What is Chiari Type II Malformation?

Chiari Type II Malformation
Syrinx morphology in Chiari II malformations. A Type 1 syrinx: the ventral portion of the cavity is ependymal lined (asterix), while the dorsal aspect is irregularly lined by a layer of hyperplastic glia (arrow). Despite the lack of dorsal ependymal lining, the impression that this is simply a wedge shaped extension of the more caudal dysraphism is difficult to escape. B Type 2 syrinx, extending in this section into the cervical cord, with a prominent paramedian and dorsal distribution. The arrow indicates the normal dorsal and median glial raphe and arrowheads the slit-like paramedian syrinx. This cavity was continuous with the dilated central canal (asterix) caudally. C Dorsal aspect of the same section as B at higher power, demonstrating glial processes lining a slit like cavity containing macrophages, with axonal tracts in the walls of the cavity. D and E: Same specimen, type 3 syrinx, demonstrating irregular extension of the syrinx both dorsolaterally (D) and ventrolaterally (E). The cavity marked by an asterix is partially lined by ependyma and is continuous with the central canal. All sections stained immunohistochemically for vimentin.Fetal syringomyelia. Guo A, Chitayat D, Blaser S, Keating S, Shannon P - Acta neuropathologica communications (2014). Not Altered. CC.

Chiari type II malformation is a relatively common congenital malformation of the spine and posterior fossa characterized by myelomeningocele and a small posterior fossa with the descent of the brainstem, vermis, and cerebellar tonsils.

What is the Pathology of Chiari Type II Malformation? 

Etiology: The cause of Chiari type II malformation is unknown.  

Pathogenesis: The sequence of events that lead to Chiari type II malformation is due to in utero malformation of the cranial structures and spine. 

Histology: The histology associated with Chiari type II malformation shows dysplastic large fibrous tissue along with choroid plexus.

How does Chiari Type II Malformation Present?

Patients with Chiari type II malformation are more often females than males, and the condition is congenital. The symptoms, features, and clinical findings associated with Chiari type II malformation include hydrocephalus, loss of strength, abnormal breathing, and depressed gag reflex. 

How is Chiari Type II Malformation Diagnosed?

Chiari type II malformation is diagnosed via MRI and CT scan. 

How is Chiari Type II Malformation Treated?

Chiari type II malformation is treated surgically. 

What is the Prognosis of Chiari Type II Malformation?

The prognosis of Chiari type II malformation is poor.