trials are needed to compare the growing rods to other non-surgical treatments as well as surgical procedures. In this situation, the growing rods are not FDA approved at this time, and are still considered investigational and therefore not medically necessary. Growing Rods Spinal Surgery Oct 15 21

22 Review History June 2007 Medical Advisory Council, Clinical Pharmacy Advisory Committee initial approval June 2008 Update. No revisions. Codes reviewed. June 2010 Update. No revisions. Codes reviewed. June 2011 Update. Added Medicare Table. No revisions. May 2012 Update no revisions May 2013 Update no revisions. Codes updated. May 2014 Update no revisions. May 2015 October 2015 Update no revisions. Update revised policy statement to consider traditional growing rods or magnetically controlled growing rods medically necessary when criteria is met This policy is based on the following evidence-based guidelines: 1. The Journal of Bone and Joint Surgery (American). 2005;87: doi: /jbjs.e What's New in Spine Surgery. 2. National Guideline Clearinghouse (NGC). Screening for idiopathic scoliosis: a brief evidence update for the U.S. Preventive Services Task Force. Rockville (MD); Agency for Healthcare Research and Quality; 2004 Jun. 7 p. 3. National Institute for Health and Care Excellence (NICE). The MAGEC system for spinal lengthening in children with scoliosis. June Available at: References Update October Akgül T, Dikici F, Şar C, et al. Growing rod instrumentation in the treatment of early onset scoliosis. Acta Orthop Belg Dec;80(4): Akbarnia BA, Cheung K, Noordeen H, et al. Next generation of growth-sparing techniques: preliminary clinical results of a magnetically controlled growing rod in 14 patients with early-onset scoliosis. Spine (Phila Pa 1976) Apr 15;38(8): Akbarnia BA, Emans JB. Complications of growth-sparing surgery in early onset scoliosis. Spine (Phila Pa 1976) Dec 1;35(25): Akbarnia BA, Pawelek JB, Cheung KM, et al. Traditional Growing Rods Versus Magnetically Controlled Growing Rods for the Surgical Treatment of Early-Onset Scoliosis: A Case-Matched 2-Year Study. Spine Deformity 2 (2014) Available at: 5. Bess S, Akbarnia BA, Thompson GH, et al. Complications of growing-rod treatment for early-onset scoliosis: analysis of one hundred and forty patients. J Bone Joint Surg Am Nov 3;92(15): Brooks JT, Sponseller PD. What's New in the Management of Neuromuscular Scoliosis. J Pediatr Orthop Apr Caniklioglu M, Gokce A, Ozturkmen Y, et al. Clinical and radiological outcome of the growing rod technique in the management of scoliosis in young children. Acta Orthop Traumatol Turc. 2012;46(5): Chandran S, McCarthy J, Noonan K,et al. Early treatment of scoliosis with growing rods in children with severe spinal muscular atrophy: a preliminary report. J Pediatr Orthop Jun;31(4): Charroin C, Abelin-Genevois K, Cunin V, et al. Direct costs associated with the management of progressive early onset scoliosis: estimations based on gold Growing Rods Spinal Surgery Oct 15 22

23 standard technique or with magnetically controlled growing rods. Orthop Traumatol Surg Res Sep;100(5): Cheung KM, Cheung JP, Samartzis D, et al. Magnetically controlled growing rods for severe spinal curvature in young children: a prospective case series. Lancet May 26;379(9830): Cunin V. Early-onset scoliosis: current treatment. Orthop Traumatol Surg Res Feb;101(1 Suppl):S Dannawi Z, Altaf F, Harshavardhana NS, et al. Early results of a remotelyoperated magnetic growth rod in early-onset scoliosis. Bone Joint J Jan;95-B(1): Dede O, Demirkiran G, Bekmez S, et al. Utilizing the "Stable-to-be Vertebra" Saves Motion Segments in Growing Rods Treatment for Early-Onset Scoliosis. J Pediatr Orthop Apr La Rosa G, Oggiano L, Ruzzini L. Magnetically Controlled Growing Rods for the Management of Early-onset Scoliosis: A Preliminary Report. J Pediatr Orthop Jul Liang J, Li S, Xu D, et al. Risk factors for predicting complications associated with growing rod surgery for early-onset scoliosis. Clin Neurol Neurosurg Sep;136: Matsumoto M, Watanabe K, Hosogane