Treatment of the Adult Chiari I Malformation

  • John D. Heiss
  • Edward H. OldfieldEmail author


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.


Chiari I Syringomyelia Craniovertebral decompression Duraplasty 

Supplementary material

Operative Video 26.1

(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)

Operative Video 26.2

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

Operative Video 26.3

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


  1. 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.CrossRefGoogle Scholar
  2. 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. 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.PubMedCrossRefGoogle Scholar
  4. 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.CrossRefGoogle Scholar
  5. 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.CrossRefGoogle Scholar
  6. 6.
    Bucy PC, Lichtenstein BW. Arnold-Chiari deformity in an adult without obvious cause. J Neurosurg. 1945;2:245–50.CrossRefGoogle Scholar
  7. 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.PubMedCrossRefGoogle Scholar
  8. 8.
    Gardner WJ. Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry. 1965;28:247–59.PubMedCrossRefGoogle Scholar
  9. 9.
    Levy WJ, Mason L, Hahn JF. Chiari malformation presenting in adults: a surgical experience in 127 cases. Neurosurgery. 1983;12:377–90.PubMedCrossRefGoogle Scholar
  10. 10.
    Pillay PK. Thecoperitoneal shunting for syringomyelia. J Neurosurg. 1991;75:835–6.PubMedGoogle Scholar
  11. 11.
    Logue V, Edwards M. Syringomyelia and its surgical treatment–an analysis of 75 patients. J Neurol Neurosurg Psychiatry. 1981;44:273–84.PubMedCrossRefGoogle Scholar
  12. 12.
    Klekamp J, Batzdorf U, Samii M, Bothe HW. The surgical treatment of Chiari I malformation. Acta Neurochir (Wien). 1996;138:788–801.CrossRefGoogle Scholar
  13. 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. 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.PubMedCrossRefGoogle Scholar
  15. 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.PubMedCrossRefGoogle Scholar
  16. 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.PubMedGoogle Scholar
  17. 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.PubMedCrossRefGoogle Scholar
  18. 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.PubMedCrossRefGoogle Scholar
  19. 19.
    Nyland H, Krogness KG. Size of posterior fossa in Chiari type 1 malformation in adults. Acta Neurochir (Wien). 1978;40:233–42.CrossRefGoogle Scholar
  20. 20.
    Cinalli G, Spennato P, Sainte-Rose C, et al. Chiari malformation in craniosynostosis. Childs Nerv Syst. 2005;21:889–901.PubMedCrossRefGoogle Scholar
  21. 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.PubMedCrossRefGoogle Scholar
  22. 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.PubMedCrossRefGoogle Scholar
  23. 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.PubMedCrossRefGoogle Scholar
  24. 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.PubMedCrossRefGoogle Scholar
  25. 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.PubMedCrossRefGoogle Scholar
  26. 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.CrossRefGoogle Scholar
  27. 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.PubMedCrossRefGoogle Scholar
  28. 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.PubMedGoogle Scholar
  29. 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.PubMedCrossRefGoogle Scholar
  30. 30.
    Kasner SE, Rosenfeld J, Farber RE. Spontaneous intracranial hypotension: headache with a reversible Arnold-Chiari malformation. Headache. 1995;35:557–9.PubMedCrossRefGoogle Scholar
  31. 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.PubMedCrossRefGoogle Scholar
  32. 32.
    Ammerman JM, Goel R, Polin RS. Resolution of Chiari malformation after treatment of acromegaly. Case illustration. J Neurosurg. 2006;104:980.PubMedCrossRefGoogle Scholar
  33. 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.PubMedCrossRefGoogle Scholar
  34. 34.
    Lemar Jr HJ, Perloff JJ, Merenich JA. Symptomatic Chiari-I malformation in a patient with acromegaly. South Med J. 1994;87:284–5.PubMedCrossRefGoogle Scholar
  35. 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.PubMedCrossRefGoogle Scholar
  36. 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.PubMedGoogle Scholar
  37. 37.
    Fenoy AJ, Menezes AH, Fenoy KA. Craniocervical junction fusions in patients with hindbrain herniation and syringohydromyelia. J Neurosurg Spine. 2008;9:1–9.PubMedCrossRefGoogle Scholar
  38. 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.PubMedCrossRefGoogle Scholar
  39. 39.
    Heiss JD, Patronas N, DeVroom HL, et al. Elucidating the pathophysiology of syringomyelia. J Neurosurg. 1999;91:553–62.PubMedCrossRefGoogle Scholar
  40. 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.PubMedCrossRefGoogle Scholar
  41. 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.PubMedCrossRefGoogle Scholar
  42. 42.
    Elster AD, Chen MY. Chiari I malformations: clinical and radiologic reappraisal. Radiology. 1992;183:347–53.PubMedGoogle Scholar
  43. 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.PubMedGoogle Scholar
  44. 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.PubMedCrossRefGoogle Scholar
  45. 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.PubMedCrossRefGoogle Scholar
  46. 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.PubMedCrossRefGoogle Scholar
  47. 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.PubMedCrossRefGoogle Scholar
  48. 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.PubMedCrossRefGoogle Scholar
  49. 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.PubMedCrossRefGoogle Scholar
  50. 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.PubMedCrossRefGoogle Scholar
  51. 51.
    Kempe LG. Operative neurosurgery. New York: Springer; 1970.Google Scholar
  52. 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.PubMedCrossRefGoogle Scholar
  53. 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.PubMedCrossRefGoogle Scholar
  54. 54.
    Mazzola CA, Fried AH. Revision surgery for Chiari malformation decompression. Neurosurg Focus. 2003;15:E3.PubMedCrossRefGoogle Scholar
  55. 55.
    Sacco D, Scott RM. Reoperation for Chiari malformations. Pediatr Neurosurg. 2003;39:171–8.PubMedCrossRefGoogle Scholar
  56. 56.
    Tubbs RS, Webb DB, Oakes WJ. Persistent syringomyelia following pediatric Chiari I decompression: radiological and surgical findings. J Neurosurg. 2004;100:460–4.PubMedGoogle Scholar
  57. 57.
    Batzdorf U, Klekamp J, Johnson JP. A critical appraisal of syrinx cavity shunting procedures. J Neurosurg. 1998;89:382–8.PubMedCrossRefGoogle Scholar
  58. 58.
    Sgouros S, Williams B. A critical appraisal of drainage in syringomyelia. J Neurosurg. 1995;82:1–10.PubMedCrossRefGoogle Scholar
  59. 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.PubMedCrossRefGoogle Scholar
  60. 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.PubMedCrossRefGoogle Scholar
  61. 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.PubMedCrossRefGoogle Scholar
  62. 62.
    Matsumoto T, Symon L. Surgical management of syringomyelia–current results. Surg Neurol. 1989;32:258–65.PubMedCrossRefGoogle Scholar
  63. 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.PubMedGoogle Scholar
  64. 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.PubMedCrossRefGoogle Scholar
  65. 65.
    Krayenbuhl H. Evaluation of the different surgical approaches in the treatment of syringomyelia. Clin Neurol Neurosurg. 1975;77:111–28.PubMedGoogle Scholar
  66. 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.PubMedGoogle Scholar
  67. 67.
    Welch K, Shillito J, Strand R, Fischer EG, Winston KR. Chiari I “malformations”–an acquired disorder? J Neurosurg. 1981;55:604–9.PubMedCrossRefGoogle Scholar
  68. 68.
    Batzdorf U. Chiari I, malformation with syringomyelia. Evaluation of surgical therapy by magnetic resonance imaging. J Neurosurg. 1988;68:726–30.PubMedCrossRefGoogle Scholar
  69. 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.PubMedGoogle Scholar
  70. 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.PubMedGoogle Scholar
  71. 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.PubMedCrossRefGoogle Scholar
  72. 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.PubMedCrossRefGoogle Scholar
  73. 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

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Surgical Neurology Branch, NINDSNational Institutes of HealthBethesdaUSA
  2. 2.Department of Neurological SurgeryUniversity of VirginiaCharlottesvilleUSA

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