Chiari malformations
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Chiari malformations are a group of defects associated with congentialcongenital caudal 'displacement' of the cerebellum and brainstem.
Initial descriptions were based on autopsy observations. Three types were described, with a fourthothers added later. Types II and III are likely to be related to each other 1.
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Chiari I malformation
- most common
- peg-like cerebellar tonsils displaced into the upper cervical canal through the foramen magnum
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Chiari 1.5 malformation
- described in the literature as both a condition in its own right as well as a variant of Chiari I malformation
- caudal descent of cerebellar tonsils and brain stem
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Chiari II malformation
- displacement of the medulla, fourth ventricle and
cerebellumcerebellar vermis through the foramen magnum - usually with associated with a lumbosacral spinal myelomeningocoele
- displacement of the medulla, fourth ventricle and
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Chiari III malformation
- features similar to Chiari II but with an occipital and/or high cervical encephalocoele
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Chiari IV malformation
- severe cerebellar hypoplasia without displacement of the cerebellum through the foramen magnum
- probably a variation of cerebellar hypoplasia
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Chiari V malformation
- absent cerebellum
- herniation of the occipital lobe through the foramen magnum
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Chiari 0 malformation
- syrinx
- no cerebellar tonsil or brain stem descent
History and etymology
It was first described by Hans Chiari, Austrian pathologist, (1851-1914) in 1891 3. In this and subsequent papers Chiari also credited Julius Arnold (1835-1915) Professor of Anatomy at Heidelberg, on the grounds of a previous publication by Arnold believed by him to be of a Chiari II malformation. It appears that this is not actually the case, and as such the term Arnold-Chiari to denote Chiari II malformations is no longer advocated 4.
-<p><strong>Chiari malformations</strong> are a group of defects associated with congential caudal 'displacement' of the cerebellum and brainstem.</p><p>Initial descriptions were based on autopsy observations. Three types were described, with a fourth added later. Types II and III are likely to be related to each other <sup>1</sup>. </p><ul>- +<p><strong>Chiari malformations</strong> are a group of defects associated with congenital caudal 'displacement' of the cerebellum and brainstem.</p><p>Initial descriptions were based on autopsy observations. Three types were described, with others added later. Types II and III are likely to be related to each other <sup>1</sup>. </p><ul>
-<a href="/articles/chiari-15-malformation">Chiari 1.5 malformation</a><ul><li>described in the literature as both a condition in its own right as well as a variant of Chiari I malformation</li></ul>- +<a href="/articles/chiari-15-malformation">Chiari 1.5 malformation</a><ul>
- +<li>described in the literature as both a condition in its own right as well as a variant of Chiari I malformation</li>
- +<li>caudal descent of cerebellar tonsils and brain stem </li>
- +</ul>
-<li>displacement of the medulla, fourth ventricle and cerebellum through the foramen magnum</li>- +<li>displacement of the medulla, fourth ventricle and cerebellar vermis through the foramen magnum</li>
- +<li>
- +<a href="/articles/chiari-v-malformation">Chiari V malformation </a><ul>
- +<li>absent cerebellum </li>
- +<li>herniation of the occipital lobe through the foramen magnum </li>
- +</ul>
- +</li>
- +<li>
- +<a href="/articles/chiari-0-malformation">Chiari 0 malformation </a><ul>
- +<li>syrinx</li>
- +<li>no cerebellar tonsil or brain stem descent </li>
- +</ul>
- +</li>
References changed:
- 5. Tubbs RS, Muhleman M, Loukas M et-al. A new form of herniation: the Chiari V malformation. Childs Nerv Syst. 2012;28 (2): 305-7. <a href="http://dx.doi.org/10.1007/s00381-011-1616-5">doi:10.1007/s00381-011-1616-5</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/22038152">Pubmed citation</a><span class="auto"></span>
- 6. Tubbs RS, Elton S, Grabb P et-al. Analysis of the posterior fossa in children with the Chiari 0 malformation. Neurosurgery. 2001;48 (5): 1050-4. <a href="http://www.ncbi.nlm.nih.gov/pubmed/11334271">Pubmed citation</a><span class="auto"></span>
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