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__NOTOC__
{{Infobox_Disease
{{Infobox_Disease
| Name           = Arnold-Chiari malformation
| Name = Arnold-Chiari malformation
| Image         = Syringomyelia.JPG
| Image = Syringomyelia.JPG
| Caption       = An uncollapsed [[Syrinx (medicine)|syrinx]] (before surgery).
| Caption = An uncollapsed [[Syrinx (medicine)|syrinx]] (before surgery).
| DiseasesDB     = 899
| DiseasesDB = 899
| ICD10         = {{ICD10|Q|07|0|q|00}}
| ICD10 = {{ICD10|Q|07|0|q|00}}
| ICD9           = {{ICD9|741.0}}
| ICD9 = {{ICD9|741.0}}
| ICDO           =  
| ICDO =  
| OMIM           = 207950
| OMIM = 207950
| MedlinePlus   =  
| MedlinePlus =  
| eMedicineSubj  =
| MeshID = D001139
| eMedicineTopic =
| MeshID         = D001139
}}
}}
{{SI}}
{{Arnold-Chiari malformation}}
{{CMG}}
{{CMG}}; {{AE}}: {{Fs}}


==Overview==
'''''Synonyms and keywords:''''' Chiari malformation; ACM.
==[[Arnold-Chiari malformation overview|Overview]]==


'''Arnold-Chiari malformation''', sometimes referred to as '''Chiari malformation''' or '''ACM''', is a congenital anomaly of the [[brain]].
==[[Arnold-Chiari malformation historical perspective|Historical Perspective]]==


Arnold-Chiari Malformation II occurs in almost all children born with both [[spina bifida]] and [[hydrocephalus]], but ACM I is typically seen in children and adults without spina bifida. The scale of severity is rated I - IV, with IV being the most severe. <ref>Barkovich AJ, Wippold FJ, Sherman JL, Citrin CM. [http://www.ajnr.org/cgi/content/abstract/7/5/795 Significance of cerebellar tonsillar position on MR.] AJNR Am J Neuroradiol. 1986 Sep-Oct;7(5):795-9.</ref> <ref>Haughton VM, Korosec FR, Medow JE, Dolar MT, Iskandar BJ. [http://www.ajnr.org/cgi/content/abstract/24/2/169 Peak systolic and diastolic CSF velocity in the foramen magnum in adult patients with Chiari I malformations and in normal control participants.] AJNR Am J Neuroradiol. 2003 Feb;24(2):169-76.</ref> <ref>Elster A, Chen M. [http://radiology.rsnajnls.org/cgi/content/abstract/183/2/347 Chiari I malformations: clinical and radiologic reappraisal.] Radiology. 1992; 183:347-353.</ref>
==[[Arnold-Chiari malformation classification|Classification]]==


==Cause==
==[[Arnold-Chiari malformation pathophysiology|Pathophysiology]]==
The [[cerebellar tonsils]] are elongated and pushed down through the opening of the base of the [[skull]] (see [[foramen magnum]]), blocking the flow of [[cerebrospinal fluid]] (CSF).


The [[brainstem]], cranial nerves, and the lower portion of the [[cerebellum]] may be stretched or compressed.
==[[Arnold-Chiari malformation causes|Causes]]==


Therefore, any of the functions controlled by these areas may be affected. The blockage of CSF flow may also cause a [[syrinx (medicine)|syrinx]] to form, eventually leading to [[syringomyelia]].  Many sufferers turn to the Chiari Institute in Long Island, NY for specialized medical attention and medication.
==[[Arnold-Chiari malformation differential diagnosis|Differentiating Arnold-Chiari malformation from other Diseases]]==


==Presentation==
==[[Arnold-Chiari malformation epidemiology and demographics|Epidemiology and Demographics]]==
In [[infant]]s, the most common symptoms are [[stridor]] and swallowing difficulties. In older children, upper (and lower as age marches on) limb weakness and breathing difficulties may occur. Patients may experience no symptoms or remain asymptomatic until early adulthood, at which point they will often experience quick onset severe headaches and neck pain. Fatigue, dizziness, vertigo, neuropathic pain, pain at the point of tethering, visual disturbances, difficulty swallowing, ringing in the ears, sleep apnea, impaired fine motor skills, muscle weakness, and palpitations and excessive clearing of the throat with no obstructions are other common symptoms. Because of the complex combination of symptoms and the lack of experience with ACM1 had by many, even outstanding neurologists and neurosurgeons, many patients are frequently misdiagnosed.


