Chiari Type 1 Deformity in Children: Pathogenetic, Clinical, Neuroimaging, and Management Aspects

Andrea Poretti, Ramy Ashmawy, Tomas Garzon-Muvdi, George I. Jallo, Thierry A.G.M. Huisman, Charles Raybaud

Research output: Contribution to journalReview article

20 Scopus citations

Abstract

Our understanding of cerebellar tonsillar herniation evolved over time and nowadays various pathomechanisms have been proposed. Causes of tonsillar herniation share a discrepancy between content (fore- and hindbrain) and container (supratentorial cranial vault, posterior fossa), may be associated with abnormalities of the craniocervical junction, and may have a developmental or acquired nature. In tonsillar herniation, the hindbrain is not malformed but deformed. Accordingly, Chiari type 1 deformity, not Chiari type 1 malformation is the correct term to characterize primary tonsillar herniation. Chiari type 1 deformity is commonly seen in pediatric neurology, neuroradiology, and neurosurgery and may have various clinical presentations depending on patient age. In addition, Chiari type 1 deformity is increasingly found by neuroimaging studies as an incidental finding in asymptomatic children. An accurate and reliable selection of patients based on clinical and neuroimaging findings is paramount for the success of neurosurgical treatment. Future studies are needed to provide selection criteria with a higher sensitivity and specificity.

Original languageEnglish (US)
Pages (from-to)293-307
Number of pages15
JournalNeuropediatrics
Volume47
Issue number5
DOIs
StatePublished - Oct 1 2016
Externally publishedYes

Keywords

  • cerebellum
  • Chiari type 1
  • children
  • neuroimaging
  • neurosurgery

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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