What do you know about Chiari Malformation Type II?


Question:
I would like any information anyone has about this condition, and if you have any advice, I would appreciate that too!

Answers:
The Chiari II malformation is a complex anomaly with skull, dural, brain, spinal, and spinal cord manifestations. This disorder is almost invariably associated with myelomeningocele. The hindbrain findings of Chiari II malformation are best explained with the theory of McLone and Knepper, which allows the hindbrain disorder to be conceptualized as resulting from a normal-sized cerebellum developing in an abnormally small posterior fossa with a low tentorial attachment.

Chiari II malformations are treated if the patient is symptomatic, and physicians have determined that there are no complications from hydrocephalus. In some patients, consideration of a tethered cord is also explored. In many infants who become symptomatic from a Chiari II malformation, the symptom onset and progression are severe and rapid, and this requires an urgent or emergency approach.

Surgical treatment of these malformations depends on the type of malformation. The goal of surgery is to relieve the symptoms, or stop the progression of the syrinx or symptoms.

Chiari II decompression is treated similarly, but is usually restricted to decompressing the tissues in the spinal canal and leaving the back of the skull alone.

The goal of Chiari surgery is:

-Optimal decompression of nerve tissue
-Reconstruction of normal CSF flow around and behind the cerebellum


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