Challenges in EOS Early Onset Spinal Deformity Treating very Many - PDF document

11/8/2013 Disclosures (Growing Spine) Magnetically Controlled Growing Rods (MCGR) for Early Onset Scoliosis (EOS) Growing Spine Foundation (a) DePuy Spine (a,b) Behrooz A. Akbarnia, MD Ellipse Tech. (a,b) Clinical Professor, University of California, San Diego Medical Director, San Diego Center for Spinal Disorders K2M (a,b) La Jolla, California Kspine (b) a. Grants/Research Support UCSF Practical Course in Advanced Spinal techniques b. Consultant Las Vegas, November 8, 2013 c. Stock/Shareholder d. Speakers’ Bureau e. Other Financial Support Challenges in EOS Early Onset Spinal Deformity Treating very • Many etiologies young children • Many different treatments with progressive • High rate of complications EOS remains • Limited outcome measures challenging … • Comparisons difficult! 1

11/8/2013 Literature Review J Ped Ortho 2010 No Evidence Based (EBM) • Options between growing rods, nonoperative treatment, VEPTR, Shilla, and fusion. Data • Practical variation exists (as each patient needs special consideration) but consensus exists on OBM ( O pinion Based) the utility of GR in EOS specifically about starting age, curve and etiology. • Vitale Study Acknowledgement Growing Spine Organization Growing Spine Foundation Growing Spine Study Group GSSG Sites 2

11/8/2013 GSSG- San Diego Site Development and Initial Validation of a Novel Classification System in Early Onset Scoliosis Brendan A. Williams 1 , MD; Hiroko Matsumoto 1 , MA; Daren J. McCalla 1 , BS; Behrooz A. Akbarnia 2 , MD; Laurel C. Blakemore 3 , MD; Randal R. Betz 4 , MD; John M. Flynn 5 , MD; Charles E. Johnston 6 , MD; Richard E. McCarthy 7 , MD; David P. Roye Jr. 1 , MD; David L. Skaggs 8 , MD; John T. Smith 9 , MD; Brian D. Snyder 10 , MD, PhD; Paul D. Sponseller 11 , MD, MBA; Peter F. Sturm 12 , MD; George H. Thompson 13 , MD; Muharrem Yazici 14 , MD; Michael G. Vitale 1 , MD, MPH. Cobb Angle Etiology Congenital/Structural Neuromuscular Rady Children’s Hospital EOS Program Syndromic Idiopathic Results Classification of EOS (C-EOS) Not Sum of Useful Essential CVR Useful Ranks COBB 0 1 14 0.87 29 Cobb APR ETIOLOGY 0 3 12 0.60 27 Etiology Kyphosis Angle Modifier KYPHOSIS 0 4 11 0.47 26 AGE 5 0 10 0.33 20 PROGRESSION 3 5 7 -0.07 19 C ongenital/ 1 : <20 ° CHEST WALL ABNORMALITIES 2 9 4 -0.47 17 (-) : <20 ° P 0 : <10 ° /yr Structural FLEXIBILITY 4 6 5 -0.33 16 OTHER CO-MORBIDITIES 3 8 4 -0.47 16 N euromus 2 : 21-50 ° PULMONARY FUNCTION 3 9 3 -0.60 15 cular AMBULATORY ABILITY 2 12 1 -0.87 14 N : 21-50 ° P 1 : 10-20 ° /yr NUTRITIONAL STATUS 5 8 2 -0.73 12 S yndromic 3 : 51-90 ° MENTAL FUNCTION 10 5 0 -1.00 5 BONE QUALITY 11 4 0 -1.00 4 (+) : >50 ° P 2 : >20 ° /yr I diopathic 4 : >90 ° Vitale et al 3

11/8/2013 Questions to be addressed: Validation Studies (ICEOS) Flynn, Vitale et al. • Why children with EOS need treatment? Risk by Classification: • What are the goals of treatment? Lower Risk of Rapid Failure • • What are the treatment options Congenital (21-50& 51-90); C2, C3 • Syndromic (21-50); S2 including growth friendly options? • Idiopathic (51-90); I3 Higher Risk of Rapid Failure • What are the expected outcomes? • Congenital (>90); C4 • Neuromuscular (>51-90); N3 • Can we minimize complications? • Neuromuscular (>90); N4 • Syndromic (51-90); S3 • What to expect in the future? Questions to be addressed: Natural History Untreated Scoliosis • Why children with EOS need • Infantile: 0 to 3 years treatment? • What are the goals of treatment? • Juvenile: 4 to 9 years • What are the options? • Non-fusion options • Can we minimize complications? • What is the expected outcomes? • Adolescent: 10 to 16 years • What to expect in the future? Pehrsson, Larssson, Oden & Nachemson, Spine, 1992 4

11/8/2013 Thoracic Insufficiency Syndrome Thoracic Insufficiency Syndrome Poor Quality of Life • Among the The Inability of the Thorax to Support lowest QOL in Pediatrics Normal Respiration observed in 100 85 pediatrics 80 75 69 Or 63 60 53 – Asthma Lung Growth 40 – JRA 20 – Heart 0 transplant TIS Asthma JRA Heart X Norms Campbell, Smith, et al. J BJS Mar, 2003 Vitale, JPO, 2008 J BJS Aug, 2004 Questions to be addressed: Treatment Goals  Deformity Correction ( spine and chest ) • Why children with EOS need and maintenance of correction treatment?  Improve pulmonary and spinal function • What are the goals of treatment?  Normalize the spinal growth and avoid • What are the options? early fusion (maintain mobility) • Non-fusion options  Minimize complications • Can we minimize complications?  Improve quality of life and the care of the • What is the expected outcomes? patient • What to expect in the future? 5

