Chiari malformation: Difference between revisions

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histology in chiari (mostly type II)
 
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*Usually a radiologic diagnosis.
*Usually a radiologic diagnosis.
*May be seen in a [[fetal autopsy]].
*May be seen in a [[fetal autopsy]].
Clinical:<ref>{{Cite journal  | last1 = Ferré Masó | first1 = A. | last2 = Poca | first2 = MA. | last3 = de la Calzada | first3 = MD. | last4 = Solana | first4 = E. | last5 = Romero Tomás | first5 = O. | last6 = Sahuquillo | first6 = J. | title = Sleep disturbance: a forgotten syndrome in patients with Chiari I malformation. | journal = Neurologia | volume =  | issue =  | pages =  | month = Mar | year = 2011 | doi = 10.1016/j.nrl.2011.01.008 | PMID = 21420201 }}</ref>
*Headaches, occipital.
*Dizziness.
*Nocturnal respiratory abnormalities.


===Classification===
===Classification===
Numbered from least severe to most severe:
Numbered from least severe to most severe:
*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, 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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{{Reflist|2}}
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Neuropathology]]
[[Category:Neuropathology]]

Latest revision as of 09:12, 16 September 2015

Chiari malformation is a developmental abnormality of the brain.

General

Clinical:[1]

  • Headaches, occipital.
  • Dizziness.
  • Nocturnal respiratory abnormalities.

Classification

Numbered from least severe to most severe:

  • Chiari type I - tonsils herniated[2] (radiologic definition: 4-6 mm below the plane of the foramen magnum).
    • Associated with: sudden death,[3] sleep apnea, cerebellar ataxia.
  • Chiari type II - often assoc. with hydrocephaly at birth. Often associated with myelomeningocele.
  • Chiari type III - cerebellum + brain stem herniate through foramen magnum +/- encephalocele.[4]
  • Chiari type IV - cerebellar hypoplasia or no cerebellum.

Histology

Surgery depends on clinical symptoms due to CSF obstructions. Specimens may include: [5]

  • Vertebral bone (decompression)
  • Hermiated cerebellar tonsils.
  • Reactive / dysplastic choroid plexus.
  • Glial nodules.
  • Arachnoidal cysts.
  • Subependymomas.

In cases with myelomeningocele at autopsy, the posterior fossa should be examined.

Images

  • Schematic display of Chiari type II malformation.
    Schematic display of Chiari type II malformation.
  • Radiology of Chiari type II.
    Radiology of Chiari type II.
  • Hypertrophic plexus choroideus in Chiari type II (low mag).
    Hypertrophic plexus choroideus in Chiari type II (low mag).
  • Fibrous tissue within a hypertrophic plexus choroideus in Chiari type II (intermed mag).
    Fibrous tissue within a hypertrophic plexus choroideus in Chiari type II (intermed mag).

See also

References

  1. ↑ Ferré Masó, A.; Poca, MA.; de la Calzada, MD.; Solana, E.; Romero Tomás, O.; Sahuquillo, J. (Mar 2011). "Sleep disturbance: a forgotten syndrome in patients with Chiari I malformation.". Neurologia. doi:10.1016/j.nrl.2011.01.008. PMID 21420201.
  2. ↑ URL: http://rarediseases.info.nih.gov/GARD/Disease.aspx?diseaseID=9230. Accessed on: 6 May 2011.
  3. ↑ Zhang, J.; Shao, Y.; Qin, Z.; Liu, N.; Zou, D.; Huang, P.; Chen, Y. (Dec 2012). "Sudden Unexpected Death due to Chiari Type I Malformation in a Road Accident Case.". J Forensic Sci. doi:10.1111/1556-4029.12051. PMID 23278920.
  4. ↑ URL: http://www.ninds.nih.gov/disorders/chiari/detail_chiari.htm. Accessed on: 6 May 2011.
  5. ↑ Piatt, JH.; D'Agostino, A. (Feb 1999). "The Chiari II malformation: lesions discovered within the fourth ventricle.". Pediatr Neurosurg 30 (2): 79-85. doi:28767. PMID 10325563.
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