Chiari II malformation

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Chiari II malformation, also known as Arnold-Chiari malformation, is a relatively common congenital malformation of the spine and posterior fossa characterised by myelomeningocoele (lumbosacral spina bifida aperta) and a small posterior fossa with a descent of the brainstem and cerebellar tonsils. Numerous associated abnormalities are also frequently encountered.  

Epidemiology

Chiari II malformations are encountered relatively commonly with an incidence of ~1:1000 live births 7. When a child is born with a myelomeningocoele the vast majority (~95%) have an associated Chiari II malformation. 

Clinical presentation

Given the wide range of anatomical severity as well as thea large number of associated abnormalities which are sometimes encountered, it should be no surprise that the clinical presentation of patients with Chiari II malformations is also varied both in character and severity. The presentation can be divided according to the age of the individual (although most will have life long sequelae) as follows 7:

Pathology

The malformation is characterised by a displacement of the medulla, fourth ventricle and cerebellum through the foramen magnum likely a result of a small posterior fossa. 

Associations

Radiographic features

Antenatal ultrasound

Classical signs described on ultrasound include

There may also be evidence of fetal ventriculomegaly due to obstructive effects as a result of downward cerebellar herniation. Additionally, many of the associated malformations (e.g. corpus callosal dysgenesis) may be identified. 

MRI

MRI is the modality of choice for detecting and characterising the full constellation of findings associated with Chiari II malformations. The key features are discussed below, whereas the wide range of associated abnormalities (see above) are discussed separately. 

Posterior fossa
  • small posterior fossa with a low attachment of the tentorium and low torcula
  • the brainstem appears 'pulled' down with an elongated and low lying fourth ventricle
  • the tectal plate appears beaked: inferior colliculus is elongated and points posteriorly, with resulting angulation of the aqueduct which results in aqueductal stenosis and hydrocephalus
  • cerebellar tonsils and vermis are displaced inferiorly through foramen magnum which appears crowded
Spine

Treatment and prognosis

Treatment of patients with Chiari II malformations is complex due to the variety and variable severity of malformations:

  • myelomeningocoele repair and management of neurogenic bladder
  • ventricular shunting (usually ventriculoperitoneal) 
    • hydrocephalus usually requires shunting and can help ameliorate cranial nerve and brainstem dysfunction
  • craniovertebral decompression
    • may also be required in neonates which brainstem dysfunction if hydrocephalus is not present or symptoms and signs do not improve with shunting
    • older patients with hind brain herniation or syringohydromyelia may also benefit

History and etymology

The Chiari malformations were first described in 1891 by Hans Chiari, Austrian pathologist (1851-1916), see the article on Chiari malformations for further details. 

Differential diagnosis

The differential is predominantly one of definition, and the term Chiari type II is often inappropriately used to designate a variety of malformations. Provided both a myelomeningocoele and brainstem descent are present the diagnosis is usually straight forward 7:

  • -<p><strong>Chiari II malformation</strong>, also known as <strong>Arnold-Chiari malformation</strong>, is a relatively common congenital malformation of the spine and posterior fossa characterised by <a href="/articles/myelomeningocoele">myelomeningocoele</a> (lumbosacral <a href="/articles/spina-bifida">spina bifida</a> aperta) and a small posterior fossa with descent of the <a href="/articles/brainstem">brainstem</a> and cerebellar tonsils. Numerous associated abnormalities are also frequently encountered.  </p><h4>Epidemiology</h4><p>Chiari II malformations are encountered relatively commonly with an incidence of ~1:1000 live births <sup>7</sup>. When a child is born with a myelomeningocoele the vast majority (~95%) have an associated Chiari II malformation. </p><h4>Clinical presentation</h4><p>Given the wide range of anatomical severity as well as the large number of associated abnormalities which are sometimes encountered, it should be no surprise that the clinical presentation of patients with Chiari II malformations is also varied both in character and severity. The presentation can be divided according to the age of the individual (although most will have life long sequelae) as follows <sup>7</sup>:</p><ul>
  • +<p><strong>Chiari II malformation</strong>, also known as <strong>Arnold-Chiari malformation</strong>, is a relatively common congenital malformation of the spine and posterior fossa characterised by <a href="/articles/myelomeningocoele">myelomeningocoele</a> (lumbosacral <a href="/articles/spina-bifida">spina bifida</a> aperta) and a small posterior fossa with a descent of the <a href="/articles/brainstem">brainstem</a> and cerebellar tonsils. Numerous associated abnormalities are also frequently encountered.  </p><h4>Epidemiology</h4><p>Chiari II malformations are encountered relatively commonly with an incidence of ~1:1000 live births <sup>7</sup>. When a child is born with a myelomeningocoele the vast majority (~95%) have an associated Chiari II malformation. </p><h4>Clinical presentation</h4><p>Given the wide range of anatomical severity as well as a large number of associated abnormalities which are sometimes encountered, it should be no surprise that the clinical presentation of patients with Chiari II malformations is also varied both in character and severity. The presentation can be divided according to the age of the individual (although most will have life long sequelae) as follows <sup>7</sup>:</p><ul>
  • -<li><a href="/articles/tectal-beaking">tectal beaking</a></li>
  • +<li><a href="/articles/tectal-beaking-midbrain">tectal beaking</a></li>
  • -<a href="/articles/lemon-sign">lemon sign</a> </li>
  • +<a href="/articles/lemon-sign-cerebellum">lemon sign</a> </li>
  • -<a href="/articles/banana-sign">banana cerebellum sign</a> </li>
  • -</ul><p>There may also be evidence of <a href="/articles/fetal-ventriculomegaly">fetal ventriculomegaly</a> due to obstructive effects as a result of downward cerebellar herniation. Additionally many of the associated malformations (e.g. <a href="/articles/dysgenesis-of-the-corpus-callosum">corpus callosal dysgenesis</a>) may be identified. </p><h5>MRI</h5><p>MRI is the modality of choice for detecting and characterising the full constellation of findings associated with Chiari II malformations. The key features are discussed below, whereas the wide range of associated abnormalities (see above) are discussed separately. </p><h6>Posterior fossa</h6><ul>
  • +<a href="/articles/banana-sign-cerebellum">banana cerebellum sign</a> </li>
  • +</ul><p>There may also be evidence of <a href="/articles/fetal-ventriculomegaly">fetal ventriculomegaly</a> due to obstructive effects as a result of downward cerebellar herniation. Additionally, many of the associated malformations (e.g. <a href="/articles/dysgenesis-of-the-corpus-callosum">corpus callosal dysgenesis</a>) may be identified. </p><h5>MRI</h5><p>MRI is the modality of choice for detecting and characterising the full constellation of findings associated with Chiari II malformations. The key features are discussed below, whereas the wide range of associated abnormalities (see above) are discussed separately. </p><h6>Posterior fossa</h6><ul>
  • -</ul><h4>History and etymology</h4><p>The Chiari malformations were first described in 1891 by <strong>Hans Chiari,</strong> Austrian pathologist (1851-1916), see article on <a href="/articles/chiari-malformations">Chiari malformations</a> for further details. </p><h4>Differential diagnosis</h4><p>The differential is predominantly one of definition, and the term Chiari type II is often inappropriately used to designate a variety of malformations. Provided both a myelomeningocoele and brainstem descent are present the diagnosis is usually straight forward <sup>7</sup>:</p><ul>
  • +</ul><h4>History and etymology</h4><p>The Chiari malformations were first described in 1891 by <strong>Hans Chiari,</strong> Austrian pathologist (1851-1916), see the article on <a href="/articles/chiari-malformations">Chiari malformations</a> for further details. </p><h4>Differential diagnosis</h4><p>The differential is predominantly one of definition, and the term Chiari type II is often inappropriately used to designate a variety of malformations. Provided both a myelomeningocoele and brainstem descent are present the diagnosis is usually straight forward <sup>7</sup>:</p><ul>

Systems changed:

  • Central Nervous System
  • Obstetrics
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