Skip to main content

Surgical Treatment in Symptomatic Chiari Malformation Type I: A Series of 25 Adult Patients Treated with Cerebellar Tonsil Shrinkage

  • Chapter
  • First Online:
New Trends in Craniovertebral Junction Surgery

Abstract

Background: The variety of symptoms and radiological findings in patients with Chiari malformation type I makes both the indication for surgery and the technical modality controversial. We report our 5-year experience, describing our technique and critically evaluating the clinical results.

Methods: Between 2012 and 2016, 25 patients (15 female and 10 male; mean age 39.2 years) underwent posterior fossa decompression for Chiari malformation type I. Their clinical complaints included headache, nuchalgia, upper limb weakness or numbness, instability, dizziness and diplopia. Syringomyelia was present in 12 patients (48%). Suboccipital craniectomy was completed in all cases with C1 laminectomy and shrinkage of the cerebellar tonsils by bipolar coagulation; duraplasty was performed with a suturable dura substitute.

Results: Gratifying results were observed in our series. Symptoms and signs were resolved in 52% of patients, and 20% of patients had an improvement in their preoperative deficits. The symptoms of six patients (24%) were essentially unchanged, and one patient (4%) deteriorated despite undergoing surgery. Generally, patients with syringomyelia on magnetic resonance imaging (MRI) showed less symptomatic improvement after surgery. The syrinx disappeared in seven of 12 patients, and complications occurred in three patients (12%).

Conclusion: Cerebellar tonsil reduction and restoration of cerebrospinal fluid (CSF) circulation provided clinical improvement and a stable reduction in the syrinx size in the vast majority of treated patients, with a low rate of complications.

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 189.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
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

References

  1. Chiari H. Über Veränderungen des Kleinhirns, des Pons und der Medulla oblongata in Folge von congenitaler Hydrocephalie des Grosshirns. Wien: K.K. Hof- und Staatsdruckerei; 1896.

    Google Scholar 

  2. Rocque BG, Oakes WJ. Surgical treatment of Chiari I malformation. Neurosurg Clin N Am. 2015;26:527–31.

    Article  Google Scholar 

  3. Elster AD, Chen MY. Chiari I malformations: clinical and radiologic reappraisal. Radiology. 1992;183:347–53.

    Article  CAS  Google Scholar 

  4. Fernández AA, Guerrero AI, Martínez MI, Vázquez ME, Fernández JB, Chesa i Octavio E, et al. Malformations of the craniocervical junction (Chiari type I and syringomyelia: classification, diagnosis and treatment). BMC Musculoskelet Disord. 2009;10(Suppl 1):S1.

    Article  Google Scholar 

  5. Meadows J, Kraut M, Guarnieri M, Haroun RI, Carson BS. Asymptomatic Chiari type I malformations identified on magnetic resonance imaging. J Neurosurg. 2000;92:920–6.

    Article  CAS  Google Scholar 

  6. Nishikawa M, Sakamoto H, Hakuba A, Nakanishi N, Inoue Y. Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa. J Neurosurg. 1997;86:40–7.

    Article  CAS  Google Scholar 

  7. Anson JA, Benzel EC, Awad IA, AANS Publications Committee. Syringomyelia and the Chiari malformations. Park Ridge: American Association of Neurological Surgeons; 1997.

    Google Scholar 

  8. Hwang HS, Moon JG, Kim CH, Oh SM, Song JH, Jeong JH. The comparative morphometric study of the posterior cranial fossa: what is effective approaches to the treatment of Chiari malformation type 1? J Korean Neurosurg Soc. 2013;54:405–10.

    Article  Google Scholar 

  9. Fernandes YB, Ramina R, Campos-Herrera CR, Borges G. Evolutionary hypothesis for Chiari type I malformation. Med Hypotheses. 2013;81:715–9.

    Article  Google Scholar 

  10. Furtado SV, Reddy K, Hegde AS. Posterior fossa morphometry in symptomatic pediatric and adult Chiari I malformation. J Clin Neurosci. 2009;16:1449–54.

    Article  Google Scholar 

  11. Milhorat TH, Nishikawa M, Kula RW, Dlugacz YD. Mechanisms of cerebellar tonsil herniation in patients with Chiari malformations as guide to clinical management. Acta Neurochir. 2010;152:1117–27.

