Early Onset Scoliosis: Defining the Extent of the Problem and Non Operative Treatment

Transcription

1 12/15/2015 vumedi Webinar: Latest Advances in the Treatment of Early Onset Scoliosis Early Onset Scoliosis: Defining the Extent of the Problem and Non Operative Treatment Suken A. Shah, MD Division Chief, Spine & Scoliosis Center Nemours/Alfred I. dupont Hospital for Children Wilmington, Delaware USA Associate Professor of Orthopaedic Surgery Thomas Jefferson University Early Onset Scoliosis Scoliosis onset before age 5 Continues to be challenging Natural history Progressive thoracic deformity Pulmonary compromise Traditional methods Not ideal deleterious effects Growing spine, chest wall, lungs Early Onset Scoliosis Scoliosis onset before age 5 Continues to be challenging Natural history Progressive thoracic deformity Pulmonary compromise Traditional methods Not ideal deleterious effects Growing spine, chest wall, lungs 1

2 12/15/2015 Early Onset Scoliosis Infantile Idiopathic Congenital Neuromuscular Syndromic Other Syndromic Early Onset Scoliosis Marfan Gene encoding fibrillin-1 (15) Autosomal dominant 25% incidence of spont mutations Larsen AD, some sporadic Arthrogryposis Heterogeneous, sporadic Neurofibromatosis - 1 Goldenhaar Jeune Spinal Growth Velocity 2

3 12/15/2015 T1-S1 Boys cm Birth 5 yr 10 yr Adult Gain Birth to 5 10 cm 5 to 10 5 cm 10 to cm A peri-vertebral arthrodesis in the T1-S1 segment at 5 years of age causes a sitting height deficit of 15 cm (T1 T12 =10 cm; L1 L5= 5 cm) Fusion formula: 0.07 cm x # fused levels x yrs of growth remaining Sanders: Spinal growth (T1-S1) is very rapid during the growth spurt Girls Boys Childhood 1.5cm/year 1.5cm/year Growth Spurt 2.5cm/year 2.5cm/year Terminal Growth 0.4cm/year 0.4cm/year Higher than reported with prior cross sectional studies Calculating Growth If you assume individual lumbar segments grow similarly and thoracic segments also grow similarly: Childhood Growth: 1.1mm/seg/yr lumbar 0.7 mm/seg/yr thoracic Adolescent Growth: 1.9 mm/seg/yr lumbar 1.3mm/seg/yr thoracic Lumbar 38% Thoracic 628% Courtesy of James Sanders MD 3

4 12/15/2015 Volumetric Growth The thorax: the fourth dimension of the spine 100% 50% 6% 30% New born 5 years 10 years 15 years The Growing Spine, Springer Verlag 1990 Pulmonary Development Alveoli 20 million at birth 250 million at 4 years Adult size at the age 8 Airways Develop before birth Pulmonary vascular tree The arteries develop to the size of the lung and thorax, not to the patient s age Early Onset Scoliosis The Concern: Life threatening health risks exist when significant curves develop before age 5 years. Thoracic insufficiency Spinal, chest wall growth 4

5 12/15/2015 Natural History / Death Rate Pehrsson, Larssson, Oden, Nachemson, Spine, 1992 Thoracic Insufficiency Syndrome (TIS) Natural History / Death Rate Pehrsson, Larssson, Oden, Nachemson, Spine,

6 12/15/2015 Natural History of EOS Vitale J Pediatr Orthop 2008 Vitale Spine 2008 EOS pts have a poor quality of life TIS, asthma, JRA, cong. heart disease Early fusion patients have Poor PFTs Shorter spines More pain than unfused Poor quality of life Goals of Treatment in EOS Control curve progression Optimize pulmonary function Maintain spinal growth Limit complications Facilitate care of the patient Improve quality of life Mechanical Strategies Non-Operative Treatment Distract posteriorly -Growing Rods, VEPTR Guide growth along rods -Luque trolley, Shilla procedure Slow convex anterior growth -Stapling, Tethering, NCS growth arrest 6

