Skip to main content
Log in

Surgical technique for cranio-cervical decompression in syringomyelia associated with Chiari type I malformation

  • Clinical Articles
  • Published:
Acta Neurochirurgica Aims and scope Submit manuscript

Summary

Our purpose is to present our results with the surgical treatment of syringomyelia associated with Chiari type I malformation. Between October 1989 and October 1995, twenty-eight patients underwent a sub-occipital craniotomy and a C1 laminectomy. After dura mater opening the cerebellar tonsils were mobilised. Neither catheter, nor plugging of the obex, nor tonsillar tissue removal was performed. The dura mater was enlarged by means of a wide graft to create a newcisterna magna of adequate size.

Postoperative MRI scans showed an ascent of the cerebellum of 4.3±4.8 mm (measured by thefastigium to basal line), as well as of the brainstem (mean migration of the mesencephalon-pons junction of 4.3±3.3 mm). The tonsils emigrated cranially 6.5±4.8 mm. While preoperative mean syringo-cord ratio was 66.3%±13.3, post-operatively was 12.1%±12.7 (p<0.0001). A complete collapse of the syrinx was observed in 39% of the patients.

Long-term improvements were obtained in 73% of the cases and 27% were unchanged. No patient got worse.

We conclude that in the treatment of syringomyelia associated with Chiari I malformation an artificial cisterma magna of sufficient size must be created. This is achieved by means of an extensive sub-occipital craniotomy and C1 laminectomy, followed by dural opening. Small bone removal with limited enlargement of theposterior fossa often results in failures of treatment and recurrences. Tonsillar removal is not necessary to obtain a good reconstruction of the cisterma magna.

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

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

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

    Google Scholar 

  2. Albert P (1978) Resultados quirúrgicos en la siringomielia. Presented at the Thirtieth Annual Meeting of the Luso-Spanish Society of Neurosurgery, Barcelona

  3. Appleby A, Foster JB, Hankinson J, Hudgson P (1968) The diagnosis and management of the Chiari anomalies in adult life. Brain 91: 131–140

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  5. Barbaro NM, Wilson CB, Gution PH, Edwards MSB (1984) Surgical treatment of syringomyelia. Favourable results with syringo-peritoneal shunting. J Neurosurg 61: 531–538

    PubMed  CAS  Google Scholar 

  6. Barkovich AJ, Wippold FJ, Sherman JL, Citrin CM (1986) Significance of cerebellar tonsilar position on MR. Am J Neuroradiol 7: 795–799

    PubMed  CAS  Google Scholar 

  7. Barkovich AJ, Sherman JL, Citrin CM, Wippold FJ (1987) MR of postoperative syringomyelia. Am J Neuroradiol 8: 319–327

    PubMed  CAS  Google Scholar 

  8. Batzdorf U (1988) Chiari I malformation with syringomyelia: Evaluation of surgical therapy by magnetic resonance imaging. J Neurosurg 68: 726–730

    PubMed  CAS  Google Scholar 

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

    Google Scholar 

  10. Bertrand G (1973) Dynamic factors in the evolution of syringomyelia and syringobulbia. Clin Neurosurg 20: 322–333

    PubMed  CAS  Google Scholar 

  11. Blagodatsky MD, Larinov SN, Monhin PA, Shanturov VA, Gladyshev YuV (1993) Surgical treatment of “hindbrain related” syringomyelia: new data for pathogenesis. Acta Neurochir (Wien) 124: 82–85

    Article  CAS  Google Scholar 

  12. Blagodatsky MD, Larionov SN (1993) Surgical treatment of “Hindbrain related” syringomyelia long-term results. Acta Neurochir (Wien) 123: 209–210

    Google Scholar 

  13. Boiardi A, Munari L, Silvani A, Porta E, Scuratti A, Lodrini S (1991) Natural history and postsurgical outcome of syringomyelia. Ital J Neurol Sci 12: 575–579

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  15. Dittmann W, Hofmann E (1993) On the evaluation of craniocervical decompression: normalisation of the cerebral spinal fluid circulation in MRI? Acta Neurochir (Wien) 123: 180–183

    CAS  Google Scholar 

  16. Donauer E, Rascher K (1993) Syringomyelia: a brief review of ontogenetic, experimental and clinical aspects. Neurosurg Rev 16: 7–13

    Article  PubMed  CAS  Google Scholar 

  17. Duddy MJ, Williams B (1991) Hindbrain migration after decompression for hindbrain hernia: a quantitative assessment using MRI. Br J Neurosurg 5: 141–152

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  19. Faulhauer K, Loew K (1978) The surgical treatment of syringomyelia. Long-term results. Acta Neurochir (Wien) 44: 215–222

    Article  CAS  Google Scholar 

  20. Fujii K, Natori Y, Nakagaki H, Fukui M (1991) Management of syringomyelia associated with Chiari malformation: comparative study of syrinx size and symptoms by magnetic resonance iamging. Surg Neurol 38: 161–162

    Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  24. Gardner WJ (ed) (1973) The dysraphic states: fro syringomyelia to anencephaly. Excerpta Medica, Amsterdam, pp 23–34

    Google Scholar 

  25. Gardner WJ (1977) Hydrodynamic factors in Dandy-Walker and Arnold-Chiari malformations. Childs Brain 3: 200–212

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  27. Gower DJ, Pollay M, Leech R (1994) Pedriatric syringomyelia. J Child Neurol 9: 14–21

    Article  PubMed  CAS  Google Scholar 

  28. Hankinson J (1978) The surgical treatment of syringomyelia. In: Krayenbühl H (ed) Advances and technical standards in neurosurgery, Vol 5, Chapter 4. Springer, Wien New York, pp 127–151

    Google Scholar 

  29. Isu T, Sasaki H, Takamura H, Kobayashi N (1993) Foramen magnum decompression with removal of the outer layer of the dura as treatment of syringomyelia occurring with Chiari I malformation. Neurosurgery 33: 845–850

    Article  Google Scholar 

  30. Klekamp J, Samii M, Bothe HW, El Azm M, Sjuts E, Babapour B (1993) Syringomyelia: a pathophysiological hypothesis with special reference to dynamic MRI imaging. Acta Neurochir (Wien) 123: 183

    Google Scholar 

  31. Kohno K, Sakaki S, Nakamura H, Sakoh M, Takeda S, Sadamoto K (1991) Foramen magnum decompression for syringomyelia associated with basilar impression and Chiari I malformation: report of three cases. Neurol Med Chir Tokyo 31: 715–719

    PubMed  CAS  Google Scholar 

  32. Kokmen E, Marsh WR, Baker HL Jr (1985) Magnetic resonance imaging in syringomyelia. Neurosurgery 17: 267–270

    Article  PubMed  CAS  Google Scholar 

  33. Krayenbühl H (1974) Evaluation of the different surgical approaches in the treatment of syringomyelia. Clin Neurol Neurosurg 2: 110–128

    Article  Google Scholar 

  34. Kuroda S, Matsuzawa H, Iwasaki Y, Hida K, Imamura H, Abe H, Saito H (1994) CSF dynamics in the patients with syringomyelia associated with Chiari’s malformation: qualitative analysis on cine MR. No To Shinkei 46: 59–64

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  36. Logue V, Edwards MR (1981) Syringomyelia and its surgical treatment—an analysis of 75 patients. J Neurol Neurosurg Psychiatry 44: 273–284

    Article  PubMed  CAS  Google Scholar 

  37. Mariani C, Cislaghi MG, Barbieri S, Filizzolo F, Di Palma F, Farina E, D’Aliberti G, Scarlato G (1991) The natural history and results of surgery in 50 cases of syringomyelia. J Neurol 238: 433–438

    Article  PubMed  CAS  Google Scholar 

  38. Marin-Padilla M, Marin-Padilla TM (1981) Morphogenesis of experimentally induced Arnold-Chiari malformation. J Neurol Sci 50: 29–55

    Article  PubMed  CAS  Google Scholar 

  39. Matsumoto T, Symon L (1989) Surgical management of syringomyelia—current results. Surg Neurol 32: 253–256

    Article  Google Scholar 

  40. Milhorat TH, Johnson RW, Johnson WD (1991) Pathogenesis of syringomyelia with description of non-communicating type that arises immediately caudal to obstructive lesions. In: Matsumoto S, Tamaki N (eds) Hydrocephalus: pathogenesis and treatment. Springer, Berlin Heidelberg New York Tokyo, pp 218–228

    Google Scholar 

  41. Milhorat TH, Johnson WD, Miller JL, Bergland RM, Hollenbergsher J (1992) Surgical treatment of syringomyelia based on magnetic resonance criteria. Neurosurgery 31: 231–245

    Article  PubMed  CAS  Google Scholar 

  42. Milhorat TH, Miller JL, Johnson WD, Adler DE, Heger IM (1993) Anatomical basis of syringomyelia occurring with hindbrain lesions. Neurosurgery 32: 748–754

    Article  PubMed  CAS  Google Scholar 

  43. Milhorat TH, Capocelli AL Jr, Anzil AP, Kotzen RM, Milhorat RH (1995) Patholgoical basis of spinal cord cavitation in syringomyelia: analysis of 105 autopsy cases. J Neurosurg 82: 802–812

    PubMed  CAS  Google Scholar 

  44. Morgan D, Williams B (1992) Syringobulbia: a surgical appraisal. J Neurol Psychiatry 55. 1132–1141

    CAS  Google Scholar 

  45. Newton EJ (1969) Syringomyelia as a manifestation of defec tive fourth ventricular drainage. Ann R Coll Surg Engl 44: 194–213

    PubMed  CAS  Google Scholar 

  46. Nyland H, Krogness KG (1978) Size of posterior fossa in Chiari type I malformation in adults. Acta Neurochir (Wien) 40: 233–242

    Article  CAS  Google Scholar 

  47. Oakes WJ (1985) Chiari malformations, hydromyelia, syringomyelia. In: Wilkins RH, Rengachary SS (eds) Neurosurgery, Vol 3. McGraw-Hill, New York, pp 2102–2124

    Google Scholar 

  48. Oldfield EH, Murasko K, Shawker TH, Patronas NJ (1994) Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils: implications for diagnosis and treatment. J Neurosurg 80: 3–15

    PubMed  CAS  Google Scholar 

  49. Park TS, Cail WS, Broaddus WC, Walker MG (1989) Lumboperitoneal shunt combined with myelotomy for treatment of syringomyelia. J Neurosurg 70: 721–727

    PubMed  CAS  Google Scholar 

  50. Paramonov LV (1991) A surgical treatment method in syringomyelia. Zh Nevropatol Psikhiatr Im S S Korsakova 91: 3–6

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  52. Pillay PK, Awad IA, Hahn JF (1993) Gardner’s hydrodynamic theory of syringomyelia revisited. Cleve Clin J Med 59: 373–380

    Google Scholar 

  53. Raftopulos C, Sanchez A, Matos C, Baleriaux D, Bank WO, Brotchi J (1993) Hydrosyringomyelia-Chiari I complex. Prospective evaluation of a modified foramen magnum decompression procedure: preliminary results. Surg Neurol 39: 163–169

    Article  Google Scholar 

  54. Raynor RB (1989) Congenital malformations of the base of the skull: The Arnold-Chiari malformation. In: The Cervical Spine Research Society Editorial Committee (ed) The cervical spine. Lippincott, Philadelphia, pp 226–235

    Google Scholar 

  55. Rhoton AL Jr (1976) Microsurgery of Arnold-Chiari malformation in adults with and without hydromyelia. J Neurosurg 45: 473–483

    PubMed  Google Scholar 

  56. Rice-Edwards JM (1989) Syringomyelia and the Chiari malformation. In: Dudley H,et al (eds) Rob and Smith’s operative surgery. Butterworths, London, pp 135–145

    Google Scholar 

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

    PubMed  CAS  Google Scholar 

  58. Sahuquillo J, Rubio E, Poca MA, Rovira A, Rodriguez-Baeza A, Cervera C (1994) 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 35: 874–884

    Article  PubMed  CAS  Google Scholar 

  59. Sakamoto H, Hakuba A, Fujitani K, Nishimura S (1991) Surgical treatment of the re-tethered spinal cord after repair of lympomyelomeningocele. J Neurosurg 74: 709–714

    Article  PubMed  CAS  Google Scholar 

  60. Samii M, Klekamp A, Sepehrnia A, Bothe HW, El Azm M, Sjuts E, Babapour B (1993) Syringomyelia associate with Arnold-Chiari I malformation and tumours of the posterior fossa. Long-term results of syringo-subarachnoid shunting and decompression of the posterior fossa. Acta Neurochir (Wien) 123: 195

    Google Scholar 

  61. Sarnat HB (1991) Embryology and dysgenesis of the posterior fossa. In: Bathdorf U (ed) Syringomyelia: current concepts in diagnosis and treatment. Williams and Wilkins, Baltimore, pp 3–34

    Google Scholar 

  62. Schady W, Metclfe RA, Butler P (1987) The incidence of cranio-cervical bony anomalies in the Chiari malformation. J Neurol Sci 82: 193–201

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  64. Stovner LJ, Bergan U, Nilsen G, Sjaastad O (1993) Posterior cranial fossa dimensions in the Chiari I malformation: relation to pathogenesis and clinical presentation. Neuroradiology 35(2): 113–118

    Article  PubMed  CAS  Google Scholar 

  65. Tator CH, Meguro K, Rowed DW (1982) Favorable results with syringo-subarachnoid shunts for treatment of syringomyelia. J Neurosurg 56: 517–523

    PubMed  CAS  Google Scholar 

  66. Terae S, Miyasaka S, Abe H, Tashiro K (1994) Increased pulsatile movement of the hindbrain in syringomyelia associated with the Chiari malformation: cine-MRI with presaturation bolus tracking. Neuroradiol 36: 125–129

    Article  CAS  Google Scholar 

  67. Tognetti F, Calbucci F (1993) Syringomyelia: syringo-subarachnoid shunt versus posterior fossa decompression. Acta Neurochir (Wien) 123: 196–197

    CAS  Google Scholar 

  68. Van Velthoven V, Jost M, Siekmann R, Eggert HR (1993) Surgical strategies and results in syringomyelia. Acta Neurochir (Wien) 123: 199–201

    Google Scholar 

  69. Van den Bergh R, Van Calenberg F (1993) Neurosurgical results and outcome of conservative treatment. Acta Neurochir (Wien) 123: 198–199

    Google Scholar 

  70. Vega A, Quintana F, Berciano J (1990) Basicondrocranium anomalies in adult Chiari type I malformation: a morphometric study. J Neurol Sci 99: 137–145

    Article  PubMed  CAS  Google Scholar 

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

    Article  CAS  Google Scholar 

  72. Wester K, Pedersen PH, Kråkenes J (1989) Spinal cord damage caused by rotation of a T-drain in a patient with syringo-peritoneal shunt. Surg Neurol 31: 224–227

    Article  PubMed  CAS  Google Scholar 

  73. Wiedemayer H, Nau HE, Rauhut F, Grote W, Gerhard L (1994) Operative treatment and prognosis of syringomyelia. Neurosurg Rev 17: 37–41

    Article  PubMed  CAS  Google Scholar 

  74. Williams B (1976) Cerebrospinal fluid pressure changes in response to coughing. Brain 99: 331–346

    Article  PubMed  CAS  Google Scholar 

  75. Williams B (1978) A critical appraisal of posterior fossa surgery for communicating syringomyelia. Brain 101: 233–250

    Article  Google Scholar 

  76. Williams B (1986) Progress in syringomyelia. Neurol Res 8: 139–145

    Google Scholar 

  77. Williams B (1991) Pathogenesis of syringomyelia. In: Batzdorf U (ed) Syringomyelia: current concepts in diagnosis and treatment. Williams and Wilkins, Baltimore, pp 59–90.

    Google Scholar 

  78. Williams B (1993) Pathogenesis of syringomyelia. Acta Neurochir (Wien) 123: 159–165

    CAS  Google Scholar 

  79. Williams B (1993) Surgery for hindbrain related syringomyelia. Adv Tech Stand Neurosurg 20: 107–164

    PubMed  CAS  Google Scholar 

  80. Williams B (1993) Surgical treatment of syringobulbia. Neurosurg Clin N Am 4: 553–571

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Vanaclocha, V., Saiz-Sapena, N. & Garcia-Casasola, M.C. Surgical technique for cranio-cervical decompression in syringomyelia associated with Chiari type I malformation. Acta Neurochir 139, 529–540 (1997). https://doi.org/10.1007/BF02750996

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02750996

Keywords

Navigation