ACM with syrinx

I was diagnosed with acm about 3 years ago. I hac decomp. surgery last year which has not worked. My syrinx runs the full length of my back, but it is not 1, but many small ones. The drs. are considering another decomp. or somehow drainig it, but are unsure if a shunt would work on me because of the multiple lakes. Are there any other alternatives? When i had the decomp. they did not do duraplasty. My symptoms get worse daily, it won~t be long before I have to stop working completely. Thank you.

[CCF-Neuro-M.D.-JT]

Your case sounds quite complicated. Decompression with duraplasty and shunting has been shown to be of benefit in adults with symptomatic Chiari and syrinx. However, your syrinx may require more extensive surgical intervention given the "multiple lakes." One study from Italy showed improvement with a technique in which they made multiple cuts (microincisions) in the dura or tissue covering the spinal cord to allow for normalization of the CSF flow. I 'm not sure this is being done here in the US, but your surgeon may have more info.

As your case is complex and your symptoms are worsening, consider going for a second opinion at a major academic center with a neurosurgeon who specializes in these procedures. Dr. Benzel here at the Clinic is an outstanding spine surgeon who takes care of Chiari patients. Good luck.

[Annika]

Dear Nikibug,

Have you been evaluated for a possible tethered cord?  I have a tethered spinal cord as a result of adhesive scar tissue that formed after removal of a cauda equina tumor.  Both Chiari and syrinx formation are associated with tethered cord.  Although a tethered cord is best known as a congenital problem (often associated with other congenital spinal disorders like myelomeningocele and spina bifida), it can develop in adults when there's some sort of insult that promotes scarring around the spinal cord or cauda equina nerve roots.  

These insults tend to be anything which releases blood into the CSF space, or causes inflammation around the arachnoid layer of the the spine.  Hence, the presence of this scar tissue is often called "arachnoiditis", even when there's no longer any "itis" (inflammation) left, but only the residual scar tissue.  Causes include spinal surgery (both inside and outside the dura), meningitis, foreign subtances injected into the CSF (such as older myelogram dyes), complications from medical procedures that have the potential to tear the dura and allow blood to enter the CSF (epidurals, spinal anesthesia, multiple LP's or bloody LP's), spinal trauma, and many, many more causes.

There are many recent papers on the pathophysiology of syrinx formation that suggest that some sort of tethering effect very often lies behind this problem.  Since tethering also causes Chiaris to develop, these abnormalities often hang together.  I am in touch with many other patients with tethered cords, and the whole issue of Chiari symptoms and syrinx symptoms comes up all the time.  I'm speaking here just as a fellow patient, though, so check this out with your doctor.  But the papers on syrinx formation are fairly new (most of them in the past 1-2 years), and not all doctors seem to have kept up with the new research on what causes a syrinx to form.

Good luck,
Annika