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Chiari I malformations with syringomyelia: long-term results of neurosurgical decompression

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Abstract

Study design

Retrospective case series.

Objectives

The objective was to assess the long-term outcomes on scoliosis following Chiari-I (CM-I) decompression in patients with CM-I and syringomyelia (SM). A secondary objective was to identify risk factors of scoliosis progression.

Background

The association between CM-I with SM and scoliosis is recognized, but it remains unclear if CM-I decompression alters the long-term evolution of scoliosis in patients with associated syringomyelia.

Methods

A retrospective review of children with scoliosis, CM-I, and SM during 1997–2015 was performed. Congenital, syndromic, and neuromuscular scoliosis were excluded. Clinical and radiographic characteristics were recorded at presentation, pre-decompression, after 1-year, and latest follow-up. A scale to measure syringomyelia area on MRI was used to evaluate SM changes post-decompression.

Results

65 children with CM-I, SM, and scoliosis and a mean age of 8.9 years (range 0.7–15.8) were identified. Mean follow-up was 6.9 years (range 2.0–20.4). Atypical curves were present in 28 (43%) children. Thirty-eight patients (58%) underwent decompression before 10 years. Syringomyelia size reduced a mean of 70% after decompression (p < 0.001). Scoliosis improved in 26 (40%), stabilized in 17 (26%), and progressed in 22 (34%) cases. Early spinal fusion was required in 7 (11%) patients after a mean of 0.5 ± 0.37 years and delayed fusion in 16 (25%) patients after 6.0 ± 3.24 years. The remaining 42 (65%) patients were followed for a median of 6.1 years (range 2.0–12.3) without spine instrumentation or fusion. Fusion patients experienced less improvement in curve magnitude 1-year post-decompression (p < 0.001) and had larger curves at presentation (43° vs. 34°; p = 0.004).

Conclusions

Syringomyelia size decreased by 70% after CM-I decompression and scoliosis stabilized or improved in two-thirds of patients. Greater curve improvement within the first year post-decompression and smaller curves at presentation decreased the risk of spinal fusion. Neurosurgical decompression is recommended in children with CM-I, SM, and scoliosis with the potential to treat all three conditions.

Level of evidence

Level IV.

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References

  1. Arai S, Ohtsuka Y, Moriya H et al (1993) Scoliosis associated with syringomyelia. Spine (Phila Pa 1976) 18(12):1591–1592

    Article  CAS  Google Scholar 

  2. Blake NS, Lynch AS, Dowling FE (1986) Spinal cord abnormalities in congenital scoliosis. Ann Radiol (Paris) 29(3–4):377–379

    CAS  Google Scholar 

  3. Bradford DS, Heithoff KB, Cohen M (1991) Intraspinal abnormalities and congenital spine deformities: a radiographic and MRI study. J Pediatr Orthop 11(1):36–41

    Article  CAS  Google Scholar 

  4. Nokes SR, Murtagh FR, Jones JD 3rd et al (1987) Childhood scoliosis: MR imaging. Radiology 164(3):791–797. https://doi.org/10.1148/radiology.164.3.3615882

    Article  CAS  PubMed  Google Scholar 

  5. Strahle J, Smith BW, Martinez M et al (2015) The association between Chiari malformation Type I, spinal syrinx, and scoliosis. J Neurosurg Pediatr 15(6):607–611. https://doi.org/10.3171/2014.11.peds14135

    Article  PubMed  Google Scholar 

  6. Jankowski PP, Bastrom T, Ciacci JD et al (2016) Intraspinal pathology associated with pediatric scoliosis: a ten-year review analyzing the effect of neurosurgery on scoliosis curve progression. Spine (Phila Pa 1976) 41(20):1600–1605. https://doi.org/10.1097/brs.0000000000001559

    Article  Google Scholar 

  7. Kelly MP, Guillaume TJ, Lenke LG (2015) Spinal deformity associated with chiari malformation. Neurosurg Clin N Am 26(4):579–585. https://doi.org/10.1016/j.nec.2015.06.005

    Article  PubMed  PubMed Central  Google Scholar 

  8. Krieger MD, Falkinstein Y, Bowen IE et al (2011) Scoliosis and Chiari malformation Type I in children. J Neurosurg Pediatr 7(1):25–29. https://doi.org/10.3171/2010.10.peds10154

    Article  PubMed  Google Scholar 

  9. Eule JM, Erickson MA, O’Brien MF et al (2002) Chiari I malformation associated with syringomyelia and scoliosis: a twenty-year review of surgical and nonsurgical treatment in a pediatric population. Spine (Phila Pa 1976) 27(13):1451–1455

    Article  Google Scholar 

  10. Huebert HT, MacKinnon WB (1969) Syringomyelia and scoliosis. J Bone Jt Surg Br 51(2):338–343

    Article  CAS  Google Scholar 

  11. Oldfield EH, Muraszko K, Shawker TH et al (1994) Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg 80(1):3–15. https://doi.org/10.3171/jns.1994.80.1.0003

    Article  CAS  PubMed  Google Scholar 

  12. Qiu Y, Zhu Z, Wang B et al (2008) Radiological presentations in relation to curve severity in scoliosis associated with syringomyelia. J Pediatr Orthop 28(1):128–133. https://doi.org/10.1097/bpo.0b013e31815ff371

    Article  PubMed  Google Scholar 

  13. Sha S, Li Y, Qiu Y et al (2017) Posterior fossa decompression in Chiari I improves denervation of the paraspinal muscles. J Neurol Neurosurg Psychiatry. https://doi.org/10.1136/jnnp-2016-315161

    Article  PubMed  Google Scholar 

  14. Zhu Z, Qiu Y, Wang B et al (2007) Abnormal spreading and subunit expression of junctional acetylcholine receptors of paraspinal muscles in scoliosis associated with syringomyelia. Spine (Phila Pa 1976) 32(22):2449–2454. https://doi.org/10.1097/brs.0b013e3181573d01

    Article  Google Scholar 

  15. Attenello FJ, McGirt MJ, Atiba A et al (2008) Suboccipital decompression for Chiari malformation-associated scoliosis: risk factors and time course of deformity progression. J Neurosurg Pediatr 1(6):456–460. https://doi.org/10.3171/ped/2008/1/6/456

    Article  PubMed  Google Scholar 

  16. Brockmeyer D, Gollogly S, Smith JT (2003) Scoliosis associated with Chiari 1 malformations: the effect of suboccipital decompression on scoliosis curve progression: a preliminary study. Spine (Phila Pa 1976) 28(22):2505–2509. https://doi.org/10.1097/01.brs.0000092381.05229.87

    Article  Google Scholar 

  17. Hwang SW, Samdani AF, Jea A et al (2012) Outcomes of Chiari I-associated scoliosis after intervention: a meta-analysis of the pediatric literature. Childs Nerv Syst 28(8):1213–1219. https://doi.org/10.1007/s00381-012-1739-3

    Article  PubMed  Google Scholar 

  18. Sengupta DK, Dorgan J, Findlay GF (2000) Can hindbrain decompression for syringomyelia lead to regression of scoliosis? Eur Spine J 9(3):198–201

    Article  CAS  Google Scholar 

  19. Yeom JS, Lee CK, Park KW et al (2007) Scoliosis associated with syringomyelia: analysis of MRI and curve progression. Eur Spine J 16(10):1629–1635. https://doi.org/10.1007/s00586-007-0472-1

    Article  PubMed  PubMed Central  Google Scholar 

  20. Ravindra VM, Onwuzulike K, Heller RS et al (2018) Chiari-related scoliosis: a single-center experience with long-term radiographic follow-up and relationship to deformity correction. J Neurosurg Pediatr 21(2):185–189. https://doi.org/10.3171/2017.8.PEDS17318

    Article  PubMed  Google Scholar 

  21. Ghanem IB, Londono C, Delalande O et al (1997) Chiari I malformation associated with syringomyelia and scoliosis. Spine (Phila Pa 1976) 22(12):1313–1317 (discussion 1318)

    Article  CAS  Google Scholar 

  22. Flynn JM, Sodha S, Lou JE et al (2004) Predictors of progression of scoliosis after decompression of an Arnold Chiari I malformation. Spine (Phila Pa 1976) 29(3):286–292

    Article  Google Scholar 

  23. Muhonen MG, Menezes AH, Sawin PD et al (1992) Scoliosis in pediatric Chiari malformations without myelodysplasia. J Neurosurg 77(1):69–77. https://doi.org/10.3171/jns.1992.77.1.0069

    Article  CAS  PubMed  Google Scholar 

  24. Ozerdemoglu RA, Transfeldt EE, Denis F (2003) Value of treating primary causes of syrinx in scoliosis associated with syringomyelia. Spine (Phila Pa 1976) 28(8):806–814

    Google Scholar 

  25. Spiegel DA, Flynn JM, Stasikelis PJ et al (2003) Scoliotic curve patterns in patients with Chiari I malformation and/or syringomyelia. Spine (Phila Pa 1976) 28(18):2139–2146. https://doi.org/10.1097/01.brs.0000084642.35146.ec

    Article  Google Scholar 

  26. Kontio K, Davidson D, Letts M (2002) Management of scoliosis and syringomyelia in children. J Pediatr Orthop 22(6):771–779

    PubMed  Google Scholar 

  27. Tubbs RS, Lyerly MJ, Loukas M et al (2007) The pediatric Chiari I malformation: a review. Childs Nerv Syst 23(11):1239–1250. https://doi.org/10.1007/s00381-007-0428-0

    Article  Google Scholar 

  28. Alzate JC, Kothbauer KF, Jallo GI et al (2001) Treatment of Chiari I malformation in patients with and without syringomyelia: a consecutive series of 66 cases. Neurosurg Focus 11(1):E3

