Skip to main content
SpringerLink
Log in

Treatment of the Adult Chiari I Malformation

  • Chapter
  • First Online:
The Chiari Malformations

Abstract

Successful surgery for Chiari I malformation in adults requires ­appropriate patient selection, the identification of the optimal procedure for the specific circumstances of that patient, and careful execution of the surgery. The goal of surgery is to reestablish the unrestricted, pulsatile movement of the cerebrospinal fluid in the subarachnoid space at the craniovertebral junction. This is accomplished almost universally by removing the posterior rim of the foramen magnum and the posterior arch of C1, opening the dura, and placing a pericranial graft to expand the dural space at that level.

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 189.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 239.00
Price excludes VAT (USA)
  • Compact, lightweight 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 infolge von congenitaler Hydrocephalie des Grosshirns (Concerning changes in the cerebellum due to hydrocephalus of the cerebrum). Deutsche Medizinische Wochenschrift. 1891;17:1172–5.

    Article  Google Scholar 

  2. Harding BN, Copp AJ. Malformations. In: Graham DI, Lantos PL, editors. Greenfield’s neuropathology. 7th ed. London: Arnold; 2002. p. 357–483.

    Google Scholar 

  3. Mortazavi MM, Tubbs RS, Hankinson TC, Pugh JA, Cohen-Gadol AA, Oakes WJ. The first posterior fossa decompression for Chiari malformation: the contributions of Cornelis Joachimus van Houweninge Graftdijk and a review of the infancy of “Chiari decompression”. Childs Nerv Syst. 2011;27:1851–6.

    Article  PubMed  Google Scholar 

  4. McConnell AA, Parker HL. A deformity of the hind-brain associated with internal hydrocephalus. Its relation to the Arnold-Chiari malformation. Brain. 1938;61:415–29.

    Article  Google Scholar 

  5. Adams RD, Schatzki R, Scovill WB. The Arnold-Chiari malformation. Diagnosis, demonstration by intraspinal Lipiodol and successful surgical treatment. N Engl J Med. 1941;225:125–31.

    Article  Google Scholar 

  6. Bucy PC, Lichtenstein BW. Arnold-Chiari deformity in an adult without obvious cause. J Neurosurg. 1945;2:245–50.

    Article  Google Scholar 

  7. Gardner WJ, Goodall RJ. The surgical treatment of Arnold-Chiari malformation in adults; an explanation of its mechanism and importance of encephalography in diagnosis. J Neurosurg. 1950;7:199–206.

    Article  PubMed  CAS  Google Scholar 

  8. Gardner WJ. Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry. 1965;28:247–59.

    Article  PubMed  CAS  Google Scholar 

  9. Levy WJ, Mason L, Hahn JF. Chiari malformation presenting in adults: a surgical experience in 127 cases. Neurosurgery. 1983;12:377–90.

    Article  PubMed  CAS  Google Scholar 

  10. Pillay PK. Thecoperitoneal shunting for syringomyelia. J Neurosurg. 1991;75:835–6.

    PubMed  CAS  Google Scholar 

  11. Logue V, Edwards M. Syringomyelia and its surgical treatment–an analysis of 75 patients. J Neurol Neurosurg Psychiatry. 1981;44:273–84.

    Article  PubMed  CAS  Google Scholar 

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

    Article  CAS  Google Scholar 

  13. Barbaro NM. Surgery for primarily spinal syringomyelia. In: Batzdorf U, editor. Syringomyelia: current concepts in diagnosis and treatment. Baltimore: Williams and Wilkins; 1991. p. 183–98.

    Google Scholar 

  14. 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  PubMed  Google Scholar 

  15. 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  PubMed  Google Scholar 

  16. Isu T, Sasaki H, Takamura H, Kobayashi N. Foramen magnum decompression with removal of the outer layer of the dura as treatment for syringomyelia ­occurring with Chiari I malformation. Neurosurgery. 1993;33:844–9; discussion 9–50.

    PubMed  CAS  Google Scholar 

  17. Kotil K, Ton T, Tari R, Savas Y. Delamination technique together with longitudinal incisions for treatment of Chiari I/syringomyelia complex: a prospective clinical study. Cerebrospinal Fluid Res. 2009;6:7.

    Article  PubMed  Google Scholar 

  18. Romero FR, Pereira CA. Suboccipital craniectomy with or without duraplasty: what is the best choice in patients with Chiari type 1 malformation? Arq Neuropsiquiatr. 2010;68:623–6.

    Article  PubMed  Google Scholar 

  19. Nyland H, Krogness KG. Size of posterior fossa in Chiari type 1 malformation in adults. Acta Neurochir (Wien). 1978;40:233–42.

    Article  CAS  Google Scholar 

  20. Cinalli G, Spennato P, Sainte-Rose C, et al. Chiari malformation in craniosynostosis. Childs Nerv Syst. 2005;21:889–901.

    Article  PubMed  Google Scholar 

  21. Buxton N, Jaspan T, Punt J. Treatment of Chiari malformation, syringomyelia and hydrocephalus by neuroendoscopic third ventriculostomy. Minim Invasive Neurosurg. 2002;45:231–4.

    Article  PubMed  CAS  Google Scholar 

  22. Decq P, Le Guerinel C, Sol JC, Brugieres P, Djindjian M, Nguyen JP. Chiari I malformation: a rare cause of noncommunicating hydrocephalus treated by third ventriculostomy. J Neurosurg. 2001;95:783–90.

    Article  PubMed  CAS  Google Scholar 

  23. Metellus P, Dufour H, Levrier O, Grisoli F. Endoscopic third ventriculostomy for treatment of noncommunicating syringomyelia associated with a Chiari I malformation and hydrocephalus: case report and pathophysiological considerations. Neurosurgery. 2002;51:500–3; discussion 3–4.

    Article  PubMed  Google Scholar 

  24. Mohanty A, Suman R, Shankar SR, Satish S, Praharaj SS. Endoscopic third ventriculostomy in the management of Chiari I malformation and syringomyelia associated with hydrocephalus. Clin Neurol Neurosurg. 2005;108:87–92.

    Article  PubMed  Google Scholar 

  25. Hayhurst C, Osman-Farah J, Das K, Mallucci C. Initial management of hydrocephalus associated with Chiari malformation Type I-syringomyelia complex via endoscopic third ventriculostomy: an outcome analysis. J Neurosurg. 2008;108:1211–4.

    Article  PubMed  Google Scholar 

  26. Mea E, Chiapparini L, Leone M, Franzini A, Messina G, Bussone G. Chronic daily headache in the adults: differential diagnosis between symptomatic Chiari I malformation and spontaneous intracranial hypotension. Neurol Sci. 2011;32 Suppl 3:291–4.

    Article  Google Scholar 

  27. Udayakumaran S. Post CSF shunting Chiari I malformation-sequel or complication? Hypertension or hypotension? Childs Nerv Syst. 2009;25:1391–2; author reply 3.

    Article  PubMed  Google Scholar 

  28. Puget S, Kondageski C, Wray A, et al. Chiari-like tonsillar herniation associated with intracranial hypotension in Marfan syndrome. Case report. J Neurosurg. 2007;106:48–52.

    PubMed  Google Scholar 

  29. Atkinson JL, Weinshenker BG, Miller GM, Piepgras DG, Mokri B. Acquired Chiari I malformation secondary to spontaneous spinal cerebrospinal fluid leakage and chronic intracranial hypotension syndrome in seven cases. J Neurosurg. 1998;88:237–42.

    Article  PubMed  CAS  Google Scholar 

  30. Kasner SE, Rosenfeld J, Farber RE. Spontaneous intracranial hypotension: headache with a reversible Arnold-Chiari malformation. Headache. 1995;35:557–9.

    Article  PubMed  CAS  Google Scholar 

  31. Agostinis C, Caverni L, Montini M, Pagani G, Bonaldi G. “Spontaneous” reduction of tonsillar herniation in acromegaly: a case report. Surg Neurol. 2000;53:396–9.

    Article  PubMed  CAS  Google Scholar 

  32. Ammerman JM, Goel R, Polin RS. Resolution of Chiari malformation after treatment of acromegaly. Case illustration. J Neurosurg. 2006;104:980.

    Article  PubMed  Google Scholar 

  33. Hara M, Ichikawa K, Minemura K, et al. Acromegaly associated with Chiari-I malformation and polycystic ovary syndrome. Intern Med. 1996;35:803–7.

    Article  PubMed  CAS  Google Scholar 

  34. Lemar Jr HJ, Perloff JJ, Merenich JA. Symptomatic Chiari-I malformation in a patient with acromegaly. South Med J. 1994;87:284–5.

    Article  PubMed  Google Scholar 

  35. Grabb PA, Mapstone TB, Oakes WJ. Ventral brain stem compression in pediatric and young adult patients with Chiari I malformations. Neurosurgery. 1999;44:520–7; discussion 7–8.

    Article  PubMed  CAS  Google Scholar 

  36. Aronson DD, Kahn RH, Canady A, Bollinger RO, Towbin R. Instability of the cervical spine after decompression in patients who have Arnold-Chiari malformation. J Bone Joint Surg Am. 1991;73:898–906.

    PubMed  CAS  Google Scholar 

  37. Fenoy AJ, Menezes AH, Fenoy KA. Craniocervical junction fusions in patients with hindbrain herniation and syringohydromyelia. J Neurosurg Spine. 2008;9:1–9.

    Article  PubMed  Google Scholar 

  38. Vernooij MW, Ikram MA, Tanghe HL, et al. Incidental findings on brain MRI in the general population. N Engl J Med. 2007;357:1821–8.

    Article  PubMed  CAS  Google Scholar 

  39. Heiss JD, Patronas N, DeVroom HL, et al. Elucidating the pathophysiology of syringomyelia. J Neurosurg. 1999;91:553–62.

    Article  PubMed  CAS  Google Scholar 

  40. McGirt MJ, Atiba A, Attenello FJ, et al. Correlation of hindbrain CSF flow and outcome after surgical decompression for Chiari I malformation. Childs Nerv Syst. 2008;24:833–40.

    Article  PubMed  Google Scholar 

  41. Oldfield EH, Muraszko K, Shawker TH, Patronas NJ. Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg. 1994;80:3–15.

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  43. Pillay PK, Awad IA, Little JR, Hahn JF. Surgical management of syringomyelia: a five year experience in the era of magnetic resonance imaging. Neurol Res. 1991;13:3–9.

    PubMed  CAS  Google Scholar 

  44. Wetjen NM, Heiss JD, Oldfield EH. Time course of syringomyelia resolution following decompression of Chiari malformation Type I. J Neurosurg Pediatr. 2008;1:118–23.

    Article  PubMed  Google Scholar 

  45. Sansur CA, Heiss JD, DeVroom HL, Eskioglu E, Ennis R, Oldfield EH. Pathophysiology of headache associated with cough in patients with Chiari I malformation. J Neurosurg. 2003;98:453–8.

    Article  PubMed  Google Scholar 

  46. Vakharia VN, Guilfoyle MR, Laing RJ. Prospective study of outcome of foramen magnum decompressions in patients with syrinx and non-syrinx associated Chiari malformations. Br J Neurosurg. 2012;26(1):7–11.

    Article  PubMed  CAS  Google Scholar 

  47. Rocque BG, George TM, Kestle J, Iskandar BJ. Treatment practices for Chiari malformation type I with syringomyelia: results of a survey of the American Society of Pediatric Neurosurgeons. J Neurosurg Pediatr. 2011;8:430–7.

    Article  PubMed  Google Scholar 

  48. Waldau B, Domeshek LF, Leigh FA, et al. Spontaneous resolution of a 13-mm Chiari malformation Type I in relation to differential growth of the posterior fossa volume. J Neurosurg Pediatr. 2009;3:110–4.

    Article  PubMed  Google Scholar 

  49. Mutchnick IS, Janjua RM, Moeller K, Moriarty TM. Decompression of Chiari malformation with and without duraplasty: morbidity versus recurrence. J Neurosurg Pediatr. 2010;5:474–8.

    Article  PubMed  Google Scholar 

  50. Yeh DD, Koch B, Crone KR. Intraoperative ultrasonography used to determine the extent of surgery necessary during posterior fossa decompression in children with Chiari malformation type I. J Neurosurg. 2006;105:26–32.

    Article  PubMed  Google Scholar 

  51. Kempe LG. Operative neurosurgery. New York: Springer; 1970.

    Google Scholar 

  52. Heiss JD, Suffredini G, Smith R, et al. Pathophysiology of persistent syringomyelia after decompressive craniocervical surgery. Clinical article. J Neurosurg Spine. 2010;13:729–42.

    Article  PubMed  Google Scholar 

  53. Hultman CS, Riski JE, Cohen SR, et al. Chiari malformation, cervical spine anomalies, and neurologic deficits in velocardiofacial syndrome. Plast Reconstr Surg. 2000;106:16–24.

    Article  PubMed  CAS  Google Scholar 

  54. Mazzola CA, Fried AH. Revision surgery for Chiari malformation decompression. Neurosurg Focus. 2003;15:E3.

    Article  PubMed  Google Scholar 

  55. Sacco D, Scott RM. Reoperation for Chiari malformations. Pediatr Neurosurg. 2003;39:171–8.

    Article  PubMed  Google Scholar 

  56. Tubbs RS, Webb DB, Oakes WJ. Persistent syringomyelia following pediatric Chiari I decompression: radiological and surgical findings. J Neurosurg. 2004;100:460–4.

    PubMed  Google Scholar 

  57. Batzdorf U, Klekamp J, Johnson JP. A critical appraisal of syrinx cavity shunting procedures. J Neurosurg. 1998;89:382–8.

    Article  PubMed  CAS  Google Scholar 

  58. Sgouros S, Williams B. A critical appraisal of drainage in syringomyelia. J Neurosurg. 1995;82:1–10.

    Article  PubMed  CAS  Google Scholar 

  59. Takayasu M, Shibuya M, Kouketsu N, Suzuki Y. Rapid enlargement of a syringomyelia cavity following syringo-subarachnoid shunt: case report. Surg Neurol. 1996;45:366–9.

    Article  PubMed  CAS  Google Scholar 

  60. Wester K, Pedersen PH, Krakenes J. Spinal cord damage caused by rotation of a T-drain in a patient with syringoperitoneal shunt. Surg Neurol. 1989;31:224–7.

    Article  PubMed  CAS  Google Scholar 

  61. Munshi I, Frim D, Stine-Reyes R, Weir BK, Hekmatpanah J, Brown F. Effects of posterior fossa decompression with and without duraplasty on Chiari malformation-associated hydromyelia. Neurosurgery. 2000;46:1384–9; discussion 9–90.

    Article  PubMed  CAS  Google Scholar 

  62. Matsumoto T, Symon L. Surgical management of syringomyelia–current results. Surg Neurol. 1989;32:258–65.

    Article  PubMed  CAS  Google Scholar 

  63. Van Calenbergh F, Hoorens G, Van den Bergh R. Syringomyelia: a retrospective study Part II: diagnostic and therapeutic approach. Acta Neurol Belg. 1990;90:100–10.

    PubMed  Google Scholar 

  64. Pare LS, Batzdorf U. Syringomyelia persistence after Chiari decompression as a result of pseudomeningocele formation: implications for syrinx pathogenesis: report of three cases. Neurosurgery. 1998;43:945–8.

    Article  PubMed  CAS  Google Scholar 

  65. Krayenbuhl H. Evaluation of the different surgical approaches in the treatment of syringomyelia. Clin Neurol Neurosurg. 1975;77:111–28.

    PubMed  CAS  Google Scholar 

  66. Lee TC, Gau YL, Lui CC. Treatment of symptomatic syringomyelia with ventriculoperitoneal shunt: report of a case. J Formos Med Assoc. 1992;91:548–51.

    PubMed  CAS  Google Scholar 

  67. Welch K, Shillito J, Strand R, Fischer EG, Winston KR. Chiari I “malformations”–an acquired disorder? J Neurosurg. 1981;55:604–9.

    Article  PubMed  CAS  Google Scholar 

  68. Batzdorf U. Chiari I, malformation with syringomyelia. Evaluation of surgical therapy by magnetic resonance imaging. J Neurosurg. 1988;68:726–30.

    Article  PubMed  CAS  Google Scholar 

  69. Fischbein NJ, Dillon WP, Cobbs C, Weinstein PR. The “presyrinx” state: a reversible myelopathic condition that may precede syringomyelia. AJNR Am J Neuroradiol. 1999;20:7–20.

    PubMed  CAS  Google Scholar 

  70. Jinkins JR, Reddy S, Leite CC, Bazan 3rd C, Xiong L. MR of parenchymal spinal cord signal change as a sign of active advancement in clinically progressive posttraumatic syringomyelia. AJNR Am J Neuroradiol. 1998;19:177–82.

    PubMed  CAS  Google Scholar 

  71. 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  PubMed  CAS  Google Scholar 

  72. Noudel R, Jovenin N, Eap C, Scherpereel B, Pierot L, Rousseaux P. Incidence of basioccipital hypoplasia in Chiari malformation type I: comparative morphometric study of the posterior cranial fossa. Clinical article. J Neurosurg. 2009;111:1046–52.

    Article  PubMed  Google Scholar 

  73. Heiss JD, Oldfield EH. Chiari malformations and syringomyelia. In: Quinones-Hinojosa A, editor. Schmidek & sweet operative neurosurgical techniques: indications, methods, and results. 6th ed. New York: Elsevier; 2012.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Surgical Neurology Branch, NINDS, National Institutes of Health, 10 Center Drive, Room 3D20, MSC-1414, 20892, Bethesda, MD, USA

    John D. Heiss MD

  2. Department of Neurological Surgery, University of Virginia, CDW Room 3530 – Hospital Drive, 800212, 22908-0212, Charlottesville, VA, USA

    Edward H. Oldfield MD, FACS

Authors
  1. John D. Heiss MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Edward H. Oldfield MD, FACS
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Edward H. Oldfield MD, FACS .

Editor information

Editors and Affiliations

  1. Children's Hospital of Alabama, 7th Ave S 1600, Birmingham, 35233-1711, Alabama, USA

    R. Shane Tubbs

  2. The Children's Hospital of Alabama, 7th Ave S 1600, Birmingham, 35233-1711, Alabama, USA

    W. Jerry Oakes

Electronic Supplementary Material

Below is the link to the electronic supplementary material.

(a) Video demonstrating the pistonlike motion of the cerebellar tonsils during the cardiac cycle with descent of the tonsils occurring during cardiac systole (WMV 26488 kb)

(b) Video demonstrating the upper pole of the syrinx and its narrowing during systole and enlargement during diastole (WMV 15940 kb)

Video demonstrating the pistonlike motion of the cerebellar tonsils during the cardiac cycle (WMV 12576 kb)

Rights and permissions

Reprints and permissions

Copyright information

© 2013 Springer Science+Business Media New York

About this chapter

Cite this chapter

Heiss, J.D., Oldfield, E.H. (2013). Treatment of the Adult Chiari I Malformation. In: Tubbs, R., Oakes, W. (eds) The Chiari Malformations. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6369-6_26

Download citation

  • DOI: https://doi.org/10.1007/978-1-4614-6369-6_26

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-6368-9

  • Online ISBN: 978-1-4614-6369-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation