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Chiari Malformations Types II, III, IV, and V

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Abstract

This chapter is addressed to the description of the forms of caudal herniation through the foramen magnum different from Chiari type I malformation, which is largely the most common variant of hindbrain herniation. Among the here-illustrated conditions, Chiari type II malformation is the most frequently encountered in the clinical practice, following the epidemiological distribution of the open spina bifida to which it is strictly related. Chiari II malformation provides diagnostic and management challenges because it is not a single malformation but a constellation of malformations involving either the neural (brain, cerebellum, spinal cord) and the bony structures (skull, spine). The clinical picture can vary significantly according to the age at onset and the severity of the brainstem dysfunction. On the other hand, the other variants are very rare, like Chiari type III malformation, or exceptional and nosographically still debated, like Chiari types IV and V malformations. These rare forms are quite often burdened by a dismal prognosis.

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  1. Pediatric Neurosurgery, A. Gemelli Hospital, Rome, Italy

    Luca Massimi & Massimo Caldarelli

  2. Department of Neurosurgery, International Neuroscience Institute, Hannover, Germany

    Concezio Di Rocco

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  1. Luca Massimi
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  2. Massimo Caldarelli
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  3. Concezio Di Rocco
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Correspondence to Luca Massimi .

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  1. International Neuroscience Institute, Hannover, Germany

    Concezio Di Rocco

  2. Oakland Medical Center, Oakland, CA, USA

    Dachling Pang

  3. University of Toronto Hospital for Sick Children, Toronto, ON, Canada

    James T. Rutka

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  1. Department of Neurosurgery, International Neuroscience Institute, Hannover, Germany

    Concezio Di Rocco

  2. Pediatric Neurosurgery, Catholic University Medical School, Rome, Italy

    G. Tamburrini

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Massimi, L., Caldarelli, M., Di Rocco, C. (2018). Chiari Malformations Types II, III, IV, and V. In: Di Rocco, C., Pang, D., Rutka, J. (eds) Textbook of Pediatric Neurosurgery. Springer, Cham. https://doi.org/10.1007/978-3-319-31512-6_9-1

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