Difference between revisions of "Chiari malformation"

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histology in chiari (mostly type II)
(histology in chiari (mostly type II))
 
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*Chiari type I - tonsils herniated<ref>URL: [http://rarediseases.info.nih.gov/GARD/Disease.aspx?diseaseID=9230 http://rarediseases.info.nih.gov/GARD/Disease.aspx?diseaseID=9230]. Accessed on: 6 May 2011.</ref> (radiologic definition: 4-6 mm below the plane of the foramen magnum).
*Chiari type I - tonsils herniated<ref>URL: [http://rarediseases.info.nih.gov/GARD/Disease.aspx?diseaseID=9230 http://rarediseases.info.nih.gov/GARD/Disease.aspx?diseaseID=9230]. Accessed on: 6 May 2011.</ref> (radiologic definition: 4-6 mm below the plane of the foramen magnum).
**Associated with: sudden death,<ref>{{Cite journal  | last1 = Zhang | first1 = J. | last2 = Shao | first2 = Y. | last3 = Qin | first3 = Z. | last4 = Liu | first4 = N. | last5 = Zou | first5 = D. | last6 = Huang | first6 = P. | last7 = Chen | first7 = Y. | title = Sudden Unexpected Death due to Chiari Type I Malformation in a Road Accident Case. | journal = J Forensic Sci | volume =  | issue =  | pages =  | month = Dec | year = 2012 | doi = 10.1111/1556-4029.12051 | PMID = 23278920 }}</ref> sleep apnea, cerebellar ataxia.
**Associated with: sudden death,<ref>{{Cite journal  | last1 = Zhang | first1 = J. | last2 = Shao | first2 = Y. | last3 = Qin | first3 = Z. | last4 = Liu | first4 = N. | last5 = Zou | first5 = D. | last6 = Huang | first6 = P. | last7 = Chen | first7 = Y. | title = Sudden Unexpected Death due to Chiari Type I Malformation in a Road Accident Case. | journal = J Forensic Sci | volume =  | issue =  | pages =  | month = Dec | year = 2012 | doi = 10.1111/1556-4029.12051 | PMID = 23278920 }}</ref> sleep apnea, cerebellar ataxia.
*Chiari type II - often assoc. with hydrocephaly at birth.
*Chiari type II - often assoc. with hydrocephaly at birth. Often associated with [[myelomeningocele]].
*Chiari type III - cerebellum + brain stem herniate through foramen magnum +/- encephalocele.<ref>URL: [http://www.ninds.nih.gov/disorders/chiari/detail_chiari.htm http://www.ninds.nih.gov/disorders/chiari/detail_chiari.htm]. Accessed on: 6 May 2011.</ref>
*Chiari type III - cerebellum + brain stem herniate through foramen magnum +/- encephalocele.<ref>URL: [http://www.ninds.nih.gov/disorders/chiari/detail_chiari.htm http://www.ninds.nih.gov/disorders/chiari/detail_chiari.htm]. Accessed on: 6 May 2011.</ref>
*Chiari type IV - cerebellar hypoplasia or no cerebellum.
*Chiari type IV - cerebellar hypoplasia or no cerebellum.
==Histology==
Surgery depends on clinical symptoms due to CSF obstructions. Specimens may include: <ref>{{Cite journal  | last1 = Piatt | first1 = JH. | last2 = D'Agostino | first2 = A. | title = The Chiari II malformation: lesions discovered within the fourth ventricle. | journal = Pediatr Neurosurg | volume = 30 | issue = 2 | pages = 79-85 | month = Feb | year = 1999 | doi = 28767 | PMID = 10325563 }}</ref>
* Vertebral bone (decompression)
* Hermiated cerebellar tonsils.
* Reactive / dysplastic [[choroid plexus]].
* Glial nodules.
* Arachnoidal cysts.
* [[Subependymoma]]s.
In cases with [[myelomeningocele]] at autopsy, the posterior fossa should be examined.
==Images==
<gallery>
File:Chiari2.jpg|Schematic display of Chiari type II malformation.
File:Chiari-Malformation_MRT_T2_sag.jpg | Radiology of Chiari type II.
File:Hypertrophic_plexus_chiari_II_low_mag.jpg | Hypertrophic plexus choroideus in Chiari type II (low mag).
File:Hypertrophic_plexus_chiari_II_intermed_mag.jpg | Fibrous tissue within a hypertrophic plexus choroideus in Chiari type II (intermed mag).
</gallery>


==See also==
==See also==
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