7 12/15/2015 Dangers of Ignoring Growth Goldberg, et al Spine 2003 fusion before age 8 leads to decreased PFT s FVC 40% pred Emans, et al SRS 2005, IMAST 2007 fusion before age 5, more than 4 segments PFT s 60% predicted vs. normals results worse if T1-T6 Day, et al Spine 1994 Congenital scoli pts who were fused had lower FVC than non surgical congenital patients J Bone Joint Surg Am patients, mixed dx (20 congenitals) 3.3 yrs at surgery, 14.6 yrs at follow up FVC 57.8% of normal, FEV % of normal Correlates: Extent of spine fused Proximal thoracic fusions to T1 or T2 Fusion vs. FVC Karol, et al, JBJS Am

8 12/15/2015 Proximal Level vs. FVC Karol, et al, JBJS Am 2008 Thoracic Height vs. FVC Karol, et al, JBJS Am 2008 Ramirez et al, JBJS Am

9 12/15/2015 Previous PSF for Infantile Scoliosis Fusion before age 8 Eliminates most truncal and spinal growth Thoracic insufficiency syndrome Crankshaft phenomenon, deformity recurrence Congenital scoliosis Resection procedure, short fusion Syndromic scoliosis with known poor natural history (SMA) Rigid segmental instrumentation Epidemiology IIS IIS comprises less than 1% of idiopathic cases Varies by continent / societal practices More common in males ( 3 : 2 ) Tend to be L-sided curves (75 90%) High rate of spontaneous resolution (~ 90%) Females with R-sided thoracic IIS do have a worse prognosis 9

10 12/15/2015 History & Physical Examination Goal eliminate associated conditions from potential diagnoses History: Prenatal History ( mother s health, previous pregnancies, & medications ) Birth History ( length of gestation, type of delivery, birth weight, and complications ) Developmental History ( is child reaching appropriate cognitive and motor milestones? ) History & Physical Examination Physical Examination: Skin café au lait spots, axillary freckles NF midline patches of hair spinal dysraphysm bruising trauma Spine Adam s Forward Bend Test Curve Flexibility Abdominal Reflexes (if abnormal, consider MRI) Notation of chest of flank asymmetry and expansion History & Physical Examination Head Check for plagiocephaly, bat-ear deformity, & congenital muscular torticollis Pelvis plagiopelvy, developmental hip dysplasia Extremities Limb-length inequality 10

11 12/15/2015 Radiographic Evaluation PA & Lat x-rays of the ENTIRE spine Include Cervical spine and Pelvis In children too young to stand, x-rays should be taken supine Radiographic Measurements Scoliosis Cobb Angle Rib-Vertebral Angle Difference (RVAD) Space Available for Lung (SAL) T1-S1 height MRI Scan 20 % incidence of neural axis abnormalities Rib-Vertebral Angle Difference (RVAD-Mehta) RVAD = (Concave Angle Convex Angle) RVAD > 20 indicates progression Rib Phase Relationship Overlap of rib head on vertebral body is indicative of curve progression 11

12 12/15/2015 Mehta s Recommendations for IIS RVAD < 20 likely to RESOLVE Cobb Angle < 25 & RVAD < 20 LOW RISK FOR PROGRESSION initiate treatment if > 10 of progression occurs RVAD > 20 or Phase II & Cobb Angle likely to PROGRESS Nonoperative Treatment for EOS Observation Phase I and RVAD < 20 Follow until resolution and adolescent growth spurt Bracing Casting Age 12 months 12

13 12/15/2015 Age 23 months Clinical Photos Clinical Photos 13

14 12/15/2015 Casting Casting Historical aspects Sayre 1876 Hibbs Turnbuckle cast Risser Localizer cast 1946 EDF cast Cotrel 1950 s Elongation, derotation, flexion Stagnara Turnbuckle cast with occipito-mandibular component mid 1980 s Casting - Biomechanics Horizontal forces (ribs, erector spinae musc.) Longitudinal force (pelvic girdle & occipitomandibular) Abdominal compression ( axial compression strength of spine) Materials Plaster: unsurpassed moldability Fiberglass: over wrap for strength 14

15 12/15/2015 Prospective study of 136 children with progressive IIS treated under 4 yrs, f/u 9 yrs. Scoliosis can be reversed by harnessing the vigorous growth of the infant by serial corrective casts. 94 patients referred & treated early, scoliosis resolved with casting (avg age 1.6 yrs, Cobb 32 ) 42 patients referred late, casting could reduce, not reverse the deformity (avg age 2.5 yrs, Cobb 52 ) 15

