1. Mohammed Attiah, MB,Ch.B. FRCSC Assistant Professor,Orthopaedic Surgery UQU Orthopaedic Specialty Hospital Jeddah - Saudi Arabia Congenital Scoliosis: Treatment Options and Challenges

2. Review Definition Spine Growth Facts Associated Anomalies Natural History Challenges Decision making Treatment Options Controversies

3. Spinal Deformity with the Presence of Vertebral-Anomalies Congenital Scoliosis

4. Big Deal, Why? Osseous Development Neural Element Sagittal Natural Curves Symmetric Growth

5. Spine Growth Longitudinal Growth Chondro-epiphyseal portion of the end plate Endo-chondral Ossification Circumferential growth –Posterior growth ( 5-8 y) Laminar growth Pedicular growth –Anterior Growth(Pre-Pub yrs) Latitudinal Growth Perichondral & Periosteal apposition Taylor,J. Anat. (1975), 120, 1, pp. 49-68

6. Spine Growth Facts ? In utero = Extremely Accelerated Birth -2y = Increased Rate 2y - 10 y = Steady Rate Pre-pub = Increased Rate Thoracic Vertebra = 0.8 mm /y Lumbar Vertebra = 1.1 mm /y Thoracic Disc = 0.2-0.6 mm /y Lumbar Disc = 0.3-0.8 mm/y Hefti, JBJS Br, 1983;65:247-54 Dimeglio, Acta Orthop Belg,1990 Taylor, J Anat,1975

7. Thoracic Spine = 1.2 cm/ Year Lumbar Spine = 0.7 Cm / year Spine Growth Facts ? Dimeglio, Acta Orthop Belg,1990 Taylor, J Anat,1975

8. Campbell, JBJS AM, 2004 Wezeka, Spine, 2004 Meehan, J Ped Ortho,1985 Associated Anomalies Pulmonary Compromise –Rib Deformity –Spinal Deformity –Lung Congenital Abnormality Congenital Heart Disease 15 % –Echo / Cardiology Consult CT chest measurement PFT volume depletion VC < 50% = Post Op Pulmonary complication Ferguson, J Ped Orthop,1996Reckles, JBJS, 1975

9. Renal Anomalies 20-40 % –Unilateral kidney –Ureteric duplication –Ureteric obstruction Associated Anomalies Renal U/S Hensinger, JBJS,1974 MacEwen, JBJS, 1972

10. Spinal Cord Anomalies 20% –Tethered cord –Diastemetomyelia –Fibrous Dural Band –Intradural Lipoma –Syringomyelia –ACM Associated Anomalies Spine MRI Spine CT scan McMaster, Spine,1998

11. Natural History McMastar, Spine, 1998 Winter, Ortho Clin North Am,1998 Type of Vertebral Anomalies Growth Potential Site of Anomalies

12. Type of Vertebral Anomalies –Uni Un-segmented Bar + –Hemi-vertebra Natural History McMastar, Spine, 1998 Winter, Ortho Clin North Am,1998

13. Natural History Growth Potential –First 2 years –Adolescent Growth Spurt McMastar, Spine, 1998 Winter, Ortho Clin North Am,1998

14. Site of Anomalies Thoracic > Lumbar Natural History McMastar, Spine, 1998 Winter, Ortho Clin North Am,1998

15. Early stage of life Cause large deformity Rigid Curve Resistant to correction Progressive Associated anomalies Congenital Curves Challenges McMaster, JBJS.1982 Fernandes, JBJS,2007 Prediction about what will happen with growth is very difficult

16. Decision Making Early Treatment Decision Magnitude of Curve Age Type / Location of Anomaly Diagnosis Full Work Up Consultation

17. Balanced Spine Stop progression Deformity Correction Growing Vertebra Growing Neural Element Torso - Leg ratio Treatment Goal What do you want? :What does the spine do?:

18. Low Risk for Progression –Cobb angle < 25˚ –RAVD < 20 ˚ High Risk for Progression –Cobb Angle > 25˚ –RAVD > 21˚ Initiation of Treatment Mehta, JBJS,Br, 1972