Some patients may go an entire lifetime without having noticeable symptoms. Or, symptoms can be minimal, then turn severe suddenly due to head trauma which alters the condition of the spine, brain, or cerebellar tonsils and begins to cause more difficulties...
==[[Arnold-Chiari malformation risk factors|Risk Factors]]==


==Diagnostic Studies==
==[[Arnold-Chiari malformation natural history, complications and prognosis|Natural History, Complications and Prognosis]]==


*'''Radiographs''' may demonstrate associated abnormalities of the skull base.
==Diagnosis==
*'''CT''' may demonstrate hydrocephalus, herniated cerebellar tonsils, and a flattened spinal cord. Rarely will CT show a syrinx.
*'''MRI''' is the imaging modality of choice to evaluate for a Chiari I malformation. MRI findings:
**Cerebellar tonsillar herniation
**Wedge shaped tonsils
**Syringohydromyelia
**Small posterior fossa
**Obstructive [[hydrocephalus]]
**Brainstem anomalies


*Tonsillar displacement is measured from the basion-opisthion line on a sigittal image.
[[Arnold-Chiari malformation diagnostic study of choice|Diagnostic Study of Choice]] |[[Arnold-Chiari malformation history and symptoms|History and Symptoms]] | [[Arnold-Chiari malformation physical examination|Physical Examination]] | [[Arnold-Chiari malformation laboratory findings|Laboratory Findings]] | [[Arnold-Chiari malformation x ray|X Ray]] | [[Arnold-Chiari malformation CT|CT]] | [[Arnold-Chiari malformation MRI|MRI]] | [[Arnold-Chiari malformation echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Arnold-Chiari malformation other imaging findings|Other Imaging Findings]] | [[Arnold-Chiari malformation other diagnostic studies|Other Diagnostic Studies]]
*Herniation is usually at least 5mm, though patients with 3-5mm herniation may also have the malformation.
*MRI CSF flow studies may prove to be helpful and are currently under investigation.
 
===Patient #1===
 
Images shown below are courtesy of RadsWiki and copylefted
 
<div align="left">
<gallery heights="125" widths="125">
Image:Chiari 1 malformation 001.jpg|Sag T2
Image:Chiari 1 malformation 002.jpg|Sag T1
Image:Chiari 1 malformation 003.jpg|Sag T1
</gallery>
</div>
 
===Patient #2===
 
Images shown below are courtesy of RadsWiki and copylefted
 
<div align="left">
<gallery heights="125" widths="125">
Image:Chiari 1 malformation 101.jpg|Sag T1
Image:Chiari 1 malformation 102.jpg|Sag T2
Image:Chiari 1 malformation 103.jpg|Ax T2
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Chiari 1 malformation 104.jpg|Ax T2
Image:Chiari 1 malformation 105.jpg|Ax T2
</gallery>
</div>
 
