Arnold-Chiari malformation classification: Difference between revisions

Jump to navigation Jump to search
VisualWikitext
Prashanthsaddala (talk | contribs)
nocaptcha
16,188 edits
Created page with "__NOTOC__ {{Arnold-Chiari malformation}} {{CMG}} ==Overview== == Classification == The Austrian pathologist Hans Chiari in the late 19th century described seemingly related ..."
 
Fahimeh Shojaei (talk | contribs)
nocaptcha, Administrators
5,051 edits
No edit summary
 
(15 intermediate revisions by 2 users not shown)
Line 1: Line 1:
__NOTOC__
__NOTOC__
{{Arnold-Chiari malformation}}
{{Arnold-Chiari malformation}}
{{CMG}}
{{CMG}} {{AE}} {{Fs}}
==Overview==
 
<br />
 
== Overview ==
Arnold chiari malformation may be classified according to [[herniation]] content into 4 subtypes. Type 1 has [[herniation]] of [[Cerebellar tonsil|cerebellar tonsils]]. Type 2 has [[herniation]] of [[Cerebellar tonsil|cerebellar tonsils]] and [[Cerebellar vermis|vermis]]. Type 3 has [[herniation]] of [[cerebellar tonsil]] and [[Cerebellar vermis|vermis]] and lower [[brain stem]]. Type 4 has [[cerebellar hypoplasia]] with [[brain stem]] in [[posterior fossa]].


== Classification ==
== Classification ==
Arnold chiari malformation may be classified according to [[herniation]] content into 4 subtypes:<ref name="pmid26246090">{{cite journal |vauthors=Cotes C, Bonfante E, Lazor J, Jadhav S, Caldas M, Swischuk L, Riascos R |title=Congenital basis of posterior fossa anomalies |journal=Neuroradiol J |volume=28 |issue=3 |pages=238–53 |date=June 2015 |pmid=26246090 |pmc=4757284 |doi=10.1177/1971400915576665 |url=}}</ref>


The Austrian pathologist Hans Chiari in the late 19th century described seemingly related anomalies of the hindbrain, the so called Chiari malformations I, II and III. Later, other investigators added a fourth (Chiari IV) malformation. The scale of severity is rated I - IV, with IV being the most severe. Types III and IV are very rare.<ref name="urlArnold Chiari Malformation">{{cite web|url=http://neurosurgery.ucla.edu/body.cfm?id=109 |title=Arnold Chiari Malformation |work= |accessdate=}}</ref>
{|
 
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Subtypes
{| class="wikitable"
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Explanation
|-
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Association
! Type
! Presentation
! Other notes
|-
| I
| A congenital malformation. Is generally asymptomatic during childhood, but often manifests with headaches and cerebellar symptoms. Herniation of [[cerebellar tonsil]]s.<ref name="pmid19246872">{{Cite journal|author=Kojima A, Mayanagi K, Okui S |title=Progression of pre-existing Chiari type I malformation secondary to cerebellar hemorrhage: case report |journal=Neurol. Med. Chir. (Tokyo) |volume=49 |issue=2 |pages=90–2 |year=2009 |month=February |pmid=19246872 |doi= 10.2176/nmc.49.90|url=http://joi.jlc.jst.go.jp/JST.JSTAGE/nmc/49.90?from=PubMed |format=}} {{Dead link|date=May 2009}}</ref><ref name="pmid16509477">{{Cite journal|author=O'Shaughnessy BA, Bendok BR, Parkinson RJ, ''et al.'' |title=Acquired Chiari malformation Type I associated with a supratentorial arteriovenous malformation. Case report and review of the literature |journal=J. Neurosurg. |volume=104 |issue=1 Suppl |pages=28–32 |year=2006 |month=January |pmid=16509477 |doi=10.3171/ped.2006.104.1.28 |url=http://thejns.org/doi/abs/10.3171/ped.2006.104.1.28?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%3dncbi.nlm.nih.gov}}</ref>
| The most common form.
|-
|-
|Syndrome of occipitoatlantoaxial hypermobility
! style="background: #DCDCDC; text-align: center;" |Type1
|An acquired Chiari I Malformation in patients with hereditary disorders of connective tissue.<ref name="Milhorat-2007"/> Patients who exhibit extreme joint [[hypermobility]] and connective tissue weakness as a result of [[Ehlers-Danlos syndrome]] or [[Marfan Syndrome]] are susceptible to instabilities of the craniocervical junction and thus acquiring a Chiari Malformation. This type is difficult to diagnose and treat.<ref name="Conquer Chiari, Connective Tissue Disorders">{{cite web|url=http://www.conquerchiari.org/subs%20only/volume%204/issue%204(10)/bland%20connective%20tissue%204(10).asp |title=Dr. Bland Discusses Chiari & EDS 4(10) |publisher=Conquerchiari.org |date=2006-11-20 |accessdate=2011-11-04}}</ref>
| style="background: #F5F5F5;" |[[Herniation]] parts: [[Cerebellar tonsils]]
|
*
| style="background: #F5F5F5;" |[[Syringomyelia]]
|-
|-
| II
! style="background: #DCDCDC; text-align: center;" |Type 2
| Usually accompanied by a [[lumbar]] [[myelomeningocele]]<ref name="urlNeuroradiology - Chiari malformation (I-IV)">{{cite web|url=http://www.mir.wustl.edu/neurorad/internal.asp?NavID=123 |title=Neuroradiology - Chiari malformation (I-IV) |work= |accessdate=}}</ref> leading to partial or complete paralysis below the spinal defect. As opposed to the less pronounced tonsillar herniation seen with Chiari I, there is a larger cerebellar vermian displacement. Low lying [[torcular herophili]], tectal beaking, and hydrocephalus with consequent clival hypoplasia are classic anatomic associations.<ref>{{cite web|url =http://www.cchs.net/pediatricradiology/imagegallery/default.asp|title =Pediatric Radiology Image Gallery|accessdate = June 14, 2010|year = 2010|title = Cleveland Clinic Children's Hospital Pediatric Radiology Image Gallery|accessdate = June 14, 2010|publisher = [[Cleveland Clinic]]| archiveurl= http://web.archive.org/web/20100627053310/http://www.cchs.net/pediatricradiology/imagegallery/default.asp| archivedate= 27 June 2010 <!--DASHBot-->| deadurl= no}}</ref> The position of the torcular herophili is important for distinction from [[Dandy-Walker syndrome]] in which it is classically upturned. This is important because the hypoplastic cerebellum of Dandy-Walker may be difficult to distinguish from a Chiari malformation that has herniated or is ectopic on imaging. [[Colpocephaly]] may be seen due to the associated neural tube defect.
| style="background: #F5F5F5;" |[[Herniation]] parts: [[Cerebellar tonsils]] and [[vermis]]
|
*
| style="background: #F5F5F5;" |Lumbosacral [[myelomeningocele]]
|-
|-
| III
! style="background: #DCDCDC; text-align: center;" |Type 3
| Causes severe neurological defects. It is associated with an [[occiput|occipital]] [[encephalocele]].<ref name="Mesh">{{MeshName|Arnold-Chiari+Malformation}}</ref>
| style="background: #F5F5F5;" |[[Herniation]] parts: [[Cerebellar tonsil]] and [[vermis]], lower [[brain stem]]  
|
*
| style="background: #F5F5F5;" |[[Occipital]] [[encephalocele]]
|-
|-
| IV
! style="background: #DCDCDC; text-align: center;" |Type 4
| Characterized by a lack of [[cerebellar]] development.<ref name="urlChiari Malformations - Department of Neurological Surgery">{{cite web|url=http://www.cumc.columbia.edu/dept/nsg/ct/chiari_malformation.html |title=Chiari Malformations - Department of Neurological Surgery |work= |accessdate=}}</ref>
| style="background: #F5F5F5;" |[[Herniation]] parts: [[Brain stem]] located in [[posterior fossa]], underdeveloped [[cerebellum]]
|
*
| style="background: #F5F5F5;" |A variation of [[cerebellar hypoplasia]]
|}
|}
[[File:Neck MRI 130850-dichromatic t1-t2-t2.png|thumb|200px|Syringomiyelia associated with Chiari malformation]]
[[File:Neck MRI 130850-dichromatic t1-t2-t2.png|thumb|200px|Syringomiyelia associated with Chiari malformation]]
Other conditions sometimes associated with Chiari Malformation include [[hydrocephalus]],<ref name="urlNeuropathology For Medical Students">{{cite web|url=http://www.pathology.vcu.edu/WirSelfInst/neuro_medStudents/devdis.html |title=Neuropathology For Medical Students |work= |accessdate=}}</ref> [[syringomyelia]], [[spinal curvature]], [[tethered spinal cord syndrome]], and connective tissue disorders<ref name="Milhorat-2007">{{Cite journal|author=Milhorat TH, Bolognese PA, Nishikawa M, McDonnell NB, Francomano CA |title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and chiari malformation type I in patients with hereditary disorders of connective tissue |journal=[[Journal of Neurosurgery|Journal of Neurosurgery: Spine]] |volume=7 |issue=6 |pages=601–9 |year=2007 |month=December |pmid=18074684 |doi=10.3171/SPI-07/12/601 |url=http://thejns.org/doi/full/10.3171/SPI-07/12/601}}</ref> such as [[Ehlers-Danlos syndrome]] and [[Marfan Syndrome]].
<br />


==References==
==References==
{{reflist|2}}
{{reflist|2}}
[[Category:Neurology]]
[[Category:Disease]]
[[Category:Nervous system]]
[[Category:Neurological disorders]]
[[Category:Needs overview]]


{{WH}}
{{WH}}
{{WS}}
{{WS}}

Latest revision as of 14:41, 8 August 2019

Arnold-Chiari malformation Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Arnold-Chiari malformation from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X Ray

Echocardiography or Ultrasound

CT

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Interventions

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Case #2

Arnold-Chiari malformation classification On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Arnold-Chiari malformation classification

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Arnold-Chiari malformation classification

CDC on Arnold-Chiari malformation classification

Arnold-Chiari malformation classification in the news

Blogs on Arnold-Chiari malformation classification

Directions to Hospitals Treating Arnold-Chiari malformation

Risk calculators and risk factors for Arnold-Chiari malformation classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Fahimeh Shojaei, M.D.


Overview

Arnold chiari malformation may be classified according to herniation content into 4 subtypes. Type 1 has herniation of cerebellar tonsils. Type 2 has herniation of cerebellar tonsils and vermis. Type 3 has herniation of cerebellar tonsil and vermis and lower brain stem. Type 4 has cerebellar hypoplasia with brain stem in posterior fossa.

Classification

Arnold chiari malformation may be classified according to herniation content into 4 subtypes:[1]

Subtypes Explanation Association
Type1 Herniation parts: Cerebellar tonsils
Syringomyelia
Type 2 Herniation parts: Cerebellar tonsils and vermis
Lumbosacral myelomeningocele
Type 3 Herniation parts: Cerebellar tonsil and vermis, lower brain stem
Occipital encephalocele
Type 4 Herniation parts: Brain stem located in posterior fossa, underdeveloped cerebellum
A variation of cerebellar hypoplasia
File:Neck MRI 130850-dichromatic t1-t2-t2.png
Syringomiyelia associated with Chiari malformation


References

  1. ↑ Cotes C, Bonfante E, Lazor J, Jadhav S, Caldas M, Swischuk L, Riascos R (June 2015). "Congenital basis of posterior fossa anomalies". Neuroradiol J. 28 (3): 238–53. doi:10.1177/1971400915576665. PMC 4757284. PMID 26246090.


Template:WH Template:WS

Retrieved from "https://www.wikidoc.org/index.php?title=Arnold-Chiari_malformation_classification&oldid=1578692"