19. When to start Treatment Anticipated or actual curve progression –Curve progression 10 ˚ –Initial curve > 30 ˚ at growth spurt stages Mehta, JBJS,Br, 1972

20. Treatment Option Bracing –Mixed anomalies –Progressive secondary curve Controlling long,flexible compensatory curve below congenital anomalies Not Successful Treatment Option

21. Prevent Future Deformity In Situ Fusion Correct Present Deformity Gradual Correction Hemiepiphysiodesis Growing Nonfusion Rod Acute Correction Instrumentation & Fusion Hemivertebra Excision Osteotomy Surgical Treatment Options

22. In situ Fusion No hope to get congenitally fused side growing again Simplest & Safest solution Stop growth on convex side –Unilateral Unsegmented Bar –Balanced –Not large curve < 40 ˚ –Early stages of life McMaster, Spine, 1998 Dubousset, J Pedi Orthop,1998 Keller,lindesth, spine.1994

23. In situ Fusion Controversy Age –Very early years of life? Anterior & Posterior fusion –? Combined –Less potential anterior growth –Abnormal anterior vessels Trans-pedicular approach Decorticating the spine Facet Fusion –One level cephalad –One level caudad McMaster, Spine, 1998 Dubousset, J Pedi Orthop,1998 Keller,lindesth, spine.1994

24. Prevent Future Deformity In Situ Fusion Correct Present Deformity Gradual Correction Hemiepiphysiodesis Growing Nonfusion Rod Acute Correction Instrumentation & Fusion Hemivertebra Excision Osteotomy Surgical Treatment Options

25. FAILURE OF FORMATION CONCAVE FUTURE GROWTH I.Single Hemi-vertebra II.Curve < 50 ˚ III.Age < 5 years Hemiepiphysiodesis & Hemiarthrodesis Keller,J Ped Orthop B,1994 Winter,J Ped Orthop,1981 Andrew, JBJS Br,1985

26. Failure to achieve correction High failure rate –30 % improvement ? –40 % no change –20 % progressed Average correction 10˚ Hemiepiphysiodesis Controversy Roaf, JBJS Br,1963 Keller, Spine,1994

27. Growing Non-Fusion Rod Very Young child Treat the extended Secondary Curve Akbarnia,McCarthy,SRS,1994

28. Growing Non-Fusion Rod Primary curve should be addressed: –In Situ fusion –Hemiepiphysiodesis –Excision –Osteotomy Not commonly used technique Lack of strong evidence & F/U

29. Hemivertebra Excision Popular procedure –Immediate –Excellent –Bradford,JBJS Am,1990 Remove the Etiology Prevent worsening Correction

30. Hemivertebra Excision Ideal indication –Hemivertebra LS junction –Ignored large curve

31. Combined Vs Staged –Anterior Approach –Posterior Approach Leatherman,JBJS Am, 1996 Single Approach –Posterior Excision –Eggshell procedure Hemivertebra Excision

32. Single Stage Posterior Approach

33. Complication Blood loss –Segmental –Epidural Neurological injury –Cord injury –Root injury Winter,spine, 1989 Wiles, JBJS Am,1951

34. Instrumentation & Fusion Safe correction Balanced spine Spinal cord status Fusion level selection 8 yrs 9 Yrs 40 80

35. Downside of Long Segment Fusion Crankshaft Phenomenon –Less anterior growth rate –No absolute grantee technique Sanders, JBJS Am,1995

36. Spine Osteotomy Short Segment Osteotomy

37. Spine Osteotomy 40 Long Segment Osteotomy –Unacceptable cosmetic –Fixed deformity –Unbalanced No other solution Salvage procedure Experienced spinal surgeon

38. Principles of Spine Osteotomy Cutting through anterior fusion mass Resection of enough bone to allow correction Temporarily stabilize curve Compressive instrumentation allow closure of osteotomy Bone graft ++++

39. Summary Challenges Decision making Treatment Options Safe Correct decision Long term Follow Up

40. Orthopaedic Specialty Hospital - OSH Jeddah - Saudi Arabia www.osh.med.sa