Early-Onset Spinal Deformity: Decision-Making

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1 Early-Onset Spinal Deformity: Decision-Making Scott J. Luhmann, M.D. Professor Department of Orthopaedic Surgery Washington University School of Medicine Chief of Staff, Shriner s Hospital for Children St. Louis, Missouri

2 Early-Onset Spinal Deformity Highly heterogenous group Diagnosis: idiopathic, syndromic, NM, congenital Deformity Magnitude and progression Location: C, CT, T, TL, L, LS Plane of deformity: Coronal, Sagittal, Transverse planes Patient size: height & weight Neurologic status Pulmonary status Medical comorbidities

3 Early-Onset Spinal Deformity: < 10 years of age Spine Growth in Childhood Boys Girls Cm/Year Age in Years Data from Dimeglio

4 Emery JL, Mithal A (1960) Hyperplasia Hypertrophy Age Thurlbeck WM. Postnatal human lung growth (Thorax 1982) Slow increment in alveolar number between ages 4 and 8 years, and little increase thereafter.

5 Pulmonary Function Following Early Thoracic Fusion in Non-Neuromuscular Scoliosis Karol et al, JBJS pts (various dx) Surgery: Mean age 3.3 years Mean 58.7% of thoracic spine fused F/u: Mean age 14.6 years FVC: 57.8% predicted [43% of patients <50% predicted] FEV1: 54.7% predicted Diminished PFT values in: Extent of spine fused (>4 segments) Rib anomalies Proximal extent of fusion: T1-2 vs. T3-5 vs. T6 and below

6 Long-Term Follow-Up of Patients with Untreated Scoliosis Pehrsson et al, Spine patients Born Mean f/u 56 yrs Mortality risk: Changed at y/o Majority d/t respiratory failure Which diagnoses?

7 Long-Term Follow-Up of Patients with Untreated Scoliosis Pehrsson et al, Spine 1992 Increased mortality IIS JIS Post-polio Rickets Unknown dx >70 degree Cobb Age at onset Highest mortality rate: IIS 3-dimensional restrictive lung disease

8 Goals of EOS treatment Maximal T1-T12, T1-S1 distance Permit radial expansion of the ribs/chest Minimize the 3-dimensional spinal deformity Fewest number of anesthetic episodes possible Low complication rate Low number of outpatient care episodes Maximize patient activity/function

9 EOS: Treatment Options Observation Bracing Casting Surgical Distraction-based TGR MCGR VEPTR Shilla/Growth Guidance Vertebral Body Stapling Spinal Tethering Fusion: rare 2 Main Goals Control spinal deformity Permit spinal growth

10 EOS < 5 years Cotrel.Spine Casting

11 2 y/o female Initial After 1 st cast In 2 nd cast 90 o 68 o 32 o Best results: Age <2 yrs Cobb <50 o Optimal: deformity correction Minimum: delay tactic

12 Indications EOS < 5 years of age Surgical Large magnitude: >60 degrees Significant progression Deformity not responsive or patient intolerant to casting Main Options: TGR vs. MCGR Shilla rarely may be an option: pt often too small to hide implants, pedicles too small

13 Comparison of Single and Dual Growing Rod Techniques Followed Through Definitive Surgery Thompson GH, et al (Spine 2005) Results: BEST in dual growing rods Preoperative initial to postoperative final percent Cobb correction Single rod/apical fusion 23% +/- 22% Single rod 36% +/-23% Dual rod 71% +/- 22% T1-S1 spinal growth % expected Single rod/apical fusion 0.3+/-1.02 cm/yr 25% Single rod 1.04+/-0.09 cm/yr 80% Dual rod 1.7+/ cm/yr 130% Conclusions Dual rods are stronger than single rods, providing better initial correction and maintenance of correction

14 Distraction-based Technologies: TGRs, MCGRs and VEPTR Cephalad Ribs Spine Caudal Spine Iliac Sacropelvic

15 EOS < 5 years of age Surgical Options: TGR vs. MCGR Avoid segmental fusion (no iatrogenic shortening) Use rib hooks and downgoing laminar hooks Unilateral rods ARE an option Pelvis S-hooks for pelvic obliquity Saves pedicle fixation for later

16 12 m/o male; IIS 91 o

17 4+4 y/o Treatment: 9 spine castings 3 TLSOs 74 o Progression to 74 degrees (5 months earlier = 65 degrees in TLSO)

18 T to L3 MAGEC 4.5 mm x 70 mm 92o 56 o T1-T mm T1-S1 215 mm T1-T mm T1-S1 254 mm

19 18 m/o female; OI; craniosynostosis 54 o 54 o 100 o 54 o 3.5 mm SS; 4 upgoing laminar hooks B

20 7 y/o male; Developmental delay, mitochondrial d/o 81 o 40 o 4.5 mm SS; 4 upgoing laminar hooks B

21 EOS > 5 years Indications for operative Treatment >50-60 degrees Progressive Not responsive or intolerant to casting Main Options: 1. Shilla (n=42) 2. MCGR (n=32) 3. TGR (n=40) Cm/Year Spine Growth in Childhood Age in Years Bo ys

22 5 ½ years s/p T3-L3 SHILLA Growth 6 y/o male 6 wks postop Guidance System: 1 reoperation 3 y postop 4 1/2 y postop s/p revision 11+5 yrs Spine growing off rod, revise if: 1. Painful 2. Lost control of deformity

23 Radiographic Outcomes of Growth Guidance System and Traditional Growing Rods through Definitive Treatment AAOS 2017; Accepted Spine Deformity Scott J. Luhmann, MD June C. Smith, MPH Anna McClung, BSN, RN Lynn McCullough, RNP, MNSc, ONC. Richard E. McCarthy, MD George Thompson, MD Growing Spine Study Group

24 Summary Reoperations: 3x higher in TGR group (3.1 vs. 9.3) Complications: /pt (NS) Major Cobb: 46-48% improvement (NS) Change in T1-T12: mm increase (NS) Change in T1-S1: mm increase (NS)

25 Ellipse Technologies: MAGEC TM MAGnetic Expansion Control In vivo lengthening may be only 50% of predicted lengthening

26 5+8 y/o female; 19.4 kg (43#) Previous TLSO tx; back pain SCM 30 o 104 o 91o T1-T mm T1-S1 215 mm

27 Immediate Postop 8 weeks halo preoperatively 58 o 40 o T1-T mm T1-S1 301 mm

28 2 years postop 46 o 104 o 91 o 40 o

29 Traditional Growing Rods Versus Magnetically Controlled Growing Rods for the Surgical Treatment of Early-Onset Scoliosis: A Case- Matched 2-Year Study Akbarnia GA, Pawelek JB, Cheung KM, et al. GSSG Spine Deformity 2014;2(6): TGR vs. 17 MCGR; Matched Results Major curve correction similar (NS) T1-S1& T1-T12 change (NS) Results Procedures TGR 73 open surgeries (56 were lengthenings) MCGR 16 open surgeries (137 noninvasive lengthenings) Unplanned revisions MCGR 3 patients TGR 4 patients

30 Conclusion EOS < 5 years Observation Casting Bracing Surgical: TGR or MCGR Use rib hooks, downgoing laminar hooks and pelvic S- hooks to avoid fusion Unilateral rods

31 Conclusion EOS > 5 years Observation Bracing Surgical: 1. Shilla 2. MCGR 3. TGR

32 Thank you

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