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The surgical treatment of Chiari I malformation

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Summary

A retrospective study was undertaken on 133 patients with a Chiari I malformation treated within the last 16 years at the Departments of Neurosurgery at the Nordstadt Hospital Hannover, Germany, and the University of California, Los Angeles, U.S.A. Ninety-seven patients presented with symptoms related to accompanying syringomyelia and 4 with associated syringobulbia. They underwent 149 surgical procedures and were followed for a mean of 39±52 months. A decompression at the foramen magnum was performed in 124 patients, while 22 of those with syringomyelia were treated by shunting (7 syringosubarachnoid shunts, 15 syringoperitoneal or -pleural shunts), and 3 by ventriculoperitoneal shunts for hydrocephalus.

Except for ventriculoperitoneal shunting, at least a short-term decrease in size of an associated syrinx was observed for all procedures in the majority of cases. However, no long-term benefit was observed for syrinx shunting operations. The best clinical longterm results were obtained with decompression of the foramen magnum in patients with (86% free of a clinical recurrence) and without syringomyelia (77% free of a clinical recurrence). We advise against syrinx shunting, a large craniectomy, and obex plugging which are associated with higher recurrence rates. Instead, surgery should consist of a small craniectomy, opening of the dura, archnoid dissection to establish normal cerebrospinal fluid (CSF) outflow from the 4th ventricle, and a fascia lata durai graft.

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References

  1. Abbe R, Coley WB (1891) Syringomyelia, operation-exploration of cord, withdrawal of fluid, exhibition of patient. J Nerv Ment Dis 19: 512–520

    Google Scholar 

  2. Aboulker J (1979) La syringomyelie et les liquides intra-rachidiens. Neurochirurgie 25 [Suppl]: 1–144

    Google Scholar 

  3. Armonda RA, Citrin CM, Foley KT,et al (1994) Quantitative cinemode magnetic resonance imaging of chiari I malformations: an analysis of cerebrospinal fluid dynamics. Neurosurgery 35: 214–224

    PubMed  Google Scholar 

  4. Aubin ML, Baleriaux D, Cosnard G,et al (1987) MRI in syringomyelia of congenital, infectious, traumatic or idiopathic origin. A study of 142 cases. J Neuroradiol 14: 313–336

    PubMed  Google Scholar 

  5. Ball MJ, Dayan AD (1972) Pathogenesis of syringomyelia. Lancet 2: 799–801

    Article  PubMed  Google Scholar 

  6. Banerji NK, Millar JHD (1974) Chiari malformation presenting in adult life: its relationship to syringomyelia. Brain 97: 157–168

    PubMed  Google Scholar 

  7. Barbaro NM, Wilson CB, Gutin PH,et al (1984) Surgical treatment of syringomyelia. J Neurosurg 61: 531–538

    PubMed  Google Scholar 

  8. de Barros MC, Farias W, Ataide L,et al (1968) Basilar impression and Arnold Chiari malformation. A study of 106 cases. J Neurol Neurosurg Psychiatry 31: 596–605

    PubMed  Google Scholar 

  9. Batzdorf U (1991) Syringomyelia related to abnormalities at the level of the craniocervical junction. In: Batzdorf U (ed) Syringomyelia. Current concepts in diagnosis and treatment. Williams and Wilkins, Baltimore, pp 163–182

    Google Scholar 

  10. Beric A, Dimitrijevic MR, Lindblom U (1988) Central dysesthesia syndrome in spinal cord injury patients. Pain 34: 109–116

    PubMed  Google Scholar 

  11. Bidzinski J (1988) Late results of the surgical treatment of syringomyelia. Acta Neurochir (Wien) [Suppl] 43: 29–31

    Google Scholar 

  12. Boiardi A, Munari L, Silvani A,et al (1991) Natural history and postsurgical outcome of syringomyelia. Ital J Neurol Sci 12: 575–579

    PubMed  Google Scholar 

  13. Cahan LD, Bentson JR (1982) Considerations in the diagnosis and treatment of syringomyelia and the Chiari malformation. J Neurosurg 57: 24–31

    PubMed  Google Scholar 

  14. Caraceni TA, Celano J, Borghi P (1978) Evaluation of the results obtained from the surgical treatment of patients affected by syringomyelia associated with Chiari type I malformation. Acta Neurochir (Wien) 44: 257

    Google Scholar 

  15. Cserr HF, Cooper DN, Milhorat TH (1977) Flow of cerebral interstitial fluid as indicated by the removal of extracellular markers from rat caudate nucleus. In: Bito LZ, Davson H, Fenstermacher JD (eds) The ocular and cerebrospinal fluids. Exp Eye Res [Suppl] 25: 461–473

  16. Dubois PJ, Drayer BP, Sage M,et al (1981) Intramedullary penetrance of metrizamide in the dog spinal cord. AJNR 2: 313–317

    PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  18. Eisenstat DDR, Bernstein M, FleMing JFR,et al (1986) Chiari malformation in adults: a review of 40 cases. Can J Neurol Sci 13: 221–228

    PubMed  Google Scholar 

  19. Ellertson AB, Greitz T (1970) The distending force in the production of communicating syringomyelia. Lancet 1: 1234

    Google Scholar 

  20. Elster AD, Chen MYM (1992) Chiari I malformations: clinical and radiological appraisal. Radiology 183: 347–353

    PubMed  Google Scholar 

  21. Enzmann DR, Pelc NJ (1991) Normal flow patterns with phase-contrast cine MRI imaging. Radiology 178: 467–474

    PubMed  Google Scholar 

  22. Erbengi A, Öge HK (1994) Congenital malformations of the craniocervical junction: classification and surgical treatment. Acta Neurochir (Wien) 127: 180–185

    Google Scholar 

  23. Feigin I, Ogata J, Budzilovich (1971) Syringomyelia: the role of edema in its pathogenesis. J Neuropathol Exp Neurol 30: 216–232

    PubMed  Google Scholar 

  24. Filizzolo F, Versari P, D'Aliberti G,et al (1988) Foramen magnum decompression versus terminal ventriculostomy for treatment of syringomyelia. Acta Neurochir (Wien) 93: 96–99

    Google Scholar 

  25. Garcia-Uria J, Leunda G, Carrillo R,et al (1981) Syringomyelia: long-term results after posterior fossa decompression. J Neurosurg 54: 380–383

    PubMed  Google Scholar 

  26. Gardner WJ, Angel J (1958) The cause of syringomyelia and its surgical treatment. Cleve Clin Q 25: 4–8

    PubMed  Google Scholar 

  27. Gardner WJ (1965) Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry 28: 247–259

    PubMed  Google Scholar 

  28. Greitz D (1993) Cerebrospinal fluid circulation and associated intracranial dynamics. A radiologic investigation using MR imaging and radionuclide cisternography. Acta Radiol [Suppl] 386: 1–23

    Google Scholar 

  29. Hankinson J (1978) The surgical treatment of syringomyelia. In: Krayenbuehl H (ed) Advances and technical standards in neurosurgery, Vol 5. Springer, Wien New York, pp 127–151

    Google Scholar 

  30. Hertel G, Kramer S, Placzek E (1973) Die Syringomyelie. Klinische Verlaufsbeobachtungen bei 323 Patienten. Nervenarzt 44: 1–13

    PubMed  Google Scholar 

  31. Hüwel N, Perneczky A, Urban V,et al (1992) Neuroendoscopic technique for the operative treatment of septated syringomyelia. Acta Neurochir (Wien) [Suppl] 54: 59–62

    Google Scholar 

  32. Hutchings M, Weller RO (1986) Anatomical relationships of the pia mater to cerebral blood vessels in man. J Neurosurg 65: 316–325

    PubMed  Google Scholar 

  33. Ikata T, Masaki K, Kashiwaguchi S (1988) Clinical and experimental studies on permeability of tracers in normal spinal cord and syringomyelia. Spine 13: 737–741

    PubMed  Google Scholar 

  34. Isherwood I, Fawcitt RA, Forbes WSC,et al (1977) Computer tomography of the spinal canal using metrizamide. Acta Radiol [Suppl] 355: 299–305

    Google Scholar 

  35. Isu T, Iwasaki Y, Akino M,et al (1990) Syringo-subarachnoid shunt for syringomyelia associated with Chiari malformation (Type I). Acta Neurochir (Wien) 107: 152–160

    Google Scholar 

  36. Kaplan EL, Meier P (1958) Nonparametric estimation from incomplete observations. J Am Stat Assoc 53: 457–481

    Google Scholar 

  37. Karnofsky DA, Burchenal JH (1949) The clinical evaluation of chemotherapeutic agents in cancer. In: McLeod CM (ed) Evaluation of chemotherapeutic agents. Columbia University Press, New York, pp 191–205

    Google Scholar 

  38. Kasantikul V, Netsky J, James AE (1979) Relation of age and cerebral ventricle size to central canal in man. Morphological analysis. J Neurosurg 51: 85–93

    PubMed  Google Scholar 

  39. Klekamp J, Samii M (1993) Introduction of a score system for the clinical evaluation of patients with spinal processes. Acta Neurochir (Wien) 123: 221–223

    Google Scholar 

  40. Levy WJ, Mason L, Hahn JF (1983) Chiari malformation presenting in adults: a surgical experience in 127 patients. Neurosurgery 12: 377–390

    PubMed  Google Scholar 

  41. Li KC, Chui MC (1987) Conventional and CT metrizamide myelography in Arnold-Chiari I malformation and syringomyelia. AJNR 8: 11–17

    PubMed  Google Scholar 

  42. Logue V (1971) Syringomyelia: a radiodiagnostic and radiotherapeutic saga. Clin Radiol 22: 2–16

    PubMed  Google Scholar 

  43. Mariani C, Cislaghi MG, Barbieri S,et al (1991) The natural history and results of surgery in 50 cases of syringomyelia. J Neurol 238: 433–438

    PubMed  Google Scholar 

  44. Matsumoto T, Symon L (1989) Surgical management of syringomyelia — current results. Surg Neurol 32: 258–265

    Google Scholar 

  45. Menezes AH (1991/1992) Chiari I malformations and hydro-myelia — complications. Pediatr Neurosurg 17: 146–154

    Google Scholar 

  46. Menezes AH, Van Gilder JC, Graf CJ,et al (1980) Craniocervical abnormalities. A comprehensive surgical approach. J Neurosurg 53: 444–445

    PubMed  Google Scholar 

  47. Mohr PD, Strang FA, Sambrook MA,et al (1977) The clinical and surgical features in 40 patients with primary cerebellar ectopia (Adult Chiari malformation). Q J Med 46: 85–96

    PubMed  Google Scholar 

  48. Newman PK, Terenty TR, Foster JB (1981) Some observations on the pathogenesis of syringomyelia. J Neurosurg Psychiatry 44: 964–969

    Google Scholar 

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

    Google Scholar 

  50. Oldfield EH, Muraszko K, Shawker TH,et al (1994) Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg 80: 3–15

    PubMed  Google Scholar 

  51. Paul KS, Lye RH, Strang FA,et al (1983) Arnold-Chiari malformation. Review of 71 cases. J Neurosurg 58: 183–187

    PubMed  Google Scholar 

  52. Peerless SJ, Durward QJ (1982) Management of syringomyelia: a pathophysiological approach. Clin Neurosurg 30: 531–576

    Google Scholar 

  53. Quencer RM, Post MJD, Hinks RS (1990) Cine MRI in the evaluation of normal and abnormal CSF flow: intracranial and intraspinal studies. Neuroradiology 32: 371–391

    PubMed  Google Scholar 

  54. Rennels ML, Gregory TF, Blaumanis OR,et al (1985) Evidence for a ‘paravascular’ fluid circulation in the mammalian central nervous system, provided by the rapid distribution of tracer protein throughout the brain from the subarachnoid space. Brain Res 326: 47–63

    PubMed  Google Scholar 

  55. Saez RJ, Onofrio BM, Yanagihara T (1976) Experience with Arnold-Chiari malformation, 1960–1970. J Neurosurg 45: 416–422

    PubMed  Google Scholar 

  56. Schliep G, Ritter U (1971) Klinik der Syringomyelie. Fortschr Neurol Psychiatry 39: 53–82

    Google Scholar 

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

    PubMed  Google Scholar 

  58. Shapiro WR (1988) Cerebrospinal fluid circulation and the blood brain barrier. Ann NY Acad Sci 531: 9–14

    PubMed  Google Scholar 

  59. Sherman JL, Citrin CM, Gangarosa RE,et al (1986) The MR appearance of CSF pulsations in the spinal canal. AJNR 7: 879–884

    PubMed  Google Scholar 

  60. Tokuno H, Hakuba A, Suzuki T,et al (1988) Operative treatment of Chiari malformation with syringomyelia. Acta Neurochir (Wien) [Suppl] 43: 22–25

    Google Scholar 

  61. Vaquero J, Martinez R, Salazar J,et al (1987) Syringosubarachnoid shunt for treatment of syringomyelia. Acta Neurochir (Wien) 84: 105–109

    Google Scholar 

  62. Williams B (1986) Progress in syringomyelia. Neurol Res 8: 130–145

    PubMed  Google Scholar 

  63. Williams B (1993) Surgery for hindbrain related syringomyelia. In: Symon Let al (eds) Advances and technical standards in neurosurgery, Vol 20. Springer, Wien New York, pp 108–164

    Google Scholar 

  64. Winkler SS, Sackett JF (1980) Explanation of metrizamide brain penetration: a review. J Comput Assist Tomogr 4: 191–193

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Medical School of Hannover, Neurosurgical Clinic, Nordstadt Hospital Hannover, Germany

    J. Klekamp, M. Samii & H. W. Bothe

  2. Division of Neurosurgery, University of California, Los Angeles, CA, USA

    U. Batzdorf

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  1. J. Klekamp
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  4. H. W. Bothe
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Klekamp, J., Batzdorf, U., Samii, M. et al. The surgical treatment of Chiari I malformation. Acta neurochir 138, 788–801 (1996). https://doi.org/10.1007/BF01411256

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