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Scoliosis and Syringomyelia

Scoliosis and Syringomyelia. M.ZERAH Department of Pediatric Neurosurgery. Hopital Necker Enfants-Malades. Université Paris V. France. Scoliosis et syringomyelia. 1933 Allen. Scoliosis and spinal cord tumor 1937 Coonrad. Left thoracic scoliosis

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Scoliosis and Syringomyelia

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  1. Scoliosis and Syringomyelia M.ZERAH Department of Pediatric Neurosurgery. Hopital Necker Enfants-Malades. Université Paris V. France

  2. Scoliosis et syringomyelia • 1933 Allen. Scoliosis and spinal cord tumor • 1937 Coonrad. Left thoracic scoliosis • 1944 Wood. Scoliosis and syringomyelia • 1979 Aboulker Scoliosis and syringomyelia or Syringomyelia and scoliosis • 1983 Baker “Isolated scoliosis” and syringomyelia

  3. Neurosurgeon Point of View Syringomyelia and Scoliosis

  4. Chiari I Chiari II Osseous or ligamental Lesions Achondroplasia Mucopolysaccharidosis Klippel-Feil, osteogenesis imperfecta, Larsen, T21, Hadju-Cheney…. Dandy-Walker et Posterior fossa cyst Craniosynostosis Birth trauma Intracranial Hypertension Tumor, AVM, pseudotumor cerebri, Vein of Galen, Sub dural hematoma, head trauma ... Malformative VS Acquired Hydrodynamic Blocade at the level of the CranioVertebral Junction (62%)

  5. Malformation Diastematomyelia Lipoma Neurenteric cyst Spinal cord compression Spinal tumor Spinal cord tumor Post traumatic syrinx Spinal Arachnoiditis Chiari II Isolated Scoliosis Spinal and spinal cord lesions (38%)

  6. P<0.0001 P<0.05 Our Series (1984 - 1998) Zerah. Neurochirurgie 1999

  7. Our Series (1984 - 1998) 399 syrinx , 313 operated

  8. Chiari I. Initial symptoms

  9. Chiari I (N = 188 ; 87% Scoliosis) • No difference concerning sex, level of chiari, size of the syrinx. • The only difference concerns the age at diagnosis : Scoliosis : Mean = 9,4 years (4 to 17 y) Neurol. Signs : Mean = 6.5 years (2 to 16 y) p < 0.001

  10. Chiari I (N = 188 ; 87% Scoliosis) Chiari + Syrinx in childhood = Surgery Surgery = CVJ decompression* * Except in case of hydrocephalus

  11. Chiari I Chiari I and Scoliosis • Improvement : 15% • Stabilization : 30% • Progression: 55% Prognostic factor of good results (p < 0.01) : Age < 10y and Curves < 40°

  12. 10 days post-op Pre-op Chiari and syringomyelia

  13. Chiari ?

  14. Chiari and or syrinx are symptomatic CVJ surgery Chiari and syrinx are asymptomatic Surveillance and MRI Low spinal deterioration Untethering ? No neurological deterioration, but deterioration of the scoliosis If spine surgery, discussion If orthopedic treatmentSurveillance Neurological and scoliosis deterioration Neurosurgery. CVJ and or untethering ? Chiari II (MMC). N = 44 (87% Scoliosis) Never forget that shunt dysfunction is the first cause of deterioration in MMC

  15. Chiari II

  16. Arachnoiditis

  17. Syrinx and Birth injury

  18. Frequency • 106 adults with syrinx • 54 history of birth injury B. Williams (1979)

  19. Birth trauma Progressive upper spinal cord deterioration (often delayed in adulthood) Syrinx without chiari related to an arachnoiditis of the cisterna magna Foramen magnum surgery (KT/V4/SAS) Neurological and spinal stabilisation (O surgery for scoliosis) Obstetrical syrinx N = 12 (42% scoliosis)

  20. Syrinx and Diastematomyelia

  21. Syrinx and Diastematomyelia

  22. Isolated syrinxN = 68 (100% Scoliosis) • Scoliosis +/- minimal neurological signs • Dorsal or lumbar syrinx. Never cervical • Never “under pressure syrinx” • Never evolutive • Needs one or two control MRI (one with gadolinium) • Never needs neurosurgery • The presence of such a cavity must not modify themanagement of the scoliosis.

