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Chiari Malformation Type II - Case 1

A 1 year-old boy had a history of a meningomyelocele repaired shortly after birth, and obstructive hydrocephalus requiring shunting.

SHOW THE FINDINGS IN THE CHIARI 2 MALFORMATION

    Tonsillar Herniation       "Beaked" Tectum       Hydrocephalus           

Chiari Malformation Type II: T1-weighted axial MRIs. These axial scans show several abnormalities, including descent of the cerebellar tonsils (tonsillar herniation), an abnormal midbrain with a malformed "beaked" tectum (fusion of the colliculi), and enlarged ventricles (hydrocephalus).

This malformation is know as a Chiari malformation, type II. It is quite complex and associated with more serious consequences than the type I malformation. The type II malformation affects the entire hindbrain, dura and bone, as well as the spinal cord, the latter presenting as spina bifida and a meningomyelocele. Among the abnormalities included in this malformation are aqueductal stenosis, hydrocephalus, tonsillar herniation, a "kinked" medulla, an elongated 4th ventricle, fused colliculi resulting in a "beaked" tectum of the midbrain, partial agenesis of the corpus callosum, syringomyelia, and a dysplastic skull.


Revised 11/30/06
Copyrighted 2006. David C Preston