    Article  CAS  Google Scholar 

  29. Schijman E, Steinbok P (2004) International survey on the management of Chiari I malformation and syringomyelia. Childs Nerv Syst 20(5):341–348. https://doi.org/10.1007/s00381-003-0882-2

    Article  PubMed  Google Scholar 

  30. Ozerdemoglu RA, Denis F, Transfeldt EE (2003) Scoliosis associated with syringomyelia: clinical and radiologic correlation. Spine (Phila Pa 1976) 28(13):1410–1417. https://doi.org/10.1097/01.brs.0000067117.07325.86

    Article  Google Scholar 

  31. Navarro R, Olavarria G, Seshadri R et al (2004) Surgical results of posterior fossa decompression for patients with Chiari I malformation. Childs Nerv Syst 20(5):349–356. https://doi.org/10.1007/s00381-003-0883-1

    Article  PubMed  Google Scholar 

  32. Park JK, Gleason PL, Madsen JR et al (1997) Presentation and management of Chiari I malformation in children. Pediatr Neurosurg 26(4):190–196

    Article  CAS  Google Scholar 

  33. Bhangoo R, Sgouros S (2006) Scoliosis in children with Chiari I-related syringomyelia. Childs Nerv Syst 22(9):1154–1157. https://doi.org/10.1007/s00381-006-0090-y

    Article  PubMed  Google Scholar 

  34. Charry O, Koop S, Winter R et al (1994) Syringomyelia and scoliosis: a review of twenty-five pediatric patients. J Pediatr Orthop 14(3):309–317

    Article  CAS  Google Scholar 

  35. Farley FA, Puryear A, Hall JM et al (2002) Curve progression in scoliosis associated with Chiari I malformation following suboccipital decompression. J Spinal Disord Tech 15(5):410–414

    Article  Google Scholar 

  36. Zhu Z, Wu T, Sha S et al (2013) Is curve direction correlated with the dominant side of tonsillar ectopia and side of syrinx deviation in patients with single thoracic scoliosis secondary to Chiari malformation and syringomyelia? Spine (Phila Pa 1976) 38(8):671–677. https://doi.org/10.1097/brs.0b013e3182796ec5

    Article  Google Scholar 

  37. Zhu Z, Yan H, Han X et al (2016) Radiological features of scoliosis in Chiari I malformation without syringomyelia. Spine (Phila Pa 1976) 41(5):E276–E281. https://doi.org/10.1097/brs.0000000000001406

    Article  Google Scholar 

  38. Mackel CE, Cahill PJ, Roguski M et al (2016) Factors associated with spinal fusion after posterior fossa decompression in pediatric patients with Chiari I malformation and scoliosis. J Neurosurg Pediatr 25(6):737–743. https://doi.org/10.3171/2016.5.peds16180

    Article  PubMed  Google Scholar 

  39. Zhu Z, Wu T, Zhou S et al (2013) Prediction of curve progression after posterior fossa decompression in pediatric patients with scoliosis secondary to Chiari malformation. Spine Deform 1(1):25–32. https://doi.org/10.1016/j.jspd.2012.07.005

    Article  PubMed  Google Scholar 

  40. Nagib MG (1994) An approach to symptomatic children (ages 4–14 years) with Chiari type I malformation. Pediatr Neurosurg 21(1):31–35

    Article  CAS  Google Scholar 

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Funding

No funding was received for this study.

Author information

Authors and Affiliations

  1. Department of Orthopaedic Surgery, Boston Children’s Hospital (Harvard Teaching Hospital), Boston, MA, USA

    Bram P. Verhofste, Eric A. Davis, Patricia E. Miller, Michael T. Hresko, John B. Emans, Lawrence I. Karlin, Daniel J. Hedequist & Brian D. Snyder

  2. Department of Neurosurgery, Boston Children’s Hospital (Harvard Teaching Hospital), Boston, MA, USA

    Edward R. Smith & Mark R. Proctor

  3. Department of Orthopaedic Surgery, University Hospital Cleveland Medical Center, Cleveland, OH, USA

    Michael P. Glotzbecker

  4. Department of Orthopaedic Surgery, Rainbow Babies and Children’s Hospital, Cleveland, OH, USA

    Michael P. Glotzbecker

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  1. Bram P. Verhofste
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  2. Eric A. Davis
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  4. Michael T. Hresko
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Contributions

All authors: substantial contributions to the conception or design of the work; or the acquisition, analysis, or interpretation of data for the work, drafting the work, or revising it critically for important intellectual content and final approval of the version to be published.

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Correspondence to Michael P. Glotzbecker.

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Investigation performed at: Department of Orthopaedic Surgery, Boston Children’s Hospital (Harvard Teaching Hospital), Boston, MA, USA

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Verhofste, B.P., Davis, E.A., Miller, P.E. et al. Chiari I malformations with syringomyelia: long-term results of neurosurgical decompression. Spine Deform 8, 233–243 (2020). https://doi.org/10.1007/s43390-019-00009-z

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