16 12/15/2015 Courtesy Jacques d Astous MD 16

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19 12/15/2015 Infantile Idiopathic Scoliosis 15 months Lt T8-L1 41 RVAD 41 Rib Phase 2 Infantile Idiopathic Scoliosis Brace 15 months 25 months 4 y.o. Infantile Idiopathic Scoliosis 8 months Lt T6-L2 52 RVAD 40º Rib Phase 2 19

20 12/15/2015 Infantile Idiopathic Scoliosis Cast 8 months 11 months 21 months 2 yof PWS 2 yof IIS 20

21 12/15/2015 Results of Casting Sanders J, D Astous J, J Pediatr Orthop 2009 Best results in less than 20 mos, 60 degrees Baulesh DM, J Pediatr Orthop 2012 Fletcher ND, J Pediatr Orthop 2012 Delay tactic, increased thoracic height Dhawale A, Shah SA, J Pediatr Orthop 2013 Increased PIP anesthesia issue Demirkiran HG, J Pediatr Orthop 2015 Effective even in congenitals to delay operative tx New Data Suggests Benefit in Delaying Surgery Decrease in complications in older children Better soft tissue Larger implants Weight gains seen only in children > 4 years old Average length gains diminish over time Force to distract increases over time Complications vs. risks of delaying surgery Management Trends in Early Onset Scoliosis Delay surgery with casting Later implantation is better Nutritional and height gains Increase T1-T12 length to improve pulm fcn Complications reduced with dual rods Decrease number of surgeries for lengthening Shilla, trolley Magnetically controlled growth rods 21

22 12/15/2015 Psychosocial Effects of Repetitive Surgeries in Children with Early Onset Scoliosis Methods Instrument used Child Behavior Checklist (a parent-report instrument) Strength and Difficulties Questionnaire (a parent report behavioural screening questionnaire) Care Giver Support Abnormal psychosocial scores observed in patients with EOS. The at risk patients are younger at the time of their initial scoliosis surgery and the number of repetitive surgeries. Evidence: Level III (Matsumoto, Williams et al. 2013) Issues with Repetitive Anesthesia Children repeatedly exposed to procedures requiring general anesthesia before age 2 years are at increased risk for the later development of ADHD even after adjusting for comorbidities. Drive Growth Predetermined interval Follow Growth Lengthening DiMeglio tables, skeletal age, height changes Sanders data How Often? 1 month, 3 months, 6 months How Much? How often should I X-ray my patient? 22

23 12/15/2015 Thank You Nemours Spine and Scoliosis Center 23

24 12/7/2015 SHILLA Growth Guidance for EOS Richard E McCarthy MD Professor University of Arkansas for Medical Sciences Department of Orthopaedics and Neurosurgery Little Rock, Arkansas December 2015 Disclosures Medtronic Consultant, teaching faculty, royalties Editorial Reviewer for Spinal Deformity and JPO Past President SRS CONCEPT Shilla 1) The apex is the center of maximal deformity VS 1

25 12/7/2015 Principles 2) Children have a natural rate and pattern of growth in the spine 3) Horizontal apophyses ( in large curves) will add to spinal height when directed vertically Method of Correction Step 1 Release apex Step2 Move apex to midline Step3 Correct sagittal plane Step4 Derotate apex Patient Example 3 yrs old Neuromuscular Blueprint 2 mo postop 2

26 12/7/2015 Shilla Procedure: depends upon Guidance of spinal growth Essentials: Correction of the apex to neutral Maintenance of correction with dual rods Using spinal growth as the engine Use of growing screws to slide along rods at the top and bottom Allow for guided growth Growing Screws Motion at : rod/screw head and screw head/post Gap for motion and sliding of rod Fixed Multiaxial Growing Screws Multiaxial screws capture rods without binding to the rod allowing the rod to slide Rod fixed at apex Leave rods 2 cm long each end 3

27 12/7/2015 Growing Screws APEX Fascial incision 1cm off midline Ponte Osteotomies Between apical levels 4

