Abstract
Bony and soft tissue morphological abnormalities of the craniovertebral junction may lead to posterior fossa compression syndromes such as Chiari 1 malformations. More subtle physiological contributions to these syndromes, such as cerebrospinal fluid (CSF) dynamics and abnormal motion at the junction, are often overlooked but equally important. Interplay of these components may lead to additional hindbrain malformation syndromes, classified as Chiari 1.5 and Chiari 0. These syndromes have clinical presentations similar to Chiari 1 malformations and are treated similarly with good outcomes.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Liu W, Wu H, Aikebaier Y, Wulabieke M, Paerhati R, Yang X. No significant difference between Chiari malformation type 1.5 and type I. Clin Neurol Neurosurg. 2017;157:34–9.
Bogdanov EI, Heiss JD, Mendelevich EG, Mikhaylov IM, Haass A. Clinical and neuroimaging features of “idiopathic” syringomyelia. Neurology. 2004;62:791–4.
Tubbs RS, Elton S, Grabb P, Dockery SE, Bartolucci AA, Oakes WJ. Analysis of the posterior fossa in children with the Chiari 0 malformation. Neurosurgery. 2001;48:1050–4.
Kyoshima K, Kuroyanagi T, Oya F, Kamijo Y, El-Noamany H, Kobayashi S. Syringomyelia without hindbrain herniation: tight cisterna magna. Report of four cases and a review of the literature. Neurosurgery. 2002;96:239–49.
Iskandar BJ, Hedlund GL, Grabb PA, Oakes WJ. The resolution of syringohydromyelia without hindbrain herniation after posterior fossa decompression. J Neurosurg. 1998;89:212–6.
Azahraa Haddad F, Qaisi I, Joudeh N, Dajani H, Jumah F, Elmashala A, et al. The newer classifications of the Chiari malformations with clarifications: an anatomical review. Clin Anat. 2018;31(3):314–22.
Chern JJ, Gordon AJ, Mortazavi MM, Tubbs RS, Oakes WJ. Pediatric Chiari malformation type 0: a 12-year institutional experience. J Neurosurg Pediatr. 2011;8:1–5.
Tubbs RS, Iskandar BJ, Bartolucci AA, Oakes WJ. A critical analysis of the Chiari 1.5 malformation. J Neurosurg Pediatr. 2004;101:179–83.
Greenlee JD, Donovan KA, Hasan DM, Menezes AH. Chiari I malformation in the very young child: the spectrum of presentations and experience in 31 children under age 6 years. Pediatrics. 2002;110:1212–9.
Strickland A, Baker CM, Siatkowski RM, Mapstone TB. Divergence insufficiency alleviated by posterior fossa decompression with duraplasty in a patient with Chiari type 1.5 malformation. J Neurosurg Pediatr. 2018;22(5):504–7.
Janjua MB, Ivasyk I, Greenfield JP. Vertebrobasilar insufficiency due to distal posterior inferior cerebellar artery compression in Chiari 1.5. World Neurosurg. 2017;4:1050.e1–e6. Epub 2017 Jun 3.
Ozsoy KM, Oktay K, Cetinalp NE, Gezercan Y, Erman T. The role of cine flow magnetic resonance imaging in patients with Chiari 0 malformation. Turk Neurosurg. 2018;28(2):251–6.
Shijman E, Steinbok P. International survey on the management of Chiari I malformation and syringomyelia. Childs Nerv Syst. 2004;20:341–8.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2020 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Rindler, R.S., Chern, J.J. (2020). Newer Subsets: Chiari 1.5 and Chiari 0 Malformations. In: Tubbs, R., Turgut, M., Oakes, W. (eds) The Chiari Malformations. Springer, Cham. https://doi.org/10.1007/978-3-030-44862-2_3
Download citation
DOI: https://doi.org/10.1007/978-3-030-44862-2_3
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-44861-5
Online ISBN: 978-3-030-44862-2
eBook Packages: MedicineMedicine (R0)