Journal of Korean Neurosurgical Society

The Korean Neurosurgical Society (대한신경외과학회)
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Chiari 1.5 Malformation : An Advanced Form of Chiari I Malformation

  • Kim, In-Kyeong (Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine) ;
  • Wang, Kyu-Chang (Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine) ;
  • Kim, In-One (Division of Pediatric Radiology, Seoul National University Children's Hospital, Seoul National University College of Medicine) ;
  • Cho, Byung-Kyu (Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine)
  • Received : 2010.08.10
  • Accepted : 2010.10.04
  • Published : 2010.10.28
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Abstract

The Chiari 1.5 malformation is defined as a tonsillar hemiation within a Chiari I malformation with additional caudal descent of the brainstem through the foramen magnum. We describe a patient with Chiari I malformation who evolved to Chiari 1.5 malformation during longitudinal follow-up. A 15-year-old girl presented with neck pain during exercise for two years. She had been diagnosed with Chiari I malformation with mild hydrocephalus after minor cervical trauma at the age of six years. At that time, she was asymptomatic. After she complained of aggravated neck pain, neuroimaging (nine years after first imaging) revealed caudal descent of the brainstem and syringomyelia in addition to progression of tonsillar hemiation. Posterior fossa decompressive surgery resulted in complete resolution of neck pain. Based on neuroimaging and operative findings, she was diagnosed as Chiari 1.5 malformation. Neuroimaging performed seven months after surgery showed an increased anterior-posterior diameter of the medulla oblongata and markedly decreased syringomyelia. This case demonstrates progressive developmental process of the Chiari 1.5 malformation as an advanced form of the Chiari I malformation.

Keywords

References

  1. Armonda RA, Citrin CM, Foley KT, Ellenbogen RG : Quantitative cine-mode magnetic resonance imaging of Chiari I malformations: an analysis of cerebrospinal fluid dynamics. Neurosurgery 35 : 214-223; discussion 223-224, 1994 https://doi.org/10.1227/00006123-199408000-00006
  2. Bersani G, Paolemili M, Quartini A, Clemente R, Gherardelli S, Iannitelli A, et al. : Neurological soli: signs and cerebral measurements investigated by means of MRI in schizophrenic patients. Neurosci Lett 413 : 82-87,2007 https://doi.org/10.1016/j.neulet.2006.11.032
  3. Capra V, De Marco p, Merello E, Baffiko AM, Baldi M, Divizia MT, et al. : Craniosynostosis, hydrocephalus, Chiari I malformation and radioulnar synostosis: probably a new syndrome. Eur J Med Genet 52 : 17-22, 2009 https://doi.org/10.1016/j.ejmg.2008.10.005
  4. Cardoso M, Keating RF : Neurosurgical management of spinal dysraphism and neurogenic scoliosis. Spine (Phila Pa 1976) 34 : 1775-1782, 2009 https://doi.org/10.1097/BRS.0b013e3181b07914
  5. Chang HS, Nakagawa H : Hypothesis on the pathophysiology of syringomyelia based on simulation of cerebrospinal fluid dynamics. J Neurol Neurosurg Psychiatry 74: 344-347, 2003 https://doi.org/10.1136/jnnp.74.3.344
  6. Cronin CG, Lohan DG, Mhuircheartigh JN, Meehan CP, Murphy J, Roche C: CT evaluation of Chamberlain's, McGregor's, and McRae's skull-base lines. Clin Radiol 64 : 64-69, 2009 https://doi.org/10.1016/j.crad.2008.03.012
  7. Greiz D : Unraveling the riddle of syringomyelia. Neurosurg Rev 29: 251-263; discussion 264, 2006 https://doi.org/10.1007/s10143-006-0029-5
  8. Koehler PJ : Chiari's description of cerebellar ectopy (1891). With a summary of Cleland's and Arnold's contributions and some early observations on neural-tube defects. J Neurosurg 75 : 823-826, 1991 https://doi.org/10.3171/jns.1991.75.5.0823
  9. Meadows J, Kraut M, Guarnieri M, Haroun RI, Carson BS : Asymptomatic Chiari Type I malformations identified on magnetic resonance imaging. J Neurosurg 92 : 920-926, 2000 https://doi.org/10.3171/jns.2000.92.6.0920
  10. Snyder P : Chiari malformation and syringomyelia. Radiol Technol 79 : 555-558, 2008
  11. Tubbs RS, Iskandar BJ, Bartolucci AA, Oakes WJ: A critical analysis of the Chiari 1.5 malformation. J Neurosurg 101: 179-183, 2004 https://doi.org/10.3171/jns.2004.101.2.0179
  12. Tubbs RS, McGirt MJ, Oakes WJ : Surgical experience in 130 pediatric patients with Chiari I malformations. J Neurosurg 99: 291-296, 2003 https://doi.org/10.3171/jns.2003.99.2.0291
  13. Tubbs RS, Wellons JC 3rd, Blount JP, Grabb PA, Oakes WJ : Inclination of the odontoid process in the pediatric Chiari I malformation. J Neurosurg 98 : 43-49, 2003 https://doi.org/10.3171/jns.2003.98.1.0043

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