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Congenital scoliosis

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Congenital Scoliosis review

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Congenital scoliosis

  1. 1. Congenital Scoliosis Richard C Rooney, MD, FACS rrooney@seattlespinegroup.com
  2. 2. Classification Defects of Segmentaion Defects of Formation Complex Spine Deformities • Proposed by Moe et al – Early concept • Van Schrick • Mac Ewen • Accepted by SRS and AAOS • Simplistic – Ignores current understanding of embryogenesis – Does not explain all deformities
  3. 3. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980
  4. 4. Embryonic Period Anomalies 1st 56 Days • Germinal layer adhesion • Notochord substance sequestration • Asynchronous hemimetameric pair development • Hemimetamer hypoplasia and aplasia • Ventral and lateral process coalition
  5. 5. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980
  6. 6. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980 Hedequist and Emans, Congenital Scoliosis, Journal of AAOS, 2004
  7. 7. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980
  8. 8. Fetal Period Anomalies 57th day to Birth • Vertebral joint failure of segmentation • Centrum hypoplasia and aplasia
  9. 9. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980
  10. 10. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980
  11. 11. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980
  12. 12. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980
  13. 13. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980
  14. 14. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980
  15. 15. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980
  16. 16. Lonstein, Congenital Spine Deformities, Ortho Clinics of NA, Vol 30, No.3, 1999
  17. 17. Genetics • Wynne-Davies 1975 – 337 pts with congenital anomalies – Most were sporadic with no risk to subsequent siblings of offspring – Multiple anomalies carried a 5~10% risk to sibs • Winter 1983 – 1200 pts – 1% with a known relative with a problem • Most twin studies show one with a defect and one without • McMaster et al JBJS, 1999, – Positive family hx with multiple levels of bilateral failure of segmentation, fused ribs, missing segments – Spondylothoracic dysplasia or Jarcho-Levin syndrome
  18. 18. Associated Anomlies • Renal 30% - Renal imaging is most important image in pre-schoolers • Cardiac 15% - Evaluate murmurs, they are never due to the scoliosis • Dysraphism 20+% – Beware of skin anomalies – Leg length discrepancy – Neurological asymmetry • Skeletal anomalies Hedequist and Emans, Congenital Scoliosis, Journal of AAOS, Vol 12, No. 4, 2004
  19. 19. Nomenclature • Segmented – has disc space • Incarcerated – adjacent level accommodates hemi Hedequist and Emans, Congenital Scoliosis, Journal of AAOS, Vol 12, No. 4, 2004
  20. 20. Midline Ectodermal Anomalies Correlate with Neural Anomalies • Skin tag • Dimples • Pigmentation • Hair patch Hedequist and Emans, Congenital Scoliosis, Journal of AAOS, Vol 12, No. 4, 2004
  21. 21. Midline skin anomalies may mean neural anomalies
  22. 22. Hedequist and Emans, Congenital Scoliosis, Journal of AAOS, Vol 12, No. 4, 2004
  23. 23. Evaluation of Entire Spinal Canal • Search for anomalies – Chiari malformation – Syringomyelia – Diatematomyelis – Filum terminale – Lipoma – Tumor • Indication – Pre-op – Neurologic deficit or asymmetry – Lower limb asymmetry – Every pt with a congenital defect? Hedequist and Emans, Congenital Scoliosis, Journal of AAOS, Vol 12, No. 4, 2004
  24. 24. Diastematomyelia
  25. 25. Diastematomyelia Radiographic Signs • Widened interpedicular distance • Narrowed disc • Midline calcification
  26. 26. Winter Sign Asymmetrical forward bend is consistent with a tethered cord
  27. 27. Tethered Cord Not Just a Problem for Children • Adults can present with tethered cord symptoms – Increasing pain or neurologic loss of function – Lower limb asymmetry or spasticity • Do not be satisfied with images of only lumbar spine
  28. 28. Lonstein, Congenital Spine Deformities, Ortho Clinics of NA, Vol 30, No.3, 1999
  29. 29. Hedequist and Emans, Congenital Scoliosis, Journal of AAOS, Vol 12, No. 4, 2004
  30. 30. Progression of Congenital Scoliosis • Highly variable • “Most progress” (10~20% nonprogressive) – Unilateral bar with contralateral hemi – worst – Unilateral unsegmented bar – Double hemivertebra – Single hemivertebra – Wedge vertebra – Block vertebra – best prognosis McMaster and Ohtsuka,JBJS, vol 64, 1982
  31. 31. Location • Thoracic curves with poorest prognosis • Hemivertebra at lumbosacral and cervicothoracic junction cause decompensation Lonstein, Congenital Spine Deformities, Ortho Clinics of NA, Vol 30, No.3, 1999
  32. 32. Non-operative Treatment • Observation – Not for high risk – Hemivertebra and mixed deformity • Bracing – limited role – Contraindicated for short stiff curves – ? Help for flexible curves
  33. 33. Lonstein, Congenital Spine Deformities, Ortho Clinics of NA, Vol 30, No.3, 1999 Treated with Observation
  34. 34. Hedequist and Emans, Congenital Scoliosis, Journal of AAOS, Vol 12, No. 4, 2004
  35. 35. Surgical Rx • Fusion-in-situ – Simple – Ineffective • Anterior epiphysiodesis & posterior hemiarthrodesis – Progressive deformity – Growing patient • Combined anterior posterior fusion • Hemivertebra excision
  36. 36. Lonstein, Congenital Spine Deformities, Ortho Clinics of NA, Vol 30, No.3, 1999 Convex Growth Arrest at 2+8
  37. 37. Hedequist and Emans, Congenital Scoliosis, Journal of AAOS, Vol 12, No. 4, 2004
  38. 38. Lonstein, Congenital Spine Deformities, Ortho Clinics of NA, Vol 30, No.3, 1999 Ant excision of hemivert and post instrumentation and fusion
  39. 39. Congenital Scoliosis with Myelodysplasia
  40. 40. Rx with ant discectomies post instr with pedicle screws at bifid vertebra and Galveston fixation
  41. 41. Kyphosis • Kyphosis with centrum aplasia – Sharp angular – Risk of paraplegia – Rx with circum-fusion +/- corpectomy • Kyphosis with anterior bar – Less neuro risk id not operated – Surgical options customized
  42. 42. Lordosis • Anterior fusion – small curve • Anterior / posterior procedure – large curves
  43. 43. Lonstein, Congenital Spine Deformities, Ortho Clinics of NA, Vol 30, No.3, 1999 Progressive lordosis treated with ant discectomies, post fusion, and post-op cast
  44. 44. Lonstein, Congenital Spine Deformities, Ortho Clinics of NA, Vol 30, No.3, 1999
  45. 45. Conclusion • Embryogenesis can be analyzed • Progression is unpredictable with exceptions – Bar with contralateral hemivertebra – Kyphosis due to centrum aplasia • Treatment – Remove deforming growth elements – Fusion-in-situ is no better than placebo

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