Skip to main content
SpringerLink
Log in

Clinical Presentation of Pediatric Chiari I Malformations

  • Chapter
  • First Online:
The Chiari Malformations

Abstract

Clinical presentation of the Chiari I malformation varies with patient age, and children typically present with shorter symptom duration than adults. Symptoms and signs of Chiari I malformation in children are most often attributable to impairment of cerebrospinal fluid (CSF) flow, direct neural compression, and/or syringomyelia. Comparison of contemporary and older literature reveals a trend toward earlier diagnosis, prior to manifestation of significant neurologic morbidity. General availability and use of magnetic resonance imaging likely explains this dramatic shift. Occipital and/or cervical pain is the most common presentation, particularly if Valsalva-induced, followed by scoliosis, syringomyelia, and lower cranial nerve dysfunction. Headache not localized to the occipital/cervical region correlates poorly with Chiari I malformation. The natural history of asymptomatic children with Chiari I malformation (absent syringomyelia) appears benign. Surgical treatment should be reserved for patients with localized pain, syringomyelia, or cervicomedullary/cerebellar dysfunction.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 169.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 249.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Similar content being viewed by others

References

  1. Dyste GN, Menezes AH. Presentation and management of pediatric Chiari malformations without myelodysplasia. Neurosurgery. 1988;23(5):589–97.

    Article  CAS  Google Scholar 

  2. Iskandar B, Oakes WJ. Chiari malformation and syringomyelia. In: Albright AL, Pollack IF, Adelson PD, editors. Principles and practice of pediatric neurosurgery. New York: Thieme; 1999. p. 165–87.

    Google Scholar 

  3. Tubbs RS, Beckman J, Naftel RP, Chern JJ, Wellons JC 3rd, Rozzelle CJ, et al. Institutional experience with 500 cases of surgically treated pediatric Chiari malformation Type I. J Neurosurg Pediatr. 2011;7(3):248–56.

    Google Scholar 

  4. Furuya K, Sano K, Segawa H, Ide K, Yoneyama H. Symptomatic tonsillar ectopia. J Neurol Neurosurg Psychiatry. 1998;64(2):221–6.

    Article  CAS  Google Scholar 

  5. Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C, et al. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 1999;44(5):1005–17.

    Article  CAS  Google Scholar 

  6. Park JK, Gleason PL, Madsen JR, Goumnerova LC, Scott RM. Presentation and management of Chiari I malformation in children. Pediatr Neurosurg. 1997;26(4):190–6.

    Article  CAS  Google Scholar 

  7. Wu YW, Chin CT, Chan KM, Barkovich AJ, Ferriero DM. Pediatric Chiari I malformations: do clinical and radiologic features correlate? Neurology. 1999;53(6):1271–6.

    Article  CAS  Google Scholar 

  8. Alehan FK. Value of neuroimaging in the evaluation of neurologically normal children with recurrent headache. J Child Neurol. 2002;17(11):807–9.

    Article  Google Scholar 

  9. Lewis DW, Dorbad D. The utility of neuroimaging in the evaluation of children with migraine or chronic daily headache who have normal neurological examinations. Headache. 2000;40(8):629–32.

    Article  CAS  Google Scholar 

  10. Schwedt TJ, Guo Y, Rothner AD. “Benign” imaging abnormalities in children and adolescents with headache. Headache. 2006;46(3):387–98.

    Article  Google Scholar 

  11. Strahle J, Muraszko KM, Kapurch J, Bapuraj JR, Garton HJ, Maher CO. Chiari malformation Type I and syrinx in children undergoing magnetic resonance imaging. J Neurosurg Pediatr. 2011;8(2):205–13.

    Article  Google Scholar 

  12. McGirt MJ, Nimjee SM, Floyd J, Bulsara KR, George TM. Correlation of cerebrospinal fluid flow dynamics and headache in Chiari I malformation. Neurosurgery. 2005;56(4):716–21; discussion −21.

    Google Scholar 

  13. Benglis D Jr, Covington D, Bhatia R, Bhatia S, Elhammady MS, Ragheb J, et al. Outcomes in pediatric patients with Chiari Malformation Type I followed up without surgery. J Neurosurg Pediatr. 2011;7(4):375–9.

    Article  Google Scholar 

  14. Isu T, Chono Y, Iwasaki Y, Koyanagi I, Akino M, Abe H, et al. Scoliosis associated with syringomyelia presenting in children. Childs Nerv Syst. 1992;8(2):97–100.

    Article  CAS  Google Scholar 

  15. Wu L, Qiu Y, Wang B, Zhu ZZ, Ma WW. The left thoracic curve pattern: a strong predictor for neural axis abnormalities in patients with “idiopathic” scoliosis. Spine (Phila Pa 1976). 2010;35(2):182–5.

    Article  Google Scholar 

  16. Zadeh HG, Sakka SA, Powell MP, Mehta MH. Absent superficial abdominal reflexes in children with scoliosis. An early indicator of syringomyelia. J Bone Joint Surg Br. 1995;77(5):762–7.

    Article  CAS  Google Scholar 

  17. Lewonowski K, King JD, Nelson MD. Routine use of magnetic resonance imaging in idiopathic scoliosis patients less than eleven years of age. Spine (Phila Pa 1976). 1992;17(6 Suppl):S109–16.

    Article  CAS  Google Scholar 

  18. Maiocco B, Deeney VF, Coulon R, Parks PF, Jr. Adolescent idiopathic scoliosis and the presence of spinal cord abnormalities. Preoperative magnetic resonance imaging analysis. Spine (Phila Pa 1976). 1997;22(21):2537–41.

    Google Scholar 

  19. Brockmeyer D, Gollogly S, Smith JT. Scoliosis associated with Chiari 1 malformations: the effect of suboccipital decompression on scoliosis curve progression: a preliminary study. Spine (Phila Pa 1976). 2003;28(22):2505–9.

    Article  Google Scholar 

  20. Hida K, Iwasaki Y, Koyanagi I, Abe H. Pediatric syringomyelia with chiari malformation: its clinical characteristics and surgical outcomes. Surg Neurol. 1999;51(4):383–90. discussion 90–1

    Article  CAS  Google Scholar 

  21. Muhonen MG, Menezes AH, Sawin PD, Weinstein SL. Scoliosis in pediatric Chiari malformations without myelodysplasia. J Neurosurg. 1992;77(1):69–77.

    Article  CAS  Google Scholar 

  22. Flynn JM, Sodha S, Lou JE, Adams SB Jr, Whitfield B, Ecker ML, et al. Predictors of progression of scoliosis after decompression of an Arnold Chiari I malformation. Spine (Phila Pa 1976). 2004;29(3):286–92.

    Article  Google Scholar 

  23. Ruff ME, Oakes WJ, Fisher SR, Spock A. Sleep apnea and vocal cord paralysis secondary to type I Chiari malformation. Pediatrics. 1987;80(2):231–4.

    CAS  PubMed  Google Scholar 

  24. Wynn R, Goldsmith AJ. Chiari type I malformation and upper airway obstruction in adolescents. Int J Pediatr Otorhinolaryngol. 2004;68(5):607–11.

    Article  Google Scholar 

  25. Nagib MG. An approach to symptomatic children (ages 4–14 years) with Chiari type I malformation. Pediatr Neurosurg. 1994;21(1):31–5.

    Article  CAS  Google Scholar 

  26. Cogan DG. Down-beat nystagmus. Arch Ophthalmol. 1968;80(6):757–68.

    Article  CAS  Google Scholar 

  27. Gingold SI, Winfield JA. Oscillopsia and primary cerebellar ectopia: case report and review of the literature. Neurosurgery. 1991;29(6):932–6.

    Article  CAS  Google Scholar 

  28. Lewis AR, Kline LB, Sharpe JA. Acquired esotropia due to Arnold-Chiari I malformation. J Neuroophthalmol. 1996;16(1):49–54.

    Article  CAS  Google Scholar 

  29. Rosetti P, Ben Taib NO, Brotchi J, De Witte O. Arnold Chiari Type I malformation presenting as a trigeminal neuralgia: case report. Neurosurgery. 1999;44(5):1122–3. discussion 3–4

    Article  CAS  Google Scholar 

  30. Sclafani AP, DeDio RM, Hendrix RA. The Chiari-I malformation. Ear Nose Throat J. 1991;70(4):208–12.

    CAS  PubMed  Google Scholar 

  31. Selmi F, Davies KG, Weeks RD. Type I Chiari deformity presenting with profound sinus bradycardia: case report and literature review. Br J Neurosurg. 1995;9(4):543–5.

    Article  CAS  Google Scholar 

  32. Yglesias A, Narbona J, Vanaclocha V, Artieda J. Chiari type I malformation, glossopharyngeal neuralgia and central sleep apnoea in a child. Dev Med Child Neurol. 1996;38(12):1126–30.

    Article  CAS  Google Scholar 

  33. Dyste GN, Menezes AH, VanGilder JC. Symptomatic Chiari malformations. An analysis of presentation, management, and long-term outcome. J Neurosurg. 1989;71(2):159–68.

    Article  CAS  Google Scholar 

  34. Galarza M, Martinez-Lage JF, Ham S, Sood S. Cerebral anomalies and Chiari type 1 malformation. Pediatr Neurosurg. 2010;46(6):442–9.

    Article  Google Scholar 

  35. Novegno F, Caldarelli M, Massa A, Chieffo D, Massimi L, Pettorini B, et al. The natural history of the Chiari type I anomaly. J Neurosurg Pediatr. 2008;2(3):179–87.

    Article  Google Scholar 

  36. Strahle J, Muraszko KM, Kapurch J, Bapuraj JR, Garton HJ, Maher CO. Natural history of Chiari malformation type I following decision for conservative treatment. J Neurosurg Pediatr. 2011;8(2):214–21.

    Article  Google Scholar 

  37. Singhal A, Bowen-Roberts T, Steinbok P, Cochrane D, Byrne AT, Kerr JM. Natural history of untreated syringomyelia in pediatric patients. Neurosurg Focus. 2011;31(6):1–5.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurosurgery, University of Alabama at Birmingham, Birmingham, AL, USA

    Curtis J. Rozzelle

Authors
  1. Curtis J. Rozzelle
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Curtis J. Rozzelle .

Editor information

Editors and Affiliations

  1. Department of Neurosurgery, Tulane University School of Medicine, New Orleans, LA, USA

    R. Shane Tubbs

  2. Department of Neurosurgery, Aydın Adnan Menderes University School of Medicine, Efeler, Aydın, Turkey

    Mehmet Turgut

  3. Departments of Neurosurgery and Pediatrics, University of Alabama, Birmingham, AL, USA

    W. Jerry Oakes

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Rozzelle, C.J. (2020). Clinical Presentation of Pediatric Chiari I Malformations. In: Tubbs, R., Turgut, M., Oakes, W. (eds) The Chiari Malformations. Springer, Cham. https://doi.org/10.1007/978-3-030-44862-2_29

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-44862-2_29

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-44861-5

  • Online ISBN: 978-3-030-44862-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation