Congenital Scoliosis review

1. Congenital Scoliosis
Richard C Rooney, MD, FACS
rrooney@seattlespinegroup.com

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. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980

6. 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

8. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980

9. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980
Hedequist and Emans, Congenital Scoliosis, Journal of AAOS, 2004

10. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980

11. Fetal Period Anomalies
57th day to Birth
• Vertebral joint failure of segmentation
• Centrum hypoplasia and aplasia

12. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980

13. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980

14. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980

15. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980

16. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980

17. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980

18. Tsou et al, Clinical Ortho and Related Research, No. 152, 1980

19. Lonstein, Congenital Spine Deformities, Ortho Clinics of NA, Vol 30, No.3, 1999

20. 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

21. 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

24. Nomenclature
• Segmented – has disc space
• Incarcerated – adjacent level accommodates
hemi
Hedequist and Emans, Congenital Scoliosis, Journal of AAOS, Vol 12, No. 4, 2004

26. 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

27. Midline skin anomalies may mean neural
anomalies

29. Hedequist and Emans, Congenital Scoliosis, Journal of AAOS, Vol 12, No. 4, 2004

30. 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

31. Diastematomyelia

32. Diastematomyelia Radiographic
Signs
• Widened interpedicular distance
• Narrowed disc
• Midline calcification

33. Winter Sign
Asymmetrical forward bend is consistent with
a tethered cord

34. 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

35. Lonstein, Congenital Spine Deformities, Ortho Clinics of NA, Vol 30, No.3, 1999

36. Hedequist and Emans, Congenital Scoliosis, Journal of AAOS, Vol 12, No. 4, 2004

37. 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

38. 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

39. Non-operative Treatment
• Observation
– Not for high risk
– Hemivertebra and mixed deformity
• Bracing – limited role
– Contraindicated for short stiff curves
– ? Help for flexible curves

40. Lonstein, Congenital Spine Deformities, Ortho Clinics of NA, Vol 30, No.3, 1999
Treated with Observation

41. Hedequist and Emans, Congenital Scoliosis, Journal of AAOS, Vol 12, No. 4, 2004

42. Surgical Rx
• Fusion-in-situ
– Simple
– Ineffective
• Anterior epiphysiodesis & posterior
hemiarthrodesis
– Progressive deformity
– Growing patient
• Combined anterior posterior fusion
• Hemivertebra excision

43. Lonstein, Congenital Spine Deformities, Ortho Clinics of NA, Vol 30, No.3, 1999
Convex Growth Arrest at 2+8

44. Hedequist and Emans, Congenital Scoliosis, Journal of AAOS, Vol 12, No. 4, 2004

45. Lonstein, Congenital Spine Deformities, Ortho Clinics of NA, Vol 30, No.3, 1999
Ant excision of hemivert and
post instrumentation and fusion

50. Congenital Scoliosis with Myelodysplasia

52. Rx with ant discectomies post instr with pedicle
screws at bifid vertebra and Galveston fixation

53. 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

56. Lordosis
• Anterior fusion – small curve
• Anterior / posterior procedure – large
curves

57. 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

58. Lonstein, Congenital Spine Deformities, Ortho Clinics of NA, Vol 30, No.3, 1999

59. 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