===Other Radiologic Findings===
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation MRI 0001.jpg|Brain: Arnold Chiari I; T1 (MRI)
Image:Arnold-Chiari Malformation CT 0001.jpg|Brain CT: Arnold Chiari I with Intrathecal Contrast; WC
Image:Arnold-Chiari Malformation MRI 0003.jpg|Brain: Arnold Chiari I, with Hydromyelia of Cervical Spinal Cord; T1 (MRI)
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation Plain Film 0001.jpg|Skull: Arnold Chiari II, Skull with Luckenschadel (Plain Film)
Image:Arnold-Chiari Malformation CT 0002.jpg|Brain: Arnold Chiari II, Hydrocephalus (CT)
Image:Arnold-Chiari Malformation CT 0003.jpg|Brain: Arnold Chiari II, 6 months Post-Shunt (CT)
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation MRI 0007.jpg|Brain: Arnold Chiari II, with Towering of The Cerebellum and Wide Tentorial Incisure;  T1 (MRI)
Image:Arnold-Chiari Malformation MRI 0008.jpg|Brain: Arnold Chiari II, with Fenestrated Falx Cerebri and Interdigitating Gyri;  T2 (MRI)
Image:Arnold-Chiari Malformation MRI 0009.jpg|Brain: Arnold Chiari II, with Tectal Beaking and Large Massa Intermedia and Stenogyria;  T1 (MRI)
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation MRI 0010.jpg|Brain: Arnold Chiari Type II with Medullary Spur and Kink (MRI)
Image:Arnold-Chiari Malformation MRI 0011.jpg|Brain: Arnold Chiari II, with Hypoplastic Corpus Callosum;  T1 (MRI)
Image:Arnold-Chiari Malformation MRI 0012.jpg|Brain: Arnold Chiari II, with Cerebellar Hemispheres that Creep Anteriorly Around the Brain Stem;  T1 (MRI)
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation MRI 0013.jpg|BRAIN: ARNOLD CHIARI II (MRI)
Image:Arnold-Chiari Malformation MRI 0014.jpg|BRAIN: ARNOLD CHIARI II WITH ELONGATED FOURTH VENTRICLES AND STENOGYRIA;  T1 (MRI)
Image:Arnold-Chiari Malformation MRI 0015.jpg|BRAIN: ARNOLD CHIARI II WITH HYPOPLASTIC CORPUS CALLOSUM AND STENOGYRIA;  T1 (MRI)
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation MRI 0016.jpg|BRAIN: ARNOLD CHIARI II WITH REPAIRED MYELOMENINGOCELE AND TETHERED SPINAL CORD;  T1 (MRI)
Image:Arnold-Chiari Malformation MRI 0017.jpg|BRAIN: ARNOLD CHIARI TYPE IV;  T1 (MRI)
Image:Arnold-Chiari Malformation MRI 0018.jpg|BRAIN: ARNOLD CHIARI TYPE IV;  T1 (MRI)
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation MRI 0019.jpg|BRAIN: ARNOLD CHIARI MALFORMATION II; 1/2 PCMC (MRI)
Image:Arnold-Chiari Malformation MRI 0020.jpg|BRAIN: ARNOLD CHIARI MALFORMATION II, HERNIATED CEREBELLAR TONSIL; 1/2 PCMC - ARROW (MRI)
Image:Arnold-Chiari Malformation MRI 0021.jpg|SPINAL CORD: ARNOLD CHIARI MALFORMATION II; 2/2 PCMC (MRI)
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation MRI 0022.jpg|SPINAL CORD: ARNOLD CHIARI MALFORMATION II; 2/2 PCMC - ARROW (MRI)
Image:Arnold-Chiari Malformation MRI 0023.jpg|BRAIN: ARNOLD CHIARI MALFORMATION; 1/3 T2 (MRI)
Image:Arnold-Chiari Malformation MRI 0024.jpg|BRAIN: ARNOLD CHIARI MALFORMATION; 1/3 - ARROW T2 (MRI)
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation MRI 0025.jpg|BRAIN: ARNOLD CHIARI MALFORMATION; 2/3 T2 (MRI)
Image:Arnold-Chiari Malformation CT 0026.jpg|SPINAL CORD: ARNOLD CHIARI MALFORMATION; 3/3 (CT)
Image:Arnold-Chiari Malformation CT 0027.jpg|BRAIN: ARNOLD CHIARI MALFORMATION (CT)
</gallery>
</div>
 
===Pathological Findings===
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0001.jpg|Brain: Arnold-Chiari Malformation: Gross fixed tissue sagittal section brain stem
Image:Arnold-Chiari Malformation 0002.jpg|Brain: Arnold Chiari Malformation: Gross fixed tissue sagittal section cerebrum brainstem and cerebellum
Image:Arnold-Chiari Malformation 0003.jpg|Brain: Arnold Chiari Malformation And Polygyria: Gross fix tissue external view
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0004.jpg|Brain: Arnold Chiari Malformation: Gross fixed tissue sagittal section brainstem close-up
Image:Arnold-Chiari Malformation 0005.jpg|Brain: Arnold Chiari Malformation: Gross fixed tissue brain stem sagittal section close-up
Image:Arnold-Chiari Malformation 0006.jpg|Brain: Arnold Chiari Malformation: Gross fixed tissue sagittal section brain stem and cerebellum
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0007.jpg|Brain: Arnold Chiari Malformation: Gross fixed tissue sagittal section brain stem cerebellum and spinal column
Image:Arnold-Chiari Malformation 0008.jpg|Brain: Hydrocephalus Secondary To Arnold Chiari Malformation: Gross fixed tissue three coronal sections cerebral hemispheres
Image:Arnold-Chiari Malformation 0009.jpg|Brain: Arnold Chiari Malformation: Gross fixed tissue cerebellum and brainstem
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0010.jpg|Spinal cord: Malformation Vertebral Bodies: Gross natural color sagittal section spinal column with malformation in region C7 T1 associated with Arnold Chiari malformation
Image:Arnold-Chiari Malformation 0011.jpg|Brain: Arnold Chiari Malformation; with Hydrocephalus, Type I
Image:Arnold-Chiari Malformation 0012.jpg|Brain: Arnold Chiari Malformation; Type II
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0013.jpg|Brain: Arnold Chiari Type II with Meningomyelocele
Image:Arnold-Chiari Malformation 0014.jpg|Brain: Arnold Chiari Malformation; Mid Sagittal
Image:Arnold-Chiari Malformation 0015.jpg|Brain: Arnold Chiari Malformation with Myelocele
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0016.jpg|Brain: Arnold Chiari Malformation with Hydrocephalus
Image:Arnold-Chiari Malformation 0017.jpg|Brain: Arnold Chiari Malformation with Hydrocephalus
Image:Arnold-Chiari Malformation 0018.jpg|Brain: Arnold Chiari Malformation
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0019.jpg|Brain: Arnold Chiari Malformation
Image:Arnold-Chiari Malformation 0020.jpg|Brain: Arnold Chiari Malformation
Image:Arnold-Chiari Malformation 0021.jpg|Brain: Arnold Chiari Malformation, a close up view
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0022.jpg|Brain: Arnold Chiari Malformation; Tonsilar Herniation of Cerebellum Compressing Medulla
Image:Arnold-Chiari Malformation 0023.jpg|Brain: Arnold Chiari Malformation; Meningomyelocele, Type II, Meningitis
Image:Arnold-Chiari Malformation 0024.jpg|Brain: Arnold Chiari Malformation, Type II; Meningomyelocele, Meningitis, Close-up of Previous one
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0025.jpg|Brain: Arnold Chiari Malformation, Intramedullary Hemorrhage
Image:Arnold-Chiari Malformation 0026.jpg|Brain: Arnold Chiari Malformation, Type II
Image:Arnold-Chiari Malformation 0027.jpg|Spinal Cord: Arnold Chiari Malformation; Type II, Meningomyelitis
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0028.jpg|Spinal Cord: Arnold Chiari Malformation; Type II, Meningomyelitis
Image:Arnold-Chiari Malformation 0029.jpg|Brain: Arnold Chiari Malformation
Image:Arnold-Chiari Malformation 0030.jpg|Brain: Arnold Chiari Malformation; Note Z-Shaped Kink in Cervical Spinal Cord
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0031.jpg|Brain: Arnold Chiari Malformation with Hydrocephalus
Image:Arnold-Chiari Malformation 0032.jpg|Skin: Myelomeningocele with Hydrocephalus; Arnold Chiari; Note Underdevelopment of Legs
</gallery>
</div>
 
