Questions posted in the Neurology and Neurosurgery Forum have been answered by doctors from The Cleveland Clinic Foundation.

Question Title: triple diagnosis

Forum: Neurology Forum
Topic: Hydrocephalus


I am a 26 year old female with S1 spina bifida. I've recently been diagnosed as having tethered cord, hydrocephalus, as well as Chiari malformation. The symptoms that are presenting themselves are: impaired gait, severe back pain, headaches, impaired balance,and decreased bladder and bowel control. I have a couple of questions which I'm tryng to get as much information about as possible.

1. Is it possible that repairing the hydrocephalus, ie. shunting, could reduce most, if not all of these symptoms?
2. Which 'abnormality' should be repaired first?
3. If shunting was performed first, what is the liklihood that the tethered cord would need to be repaired at least somewhere down the road?

Any information that you could provide would be of great assistance. Thank you.



Dear Tonya,

You have a number of developmental anomalies and the symptoms from the hydrocephalus,
the Chiari malformation and the tethered cord may overlap a bit.

Hydrocephalus may be communicating, where CSF cannot flow back into the venous
system, or non-communicating, as in a tumor physically obstructing CSF pathways.
The MRI will be useful in this regard. Symptoms from hydrocephalus are headaches,
nausea, vomiting and lethargy.

Chiari malformations are the physical herniation of the cerebellar tonsils
out the foramen magnum. Symptoms may include headaches, dizziness and cranial
nerve deficits. There are three different types of Chiari malformations, with
the Chiari I malformation being most common. This is likely what you have.

Tethered cord syndrome occurs when the terminal end of the spinal cord, the
filum terminale, is tethered to the sacrum in development. The conus medullaris
is abnormally low and there is often an associated lipoma in the region. You
may have findings on the skin in the sacral region including a hairy patch,
a dermal sinus or just discoloration of the skin. Symptoms include the skin
findings and gait instability, numbness and atrophy of the legs with variable
degrees of bowel and bladder incontinence.

Shunting will not resolve the symptoms of the tethered cord, specifically with
regard to incontinence. It is difficult to say definitively the cause
of your gait instability and impaired balance, as any of the three conditions
discussed may contribute to the situation.

You should talk with your surgeon about what he considers the most pressing
issue for you neurologically. This is the problem that should be corrected
first. If each is only mildly symptomatic then one may choose. Your hydrocephalus
may be relieved by decompression of the Chiari malformation, but this remains
to be seen. Speak with your surgeon in this regard to come up with a good,
comprehensive plan for your care.

Good luck.






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