N, Toyama Y. Updates on surgical treatments for pediatric scoliosis. J Orthop Sci Jan;19(1): McElroy MJ, Sponseller PD, Dattilo JR, et al. Growing rods for the treatment of scoliosis in children with cerebral palsy: a critical assessment. Spine (Phila Pa 1976) Nov 15;37(24):E McElroy MJ, Shaner AC, Crawford TO, et al. Growing rods for scoliosis in spinal muscular atrophy: structural effects, complications, and hospital stays. Spine (Phila Pa 1976) Jul 15;36(16): Rolton D, Thakar C, Wilson-MacDonald J, Nnadi C. Radiological and clinical assessment of the distraction achieved with remotely expandable growing rods in early onset scoliosis. Eur Spine J Sep Sánchez Márquez JM, Sánchez Pérez-Grueso FJ, Fernández-Baíllo N, García Fernández A. Growing rods in early-onset scoliosis. Do they really help to control the deformity and spinal and thoracic growth?. Rev Esp Cir Ortop Traumatol May-Jun;57(3): Sankar WN, Acevedo DC, Skaggs DL. Comparison of complications among growing spinal implants. Spine (Phila Pa 1976) Nov 1;35(23): Skaggs DL, Akbarnia BA, Flynn JM, et al. A classification of growth friendly spine implants. Chest Wall and Spine Deformity Study Group; Growing Spine Study Group; Pediatric Orthopaedic Society of North America; Scoliosis Research Society Growing Spine Study Committee. J Pediatr Orthop Apr- May;34(3): Smith JT, Johnston C, Skaggs D, et al. A New Classification System to Report Complications in Growing Spine Surgery: A Multicenter Consensus Study. J Pediatr Orthop Jan Tis JE, Karlin LI, Akbarnia BA, et al. Early onset scoliosis: modern treatment and results. J Pediatr Orthop Oct-Nov;32(7): U.S. Food and Drug Administration. 510 K Summary. MACEC Spinal Bracing and Distraction System. Premarket Notification Number: K Feb Available at: Wang S, Zhang J, Qiu G, et al. Dual growing rods technique for congenital scoliosis: more than 2 years outcomes: preliminary results of a single center. Spine (Phila Pa 1976) Dec 15;37(26):E Growing Rods Spinal Surgery Oct 15 23

24 27. Zhao Y, Qiu GX, Wang YP, et al. Comparison of initial efficacy between single and dual growing rods in treatment of early onset scoliosis. Chin Med J (Engl) Aug;125(16): References Update May Kabirian N, Akbarnia BA, Pawelek JB, et al. Deep Surgical Site Infection Following 2344 Growing-Rod Procedures for Early-Onset Scoliosis: Risk Factors and Clinical Consequences. J Bone Joint Surg Am Aug 6;96(15):e Kamaci S, Demirkiran G, Ismayilov V, et al. The effect of dual growing rod instrumentation on the apical vertebral rotation in early-onset idiopathic scoliosis. J Pediatr Orthop Sep;34(6): Pizones J, Rodríguez-López T, Zúñiga L, et al. Treatment of juvenile scoliosis: Increasing the lengthening interval with the growing rod technique should not necessarily compromise thoracic growth. Rev Esp Cir Ortop Traumatol Sep-Oct;58(5): Ramirez N, Flynn JM, Smith JT, et al. Use of the S-hook for Pelvic Fixation in Rib Based Treatment of Early Onset Scoliosis: A Multicenter Study. Spine (Phila Pa 1976) Jun Shah SA, Karatas AF, Dhawale AA, et al. The effect of serial growing rod lengthening on the sagittal profile and pelvic parameters in early-onset scoliosis. Spine (Phila Pa 1976) Oct 15;39(22):E Stokes OM, O'Donovan EJ, Samartzis D, et al. Reducing radiation exposure in early-onset scoliosis surgery patients: novel use of ultrasonography to measure lengthening in magnetically-controlled growing rods. Spine J Oct 1;14(10): Sun ZJ, Qiu GX, Zhao Y, et al. Dual growing rod treatment in early onset scoliosis: the effect of repeated lengthening surgeries on thoracic growth and dimensions. Eur Spine J Nov Watanabe K, Uno K, Suzuki T, et al. Risk Factors for Proximal Junctional Kyphosis Associated with Dual-Rod Growing-Rod Surgery for Early-Onset Scoliosis. J Spinal Disord Tech Jul 11 References Update May Dede O, Demirkiran G, Yazici M Update on the 'growing spine surgery' for young children with scoliosis. Curr Opin Pediatr Feb;26(1): Flynn JM, Tomlinson LA, Pawelek J, et al. Growing-rod graduates: lessons learned from ninety-nine patients who completed