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Chiari Malformation Type II - Case 2

A 9 month-old boy presented with an enlarging head and a delay in motor milestones.

SHOW THE FINDINGS IN THE CHIARI 2 MALFORMATION

Show the Midbrain and the "Beaked" Tectum   Note the Low Lying Cerebellum (axial scan)   Note the Cerebellum Below the Foramen Magnum

Show the "Kinked" Medulla

Chiari Malformation Type II: (Left and Middle) T2-weighted axial MRIs; (Right) T1-weighted sagittal MRI. The axial scans show several abnormalities, including descent of the cerebellar tonsils, an abnormal midbrain with a malformed "beaked" tectum (fusion of the colliculi), and enlarged ventricles (hydrocephalus). The sagittal scan shows several abnormalities, including descent of the cerebellar tonsils, a "kinked" medulla, and a very tight posterior fossa.

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 11/22/06
Copyrighted 2006. David C Preston