28 12/7/2015 Maximal Screw Fixation Correction Procedure Convex side Provisional Rod use a temporary rod on the convex side through the heads of most of the Shilla screws and into all the apical ones Correction Procedure Convex side provisional rod In situ bending Objective: to align coronal and sagittal plane and deliver the concave screws to the definitive rod 5

29 12/7/2015 Correction Procedure Derotation Place one rod forceps on each rod. * Someone must hold them during the derotation procedure. Correction Two permanent rods of equal length are placed 2-3 cm long a Cross link placed just below the apex to control the tendency of rods to spring back In child less than 5 yrs old, use sliding crosslink to allow growth of canal diameter What have we learned about SHILLA in the first 10yrs? 2 ½ yrs old 86⁰ 7 yrs postop 10 yrs posto p 1 revision Final after definitive fusion 14yo 6

30 12/7/ yrs postop 3 yrs old 80⁰ 9 1/2 yrs postop 2 revisions Simple reality: Fixed rods in a mobile child eventually break 3.5 mm 2-3 yrs 4.5 mm 4-5 yrs 5.5 mm 6 yrs or more Metallosis is a reality in growing rods Micro 7

31 12/7/2015 Rigid Pelvic Obliquity Fix to pelvis with firm fixation and Sliding Domino between Double Major Curves treated in similar manner What to do at maturity? Recommendations for the future: if residual deformity exists then correct with final fusion and instrumentation if acceptable remove metal alone 4 yr Pre-op 7 yrs old Post growth guidance Pre definitive fusion Post op definitive fusion 8

32 12/7/2015 Results at 5 year follow-up 40 pts. with variety of diagnoses 33 with long term follow up Age at index procedure: 6+11 yrs (23 moto yrs Follow-up: 6 yrs (5 to 10 yrs) Preoperative curves: 68.5 (41 to 116 ) Curve at last follow-up: 44 (16-74 ) Curve after definitive procedure: 14 to 49 PJK: 3 pts. (2 have not had definitive procedure Results at 5 year follow up SAL: Increased overall 34.1% PFT s in graduates able to complete (6) Mean FVC 67% Mean FEV1 62% All but 1 SMA pt. had FVC and FEV1 >50% Predicted No neurologic complications 9

33 12/7/2015 Results at 5 Year Follow up 73% Reduction in # of Operations compared to distraction methods 47 implant related complications Screw pullout (9), broken cables (1), rod prominence (14), growth off the rods (5), or rod fracture (18) Results at 5 year follow-up Complications (any return to the OR) Secondary infections 6 pts. Alignment issues 8 pts. Implant related 24 pts. Complications in Initial 40 Pts We recognize the effect of the learning curve in developing a new surgical technique However- 27% No complications No return to hospital 10

34 12/7/2015 Patient Example 4yo 75degree 31 Post op 6mo 24mo 32 Patient A 28mo 35mo 36 mo post index 33 11

35 12/7/ yrs post index 6 yrs post index 10+8 yrs of age yrs of age 6+7 yrs post index with 1 revision 4yo 75degree yo 35 12

36 12/14/2015 EARLY ONSET SCOLIOSIS: MAGNETICALLY CONTROLLED GROWING RODS Gregory M Mundis Jr., MD San Diego Spine Foundation Scripps Clinic Medical Group 5 th Annual XLIF User Group Meeting Rady Children s Hospital March 21-22, 2015 Melbourne, AUS December 15, 2015 VuMedi EOS Webinar DISCLOSURES CONSULTING: 1. Ellipse 2. Nuvasive 3. Medicrea 4. Misonix 5. K2M Royalties: 1. Nuvasive 2. K2M Research Support 1. Nuvasive 2. DePuy 3. ISSGF Early Onset Scoliosis Team- San Diego La Jolla, California Rady Children s Hospital EOS Program