    Article  Google Scholar 

  12. Schijman E. History, anatomic forms, and pathogenesis of Chiari I malformations. Childs Nerv Syst. 2004;20:323–8.

    Article  Google Scholar 

  13. Briganti F, Leone G, Briganti G, Orefice G, Caranci F, Maiuri F. Spontaneous resolution of Chiari type 1 malformation. A case report and literature review. Neuroradiol J. 2013;26:304–9.

    Article  CAS  Google Scholar 

  14. Chavez A, Roguski M, Killeen A, Heilman C, Hwang S. Comparison of operative and non-operative outcomes based on surgical selection criteria for patients with Chiari I malformations. J Clin Neurosci. 2014;21:2201–6.

    Article  Google Scholar 

  15. Sindou M, Chávez-Machuca J, Hashish H. Cranio-cervical decompression for Chiari type I-malformation, adding extreme lateral foramen magnum opening and expansile duroplasty with arachnoid preservation. Technique and long-term functional results in 44 consecutive adult cases—comparison with literature data. Acta Neurochir. 2002;144:1005–19.

    CAS  PubMed  Google Scholar 

  16. Blagodatsky MD, Larionov SN, Alexandrov YA, Velm AI. Surgical treatment of Chiari I malformation with or without syringomyelia. Acta Neurochir. 1999;141:963–8.

    Article  CAS  Google Scholar 

  17. Ellenbogen RG, Armonda RA, Shaw DW, Winn HR. Toward a rational treatment of Chiari I malformation and syringomyelia. Neurosurg Focus. 2000;8:E6.

    CAS  PubMed  Google Scholar 

  18. Sahuquillo J, Rubio E, Poca MA, Rovira A, Rodriguez-Baeza A, Cervera C. Posterior fossa reconstruction: a surgical technique for the treatment of Chiari I malformation and Chiari I/syringomyelia complex—preliminary results and magnetic resonance imaging quantitative assessment of hindbrain migration. Neurosurgery. 1994;35:874–85.

    Article  CAS  Google Scholar 

  19. Sakamoto H, Nishikawa M, Hakuba A, Yasui T, Kitano S, Nakanishi N, Inoue Y. Expansive suboccipital cranioplasty for the treatment of syringomyelia associated with Chiari malformation. Acta Neurochir. 1999;141:949–61.

    Article  CAS  Google Scholar 

  20. Vanaclocha V, Saiz-Sapena N. Duraplasty with freeze-dried cadaveric dura versus occipital pericranium for Chiari type I malformation: comparative study. Acta Neurochir. 1997;139:112–9.

    Article  CAS  Google Scholar 

  21. Vanaclocha V, Saiz-Sapena N, Garcia-Casasola MC. Surgical technique for cranio-cervical decompression in syringomyelia associated with Chiari type I malformation. Acta Neurochir. 1997;139:529–40.

    Article  CAS  Google Scholar 

  22. Klekamp J, Batzdorf U, Samii M, Bothe HW. The surgical treatment of Chiari I malformation. Acta Neurochir. 1996;138:788–801.

    Article  CAS  Google Scholar 

  23. Pillay PK, Awad IA, Little JR, Hahn JF. Symptomatic Chiari malformation in adults: a new classification based on magnetic resonance imaging with clinical and prognostic significance. Neurosurgery. 1991;28:639–45.

    Article  CAS  Google Scholar 

  24. Versari PP, D’Aliberti G, Talamonti G, Collice M. Foraminal syringomyelia: suggestion for a grading system. Acta Neurochir. 1993;125:97–104.

    Article  CAS  Google Scholar 

  25. Fischer EG. Posterior fossa decompression for Chiari I deformity, including resection of the cerebellar tonsils. Childs Nerv Syst. 1995;11:625–9.

    Article  CAS  Google Scholar 

  26. Guyotat J, Bret P, Jouanneau E, Ricci AC, Lapras C. Syringomyelia associated with type I Chiari malformation. A 21-year retrospective study on 75 cases treated by foramen magnum decompression with a special emphasis on the value of tonsils resection. Acta Neurochir. 1998;140:745–54.

    Article  CAS  Google Scholar 

  27. Raftopoulos C. Surgical treatment of syringomyelia based on magnetic resonance imaging criteria. Neurosurgery. 1993;33:535–6.

    CAS  PubMed  Google Scholar 

  28. Kalb S, Perez-Orribo L, Mahan M, Theodore N, Nakaji P, Bristol RE. Evaluation of operative procedures for symptomatic outcome after decompression surgery for Chiari type I malformation. J Clin Neurosci. 2012;19:1268–72.

