Chiari malformation: Difference between revisions

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'''Chiari malformation''' is a developmental abnormality of the brain.

==Classification==

==General==

*Usually a radiologic diagnosis.

*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===

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).

**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 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==

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==

*[[Fetal autopsy]].

*[[Neuropathology]].

==References==

[[Category:Diagnosis]]

[[Category:Neuropathology]]

@@ Line 1: / Line 1: @@
 '''Chiari malformation''' is a developmental abnormality of the brain.
-==Classification==
+==General==
+*Usually a radiologic diagnosis.
+*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===
 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).
-**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 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==
+*[[Fetal autopsy]].
+*[[Neuropathology]].
 ==References==
 {{Reflist|2}}
+[[Category:Diagnosis]]
 [[Category:Neuropathology]]

Chiari malformation: Difference between revisions

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