37 12/14/2015 Early Onset Spinal Deformity The big challenge we face is how to correct the TREATING VERY keep the spine mobile! YOUNG CHILDREN deformity and WITH EOS maintain growth of: REMAINS -spine -thorax CHALLENGING -lung Growth modulation with current Growing Rod (GR) techniques require frequent surgical lengthenings and are associated with high risk of complications The complication risk increased by 24% for each additional surgical procedure. JBJS, December 2010 Goal of remotely controlled devices: To reduce frequency of surgeries 7 mm of remote distraction was performed weekly for 7 weeks in experimental group (EG) under sedation Implants were removed at week #7 Animals were sacrificed 3 weeks after implant removal The MCGR was safely implanted. EG had significantly more spinal growth than the Sham group( P < 0.05) No complications were associated with the MCGR. The MCGR, shown to provide 80% of predicted spinal growth by noninvasive remote distraction 2

38 12/14/2015 SURGICAL CHALLENGES FOR MCGR PRE-OP PLANNING Classification C-EOS (etiology) Sagittal alignment Patient size Family commitment EOSQ-24 Behrooz A. Akbarnia, M.D., Kenneth Cheung, M.D., Hilali Noordeen, FRCS, Hazem Elsebaie, M.D., Muharrem Yazici, M.D.,Zaher Dannawi, FRCS., and Nima Kabirian, M.D. Annual Meeting of Pediatric Orthopaedic Society of North America (POSNA) May, 17-19, 2012 Denver, Colorado, USA There were no major implant-related complication Dual rod patients showed better initial correction of coronal deformity (p>0.05) and better monthly height increase of T1-T12 (p>0.05) and T1-S1 (p<0.05) CONCLUSION 3

39 12/14/2015 OTHER STUDIES Early clinical results of MCGR: - Safe and effective - Significant reduction in the number of surgical procedures TRADITIONAL GROWING RODS VERSUS MAGNETICALLY CONTROLLED GROWING RODS IN EARLY ONSET SCOLIOSIS: A CASE-MATCHED TWO YEAR STUDY B. A. Akbarnia, K. Cheung, G. Demirkiran, H. Elsebaie, J. Emans, C. Johnston, G. Mundis, H. Noordeen, J. Pawelek M. Shaw, D. Skaggs, P. Sponseller, G. Thompson, M. Yazici, Growing Spine Study Group The purpose of this study was to perform a case-matched comparison of MCGR and TGR patients with 2 years of follow-up TGR MCGR vs. TGR MCGR 4

40 12/14/2015 METHODS Retrospective review of MCGR patients who met the following criteria: - < 10 years old - Major curve >30º - T1-T12 <22 cm - No previous spine surgery - > 2-year follow-up 17 MCGR patients met the inclusion criteria 12 of 17 patients had complete data available for analysis T1-S1 Growth Spinal growth calculation: Annual T1-S1 Growth Annual T1-S1 Growth (mm/year) = Δ in T1-S1 from post index to latest F/U Length of follow-up RESULTS MCGR patients: - Mean age = 6.8 years - Mean follow-up = 2.5 years Follow-up was greater for TGR patients by 1.6 years Distribution of etiologies: - 4 neuromuscular - 4 syndromic - 3 idiopathic - 1 congenital 5

41 12/14/2015 RESULTS Pre-op (mean) Initial Post-op (mean) >2 YR Post-op (mean) Major Curve T1-S1 Spinal Length NO STATISTICAL DIFFERENCES MCGR 59 43% 32-25% 38 35% TGR 60 47% 31-27% 41 32% MCGR 270 mm Δ mm Δ mm TGR 264 mm Δ mm Δ mm RESULTS Overall curve correction was similar between MCGR (35%) and TGR (32%) throughout treatment Mean T1-S1 increase after index surgery was greater in TGR compared to MCGR Annual T1-S1 growth was 7.1 mm/year for MCGR and 10.6 mm/year for TGR patients RESULTS (Procedures) Total # of Surgeries Total # of Lengthenings Total # of Revisions MCGR TGR (42% of patients) 8 (67% of patients) 6

42 12/14/2015 In this small yet carefully matched series, major curve correction was similar between MCGR and TGR patients throughout treatment MCGR patients had 52 fewer surgical procedures than TGR patients While curve correction was similar, annual T1-S1 growth was 3.5 mm/year greater in TGR patients compared to MCGR patients CONCLUSION Incidence of unplanned surgery was similar MCGR patients had 57 fewer surgical procedures than TGR patients MAGNETICALLY CONTROLLED GROWING RODS FOR EARLY ONSET SCOLIOSIS: A MULTICENTER STUDY OF 23 CASES WITH MINIMUM 2 YEARS FDA approval required stringent data collection FOLLOW-UP This study reported the subset of patients with 2 year data INCLUSION 1. diagnosis of EOS of any etiology; years and younger at time of index surgery; 3. pre-op major curve size >30 ; 4. pre-op thoracic spine height <22 cm. 54 patients were enrolled in the study 7

43 12/14/2015 METHODS 54 patients enrolled at 15 sites 23 patients had 2-year follow-up with complete data Patients were evaluated at baseline, 6, 12, 18, and 24 months following the MCGR procedure. Both de novo and conversion cases were included RESULTS/CONCLUSION De Novo implantation had more improvement than conversion at baseline and with subsequent lengthenings 41 adverse events occurred in all 23 subjects with 2-year follow up, of which 13 (32%) were device related. IMPLANT COMPLICATIONS AFTER MAGNETICALLY CONTROLLED GROWING RODS FOR EARLY ONSET SCOLIOSIS: A MULTICENTER RETROSPECTIVE REVIEW Edmund Choi 1, Pooria Hosseini 1, Gregory Mundis 2, Behrooz Akbarnia 3, Haluk Berk 4, Ilkka Helenius 5, John Ferguson 6, Tiziana Greggi 7, Guido La Rosa 8, Ahmet Alanay 9, Alpasian Senkoylu 10, Kenneth Cheung 11, Jeff Pawelek 1, Burt Yaszay 12 1 San Diego Center for Spinal Disorders, 2 Scripps Health, 3 Orthopaedic Surgery, Universitoy of California, San Diego, 4 Dept. Of Ortho & Trauma, Dokuz Eylul Univ. School of Medicine, 5 Turku University Central Hospital, Department of Paediatric Orthopaedic Surgery, 6 Orthopaedics, Starship Children s Hospital/Auckland Bone and Joint Surgery, 7 Rizzoli Orthopaedic Institute, 8 Department of surgery-orthopedic Unit, Bambino Gesu Children s Hospital, 9 Acibadem University School of Medicine, 10 Gazi University, 11 The University of Hong Kong, 12 Children s Specialists, San Diego 8

44 12/14/2015 IMPLANT COMPLICATIONS AFTER MAGNETIC- CONTROLLED GROWING RODS FOR EARLY ONSET SCOLIOSIS: A MULTICENTER RETROSPECTIVE REVIEW Complications (COMP) were categorized as wound-related and instrumentation-related. COMP were also classified as early (< 6 months) versus late. Distraction technique and interval of distraction was surgeon preference without standardization across sites RESULTS 21 of 54 patients had at least 1 COMP 15/21 of those had revision surgeries. 6 had broken rods and mm rods 2/4 5.5 mm rods failed early (4 mo) and 4 late (mean = 14.5 mo). 6 experienced 1 episode of lack or loss of lengthening of which 4 lengthened subsequently. 7 had either proximal or distal fixation-related CMP at avg of 8.4 mo. 2 had infections requiring I&D, one early (2 wks) with wound drainage and one late (8 mo). The late case required explantation of one of the dual rods CONCLUSION There is a low infection rate (3.7%) with MCGR MCGR does not appear to prevent common implant related complications such as rod or foundation failure. The lack of lengthening seen at some visits is unique to MCGR. The long term implication of this remains to be determined 9

45 12/14/2015 COMPARISON OF PRIMARY VS. CONVERSION SURGERY WITH MCGR RODS IN CHILDREN WITH EOS 27 Primary (mean age 7.0±2.2 years at surgery) 23 C patients (mean age 7.7±2.4 years) P underwent mean 8.0±5.5 and C 3.5±2.4 lengthenings at last follow-up (p=0.0006) 22 patients having a minimum 2-yr FU (mean FU time 22.4±7.9 months for P and 17.3±5.9 for C, p<0.05) RESULTS COMPLICATIONS: 8 patients needed surgical intervention (29.6 %) in P and 7 (30.4 %) in C (p=0.95). 1 patient in C developed a deep wound infection. CONCLUSIONS Satisfactory correction can be achieved with primary MCGR and maintained following conversion to MCGR Spinal growth with subsequent non invasive lengthenings is less in conversion patients compared to primary 10

