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• 1944 Wood. Scoliosis and syringomyelia• 1979 Aboulker Scoliosis and syringomyelia

orSyringomyelia and scoliosis

• 1983 Baker “Isolated scoliosis” and syringomyelia

Neurosurgeon Point of View

Syringomyelia and Scoliosis

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 ...

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

Our Series (1984 - 1998)N Operated %Scoliosis

Chiari I 188 188 87%Chiari I without Syrinx 54 34 38%Chiari II (MMC) 44 22 87%Spinal Cord Tumor 43 43 52%Spinal cord tumorwithout cyst

12 12 37%

Lu mbar dysraphism 74 71 27%Isolated syrinx 34 1 100%Diastematomyelia 15 15 92%Birth injury 12 12 42%Achondroplasia 3 3 0%Mucopolysaccharidosis 5 5 80%Dandy-Walker 3 3 0%

Zerah. Neurochirurgie 1999

P<0.0001

P<0.05

Our Series (1984 - 1998)

N Operated %Scoliosis

Neonatal spinal injury 4 2 100%Craniosynostosis 4 4 0%Neurenteric cysts 3 3 100%Arachnoiditis 2 2 100%Congenital spinal cordsection

2 0 100%

Brain tumor 1 1 0%BrainRadionecrosis 1 1 0%NF I without Chiari 1 0 100%Ventricule Terminalis 3 0 0%Total 442 356 76%

399 syrinx , 313 operated

Chiari I. Initial symptoms

Scoliosis + ModeratedNeurological Signs

85 45%

Isolated scoliosis 38 20%Pyramidal syndrom 30 16%Headaches 11 6%Cranial nerves 9 5%Sphincterian signs 6 3%Pain 6 3%Hydrocephalus 4 2%

188 100%

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 < 0.001p < 0.001

Chiari I (N = 188 ; 87% Scoliosis)

Chiari + Syrinx in childhood = Chiari + Syrinx in childhood = SurgerySurgery

Chiari + Syrinx in childhood = Chiari + Syrinx in childhood = SurgerySurgery

Surgery = CVJ decompression*Surgery = CVJ decompression*Surgery = CVJ decompression*Surgery = CVJ decompression*

* Except in case of hydrocephalus

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°

Chiari and syringomyelia

Pre-op

10 dayspost-op

Chiari ?

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

Never forget that shunt dysfunction is the first cause of deterioration in MMC

Chiari II

Cervical Low dorsal Panmedullary Total

Systematic diagnosis 5 3 4 12Chiari-like deteriorationStridor, swallowing Pb, Sleepapnea, upper limb pain or deficit

4 0 5 9

Low spinal deterioration(Tethering cord Sd)Lower limb pain deficit orspatialité. Bladder Pb

2 4 2 8

Spinal deterioration 2 3 4 9

Mixed deterioration 2 1 3 6

Total 15 11 18 44

Arachnoiditis

Syrinx and Birth injury

Frequency

• 106 adults with syrinx

• 54 history of birth injury

B. Williams (1979)

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)

Syrinx and Diastematomyelia

Withsyrinx

WithoutSyrinx

Scoliosis 14 93% 9 56% p < 0,05Cutaneous signs 12 80% 15 94% NSUrinary troubles 4 27% 4 25% NSNeurological Signs 3 20% 3 19% NSAssociated with another dysraphysm

4 27% 3 19% NS

15 16

Syrinx and Diastematomyelia

QuickTime™ et undécompresseur TIFF (non compressé)sont requis pour visionner cette image.

QuickTime™ et undécompresseur TIFF (non compressé)sont requis pour visionner cette image.

QuickTime™ et undécompresseur TIFF (non compressé)sont requis pour visionner cette image.

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.

Syrinx et Isolated scoliosis (n = 68)

Asymptomatic 36 53 % Aboliti on of cutaneal abdominal reflexes

28 41 %

Sens ory Tr . 20 28 % Pyramidal S. 8 11 % Motor S. 4 6 % Sphinct er Pb 2 2 %

Syrinx Isolated scoliosis (n = 68)

CurveSingle curve 30 44%

Double curve 38 56%

ConvexityRight 40 59%Left 28 41%

Cobb angle> 50° 4 6%

25 - 50° 38 56%< 25° 26 38%

CurveSingle curve 30 44%

Double curve 38 56%

ConvexityRight 40 59%Left 28 41%

Cobb angle> 50° 4 6%

25 - 50° 38 56%< 25° 26 38%

Isolated scoliosisand

Syringomyelia

The Orthopedic (Spinal) Surgeon point of view

Scoliosis and Syringomyelia

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 ?

What is the real risk to have a “Neurologic Scoliosis” in front of a

“Adolescent Idiopathic Scoliosis (AIS)” ?

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 ?

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

Scoliosis, pain et spinal or spinal cord lesions

2442 “idiopathic scoliosis”2442 “idiopathic scoliosis”

770 (32%) painfull scoliosis770 (32%) painfull scoliosis

20 spondylolysis or spondylolystesis8 Scheuermann6 syringomyelias2 disc hernia1 tethered cord N = 481 spinal cord tumor

20 spondylolysis or spondylolystesis8 Scheuermann6 syringomyelias2 disc hernia1 tethered cord N = 481 spinal cord tumor

33 left thoracic scoliosis, or with one

neurological sign

8 Spinal or spinal cord lesion

Ramirez(1997)Ramirez(1997)

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 %

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

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

P < 0.005 NS

< 10y at first visit Inoue,Ozerdemoglu, Brockmeyer,Eule

Curve severity (>30°) Morcuende, Inoue(2004) Inoue (2003)

Left thoracic Morcuende,Inoue (2004), Ono, Spiegel, Ouellet, Brockmeyer, Eule

Inoue (2003)

Dickson’s 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

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

risk of scoliosis surgery ?

Chiari, Scoliosis and Syrinx

14 4 2

Right convex

13 2 1

Left

convex

1 2 1

• 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

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”

What is the real impact on the Scoliosis Progression ?

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

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°

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