1. Burt Yaszay, MD Jeff B. Pawelek, BS John Emans, MD Patrick Cahill, MD Bo Robertson Gregory M. Mundis, MD Behrooz A. Akbarnia, MD Children’s Spine Study Group Growing Spine Study Group The utility of growth-friendly surgery in early- onset scoliosis secondary to Beals syndrome International Congress on Early Onset Scoliosis Boston, MA – November 19-20, 2015

2. DISCLOSURES Burt Yaszay, MD (a) DePuy Synthes; Children’s Spine Foundation; (b) Depuy-Synthes, K2M, Nuvasive; (d) DePuy-Synthes, K2M; (e) K2M, OrthoPediatrics Jeff B. Pawelek, BSNone John Emans, MD (b) Medtronic Sofamor Danek; (b, e) Synthes Patrick Cahill, MD (b, d, e) DePuy Synthes Spine; (b, d) Ellipse Technologies; (d) Globus Medical; (b, d) Medtronic Bo RoberstonNone Gregory M. Mundis, Jr., MD(a,b,d) Nuvasive; (a,b) K2M; (a,e) DePuy Synthes Behrooz A. Akbarnia, MD (a) DePuy Synthes, Nuvasive; (b) Nuvasive, K2M, Ellipse; (c) Alphatec, Nuvasive, Ellipse, Nocimed; (e) Depuy Synthes, Nuvasive, K2M, Springer Children’s Spine Study Group (a) DePuy Synthes; (a) Children’s Spine Foundation Growing Spine Study Group(a) Growing Spine Foundation a. Grants/Research Support b. Consultant c. Stock/Shareholder d. Speakers’ Bureau e. Other Financial Support

3.  Beals syndrome is an extremely rare disease that is phenotypically similar to Marfan syndrome.  However, in addition to skeletal anomalies and aortic enlargement Beals patients also suffer from joint contractures.  A paucity of literature exists on how orthopaedic surgeons manage early onset scoliosis (EOS) secondary to Beals syndrome. INTRODUCTION

4. PURPOSE:  The purpose of this review was to characterize growth- friendly treatment options for EOS patients who suffer from Beals syndrome. HYPOTHESIS:  EOS patients with Beals syndrome who receive distraction-based have similar radiographic outcomes and complications rates compared to previously published data.

5.  Two multi-center EOS databases were queried to identify patients who had: Confirmed Beals sydrome Distraction-based surgical treatment Minimum 5-year follow up Complete medical and x-ray records METHODS

6.  Study time points Pre-op 1 st post-op Latest follow up 3 qualified patients 2 traditional growing rods 1 VEPTR METHODS

7. RESULTS Tx TypeGender Age at Insertion Follow up Patient #1TGRMale6.7 years6.6 years Patient #2TGRMale6.4 years5.2 years Patient #3VEPTRMale2.8 years8.0 years MEAN--5.3 years6.6 years  Demographics

8. RESULTS Tx TypePre-opPost-opLatest Patient #1TGR78º32º38º Patient #2TGR63º52º54º Patient #3VEPTR85º65º88º MEAN75º50º60º Overall mean % curve correction21%  Major Curve Correction

9. RESULTS Tx TypePre-opPost-opLatest Patient #1TGR273 mm336 mm412 mm Patient #2TGR281 mm287 mm324 mm Patient #3VEPTR232 mm271 mm346 mm MEAN262 mm298 mm361 mm  Spinal Height (T1-S1)

10. RESULTS  Complications Tx TypeImplantSurgical SiteOther Patient #1TGR3 rod fractures Patient #2TGR4 rod fractures1 infection 1 postural problem; 1 atelectasis Patient #3VEPTR 2 device migrations 6 infections 2 unexpected pain 5.3 complications per patient compared to 2.2 complications/patient reported by Bess, et al. for growing rods and 2.4 complications/patient reported by Sankar, et al. for VEPTR

11. DISCUSSION Distraction-based surgery in EOS secondary to Beals syndrome is a viable treatment option for this challenging and rare patient population. The complication rate was higher than previous reports; however, the overall increase in spinal height were favorable and consistent with current literature. Mean curve correction (21%) was lower compared to previously published data.