Skip to main content

Advertisement

Log in

Scoliosis in a child with Chiari I malformation and the absence of syringomyelia: case report and a review of the literature

  • Case Report
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Background

Scoliosis is a common finding in children with Chiari I malformation and syringomyelia. The development of scoliosis associated with a Chiari malformation typically has been ascribed to the presence of syringomyelia. Decompression of the hindbrain hernia will often alleviate the syringomyelia and, in many cases, stabilize or reverse the scoliosis.

Case report

We report a child with Chiari I malformation and scoliosis who presented with Valsalva-induced headache/neck pain. MRI revealed no syringomyelia or hydrocephalus. Radiographs revealed that her scoliotic curvature was approximately 13° and was a single levoscoliotic curve. This patient underwent a posterior fossa decompression with duraplasty for her Valsalva-induced pain. Postoperatively, she had resolution of her pain and there has been no progression of her scoliosis at 3 years follow-up.

Prognosis

Intriguingly, and scattered throughout the medical literature, many have noted cases of scoliosis in patients with only a Chiari I malformation and no syringomyelia. Moreover, experimental studies have induced scoliosis in animals after compression of the dorsal columns. After a review of the medical literature regarding a potential cause and effect of herniated hindbrain-induced scoliosis in the absence of syringomyelia, this association although rare, does seem plausible.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Alexander MA, Bunch WH, Ebbeson SOE (1972) Can experimental dorsal rhizotomy produce scoliosis? J Bone Joint Surg 54A:1509–1513

    Google Scholar 

  2. Alzate JC, Kothbauer KF, Jallo GI et al (2001) Treatment of Chiari type I malformation in patients with and without syringomyelia: a consecutive series of 66 cases. Neurosurgical Focus 11:1–5

    Google Scholar 

  3. Arai S, Ohsuka Y, Moriya H et al (1993) Scoliosis associated with syringomyelia. Spine 18:1591–1592

    PubMed  CAS  Google Scholar 

  4. Barrack RL, Whitecloud TS, Burke SW et al (1984) Proprioception in idiopathic scoliosis. Spine 9:681–685

    Article  PubMed  CAS  Google Scholar 

  5. Brockmeyer D, Gollogly S, Smith JT (2003) Scoliosis associated with Chiari I malformations: The effect of suboccipital decompression on scoliosis curve progression. A preliminary study. Spine 28:2505–2509

    Article  PubMed  Google Scholar 

  6. Cheng JCY, Guo X, Sher AHL et al (1999) Correlation between curve severity, somatosensory evoked potentials, and magnetic resonance imaging in adolescent idiopathic scoliosis. Spine 24:1679–1684

    Article  PubMed  CAS  Google Scholar 

  7. Dure LS, Percy AK, Cheek WR et al (1989) Chiari type I malformation in children. J Pediatrics 115:573–576

    Article  CAS  Google Scholar 

  8. Dyste GN, Menezes AH, BVanGilder JC (1989) Symptomatic Chiari malformations. An analysis of presentation, management, and long-term outcome. J Neurosurg 71:159–168

    Article  PubMed  CAS  Google Scholar 

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

    Google Scholar 

  10. Farley FA, Song KM, Birch JG et al (1995) Syringomyelia and scoliosis in children. J Pediatr Ortho 15:187–192

    CAS  Google Scholar 

  11. Fischbein NJ, Dillon WP, Cobbs C et al (1999) The “presyrinx” state: a reversible myelopathic condition that may precede syringomyelia. AJNR Am J Neuroradiol 20:7–20

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  13. Ghanem IB, Londono C, Delalande O et al (1997) Chiari I malformation associated with syringomyelia and scoliosis. Spine 22:1313–1317

    Article  PubMed  CAS  Google Scholar 

  14. Glassman SD, Nazar GB, Dimar JR et al (1994) Neurogenic scoliosis in children. KMA Journal 92:19–24

    CAS  Google Scholar 

  15. Gupta R, Sharma R, Vashisht S et al (1999) Magnetic resonance evaluation of idiopathic scoliosis: a prospective study. Australas Radiol 43:461–465

    Article  PubMed  CAS  Google Scholar 

  16. Hida K, Iwasaki Y, Koyanagi I et al (1999) Pediatric syringomyelia with Chiari malformation: its clinical characteristics and surgical outcomes. Surg Neurol 51:383–391

    Article  PubMed  CAS  Google Scholar 

  17. Jacob P, Rhoton AL (1997) The Chiari I Malformation in Anson JA, Benzel EC, Awad IA (eds) Syringomyelia and the Chiari Malformations. Park Ridge, Illinois, American Association of Neurological Surgeons, pp 57–67

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

    Article  Google Scholar 

  19. Lewonowski K, King JD, Nelson MD (1992) Routine use of magnetic resonance imaging in idiopathic scoliosis patients less than eleven years of age. Spine 17:109–116

    Google Scholar 

  20. Lloyd-Roberts GC, Pincott JR, MacMeniman P et al (1978) Progression in idiopathic scoliosis: a preliminary report of a possible mechanism. J Bone Joint Surg 60:451–460

    Google Scholar 

  21. MacEwen GD (1973) Experimental scoliosis. Clin Orthop 93:69–74

    PubMed  Google Scholar 

  22. Muhonen MG, Menezes AH, Swain PD et al (1992) Scoliosis in pediatric Chiari malformations without myelodysplasia. J Neurosurg 77:69–77

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  24. Nohria V, Oakes WJ (1990–1991) Chiari I malformation: a review of 43 patients. Pediatr Neurosurg 16:222–227

    PubMed  Google Scholar 

  25. Nokes SR, Murtagh FR, Jones JD et al (1987) Childhood scoliosis: mr imaging. Radiology 164:791–797

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  27. Pincott JR, Feview JS, Tafts LF (1984) Scoliosis caused by section of dorsal spinal nerve roots. J Bone Joint Surg 66:27–29

    CAS  Google Scholar 

  28. Piper JG, Menezes AH (1996) Chiari Malformation in the Adult in Menezes AH and Sonntag VKH (eds) Principles of Spinal Surgery New York, McGraw-Hill pp 379–382

  29. Sahlstrand T, Orrtengreen R, Nachemson A(1978) Postural equilibrium in adolescent idiopathic scoliosis. Acta Orthop Scand 49:354–365

    Article  PubMed  CAS  Google Scholar 

  30. Spiegel DA, Flynn JM, Stasikelis PJ et al (2003) Scoliotic curve patterns in patients with Chiari I malformation and/or syringomyelia. Spine 28:2139–2146

    Article  PubMed  Google Scholar 

  31. Suk SI, Song HS, Lee CK (1989) Scoliosis induced by anterior and posterior rhizotomy. Spine 14:692–697

    Article  PubMed  CAS  Google Scholar 

  32. Tubbs RS, Wellons JC, III, Oakes WJ (2002) Asymmetry of tonsillar ectopia in Chiari I malformation. Pediatr Neurosurg 37:199–202

    Article  PubMed  Google Scholar 

  33. Willimas B (1979) Orthopaedic features in the presentation of syringomyelia. J Bone Joint Surg 61:314–323

    Google Scholar 

  34. Winter RB, Lonstein JE, Denis F et al (1991) Prevalence of spinal canal or cord abnormalities in idiopathic, congenital, and neuromuscular scoliosis. Presented at the Scoliosis Research Society Meeting, Minneapolis, MN

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to R. Shane Tubbs.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Tubbs, R.S., Doyle, S., Conklin, M. et al. Scoliosis in a child with Chiari I malformation and the absence of syringomyelia: case report and a review of the literature. Childs Nerv Syst 22, 1351–1354 (2006). https://doi.org/10.1007/s00381-006-0079-6

Download citation

  • Received:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-006-0079-6

Keywords

Navigation