 
<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0033.jpg|Brain: Cerebellum Arnold Chiari: Gross fixed tissue flattened cerebellum
Image:Arnold-Chiari Malformation 0034.jpg|Brain: Polymicrogyria: Gross fixed brain lateral view of left cerebral hemisphere case of Arnold Chiari cerebellum
</gallery>
</div>


==Treatment==
==Treatment==


Once these "onset of symptoms" occurs, the most frequent treatment is decompression surgery, in which a neurosurgeon seeks to open the base of the skull and through various methods unrestrict CSF flow to the spine.
[[Arnold-Chiari malformation medical therapy|Medical Therapy]] | [[Arnold-Chiari malformation surgery|Surgery]] | [[Arnold-Chiari malformation primary prevention|Primary Prevention]] | [[Arnold-Chiari malformation cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Arnold-Chiari malformation future or investigational therapies|Future or Investigational Therapies]]


This treatment is under observation and review.  Decompression is a very taxing surgical procedure and is now, in some circles, disdained in lieu of tethered cord detachment at the base of the spine.  Some neurological surgeons find that detethering the spinal cord relieves the compression of the brain against the skull opening (foramen magnum) obviating the need for decompression surgery and associated trauma.  It should be noted that the alternative spinal surgery is not without risk.
==Case Studies==


==History==
[[Arnold-Chiari malformation case study one|Case #1]] | [[Arnold-Chiari malformation case study two|Case #2]]
An Austrian [[pathologist]], [[Hans Chiari]], first described these hindbrain malformations in the 1890s.  A colleague of Professor Chiari, Dr. Julius Arnold later contributed to the definition of the condition, and students of Dr. Arnold suggested the term "Arnold-Chiari malformation" to henceforth refer to the condition.<ref>{{WhoNamedIt|synd|1154|Arnold-Chiari malformation}}</ref>
 
==References==
{{reflist|2}}


{{Congenital malformations and deformations of nervous system}}
{{Congenital malformations and deformations of nervous system}}
{{SIB}}


[[Category:Overview complete]]
[[Category:Overview complete]]
[[Category:Neurology]]
[[Category:Neurology]]
[[Category:Needs patient information]]
[[Category:Disease]]
 
[[ca:Malformació d'Arnold-Chiari]]
[[ca:Malformació d'Arnold-Chiari]]
[[de:Chiari-Malformation]]
[[de:Chiari-Malformation]]

Latest revision as of 21:31, 28 September 2019

Arnold-Chiari malformation
An uncollapsed syrinx (before surgery).
ICD-10 Q07.0
ICD-9 741.0
OMIM 207950
DiseasesDB 899
MeSH D001139

Arnold-Chiari malformation Microchapters

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

Synonyms and keywords: Chiari malformation; ACM.

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Arnold-Chiari malformation from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice |History and Symptoms | Physical Examination | Laboratory Findings | X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

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