  23. Syrinx et Isolated scoliosis (n = 68)

  24. Syrinx Isolated scoliosis (n = 68)

  25. Isolated scoliosis and Syringomyelia

  26. The Orthopedic (Spinal) Surgeon point of view Scoliosis and Syringomyelia

  27. 3 Main Questions • What is the real risk to have a “Neurologic Scoliosis” in front of a “Adolescent Idiopathic Scoliosis (AIS)” ? • Does it need a systematic neurosurgical surgery (prior to the scoliosis one). Does it improve the risk of scoliosis surgery ? • What is the real impact on the Scoliosis Progression ?

  28. What is the real risk to have a “Neurologic Scoliosis” in front of a “Adolescent Idiopathic Scoliosis (AIS)” ?

  29. Idiopathic Scoliosis • 500 000 Scoliosis in US. 125 000 in France • Idiopathic Scoliosis : • No Spinal Malformation or lesion • No Neurological or Muscular diseases • Usually in adolescent girl • 65 % Idiopathic : 330 000 in US. 40 000 in France • How many are Neurologic ? Who needs an MR ?

  30. Scoliosis et syringomyelia • Systematic MRI : 1 to 4% of syrinx associated to scoliosis • Predicting factor : • Left scoliosis or one curve • < 10 y • Abolition of the abdominal cutaneous reflexes

  31. Scoliosis, pain et spinal or spinal cord lesions 33 left thoracic scoliosis, or with one neurological sign 2442 “idiopathic scoliosis” 770 (32%) painfull scoliosis 8 Spinal or spinal cord lesion 20 spondylolysis or spondylolystesis 8 Scheuermann 6 syringomyelias 2 disc hernia 1 tethered cord N = 48 1 spinal cord tumor Ramirez (1997)

  32. Risk of having a positive MR Agreement between test & MRI 75 %. Specificity 74 %. Sensitivity 82 % Morcuende Spine 2003

  33. Apical lordosis was present in 97% of children with AIS and normal MR but absent in 75 % in case of syringomyelia (n) 93) Left curve (p < 0.0001) Male predominance (p<0.001) Sagittal Plane deformity (Dickson deformity) Ouellet. Spine 2003

  34. AIS. Familial Genetic disease ? • 71 patients with AIS • 9 (13%) showed neurologic abnormality in MRI (Syrinx and/ or Chiari or tonsillar ectopia) • Among the relative of these patients 4 /15 affected with scoliosis also showed neurologic abnormality on MR Inoue. Spine 2003

  35. Does it need a systematic neurosurgical surgery (prior to the scoliosis one). Does it improve the risk of scoliosis surgery ?

  36. No correlation between the degree of tonsillar descent and scoliosis progression No correlation between the configuration of syrinx and scoliosis progression Chiari, Scoliosis and Syrinx P < O.O5 Ono. Spine. 2002

  37. Risk of permanent deficit after scoliosis surgery without previous FMD in case of Chiari • Most of the authors are in favor of treatment of Syrinx (Chiari ?) prior to Scoliosis surgery (PSAANS, ISPN) • Few prospective studies • Inoue . Spine. 2004. Prospective study (N = 250) • 44 MRI abnormalities • 12 Neurological signs = FMD = No post-op complications • 32 asymptomatic = No FMD = 1 transient deficit • “patients with neurogically asymptomatic hindbrain and spinal cord abnormalities have little risk of neurologic complications as a result of scoliosis surgery even if these patients show neural axis malformations on MRI”

  38. What is the real impact on the Scoliosis Progression ?

  39. Value of treating primary cause of syrinx in scoliosis associated with syringomyelia • Arnold Chiari I • Suboccipital decompression : 7/12 • Syrinx shunting 0 /2 • All the 7 children improved were under 10 • Myelomeningocele 0/26 • Congenital Scoliosis 0/22 Ozerdemoglu. Spine 2003

  40. Effect of FMD on scoliosis • 31% Improvement/ 31 % Stabilization / 38% Progression (Brockmeyer 2003) • 8I / 1S / 2 P (Muhonen 1992) • 6 I + S / 10 P (Sengupta 2000) • 5 I / 14 S + P (Eule 2002) • 1 I / 1 S / 5 P (Ghanem1997) Main factor of good results : Age < 10y and Curves < 40°

  41. Conclusion • Idiopathic scoliosis in case of pain and /or neurological signs and/or abnormal X-Rays (left, kyphosis…) must have an MRI • The consensus is still in favor of neurosurgery prior to spine surgery but … • It is difficult to appraise the real impact of this surgery on the progresion of the scoliosis • Progress on the understanding of the “primum movens” of the scoliosis

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