Syringomyelia Surgery and Shunt Rejection

[ktriddle]

I am very concerned about my son's condition.  A little background.  He is 24 years old and has been a Type 1 diabetic since he was 14 months old.  About 3 years ago he began experiencing pain an weakness in his lower extremities.  This was attributed to peripheral neuropathy and he was given Lyrica to help with the pain.  As the pain continued to worsen, and it became more and more difficult for him to walk, we continued to seek medical advice, only to be told that this was due his diabetes.  Different doctors ordered MRI's of his brain and his lower back, which showed nothing.   Finally, a neurologist performed a nerve conduction test and determined that the problem was in his spine and ordered and MRI of his cervical and thoracic region.  A small syrinx was found in his spine and we were referred to a surgeon.  After ordering more MRI's, the surgeon advised that surgery to drain the syrinx was necessary.  Without surgery, he said, my son's condition would continue to worsen to the point where he would loose all use of his legs and lower extremities and all control of his bowels and bladder.  Needless to say, we opted for the surgery, which was performed in June 2008.  A shunt was inserted to drain the syrinx into his chest cavity.

After surgery, my son was prescribed physical therapy to regain the use of his legs.  For the first month, he made great progress and was even able to graduate from walking with a walker to walking with crutches.  The outlook was bright.  Then one day he experienced tone and spasms in his legs and began regressing.  After calling the surgeon, another MRI was done and showed that the syrinx was draining as scheduled.  We were told that his regression may be due to a urinary tract or bladder infection.  Tests for that proved negative, and the regression continued.  Since last August, my son has gone from progressing to walking with crutches to total loss of his lower extremities and loss of all bladder and bowel functions.  Everything we were told would happen without the surgery has happened with the surgery.

Over the past 9 months, the regression has been slow but continually downward.  Until a month ago he was able to stand with the aid of a walker, though not take any steps, and could tell when he had to have a bowel movement.  Now, he cannot even do any of that.

In October, 4 months after his surgery and when he could still take a few steps with his walker, a lumbar puncture was done.  An entire battery of tests were run, all of which were negative.  The only thing the neurologist said showed up was an abundance of protein in his spinal fluid.  After consulting with 3 other neurologists, they came to the conclusion that there was a possibility his body was rejecting the shunt and this was causing the problems.  We were referred back to the surgeon who of course said there was no way that was happening.  We were then referred to another neurologist at Duke who ordered yet another MRI.  When that MRI came back negative, he politely told us that there was nothing he could do.

So here we are, 9 months after my son started getting worse, and his downward slide continues day after day, yet no one can diagnose what is wrong with him.  The first neurologist still thinks it's a matter of the shunt being rejected.  

What other tests are available to diagnose spinal diseases other than MRI's and lumbar punctures?  All anyone seems to want to do is an MRI, and he's 9 of those in the past 10 months, on his brain and spine, with and without contrast, yet nothing shows up other than the fact that the original syrinx is completely drained.  Is the possibility that his body has rejected the shunt a viable solution?  Is this common?  According to the surgeon, it's so rare that it's not a possibility in his mind.  We are getting ready to admit my son into a rehab facility to help him cope with his condition, but that doesn't give us a diagnosis of what's wrong.  If this has continued to progressively get worse for the past 9 months, I can only shudder to think what the future will hold if his problems are not diagnosed.

Any suggestions anyone has would be greatly appreciated.

Thank you

[ktriddle]

His last brain MRI was in February, as was his last thoracic spine MRI.  The last MRI he had done on his lumbar spine was last May before the thoracic MRI that found the syrinx.

The doctor that did his surgery is a specialist and has done many syringomyelia surgeries.  That is why the local neurologist referred us to him...because of his expertise.  Since his surgery, he has had numerous MRI's done, but all have been on his thoracic spine except for the 1 on his brain.  The syrinx has shown as drained since last September on all MRI's done.  

Would the tethered cord show up on an MRI?  If not, what tests are necessary to diagnose it?  After you mentioned it, I looked it up online and the symptoms seem to coincide with the deterioration of my son's condition, especially the fact that it may take a few months to a year to begin showing up.  As I mentioned in the first post, 2 months after the surgery he was progressing nicely, but all of a sudden something happened and it's been down hill ever since.  

[selmaS]

May I ask, did he have MRI's of his lumbar spine? thoracic spine?

And are his drs specialists in syringomyelia/chiari? It is very important to have a dr that this is his primary cases.

I have heard of issues with the shunts...getting clogged...just stopping....ect.

I went to several NS and NL and none mentioned my tethered cord either...I found it on the report.....

A syrinx from a trauma is a way to aquire this condition.....did they say whether or not the syrinx shrunk?....is it still filling up?...I can see the issues being the shunt is no longer working, but if the syrinx is empty, then y all the symptoms??

If the shunt drains too fast it can cause a chiari to form.How long since the last Brain MRI?

[ktriddle]

Thank you  for the comment and the information.  When he was first diagnosed, the surgeon said it was not chiari, and was probably the result of an injury he suffered to his back when he was in high school.  He did MRI's to see if there were anything else causing the syrinx and could find nothing.  Spinal cord tethering was never mentioned by any of the neurologists or surgeon we have seen.

So far, the only thing anyone has come up with is the possible rejection of the shunt.  He was prescribed Methotrexate by his neurologist, but came down with a really bad cold that turned into bronchitis about a month ago.  It was when that happened that he lost all bladder and bowel control.  He was given antibiotics and was told not to take the Methotrexate if there was any hint of any kind of virus or infection in his body, so he never took them to see if they helped or not.

So, we just go on from day to day hoping someone can come up with something, but so far it's been a dead end at every turn.  The only certainty is that his condition continues to worsen with no relief in sight.

[selmaS]

Hi...I am sorry ur son's condition is regressing again.Syringomyelia/syrinx is a related condition to chiari malformation.Many that have a syrinx also have chiari, but not all.There are other conditions like tethered cord which could cause the bladder and lower extremities to loose control.

I was recently dx with TC and I also have chiari....anad EDS.I wonder if ur son's dr looked for the cause of the syrinx.

U may want to see a Chairi specialist as they specialize in both conditions.

We have a chiari forum here on MedHelp, I am the CL there and if I can be of ne assistance please let me know.

"selma"