Cite

Copy

Tap on and choose 'Add to Home Screen' to create a shortcut app

Tap on and choose 'Install/Install App' to create a shortcut app

Features of Chiari Malformations in Children

This page was last updated on May 9th, 2017

Chiari Type Anatomical Anomaly Frequency of Hydrocephalus Associated CNS Anoimalies
I Tonsillar herniation ≥5 mm below plane of foramen magnum with moderate frequency of syringomyelia Low Multiple
II Caudal herniation of vermis, brainstem, and fourth ventricle with high frequency of syringomyelia High Associated with myelomeningocele and multiple brain anomalies
III Occipital encephalocele containing dysmorphic cerebellum and brainstem N/A N/A
IV Hypoplasia or aplasia of cerebellum N/A N/A

 

Anomalies associated with Chiari I malformation

  • Syndromic craniosynostosis: These disorders include Apert’s and Crouzon’s syndromes
  • Endocrinopathies: These include achondroplasia, acromegaly growth hormone deficiency, and osteopetrosis or Paget’s disease.
  • Neurocutaneous disorders: These disorders include NF1, acanthosis nigricans, blue rubber bleb nevus syndrome, giant congenital melanocytic nevi, LEOPARD syndrome, and Waardenburg syndrome.
  • Beckwith-Wiedemann syndrome
  • Ehlers-Danlos syndrome
  • Craniocervical anomalies: These include basilar invagination, atlantoaxial assimilation and Klippel-Feil syndrome.
  • Acquired tonsillar descent: Elevation in ICP due to conditions such as brain tumors and hydrocephalus may cause acquired tonsillar descent (56).

Your donations keep us going

The ISPN Guide is free to use, but we rely on donations to fund our ongoing work and to maintain more than a thousand pages of information created to disseminate the most up-to-date knowledge in the field of paediatric neurosurgery.

By making a donation to The ISPN Guide you are also indirectly helping the many thousands of children around the world whose treatment depends on well-informed surgeons.

Please consider making a donation today.

Use the app

The ISPN Guide can be used as a standalone app, both on mobile devices and desktop computers. It’s quick and easy to use.

Fully featured

Free registration grants you full access to The Guide and host of featured designed to help further your own education.

Stay updated

The ISPN Guide continues to expand both in breadth and depth. Join our mailing list to stay up-to-date with our progress.