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 AboulkerScoliosis and syringomyelia or Syringomyelia and scoliosis 1983 Baker“Isolated scoliosis” and syringomyelia

3. Neurosurgeon Point of View Syringomyelia and Scoliosis

4. Hydrodynamic Blocade at the level of the CranioVertebral Junction (62%) 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...

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

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

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

12. Chiari I (N = 188 ; 87% Scoliosis) Diagnosis = Clinical Exam. + MRI Clinical S. : Evolutivity MRI : Topography ( C1/C2) Syrinx (Evolutivity) Hydrocephalus Associated Abnormalities CTBone (CVJ + Spine)

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

14. Chiari ?

15. Chiari II (MMC). N = 44 (87% Scoliosis) 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 treatment Surveillance Neurological and scoliosis deterioration –Neurosurgery. CVJ and or untethering ? Never forget that shunt dysfunction is the first cause of deterioration in MMC

16. Chiari II

17. Chiari II (n = 44) 15 Shunt revision7 CVJ Decompression 7 untethering2 Syringoperitoneal shunting 15 Shunt revision7 CVJ Decompression 7 untethering2 Syringoperitoneal shunting

18. Arachnoiditis

19. Syrinx and Birth injury

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

21. Frequency 10 to 33% of lesions at the level of the CVJ or the upper spinal cord in autopsy for neonates dead after birth injury (A. Tobwin) 7 panhypopituitarism with traumatic pituitary stalk section associated to a syrinx (5 minimal chiari) : 7 histories of birth injury (K. Fujita)

22. Obstetrical syrinx N = 12 (42% scoliosis) 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)

23. Syrinx and Diastematomyelia

25. Syrinx and scoliosis

26. Isolated syrinx N = 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 the management of the scoliosis.

27. Syrinx et Isolated scoliosis (n = 68)

28. Syrinx Isolated scoliosis (n = 68)

29. Isolated scoliosis and Syringomyelia

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

31. 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 ?

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

33. 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 ?

34. 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

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

36. 86 % if Severe curve despite immature skeletal immaturity and abnormal neurologic examination 32 % if Severe curve despite immature skeletal immaturity and absence of abnormal neurologic examination 29 % if not Severe curve despite immature skeletal immaturity but abnormal neurologic examination 3% if not Severe curve despite immature skeletal immaturity and not abnormal neurologic examination Risk of having a positive MR Morcuende Spine 2003

37. Risk of having a positive MR Morcuende Spine 2003 Severe curve despite skeletal immaturity Nonsevere curve Abnormal Neurologic examination 86 %29 % Normal Neurologic examination 32 %3 % Agreement between test & MRI 75 %. Specificity 74 %. Sensitivity 82 %

38. Sagittal Plane deformity (Dickson deformity) 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) Ouellet. Spine 2003

39. 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

40. P < 0.005NS < 10y at first visitInoue,Ozerdemoglu, Brockmeyer,Eule Curve severity (>30°)Morcuende, Inoue(2004)Inoue (2003) Left thoracicMorcuende,Inoue (2004), Ono, Spiegel, Ouellet, Brockmeyer, Eule Inoue (2003) Dickson’s sagittal deformity Ouellet KyphosisInoue, Ono, Spiegel, Withaker MaleInoue, Spiegel, Eule, Ouellet Morcuende Neurologic deficitInoue, Morcuende, Ono, Spiegel, Cheng … Headache, neck painInoue, EuleMorcuende

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

42. Chiari, Scoliosis and Syrinx 1442 Right convex 1321 Left convex 121 No correlation between the degree of tonsillar descent and scoliosis progression No correlation between the configuration of syrinx and scoliosis progression Ono. Spine. 2002 P < O.O5

43. 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”

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

45. Value of treating primary cause of syrinx in scoliosis associated with syringomyelia Arnold Chiari I –Suboccipital decompression :7/12 –Syrinx shunting0 /2 All the 7 children improved were under 10 Myelomeningocele0/26 Congenital Scoliosis0/22 Ozerdemoglu. Spine 2003

46. 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°

47. 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