Chiari Type 1 Deformity in Children

Pathogenetic, Clinical, Neuroimaging, and Management Aspects

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

Research output: Contribution to journalArticle

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)
JournalNeuropediatrics
DOIs
StateAccepted/In press - Apr 27 2016

Fingerprint

Encephalocele
Neuroimaging
Rhombencephalon
Patient Selection
Arnold-Chiari Malformation
Posterior Cranial Fossa
Incidental Findings
Neurosurgery
Neurology
Prosencephalon
Pediatrics
Sensitivity and Specificity

Keywords

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

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

Cite this

Chiari Type 1 Deformity in Children : Pathogenetic, Clinical, Neuroimaging, and Management Aspects. / Poretti, Andrea; Ashmawy, Ramy; Garzon-Muvdi, Tomas; Jallo, George; Huisman, Thierry A G M; Raybaud, Charles.

In: Neuropediatrics, 27.04.2016.

Research output: Contribution to journalArticle

Poretti, Andrea ; Ashmawy, Ramy ; Garzon-Muvdi, Tomas ; Jallo, George ; Huisman, Thierry A G M ; Raybaud, Charles. / Chiari Type 1 Deformity in Children : Pathogenetic, Clinical, Neuroimaging, and Management Aspects. In: Neuropediatrics. 2016.
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