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Chiari Malformations Types II, III, IV, and V

  • Luca Massimi
  • Massimo Caldarelli
  • Concezio Di Rocco
Living reference work entry

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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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Luca Massimi
    • 1
  • Massimo Caldarelli
    • 1
  • Concezio Di Rocco
    • 2
  1. 1.Pediatric NeurosurgeryA. Gemelli HospitalRomeItaly
  2. 2.Department of NeurosurgeryInternational Neuroscience InstituteHannoverGermany

Section editors and affiliations

  • Concezio Di Rocco
    • 1
  • G. Tamburrini
    • 2
  1. 1.Department of NeurosurgeryInternational Neuroscience InstituteHannoverGermany
  2. 2.Pediatric NeurosurgeryCatholic University Medical SchoolRomeItaly

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