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The Chiari Malformations pp 55–72Cite as

Embryology and Pathophysiology of the Chiari I and II Malformations

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Abstract

Hindbrain herniation is only one component of the Chiari malformations. These malformations often have other associated intracranial anomalies and malformations of the vertebral column. Depending on embryologic timing, ­herniation of the cerebellar vermis or tonsils occurs. Additionally, the herniated hindbrain may include the medulla oblongata and fourth ventricle. Currently, no single theory explains all of the malformations seen in the Chiari I and II malformations. These pathologic derailments seem to result from a heterogeneous spectrum of ontogenetic errors and pathological mechanisms, which share some common phenotypical presentations. In this chapter, the theories pertinent to the embryology and pathophysiology of Chiari I and II malformations and their associated anomalies are discussed.

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Authors and Affiliations

  1. Section of Pediatric Neurosurgery, Children’s Hospital of Alabama, Birmingham, AL, USA

    Mohammadali M. Shoja MD

  2. Section of Pediatric Neurosurgery, Children’s of Alabama, 1600 7th Ave. S, Birmingham, AL, 35233, USA

    R. Shane Tubbs MS, PA-C, PhD

  3. Section of Pediatric Neurosurgery, Children’s of Alabama, 1600 7th Ave. S, Lowder 400, Birmingham, AL, 35233-1711, USA

    W. Jerry Oakes MD

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  1. Mohammadali M. Shoja MD
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  2. R. Shane Tubbs MS, PA-C, PhD
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  3. W. Jerry Oakes MD
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Correspondence to R. Shane Tubbs MS, PA-C, PhD .

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Editors and Affiliations

  1. Children's Hospital of Alabama, 7th Ave S 1600, Birmingham, 35233-1711, Alabama, USA

    R. Shane Tubbs

  2. The Children's Hospital of Alabama, 7th Ave S 1600, Birmingham, 35233-1711, Alabama, USA

    W. Jerry Oakes

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Shoja, M.M., Tubbs, R.S., Oakes, W.J. (2013). Embryology and Pathophysiology of the Chiari I and II Malformations. In: Tubbs, R., Oakes, W. (eds) The Chiari Malformations. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6369-6_4

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  • DOI: https://doi.org/10.1007/978-1-4614-6369-6_4

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