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Clinical Presentation of Pediatric Chiari I Malformations

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The Chiari Malformations

Abstract

Clinical presentation of the Chiari I malformation varies with patient age, and children typically present with shorter symptom duration than adults. Symptoms and signs of Chiari I malformation in children are most often attributable to impairment of CSF flow, direct neural compression, and/or syringomyelia. Comparison of contemporary and older literature reveals a trend toward earlier diagnosis, prior to manifestation of significant neurologic morbidity. General availability and use of magnetic resonance imaging likely explains this dramatic shift. Occipital and/or cervical pain is the most common presentation, particularly if Valsalva-induced, followed by scoliosis, syringomyelia, and lower cranial nerve dysfunction. Headache not localized to the occipital/cervical region correlates poorly with Chiari I malformation. The natural history of asymptomatic children with Chiari I malformation (absent syringomyelia) appears benign. Surgical treatment should be reserved for patients with localized pain, syringomyelia, or cervicomedullary/cerebellar dysfunction.

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Authors and Affiliations

  1. Section of Pediatric Neurosurgery, Children’s Hospital of Alabama, 1600 7th Ave. S., JFL 400, 35233, Birmingham, AL, USA

    Curtis J. Rozzelle MD

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  1. Curtis J. Rozzelle MD
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Correspondence to Curtis J. Rozzelle 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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Rozzelle, C.J. (2013). Clinical Presentation of Pediatric Chiari I Malformations. In: Tubbs, R., Oakes, W. (eds) The Chiari Malformations. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6369-6_20

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  • DOI: https://doi.org/10.1007/978-1-4614-6369-6_20

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