11/8/2013 Questions to be addressed: Fusing scoliosis early may contribute to shortening of the thoracic spine, TIS, and respiratory insufficiency • 28 pts, early thoracic fusion before the age 9 years, • Why children with EOS need – evaluated by pulmonary function testing at a minimum of 5 years f/u – compared to age matched controls treatment? • Average age at surgery was 3.3 yrs and at follow up was 14.6 yrs. • What are the goals of treatment? • What are the options? • Thoracic spinal height FVC < 50% < 18 cm 63% pts • Non-fusion options 13 cm 18 to 22 cm 25% pts • Can we minimize complications? 22 cm to normal † 0% pts • What is the expected outcomes? † normal 28 cm males, 26 cm females Deceased • What to expect in the future? Karol, et al., JBJS 2008, 90: 1272-1281 . Treatment Options History • Non-operative treatment (cast, Brace, traction) • Spine based growing rods • Rib-based distraction • Hybrid? • Growth modulation • Guided growth • Early fusion 6

11/8/2013 Questions to be addressed: Casting and Bracing is well tolerated • Why children with EOS need treatment? • What are the goals of treatment? • What are the options? • Non-fusion options • What is the expected outcomes? • Can we minimize complications? • What to expect in the future? Growth Friendly Implant Classification Indications for Growth-Friendly Surgery < age 8 ? 1. Distraction based All etiologies < age 9 ? – Growing Rods ONLY VEPTR IS All etiologies – VEPTR • Progressive curves not controlled or APPROVED in – MCGR amenable to bracing or casting USA 2. Guided Growth • Curves where growth preservation < age 9 ? – Luque-Trolley would be beneficial All etiologies – Shilla • Curves that require management of both the chest wall and the scoliosis 3. Tension Based >age 8 – Tether Non-congenital – Staple Skaggs 7

11/8/2013 Shilla Absent Ribs: Expansion Open Screws – no fusion Thoracoplasty by Multiple Devices no bone exposed allow rod to slide multiaxial 3 level fusion compression distraction derotation Richard McCarthy 1 year Postop Preop Postop Single Rod Techniques Newton Growing Rods 8

11/8/2013 Growing Rods Distraction Based – Rib Anchors +/- thorocotomy Comparison of spine and Rib anchors Rib to Spine All specimens eventually failed at the bone-anchor interface. • No failures were observed in the instrumentation utilized. Construct Type Maximum load for failure (Mean & Standard Deviation) 349  89 N (Screw-Screw) SS 283  48 N (Laminar Hook-Hook) HH 429  133 N (Rib Hook-Hook) RR 236  60 N (Transverse Process-Laminar Hook-Hook) TPL Young’s Modulus was calculated for each construct type and • no statistically significant difference was determined. 9

11/8/2013 Questions to be addressed: Quality of Life Outcomes • Why children with EOS need treatment? • EOSQ being collected prospectively • What are the goals of treatment? • What are the options? • Non-fusion options • What is the expected outcomes? • Can we minimize complications? • What to expect in the future? Growth per Year (cm) RESULTS (cont’d) GROUP Cobb Angle % Increase in (Pre-Initial to Correction T1-S1 • Total Group 1.21 Post Final) Length • Under 5 years 1.19 85° → 65 ° Single with 23% 6.4cm • 5-10 year 1.13 apical • Under treatment 1.01 61° → 39 ° Single w/o 36% 7.6cm • Post final fusion group 1.66 apical 92° → 26 ° Dual w/o 71% 11.8cm apical 10

11/8/2013 RESULTS 3 + 10 Year Old Girl With Marfan’s 7 + 8 yrs Follow Up 410 100 Number of Spinal Distractions 90 390 80 13 cm 80.00 370 Mean # of distractions = 5.4 70 (Range: 3 – 11) 70.00 350 60.00 60 50.00 330 50 >7 mm 40.00 4-6 40 (n=15) 310 30.00 (n=19) 3 30 Final Fusion 20.00 290 10.00 (n=17) 20 T1-S1 Length 270 .00 Length of Implant 10 Cobb Angle Change in T1-T12 (Pre-op to Latest Follow-up) 250 0 Apr-98 Dec-98 Sep-00 May-01 Sep-01 May-03 Feb-04 Jan-05 Dec-06 Oct-99 Oct-99 Oct-01 ∆ in T1-T12 was greater in the 4-6 and ≥ 7 groups (p<0.001) Age 12 + 7 Age 3 +11 Early onset scoliosis treated with Growing Rods has more growth and better Cobb correction but Progression more surgeries compared to Shilla Lindsay M. Andras, MD 1 ; Elizabeth R. A. Joiner, BS 1 ; Richard E. McCarthy, MD 2 ; Scott J. Luhmann, MD 3 ; Paul D. Sponseller, MD 4 ; John B. Emans 5 , MD; David L. Skaggs, MD 1 and Growing Spine Study Group • 37 GR 37 Shilla 134 • Same FU (4.1 vs 4.6) 128 • T1-S1 ( 8.5 vs 6.4) 103 33 86 ° • Cobb angle Change ( 36 vs 23) • Number of surgeries (7 vs 2.8) 2+6 ICEOS 2012 11