lengthening. J Bone Joint Surg Am Oct 2;95(19): Hickey BA, Towriss C, Baxter G, et al. Early experience of MAGEC magnetic growing rods in the treatment of early onset scoliosis. Eur Spine J Apr;23 Suppl 1: Kamaci S, Demirkiran G, Ismayilov V, et al. The Effect of Dual Growing Rod Instrumentation on the Apical Vertebral Rotation in Early-onset Idiopathic Scoliosis. J Pediatr Orthop Mar Latalski M, Fatyga M, Kołtowski K, et al. Guided-growth implants in the treatment of early onset scoliosis. A pilot study. Ortop Traumatol Rehabil Jan-Feb;15(1): Sánchez Márquez JM, Sánchez Pérez-Grueso FJ, Fernández-Baíllo N, García Fernández A. Growing rods in early-onset scoliosis. Do they really help to control the deformity and spinal and thoracic growth?. Rev Esp Cir Ortop Traumatol May-Jun;57(3): References Update May 2013 Growing Rods Spinal Surgery Oct 15 24

25 1. Canale & Beaty: Campbell's Operative Orthopaedics, 12th ed Mosby, An Imprint of Elsevier. Juvenile Idiopathic Scoliosis. 2. Miller DJ, Franzone JM, Matsumoto H, et al. Electronic monitoring improves brace-wearing compliance in patients with adolescent idiopathic scoliosis: a randomized clinical trial. Spine. 2012; 37: Romano M, Minozzi S, Bettany-Saltikov J, et al. Exercises for adolescent idiopathic scoliosis. Cochrane Database Syst Rev 2012; 8:CD Sanders JO, Newton PO, Browne RH, et al. Bracing in adolescent idiopathic scoliosis, surrogate outcomes, and the number needed to treat. J Pediatr Orthop 2012; 32 Suppl 2:S Watanabe K, Uno K, Suzuki T, et al. Risk Factors for Complications Associated With Growing-Rod Surgery for Early-Onset Scoliosis. Spine Jan 30. References - Update May Farooq N, Garrido E, Altaf F, et al. Minimizing complications with single submuscular growing rods: a review of technique and results on 88 patients with minimum two-year follow-up. Spine (Phila Pa 1976) Dec 1;35(25): McElroy MJ, Shaner AC, Crawford TO, et al. Growing rods for scoliosis in spinal muscular atrophy: structural effects, complications, and hospital stays. Spine (Phila Pa 1976) Jul 15;36(16): Sankar WN, Skaggs DL, Yazici M, et al. Lengthening of dual growing rods and the law of diminishing returns. Spine (Phila Pa 1976) May 1;36(10): Schroerlucke SR, Akbarnia BA, Pawelek JB, et al. How Does Thoracic Kyphosis Affect Patient Outcomes in Growing Rod Surgery? Spine (Phila Pa 1976) Dec White KK, Song KM, Frost N, Daines BK. VEPTR growing rods for early-onset neuromuscular scoliosis: feasible and effective. Clin Orthop Relat Res May;469(5): Yang JS, Sponseller PD, Thompson GH, et al. Growing rod fractures: risk factors and opportunities for prevention Spine (Phila Pa 1976) Sep 15;36(20): References Update June Elsebai HB. Safety and efficacy of growing rod technique for pediatric congenital spinal deformities. J Pediatr Orthop. 01-JAN-2011; 31(1): Scheryl SA. Treatment and prognosis of adolescent idiopathic scoliosis. October 13, Updated October 22, Mason: Murray and Nadel's Textbook of Respiratory Medicine, 5th ed Saunders, An Imprint of Elsevier. Kyphoscoliosis. Diagnosis and Etiology. References Update June Yang JS, McElroy MJ, Akbarnia BA, et al. Growing rods for spinal deformity: characterizing consensus and variation in current use. J Pediatr Orthop Apr-May;30(3): Muharrem Y, Emans J. Fusionless Instrumentation Systems for Congenital Scoliosis: Expandable Spinal Rods and Vertical Expandable Prosthetic Titanium Rib in the Management of Congenital Spine Deformities in the Growing Child. Spine: 1 August Volume 34 - Issue 17 - pp Growing Rods Spinal Surgery Oct 15 25

