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Natural history of Chiari type I malformation in children

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Abstract

The decision on whether or not to operate children with Chiari type I malformation (CIM) is difficult and controversial, because of the lack of information about the natural evolution of such a disease. Herein, we report on the evolution of 16 asymptomatic children with incidentally diagnosed CIM (mean age: 6.7 years; mean follow-up: 5.8 years). No patients required suboccipital decompression. Thirteen children remained asymptomatic, with stable or improved radiological picture (worsening in 2 cases). Three cases showed appearance of symptoms: one did not require any treatment; the remaining two underwent endoscopic third ventriculostomy because of hydrocephalus, which is a possible consequence of CIM. This analysis shows a favorable natural outcome of CIM in children, thus suggesting a conservative management in asymptomatic cases. However, multicentric studies are required to validate this data.

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References

  1. Genitori L, Peretta P, Nurisso C, Macinante L, Mussa F (2000) Chiari type I anomalies in children and adolescents: minimally invasive management in a series of 53 cases. Childs Nerv Syst 16:707–718

    Article  PubMed  CAS  Google Scholar 

  2. Navarro R, Olavarria G, Seshadri R, Gonzales-Portillo G, McLone DG, Tomita T (2004) Surgical results of posterior fossa decompression for patients with Chiari I malformation. Childs Nerv Syst 20:349–356

    Article  PubMed  Google Scholar 

  3. 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 33:311–317

    Article  PubMed  CAS  Google Scholar 

  4. Schijman E, Steinbok P (2004) International survey on the management of Chiari I malformation and syringomyelia. Childs Nerv Syst 20:341–348

    Article  PubMed  Google Scholar 

  5. Elster AD, Chen MY (1992) Chiari I malformations: clinical and radiological reappraisal. Radiology 183:347–353

    PubMed  CAS  Google Scholar 

  6. Meadows J, Kraut M, Guarnieri M, Haroun RI, Carson BS (2000) Asymptomatic Chiari type I malformations identified on magnetic resonance imaging. J Neurosurg 92:920–926

    Article  PubMed  CAS  Google Scholar 

  7. Kojima A, Mayanagi K, Okui S (2009) Progression of pre-existing Chiari type I malformation secondary to cerebellar hemorrhage. Neurol Med Chir (Tokyo) 49:90–92

    Article  Google Scholar 

  8. Novegno F, Caldarelli M, Massa A, Chieffo D, Massimi L, Pettorini B, Tamburrini G, Di Rocco C (2008) The natural history of the Chiari type I anomaly. J Neurosurg Pediatr 2:179–187

    Article  PubMed  Google Scholar 

  9. Miller JH, Limbrick DD Jr, Callen M, Smyth MD (2008) Spontaneous resolution of Chiari malformation type I in monozygotic twins. J Neurosurg Pediatr 2:317–319

    Article  PubMed  Google Scholar 

  10. 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 49:637–640

    PubMed  CAS  Google Scholar 

  11. Tokunaga M, Minami S, Isobe K, Moriya H, Kitahara H, Nakata Y (2001) Natural history of scoliosis in children with syringomyelia. J Bone Jt Surg Br 83:371–376

    Article  CAS  Google Scholar 

  12. Javatallabhula NS, Armstrong J, Sgouros S, Whitehouse W (2006) Spontaneous resolution of isolated Chiari I malformation. Childs Nerv Syst 22:201–203

    Article  Google Scholar 

  13. Sun JC, Steinbock P, Cochrane DD (2000) Spontaneous resolution and recurrence of a Chiari I malformation and associated syringomyelia. Case report. J Neurosurg 92:207–210

    Article  PubMed  CAS  Google Scholar 

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The authors declare that there is no actual or potential conflict of interest in relation to this article.

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Correspondence to Luca Massimi.

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Massimi, L., Caldarelli, M., Frassanito, P. et al. Natural history of Chiari type I malformation in children. Neurol Sci 32 (Suppl 3), 275–277 (2011). https://doi.org/10.1007/s10072-011-0684-3

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