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The Chiari Malformations and Hydrocephalus

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The Chiari Malformations

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Abstract

The association of hydrocephalus and the Chiari malformations has been described from the time of Hans Chiari’s initial report in 1891. Whether hydrocephalus is the cause of or the result of hindbrain herniation remains a subject of long-standing controversy, but recent advances in vascular and volumetric imaging may eventually provide definitive information to settle the debate. Though there is a wide range of hindbrain herniations that fall under the Chiari rubric, most authors would agree that coexisting hydrocephalus should be managed with CSF diversion first, either by shunting or endoscopic third ventriculostomy, before consideration is given to posterior fossa decompression. It is crucial to keep in mind that pseudotumor cerebri may also present with symptoms that mimic those seen in Chiari malformation, making it critical that the neurosurgeon strive to differentiate these two groups prior to surgical intervention for optimal outcome.

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Authors and Affiliations

  1. Department of Neurological Surgery, Monroe Carell Jr Children’s Hospital at Vanderbilt/Vanderbilt University, Nashville, TN, USA

    Robert P. Naftel

  2. Division of Pediatric Neurosurgery, Department of Neurosurgery, University of Alabama at Birmingham, Children’s of Alabama, Birmingham, AL, USA

    James M. Johnston

  3. Department of Neurological Surgery, Division of Pediatric Neurosurgery, Monroe Carell Jr Children’s Hospital at Vanderbilt/Vanderbilt University, Nashville, TN, USA

    John C. Wellons III

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  1. Robert P. Naftel
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  2. James M. Johnston
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  3. John C. Wellons III
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Correspondence to Robert P. Naftel .

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Editors and Affiliations

  1. Department of Neurosurgery, Tulane University School of Medicine, New Orleans, LA, USA

    R. Shane Tubbs

  2. Department of Neurosurgery, Aydın Adnan Menderes University School of Medicine, Efeler, Aydın, Turkey

    Mehmet Turgut

  3. Departments of Neurosurgery and Pediatrics, University of Alabama, Birmingham, AL, USA

    W. Jerry Oakes

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Naftel, R.P., Johnston, J.M., Wellons, J.C. (2020). The Chiari Malformations and Hydrocephalus. In: Tubbs, R., Turgut, M., Oakes, W. (eds) The Chiari Malformations. Springer, Cham. https://doi.org/10.1007/978-3-030-44862-2_13

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