26 3. Akbarnia BA, Breakwell LM, Marks DS, et al. Dual growing rod technique followed for three to eleven years until final fusion: the effect of frequency of lengthening. Spine 33: , Yazici M Emans J. Fusionless instrumentation systems for congenital scoliosis: expandable spinal rods and vertical expandable prosthetic titanium rib in the management of congenital spine deformities in the growing child. Spine (Phila Pa 1976) Aug 1;34(17): Clinical Trials.gov. Shilla Growth Permitting Spinal Instrumentation System for Treatment of Scoliosis in the Immature Spine ClinicalTrials.gov Identifier: NCT Available at: =1 6. Wick JM, Konze J, Alexander K, et al. Infantile and juvenile scoliosis: the crooked path to diagnosis and treatment. AORN J Sep; 90 (3): Sponseller PD, Yang JS, Thompson GH, et al. Pelvic fixation of growing rods: comparison of constructs. Spine (Phila Pa 1976) Jul 15; 34 (16): Hedequist DJ. Surgical Treatment for Congenital Scoliosis. Orthopedic Clinics of America - Volume 38, Issue 4 (October 2007). 9. Thompson GH. Comparison of single and dual growing rod techniques followed through definitive surgery: a preliminary study. Spine (Phila Pa 1976) 15-SEP- 2005; 30(18): References Update June Thompson G, Akbarnia BA, Campbell RM. Growing Rod Techniques in Early- Onset Scoliosis. Journal of Pediatric Orthopaedics. 27(3): , April/May Juvenile Idiopathic Scoliosis. Canale & Beaty: Campbell's Operative Orthopaedics, 11th ed Smith JT. The Use of Growth-Sparing Instrumentation in Pediatric Spinal Deformity. Orthopedic Clinics of North America - Volume 38, Issue 4 (October 2007). 4. Brewer M, Zhang T, Dong W, et al. Future Approaches of Nanomedicine in Clinical Science. Medical Clinics of North America - Volume 91, Issue 5 (September 2007) References Initial 1. Campbell R, Smith M. Thoracic Insufficiency Syndrome and Exotic Scoliosis. The Journal of Bone and Joint Surgery (American). 2007;89: doi: /jbjs.f Sucato D, Kim Y. What's New in Pediatric Orthopaedics. The Journal of Bone and Joint Surgery (American). 2007;89: doi: /jbjs.f Haefeli M, Elfering A, Kilian R, et al. Nonoperative treatment for adolescent idiopathic scoliosis: a 10- to 60-year follow-up with special reference to healthrelated quality of life. Spine 2006; 31: Andersen MO, Christensen SB, Thomsen K. Outcome at 10 years after treatment for adolescent idiopathic scoliosis. Spine 2006; 31: Coe JD, Arlet V, Donaldson W, et al. Complications in spinal fusion for adolescent idiopathic scoliosis in the new millennium. A report of the Scoliosis Research Society Morbidity and Mortality Committee. Spine 2006; 31: Newton PO, Wenger DR. Idiopathic scoliosis. In: Lovell and Winter's Pediatric Orthopaedics, 6th ed, Morrissy, RT, Weinstein, SL (Eds), p Growing Rods Spinal Surgery Oct 15 26

27 7. Sponseller PD. Bone, joint, and muscle problems. In: Oski's Pediatrics. Principles and Practice, 4th ed, p Lonstein JE. Scoliosis: surgical versus nonsurgical treatment. Clin Orthop Relat Res 2006; 443: Spiegel D, Walker J. Early Treatment of Scoliosis in Spinal Muscular Atrophy O'Neill PJ, Karol LA, Shindle MK, et al. Decreased orthotic effectiveness in overweight patients with adolescent idiopathic scoliosis. J Bone Joint Surg Am 2005; 87: Lenssinck ML, Frijlink AC, Berger MY, et al. Effect of bracing and other conservative interventions in the treatment of idiopathic scoliosis in adolescents: a systematic review of clinical trials. Phys Ther 2005; 85: Akbarnia B. Dual Growing Rods for Treatment of Progressive Early Onset Scoliosis: A Multicenter Study. Paper No: 286. February 25, The American Academy of Orthopedic Surgeons. 13. Cunningham M, Frelinghuysen P, Roh H, et al. Comparison of Single and Dual Growing Rod Techniques Followed Through Definitive Surgery. Scoliosis Research Society U.S. Food and Drug Administration. (FDA). Depuy Spine Inc. Depuy Acromed Lengthening Growing Spinal Rod. Available at: I ID= Weinstein SL, Dolan LA, Spratt KF, et al. Health and function of patients with untreated idiopathic scoliosis: a 50-year natural history study. JAMA 2003; 289: Nicholson GP, Ferguson-Pell MW, Smith K, et al. The objective measurement of spinal orthosis use for the treatment of adolescent idiopathic scoliosis. Spine 2003; 28: Rigo M, Reiter Ch, Weiss HR. Effect of conservative management on the prevalence of surgery in patients with adolescent idiopathic scoliosis. Pediatr Rehabil 2003; 6: Sponseller, PD. Sizing up scoliosis. JAMA 2003; 289: Grayhack J. The Hidden Twist Scoliosis. May 29, Merola AA, Haher TR, Brkaric M, et al. A multicenter study of the outcomes of the surgical treatment of adolescent idiopathic scoliosis using the Scoliosis Research Society (SRS) outcome instrument. Spine 2002; 27: Rowe DE. Results of Charleston Bracing in skeletally immature patients with idiopathic scoliosis. J Pediatr Orthop. 