Scoliosis%20and%20Syringomyelia

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Scoliosis and Syringomyelia

• M.ZERAH
• Department of Pediatric Neurosurgery.
• Hopital Necker Enfants-Malades.
• Université Paris V.
• France

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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

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Neurosurgeon Point of View

• Syringomyelia and Scoliosis

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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 ...

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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

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Our Series (1984 - 1998)
Zerah. Neurochirurgie 1999
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Our Series (1984 - 1998)
399 syrinx , 313 operated
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Chiari I. Initial symptoms
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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 lt 0.001
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Chiari I (N 188 87 Scoliosis)
Chiari Syrinx in childhood Surgery
Surgery CVJ decompression
Except in case of hydrocephalus
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Chiari I and Scoliosis
Chiari I

• Improvement 15
• Stabilization 30
• Progression 55

Prognostic factor of good results (p lt 0.01)
Age lt 10y and Curves lt 40
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Chiari I (N 188 87 Scoliosis)
Diagnosis Clinical Exam. MRI

• Clinical S. Evolutivity
• MRI Topography ( C1/C2) Syrinx
  (Evolutivity) Hydrocephalus Associated
  Abnormalities
• CT Bone (CVJ Spine)

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Chiari and syringomyelia
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Chiari ?
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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 treatmentSurveillance
• Neurological and scoliosis deterioration
• Neurosurgery. CVJ and or untethering ?

Never forget that shunt dysfunction is the first
cause of deterioration in MMC
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Chiari II
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Chiari II (n 44)
15 Shunt revision 7 CVJ Decompression 7
untethering 2 Syringoperitoneal shunting
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Arachnoiditis
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Syrinx and Birth injury
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Frequency

• 106 adults with syrinx
• 54 history of birth injury

B. Williams (1979)
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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)

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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)

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Syrinx and Diastematomyelia
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Syrinx and Diastematomyelia
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Syrinx and scoliosis
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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 the
  management of the scoliosis.

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Syrinx et Isolated scoliosis (n 68)
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Syrinx Isolated scoliosis (n 68)
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Isolated scoliosis and Syringomyelia
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The Orthopedic (Spinal) Surgeon point of view

• Scoliosis and Syringomyelia

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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 ?

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

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Scoliosis et syringomyelia

• Systematic MRI 1 to 4 of syrinx associated to
  scoliosis
• Predicting factor
• Left scoliosis or one curve
• lt 10 y
• Abolition of the abdominal cutaneous reflexes

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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)
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Risk of having a positive MR

• 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

Morcuende Spine 2003
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Risk of having a positive MR
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
Morcuende Spine 2003
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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 lt 0.0001)
• Male predominance (plt0.001)

Ouellet. Spine 2003
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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
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P lt 0.005 NS
lt 10y at first visit Inoue,Ozerdemoglu, Brockmeyer,Eule
Curve severity (gt30) Morcuende, Inoue(2004) Inoue (2003)
Left thoracic Morcuende,Inoue (2004), Ono, Spiegel, Ouellet, Brockmeyer, Eule Inoue (2003)
Dicksons sagittal deformity Ouellet
Kyphosis Inoue, Ono, Spiegel, Withaker
Male Inoue, Spiegel, Eule, Ouellet Morcuende
Neurologic deficit Inoue, Morcuende, Ono, Spiegel, Cheng
Headache, neck pain Inoue, Eule Morcuende
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Does it need a systematic neurosurgical surgery
(prior to the scoliosis one). Does it improve the
risk of scoliosis surgery ?
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Chiari, Scoliosis and Syrinx

• No correlation between the degree of tonsillar
  descent and scoliosis progression
• No correlation between the configuration of
  syrinx and scoliosis progression

14 4 2
Right convex 13 2 1
Left convex 1 2 1
P lt O.O5
Ono. Spine. 2002
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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

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What is the real impact on the Scoliosis
Progression ?
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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
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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 lt 10y and
Curves lt 40
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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