46 12/14/2015 UK NATIONAL INSTITUTE FOR HEALTH AND CARE EXCELLENCE (NICE) Provisional Recommendations: 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. Findings from cost modelling estimate that using the MAGEC system is cost saving compared with conventional growth rods from about 3 years after the initial insertion procedure. The estimated cost saving per patient after 6 years is around 12,077 (~$20,000 USD). The cost savings remained robust in sensitivity analyses. MCGRs are not reimbursed by insurance plans; their use is currently supported by hospital budgets. Results: With a time horizon of 4 years, the estimated direct costs of TGR and MCGR strategies were 49,067 D and 42,752 D, respectively leading to an incremental costs of 6135 D in favor of MCGR strategy. The study emphasizes that conventional strategy using TGR leads to substantial costs for the French sickness fund even though the overall economic burden is rather limited considering the rarity of EOS cases treated surgically. PROXIMAL JUNCTIONAL KYPHOSIS ASSOCIATED WITH MAGNETICALLY CONTROLLED GROWING ROD SURGERY FOR EARLY ONSET SCOLIOSIS Kenneth Cheung 1, Kenny Kwan 1, John Ferguson 2, Colin Nnadi 3, Ahmet Alanay 4, Muharrem Yazici 5, Gokhan Demirikiran 5, Behrooz Akbarnia 6 1 Queen Mary Hospital, The University of Hong Kong; 2 Starship Children Hospital, New Zealand; 3 Nuffield Department of Orthopaedics, Oxford, UK; 4 Bilim University Faculty of Medicine, Istanbul, Turkey; 5 Hacettepe University, Ankara, Turkey; 6 San Diego Centre for Spinal Disorders, La Jolla CA, USA 11

47 12/14/2015 PJK PJK occurred in 5 of 23 patients (21.7%) 3 of 5 patients had proximal anchor dislodgement; all constructs were revised, 4 of which were due to problems of rod distraction, while one patient had implant breakage At the time of revision surgery, 2 cases were found to have autofusion at the apex, and were converted to definitive fusion, and 3 had new MCGR rods and anchors implanted and distraction was continued Risk factors for PJK in TGR including proximal thoracic scoliosis, thoracic kyphosis, and proximal pedicle screws were not present in these cases No difference in the frequency of distraction (range, 1 to 3 months) between those that developed PJK (n=5) and those that did not (n=17) CASE #1: MCGR i 4 + P2 Major Cobb (T5-L1)= 105, T1-T12 height= 157 mm, T1-S1 height= 264 mm SAL ratio= 0.81, Lumbar lordosis= 69, Thoracic kyphosis= 77 PUSH PRONE 12

48 12/14/2015 PRE OPERATIVE top right left MAY 2013: Post-op X-Rays Major Cobb (T6-L1)= 55, T1-T6= 35, L1-L4= 16 T1-T12 height= 183 mm, T1-S1 height= 312 mm POST OPERATIVE top right left 13

49 12/14/2015 S/P 3 NON-OPERATIVE LENGTHENINGS SEPT. 2014: US LENGTHENING WITHOUT PRE LENGTHENING XRAYS PAIN WITH LENGTHENING POST US XRAYS 14

50 12/14/2015 INTRA-OP REVISION ROUTINE LENGTHENING X2 ONE MONTH LATER NEW PAIN AND A POP WHILE HIKING RIGHT ROD 15

51 12/14/2015 REVISION #2, APRIL 2014 REVIEW Despite improved technology, the issues with rigid implants still exists Unique issues: Large curve: >60 after MCGR Large patient complications happened at age 9 and 10 Pt remains Risser 0 with open triradiate cartilage Still has significant growth remaining Cost of implants Has needed 3 total surgeries in 24 months still better than 5? COMPLICATION - NEUROLOGIC HISTORY: 7 yo female? Adopted Family history unknown Cardiac surgery as a young girl PE: 5/5 strength, no sensory deficitis Curve moderately flexible in the prone position clinically Speech delay but learning rapidly Extremely active Small for stated age (age in question secondary to social history) 16

52 12/14/2015 CASE 2: 6 YO FEMALE; S3KP LOW CONUS- TETHERED CORD Underwent tethered cord release one week before MCGR surgery MCGR- SURGERY 17

