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Pathology of Chiari I and II Malformations

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The Chiari Malformations

Abstract

Chiari I and Chiari II malformations are generally categorized as primary cerebellar anomalies although critical review of their morphological features indicates that the cerebellar component more often is a secondary abnormality rather than a primary developmental defect. The basic malformation involves that portion of basicranium which comprises the posterior fossa; essentially it is too small. With a few exceptions, the non-bony abnormalities associated with Chiari I are secondary. In contrast the constellation of lesions that form Chiari II are complex and affect all levels of the neuraxis to a greater or lesser degree and consist of a combination of primary malformation and acquired lesions. Ironically, the cerebellar abnormalities more often fall into the acquired category.

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Author information

Authors and Affiliations

  1. Department of Anatomic Pathology, The Children’s Hospital of Philadelphia, 324 34th Street, Philadelphia, PA, 19104, USA

    Lucy B. Rorke-Adams MD

Authors
  1. Lucy B. Rorke-Adams MD
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Correspondence to Lucy B. Rorke-Adams MD .

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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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Rorke-Adams, L.B. (2013). Pathology of 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_8

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  • DOI: https://doi.org/10.1007/978-1-4614-6369-6_8

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-6368-9

  • Online ISBN: 978-1-4614-6369-6

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