2002; 22(4): Karol LA. Effectiveness of bracing in male patients with idiopathic scoliosis. Spine 2001; 26: Danielsson AJ, Nachemson AL. Radiologic findings and curve progression 22 years after treatment for adolescent idiopathic scoliosis: comparison of brace and surgical treatment with matching control group of straight individuals. Spine 2001; 26: Scoliosis. In: Essentials of Musculoskeletal Care, 2nd ed, Greene, American Academy of Orthopedic Surgeons, Rosemont, IL p Reamy BV. Adolescent idiopathic scoliosis: review and current concepts. Am Fam Physician 2001; 64: Winter RB. Expert Editorial:.Bracing for Scoliosis: Where do we go now? J Prosthet Orthot. 2000;12(1):2-4. General Purpose. Important Notice Growing Rods Spinal Surgery Oct 15 27

28 Health Net's National Medical Policies (the "Policies") are developed to assist Health Net in administering plan benefits and determining whether a particular procedure, drug, service or supply is medically necessary. The Policies are based upon a review of the available clinical information including clinical outcome studies in the peer-reviewed published medical literature, regulatory status of the drug or device, evidence-based guidelines of governmental bodies, and evidence-based guidelines and positions of select national health professional organizations. Coverage determinations are made on a case-by-case basis and are subject to all of the terms, conditions, limitations, and exclusions of the member's contract, including medical necessity requirements. Health Net may use the Policies to determine whether under the facts and circumstances of a particular case, the proposed procedure, drug, service or supply is medically necessary. The conclusion that a procedure, drug, service or supply is medically necessary does not constitute coverage. The member's contract defines which procedure, drug, service or supply is covered, excluded, limited, or subject to dollar caps. The policy provides for clearly written, reasonable and current criteria that have been approved by Health Net s National Medical Advisory Council (MAC). The clinical criteria and medical policies provide guidelines for determining the medical necessity criteria for specific procedures, equipment, and services. In order to be eligible, all services must be medically necessary and otherwise defined in the member's benefits contract as described this "Important Notice" disclaimer. In all cases, final benefit determinations are based on the applicable contract language. To the extent there are any conflicts between medical policy guidelines and applicable contract language, the contract language prevails. Medical policy is not intended to override the policy that defines the member s benefits, nor is it intended to dictate to providers how to practice medicine. Policy Effective Date and Defined Terms. The date of posting is not the effective date of the Policy. The Policy is effective as of the date determined by Health Net. All policies are subject to applicable legal and regulatory mandates and requirements for prior notification. If there is a discrepancy between the policy effective date and legal mandates and regulatory requirements, the requirements of law and regulation shall govern. * In some states, prior notice or posting on the website is required before a policy is deemed effective. For information regarding the effective dates of Policies, contact your provider representative. The Policies do not include definitions. All terms are defined by Health Net. For information regarding the definitions of terms used in the Policies, contact your provider representative. Policy Amendment without Notice. Health Net reserves the right to amend the Policies without notice to providers or Members. In some states, prior notice or website posting is required before an amendment is deemed effective. No Medical Advice. The Policies do not constitute medical advice. Health Net does not provide or recommend treatment to members. Members should consult with their treating physician in connection with diagnosis and treatment decisions. No Authorization or Guarantee of Coverage. The Policies do not constitute authorization or guarantee of coverage of particular procedure, drug, service or supply. Members and providers should refer to the Member contract to determine if exclusions, limitations, and dollar caps apply to a particular procedure, drug, service or supply. Policy Limitation: Member s Contract Controls Coverage Determinations. Statutory Notice to Members: The materials provided to you are guidelines used by this plan to authorize, modify, or deny care for persons with similar illnesses or conditions. Specific care and treatment may vary depending on individual need and the benefits covered under your contract. The determination of coverage for a particular procedure, drug, service or supply is not based upon the Policies, but rather is subject to the facts of the individual clinical case, terms and conditions of the member s contract, and requirements of applicable laws and regulations. The contract language contains specific terms and conditions, including pre-existing conditions, limitations, exclusions, benefit maximums, eligibility, and other relevant terms and conditions of coverage. In the event the Member s contract (also known as the benefit contract, coverage document, or evidence of coverage) conflicts with the Policies, the Member s contract shall govern. The Policies do not replace or amend the Member s contract. Policy Limitation: Legal and Regulatory Mandates and Requirements The determinations of coverage for a particular procedure, drug, service or supply is subject to applicable legal and regulatory mandates and requirements. If there is a discrepancy between the Policies and legal mandates and regulatory requirements, the requirements of law and regulation shall govern. Reconstructive Surgery CA Health and Safety Code requires health care service plans to cover reconstructive surgery. Reconstructive surgery means surgery performed to correct or repair abnormal structures of the body Growing Rods Spinal Surgery Oct 15 28

29 caused by congenital defects, developmental abnormalities, trauma, infection, tumors, or disease to do either of the following: (1) To improve function or (2) To create a normal appearance, to the extent possible. Reconstructive surgery does not mean cosmetic surgery," which is surgery performed to alter or reshape normal structures of the body in order to improve appearance. Requests for reconstructive surgery may be denied, if the proposed procedure offers only a minimal improvement in the appearance of the enrollee, in accordance with the standard of care as practiced by physicians specializing in reconstructive surgery. Reconstructive Surgery after Mastectomy California Health and Safety Code requires treatment for breast cancer to cover prosthetic devices or reconstructive surgery to restore and achieve symmetry for the patient incident to a mastectomy. Coverage for prosthetic devices and reconstructive surgery shall be subject to the co-payment, or deductible and coinsurance conditions, that are applicable to the mastectomy and all other terms and conditions applicable to other benefits. "Mastectomy" means the removal of all or part of the breast for medically necessary reasons, as determined by a licensed physician and surgeon. Policy Limitations: Medicare and Medicaid Policies specifically developed to assist Health Net in administering Medicare or Medicaid plan benefits and determining coverage for a particular procedure, drug, service or supply for Medicare or Medicaid members shall not be construed to apply to any other Health Net plans and members. The Policies shall not be interpreted to limit the benefits afforded Medicare and Medicaid members by law and regulation. Growing Rods Spinal Surgery Oct 15 29