53 12/14/2015 EVENTS OF SURGERY 1. MCGR placed with manual lengthening Very good correction During closure, loss of signals 2. MCGR removed Signals returned 3. In situ correction applied Still with good correction At time of closure loss of signals again 4. Decision made to just leave anchors in place, allow for fusion and return later POST FOUNDATION XRAY 76 18

54 12/14/ KG HALO TRACTION 65 PLAN Placement of MCGR after 3 weeks of Halo traction Concept Allow for gradual stretch of the spinal cord Thoughts: Incomplete tethered cord released? Too much correction to be tolerated by the spinal cord 3 MONTHS POST OP 19

55 12/14/2015 BUMPY ROAD TO SUCCESS MCGR has substantially improved technology It is still placing a stiff structure in a growing spine The implant complication rate will unlikely change compared to TGR as the surgery is very similar The ill effects of repetitive lengthening surgery is minimized Promising cost effectiveness data MOST IMPORTANTLY the kids need our help, and it is our job to ensure that these children are given the opportunity to succeed THANK YOU! WWW. G LOBA LSPI N E O U T R E A C H. O R G WWW. S A N D I E G O S PI N E F O U N D AT I O N. O R G 20

56 12/14/2015 Current Advancements in Rib- Based Distraction Systems John T. Smith, MD Mary Scowcroft Peery Presidential Endowed Chair of Orthopedics Chief, scoliosis service, Primary Children s Hospital Professor University of Utah Salt Lake City, UT Disclosures Consultant: Depuy-Synthes; Zimmer- Biomet; Spineguard; Globus medical; Ellipse (wife) Board of Directors: Children s Spine Foundation Royalties: VEPTR 2 device Disclosures Much of this presentation describes physician directed (off-label) use of spinal instrumentation. At no time does this endorse or promote or recommend the use of a specific company s products 1

57 12/14/2015 Mid 1990 s; a new approach: Ribbased distraction Bob Campbell and Melvin Smith: Defined Thoracic Insufficiency Syndrome Combined treatment of both the chest wall and spine deformity Changed the way congenital scoliosis is treated Historical Perspective: The garage version.. R. Campbell and M. Smith: Treatment of Lethal Thoracic Insufficiency with a Titanium Rib WOA, August, 1993 Rib-based constructs are now used to treat a variety of EOS conditions 2

58 12/14/2015 Infantile Idiopathic EOS Congenital EOS Congenital EOS; expansion thoracostomy with RBD 3

59 12/14/2015 Congenital Myopathy Neuromuscular EOS; rib-to-pelvis RBD Kyphosis of Myelodysplasia treated with RBD only Smith, Novais: JBJS,

60 12/14/2015 Why use Ribs vs. Spine? Low profile Multiple points of attachment Failure mode is benign (safe ) Magnetically Controlled Growing Rods using rib-based constructs Recently approved for pediatric indications by the FDA (pediatric pedicle screws rode the same train..) May be lengthened in the office Has not eliminated repetitive surgeries Distraction-based problems still present Many configurations Constructs for Distraction-Based Growth Friendly Surgery Spine to Spine Rib to Spine Spine to Pelvis Rib to Rib Rib to pelvis 5

61 12/14/2015 MCGR: Idiopathic EOS Size Challenges when selecting implants 3.5, and 6.0 rods multiple implant types, sizes for insertion No approved rib fixation or pelvic hooks (other than VEPTR) Requires improvisation and off label constructs Need to think through your construct choices and options BEFORE surgery But now, patients are choosing what they want.. 6

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63 12/14/2015 VEPTR Ingenious invention of Bob Campbell and Melvin Smith Changed the management of chest wall and spine deformity from growing rods alone However, Requires repetitive surgeries to lengthen Significant complication rate as with all growth-friendly surgery Still has a role in the low-volume thorax requiring a thoracostomy Rib-based constructs can be used with all growing systems 8

64 12/14/2015 VEPTR? But. There are still many ways/devices/constructs where ribs can be used for distraction fixation Construct planning is essential to have implants that match in size and versatility Ribs expand options for distraction in the EOS population Thank you Future spine surgeon???? 9