    Article  Google Scholar 

  29. Pryse-Phillips W. Evaluating migraine disability: the headache impact test instrument in context. Can J Neurol Sci. 2002;29(Suppl 2):S11–5.

    Article  Google Scholar 

  30. Caldarelli M, Novegno F, Vassimi L, Romani R, Tamburrini G, Di Rocco C. The role of limited posterior fossa craniectomy in the surgical treatment of Chiari malformation type I: experience with a pediatric series. J Neurosurg. 2007;106:187–95.

    PubMed  Google Scholar 

  31. Chauvet D, Carpentier A, George B. Dura splitting decompression in Chiari type 1 malformation: clinical experience and radiological findings. Neurosurg Rev. 2009;32:465–70.

    Article  Google Scholar 

  32. Durham SR, Fjeld-Olenec K. Comparison of posterior fossa decompression with and without duraplasty for the surgical treatment of Chiari malformation type I in pediatric patients: a meta-analysis. J Neurosurg Pediatr. 2008;2:42–9.

    Article  Google Scholar 

  33. Limonadi FM, Selden NR. Dura-splitting decompression of the craniocervical junction: reduced operative time, hospital stay, and cost with equivalent early outcome. J Neurosurg. 2004;101:184–8.

    PubMed  Google Scholar 

  34. Xu H, Chu L, He R, Ge C, Lei T. Posterior fossa decompression with and without duraplasty for the treatment of Chiari malformation type I—a systematic review and meta-analysis. Neurosurg Rev. 2017;40(2):213–21.

    Article  Google Scholar 

  35. Galarza M, Sood S, Ham S. Relevance of surgical strategies for the management of pediatric Chiari type I malformation. Childs Nerv Syst. 2007;23:691–6.

    Article  Google Scholar 

  36. Abd-El-Barr M, Groff MW. Less is more: limiting the size of posterior fossa decompressions in Chiari I malformations. World Neurosurg. 2014;81:706–7.

    Article  Google Scholar 

  37. Hankinson T, Tubbs RS, Wellons JC. Duraplasty or not? An evidence-based review of the pediatric Chiari I malformation. Childs Nerv Syst. 2011;27:35–40.

    Article  Google Scholar 

  38. Navarro R, Olavarria G, Seshadri R, Gonzales-Portillo G, McLone DG, Tomita T. Surgical results of posterior fossa decompression for patients with Chiari I malformation. Childs Nerv Syst. 2004;20:349–56.

    Article  Google Scholar 

  39. Hoffman CE, Souweidane MM. Cerebrospinal fluid-related complications with autologous duraplasty and arachnoid sparing in type I Chiari malformation. Neurosurgery. 2008;62:156–61.

    PubMed  Google Scholar 

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

    Article  Google Scholar 

  41. Tubbs RS, Smyth MD, Wellons JC, Oakes WJ. Arachnoid veils and the Chiari I malformation. J Neurosurg. 2004;100:465–7.

    PubMed  Google Scholar 

  42. Abla AA, Link T, Fusco D, Wilson DA, Sonntag VK. Comparison of dural grafts in Chiari decompression surgery: review of the literature. J Craniovertebr Junction Spine. 2010;1:29–37.

    Article  Google Scholar 

  43. Attenello FJ, McGirt MJ, Garcés-Ambrossi GL, Chaichana KL, Carson B, Jallo GI. Suboccipital decompression for Chiari I malformation: outcome comparison of duraplasty with expanded polytetrafluoroethylene dural substitute versus pericranial autograft. Childs Nerv Syst. 2009;25:183–90.

    Article  Google Scholar 

  44. Danish SF, Samdani A, Hanna A, Storm P, Sutton L. Experience with acellular human dura and bovine collagen matrix for duraplasty after posterior fossa decompression for Chiari malformations. J Neurosurg. 2006;104:16–20.

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer International Publishing AG, part of Springer Nature

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Villa, A., Imperato, A., Maugeri, R., Visocchi, M., Iacopino, D.G., Francaviglia, N. (2019). Surgical Treatment in Symptomatic Chiari Malformation Type I: A Series of 25 Adult Patients Treated with Cerebellar Tonsil Shrinkage. In: Visocchi, M. (eds) New Trends in Craniovertebral Junction Surgery. Acta Neurochirurgica Supplement, vol 125. Springer, Cham. https://doi.org/10.1007/978-3-319-62515-7_18

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-62515-7_18

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-62514-0

  • Online ISBN: 978-3-319-62515-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics