Advertisement

Child's Nervous System

, Volume 20, Issue 5, pp 341–348 | Cite as

International survey on the management of Chiari I malformation and syringomyelia

  • Edgardo SchijmanEmail author
  • Paul Steinbok
Focus Session

Abstract

Introduction

The availability of magnetic resonance imaging (MRI) has resulted in an increasing number of asymptomatic, minimally symptomatic, and doubtfully symptomatic patients being diagnosed with a Chiari I malformation with or without syringomyelia. In an attempt to clarify how neurosurgeons manage these clinical problems, an international survey on the Chiari I malformation and related syringomyelia was undertaken.

Method

A questionnaire on the expected natural course of the disease and on aspects of the surgical technique for a number of hypothetical cases relating to Chiari I malformation with and without syringomyelia was used to survey Pediatric Neurosurgeons worldwide.

Results

Of 246 questionnaires distributed, 76 (30.8%) were completed and returned. There was a consensus that no operation should be carried out in asymptomatic patients with a Chiari I malformation, unless there is associated syringomyelia. There was a consensus that decompression of the Chiari malformation should be performed in patients with scoliosis when syringomyelia is present, and the majority decompressed the Chiari malformation in scoliotic patients even in the absence of syringomyelia. Suboccipital decompression was the standard surgical procedure for Chiari I malformations. The majority of respondents favored routine dural opening at surgery and closure with a pericranial or synthetic patch graft. In the case of a persistent or progressive syrinx after suboccipital decompression, the majority recommended shunting of the syrinx to the subarachnoid space or to the pleural cavity.

Conclusion

There continues to be much variation in the management of the Chiari I malformation.

Keywords

Chiari I malformation Syringomyelia International survey Treatment 

Notes

Acknowledgements

We wish to thank those who completed the questionnaire survey: I.R. Abbott, J.M. Abdullah, D. Adelson, V. Balakrishnan, L.T. Basauri, M. Besser, R. Boop, W. Butler, M. Caldarelli, J. Cappelen, S. Constantini, E.M.J. Cornips, A. Czorni, P. Dhellemmes, M.S. Dias, B. Due-Tonnessen, M. Edwards, R.G. Ellenbogen, E. Erdogan, J.P. Farmer, S. Glazier, J.H. Guajardo Torres, S. Haines, A. Hanieh, R. Hayward, M.D. Heafner, E. Helseth, E.J. Herrera, R. Hollenberg, S.K. Hwang, M.A. Ibáñez, Indianapolis Neurosurgery, B.A. Kaufman, Y. Kushel, J.R.S. Leggate, J.F. Martinez-Lage, P.L. May, C. Mazza, J.G. McComb, M. Medlock, G. Meyer, N. Morota, A. Murgio, J. Nadell, M. Panigrahi, A.D. Parent, M. Partington, J.C. Peter, G. Pinna, H. Portnoy, J. Punt, A.G. Ramírez Reyes, H.L. Rekate, P. Richards, A.M. Ritter, F. Rueda Franco, H. Sakamoto, S.K. Sankhla, M. Scott, S. Sgouros, H.J. Shin, M. Shinoda, The Montreal Children’s Hospital Neurosurgery Division, T. Tomita, T. Tsuchida, F. Van Calenbergh, S. Valenzuela, W.P. Vandertop, P. van Ouwerkerk, M. Vassilyadi, E.C.G. Ventureyra, J.H.C. Voormolen, M. Vural, M.L. Walker, K.C. Wang, M. Yamasaki, Y. Yoshida, M. Zerah, G. Zuccaro.

References

  1. 1.
    Batzdorf U (1988) Chiari I malformation with syringomyelia. J Neurosurg 68:726–730PubMedGoogle Scholar
  2. 2.
    Batzdorf U (1996) Syringomyelia, Chiari malformation and hydromyelia. In: Youmans J (ed) Neurological surgery, 4th edn. Saunders, PhiladelphiaGoogle Scholar
  3. 3.
    Batzdorf U (2001) Comment. Neurosurgery 48:228Google Scholar
  4. 4.
    Batzdorf U, Klekamp J, Johnson JP (1984) A critical appraisal of syrinx cavity shunting procedures. J Neurosurg 61:531–538PubMedGoogle Scholar
  5. 5.
    Bejjani GK (2001) Definition of the adult Chiari malformation: a brief historical overview. Neurosurg Focus 11, article 1Google Scholar
  6. 6.
    Duddy JM, Williams B (1991) Hindbrain migration after decompression for hindbrain hernia: a quantitative assessment. Br J Neurosurg 5:141–152PubMedGoogle Scholar
  7. 7.
    Dyste GN, Menezes AH, VanGilder JC (1989) Symptomatic Chiari malformations. An analysis of presentation, management, and long-term outcome. J Neurosurg 71:159–168PubMedGoogle Scholar
  8. 8.
    Feldstein NA, Choudhari TF (1999) Management of Chiari I malformations with holocord syringo-hydromyelia. Pediatr Neurosurg 31:143–149CrossRefPubMedGoogle Scholar
  9. 9.
    Fischer EG (1985) Posterior fossa decompression for Chiari I deformity, including resection of the cerebellar tonsils. Childs Nerv Syst 11:625–629Google Scholar
  10. 10.
    Gambardella G, Caruso G, Caffo M, Germanò A, La Rosa G, Tomasello F (1998) Transverse microincisions of the outer layer of the dura mater combined with foramen magnum decompression as a treatment for syringomyelia with Chiari malformation. Acta Neurochir (Wien) 140:134–139Google Scholar
  11. 11.
    Hanieh A, Sutherland A, Foster B, Cundy P (2000) Syringomyelia in children with primary scoliosis. Childs Nerv Syst 16:200–202CrossRefPubMedGoogle Scholar
  12. 12.
    Haroun RI, Guarnieri M, Meadow JJ, Kraut M, Carson B (2000) Current opinions for the treatment of syringomyelia and Chiari malformations: survey of the Pediatric Section of the American Association of Neurological Surgeons. Pediatr Neurosurg 33:311–317CrossRefPubMedGoogle Scholar
  13. 13.
    Hida T, Sasaki H, Takamura H, Kobayashi N (1993) Foramen magnum decompression with removal of the outer layer of the dura as a treatment for syringomyelia occurring with Chiari I malformations. Neurosurgery 33:845–849Google Scholar
  14. 14.
    Iwasaki Y, Hida K, Abe H, Koyanagi I (1997) Etiology and treatment of syringomyelia. Neurosurgeons 16:108–112Google Scholar
  15. 15.
    Iwasaki Y, Hida K, Koyanagi I, Abe H (2000) Reevaluation of syringosubarachnoid shunt for syringomyelia with Chiari malformation. Neurosurgery 46:407–413PubMedGoogle Scholar
  16. 16.
    James HE, Brant A (2002) Treatment of the Chiari malformation with bone decompression without durotomy in children and young adults. Childs Nerv Syst 18:202–206PubMedGoogle Scholar
  17. 17.
    Koyanagi I, Iwasaki Y, Ysu T, Akino M, Abe H, Ikota T, Mitsumori K (1991) Surgical treatment of syringomyelia with Chiari malformation. Effect of foramen magnum decompression. Spinal Surg (Tokyo) 5:43–49Google Scholar
  18. 18.
    Krieger MD, McComb JG, Levy ML (1999) Towards a simpler surgical management of Chiari I malformation in pediatric population. Pediatr Neurosurg 30:113–121PubMedGoogle Scholar
  19. 19.
    Matsumoto T, Symon S (1989) Surgical management of syringomyelia. Surg Neurol 32:258–265PubMedGoogle Scholar
  20. 20.
    Milhorat TH (1993) Comment. Neurosurgery 33:849–850Google Scholar
  21. 21.
    Milhorat TM (2000) Comment. Neurosurgery 46:412Google Scholar
  22. 22.
    Nishizawa S, Yokoyama T, Yokota N, Tokuyama T, Ohta S (2001) Incidentally identified syringomyelia associated with Chiari I malformations: is early interventional surgery necessary? Neurosurgery 49:637–641PubMedGoogle Scholar
  23. 23.
    Parizek J, Sercl M, Michl A, Mericka P, Nemecek S, Nemeckova J, Jakubec J (1994) Posterior fossa duraplasty in children: remarks on surgery and clinical and CT following. Childs Nerv Syst 10:444–449PubMedGoogle Scholar
  24. 24.
    Park JK, Gleason PL, Madsen JR, Goumnerova LC, Scott RM (1997) Presentation and management of Chiari I malformation in children. Pediatr Neurosurg 26:190–196PubMedGoogle Scholar
  25. 25.
    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 35:874–885PubMedGoogle Scholar
  26. 26.
    Samii M (1998) Comment. Acta Neurochir (Wien) 140:139Google Scholar
  27. 27.
    Williams B (1993) Surgery for hindbrain related syringomyelia. In: Symon L et al (eds) Advances and technical standards in neurosurgery. Springer, New York Berlin HeidelbergGoogle Scholar

Copyright information

© Springer-Verlag 2004

Authors and Affiliations

  1. 1.Division of NeurosurgeryUniversity of Buenos Aires School of MedicineBuenos AiresArgentina
  2. 2.Section of NeurosurgeryHospital C. DurandBuenos AiresArgentina
  3. 3.Division of Pediatric NeurosurgeryB.C.’s Children’s Hospital, a part of Children’s and Women’s Health CenterVancouverCanada
  4. 4.Department of SurgeryUniversity of British ColumbiaVancouverCanada

Personalised recommendations