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Thread: Tethered cord/compressed nerves

  1. #1

    Tethered cord/compressed nerves

    Hi. I'm new to this community - not sure if this is the right forum to post...

    My 8 year old daughter has had 2 tethered cord releases. One in Sep 1997 (diagnosed June 1997) which involved snipping the fatty filum terminale. It didn't really resolve any problems.

    At the time, she had many progressive neurological symptoms (various oculomotor, vision, hearing and respiratory-related symptoms, headaches, neck/shoulder pains, tremors, head sweats, fatigue) in addition to constant tip-toeing, leg and back pains and bladder and bowel control problems. The fact of having so many symptoms complicated matters.

    In May 1998, at almost 5 years of age, she was diagnosed with Chiari I malformation (no tonsillar herniation below level of foramen magnum) without a syrinx. Over a dozen specialists said there was no Chiari after reviewing the MRI scans specifically for it. We obtained a concurring diagnosis in Sep 1998. She underwent Chiari posterior fossa decompression surgery Jan 1999 and all her symptoms were allevaited (other than the leg, back, bladder and bowel problems). She's about 95% better today from all the Chiari symptoms. Just before Chiari surgery, her apnea, dysphagia and gagging/choking symptoms were reaching a dangerous level, as mentioned by 3 neurosurgeons. She was also having more and more bouts of blindness. These were the more critical problems.

    In June 2001, she underwent a 2nd tethered cord surgery and not only had the spinal cord retethered to the column, causing substantial spinal cord tension (in doctor's words), but some nerves had also become encased and compressed in a mass of dural tissue, which had come undone from the first tethered cord surgery. The cord was released and the dural mass was cut away as much as possible to free up the nerves but only so much could be done so as to not risk cutting nerves. Therefore, there are still some nerves caught in the mass and this being compressed. Her continuing problems are being blamed on this, which could very well be, but one can argue it's speculative to a certain degree.

    Unfortunately, her leg/back/bladder/bowel problems persist and her leg pains (always behind both knees) have become worse in the last 3 months or so as have her back pains - the leg pains are the worst and she can often not walk or stand - this happens a couple of times a day or so. She can only evacuate bowel movements while kneeling down - won't work with her back flexed (e.g., sitting down). She is bladder incontinent and thus still in diapers.

    Questions:

    1. Is there anything which can be done, anywhere, for this nerve compression? Are there trials or studies being done on this type of thing?

    2. What pains meds are recommended for an 8 year old with severe leg pain? She's tried Neurontin and Tegretol with little success, if any. She's tried acupuncture (not the traditional needle type - some electo-stimulus type) but it caused even more severe pain - she couldn't stand or walk for the rest of the day.

    3. She's had recent spine MRIs to review for possible retethering but the cord is not apparently attached dorsally. Quite frankly, I don't trust MRI or any other imaging as definitive. These tools are only indicative. Any recommendations on other imaging which may help further indicate or contradict retethering? For example, can CINE MRI help monitor CSF flow within the spinal canal to confirm whether or not there is blockage and perhaps suggest retethering in the lumbar area or perhaps even a hidden problem elsewhere?

    Thanks.

  2. #2
    I will defer to Dr. Young on these complex questions. As I am sure you know, many children with Chiari malformations also have spina bifida occulta. I assume that this has been ruled out as a factor in her impairments?

    Cord tethtering after traumatic SCI is probably a different issue that what you are confronting, but I would still want to be sure that your daughter is getting appropriate rehabilitation care. Esp. in relationship to her bowel and bladder functioning, I would get her to a good pediatric rehabilitation specialist or clinic. Diapers are rarely required for bladder management if it is done right, and we usually try to not have children in diapers by school age.

    Often pain in children is under-treated. This is cruel. Have you had her referred to a pain clinic? There are other medications and interventions that can be tried. A program specializing in neuropathic pain would be best.

    I will ask Dr. Young to review the rest of your question and see if there are additional resources for you.

    (KLD)

  3. #3

    Re: Tethered cord/compressed nerves

    Thanks for the reply, KLD.

    Unfortunately, medical services here in Canada stink. Not only does it take forever to get anything done, but services are always trying to be minimized to reduce costs. In addition, the latest and greatest in medical equipment and treatments are not readily available. We've been at this for 8 years now and have resorted to medical consultations and treatments in the U.S., albeit at a high cost for us. Regardless, it's saved our daughter's life on one occasion and possibly a second.

    We don't have "Pain clinics" as far as I know. It's just go to the pediatrician or a specialist and hope they are decent enough to prescribe some pain med, which they are often leery of doing, as you remarked.

    What are the alternatives to diaper use?
    She's had several spine MRIs and Xrays, so I would guess Spina Bifida Occulta would have been detected by now, would it not?

  4. #4

    Resources

    Even in Canada there are excellent SCI rehabilitation centers. I know of ones in Toronto, Vancouver and Calgary for example. Many Canadians post here and on www.newmobility.com and may be able to give you some leads.

    I would be most surprised if there are no pain clinics/rehab programs in Canada as this is a growing and important medical specialty. I would suggest checking the major teaching hospitals such as McGill for referrals.

    A experiences SCI/Rehabilitation nurse should be able to help you explore alternatives to diapers. For bladder management, intermittent cath is done most often. Children in the USA who do this are usually expected to be independent in the prior to starting school. A good bowel program should prevent bowel accidents and the need to use diapers for this.

    (KLD)

  5. #5
    Rico,

    Let me first compliment you on one of the clearest description that I have read on this forum of a very complicated problem.

    For the sake of others who may be reading this post, let me first describe briefly (and inadequately) what a Chiari malformation is. The junction between the brain and the spinal cord, where the spinal cord exits from the cranial cavity, is vulnerable to a number of problems. If the opening (the foramen magnum) is narrow or brain herniates into the opening, the spinal cord may be compressed. Such compression may damage the spinal cord, occlude flow of cerebrospinal fluid between the brain and the spinal cord, and lead to the formation of syringomyelia (an enlargement of the central canal in the spinal cord). Surgery aims to decompress the brainstem and spinal cord, to improve the flow of CSF, and to prevent further compression. Unfortunately, surgery itself may cause scarring and tethering of the spinal cord. The symptoms of Chiari syndrome include sympathetic dysfunction, headaches, progressive neurological loss, and pain. These symptoms may appear in childhood or adulthood. Some people may not discover that they have a Chiari problem until they are adults.

    Rico, I really admire the tenacity with which your family has approached this problem. In the absence of definitive radiological signs of a Chiari malformation, most surgeons were probably unwilling to operate because the surgery itself may introduce scarring and tethering of the cord. Indeed, the dural scarring that you describe is very likely to have been related to her original surgery. Given her symptoms, however, it sounds as if the decompression was well-justified and relieved much of the symptoms. As I understand it, the main problems now are the incontinence, pain, and walking. Let me address these backward in sequence.

    Walking. Does your daughter have spasticity (you indicated that she was tip-toeing before the Chiari decompression surgery)? She may have tight gastrocnemius muscles and they may be undergoing spasms. If so, this may account for the pattern of pain that she is having, localizing to the kneepits. When I was in medical school in the 1970's, I worked with a wonderful orthopedic surgeon by the name of Eugene Bleck (he is now retired) who saw many children with similar problems. If she does have spasticity (increased reflexes and resistance to movement), electrical stimulation, botox injections, or tendon releases might provide some relief. The first is the least invasive and probably worthwhile investigating. For example, if the anterior tibialis is electrically stimulated during walking or when she has pain, this should relax the gastrocnemius. Inhibition of the antagonistic muscle is the tightest and most powerful reflexive tendencies of the spinal cord.

    Pain. From your description of the location and intermittent nature of the pain, I think that her pain may be related to spasms or spasticity, as opposed to neuropathic pain. Neuropathic pain usually manifests in the form of burning, freezing, or aching pains that radiate from the pelvis downward. There are some surgical procedures (such as dorsal root rhizotomies) that I would not recommend until it is clear that the less invasive solutions are not working.

    Incontinence. I presume that your daughter has been investigated urodynamically for the presence of bladder spasticity. If not, I would recommend that this be done. If she has bladder spasticity, this can be addressed with ditropan or other bladder antispasticity drugs. Most hospitals in Canada and the U.S. have very good pediatric urology units that should be able to help you with this issue. You may also want to consider the possibility of biofeedback training for bladder control. One of the problems with bladder control is that it usually lies outside the realms of our consciousness and biofeedback training may help her awareness of the bladder. The development of thin superabsorbent materials have made "diapers" so much more efficient these days that limited incontinence should not interfere with her daily life.

    Given the number of surgical procedures that your daughter has had, your description that 95% of her Chiari symptoms were resolved by the decompressive surgery, and the progress that she has had in the last year, if she were my daughter, I would probably avoid further surgical manipulation of the spinal cord unless there is strong evidence of progressive scarring and cord compression. I suspect that all the hospitalization and visits to doctors have interfered with her education and socialization with her peers. If so, she needs to get back on course with her schoolwork and becoming attuned to her peers.

    You do need to be aware that your daughter is at a higher than usual risk of developing scoliosis which usually appears at about the time of puberty. Therefore, I would suggest that you develop a relationship with a good pediatric orthopedic surgeon whom she trusts to help on the spasticity and scoliosis issues. Finally, if your daughter has any siblings, go to great lengths to make sure that they feel that they are loved and appreciated.

    Wise.

  6. #6

    Re: Tethered cord/compressed nerves

    Thank you very much for your reply, Wise.

    Perhaps I should clarify some things. My daughter's Chiari surgery has so far (3 years) been very successful, alleviating many "brain" symptoms. It did not cause her tethered cord because the tethered cord was diagnosed and treated (first of 2 surgeries) before the Chiari was even diagnosed. Here's a summary of events:

    - our daughter was born July 1993
    - accidentally found liver covered with hemangiomas Sep 93 while doing ultrasound of heart for murmurs
    - was put on corticosteroids which shrunk 3 external hemangiomas but did nothing for internal ones
    - we (the parents) found a clinical trial at Boston Children's for life-threatening hemangioma with Interferon Alpha-2a - she entered the protocol Nov 93
    - taken off Interferon April 94 since was starting to slow developmentally - luckily, the Interferon did its trick and the hepatic tumors shrunk over time, saving her life - she rebounded developmentally
    - at around 6 months, started having symptoms such as a disordered upward eye movement and unexplainable head sweats
    - even earlier than 6 months, my wife suspected bowel control problems and headaches - this is a mother's instinct as I thought she was worrying too much but, over time, I saw she was right!
    - all other symptoms evolved with time - it made it very difficult to determine what was causing what, especially when we still hadn't had a tethered cord or Chiari diagnosis - we suspected the Interferon at first but then suspected something else
    - as her symptoms were growing both in number and severity, we began travelling all over eastern Canada and U.S. to try to get answers to her multiple problems - all local medical people were either dumbfounded with no diagnosis or misdiagnosed her (some misdiagnoses sounded right at first, but when the symptoms changed, we knew it was wrong - others just didn't fit the picture to start with)
    - At age 4 years, a neurologist suspected a tumor at the base of her spine - had spine MRI in Boston and found tethered cord (June, 1997)
    - Tethered cord surgery 1997 (snipped filum) - no resolution of symptoms - we had hoped it would have helped the leg, back, bladder and bowel control problems - I refer to these as her "spine" symptoms to distinguish from the "brain" symptoms which were alleviated from the Chiari surgery she had later
    - Fall of 1997, some nurses mentioned that her "brain" symptoms sounded like Arnold-Chiari. Researched on the web and found very little, other than one forum/mailing list.
    - Jan 98 had brain MRI - no evidence of Chiari - still no answer
    - on web mailing list, one doctor was being talked about alot as a Chiari "expert" since he was diagnosing people with minimal or no cerebellar tonsillar herniation with Chiari I as long as they met other criteria (lack of CSF flow issues, mainly) - a major and controversial Chiari paper was published from this doc in 1999
    - May 98, we visited this neurosurgeon in NY and he diagnosed Chiari I - this did not go down well with local doctors - stated he was a quack (they would later realize they were wrong with their criticism)
    - Summer of 1998, consulted with many other neurosurgeons and neuroradiologists by mailing them MRI scans and medical history and asked to pay special attention to Chiari as a possible diagnosis - all said no
    - On Chiari mailing list, found another neurosurgeon in Chicago who was not diagnosing Chiari I solely on the basis of tonsillar herniation
    - visited Chicago doc late Sep 98 and he diagnosed Chiari I syndrome
    - local docs were still not convinced of diagnosis - they refused to talk with NY or Chicago docs at our request - this, knowing full well the progressive and dangerous nature of her symptoms and having no definitive diagnosis nor treatment for her!
    - neurosurgeons from NY and Chicago told us our daughter's respiratory-related symptoms were becoming dangerous (a neurosurgeon from the NIH told us via phone to "stop shopping around and get something done about it" - he hadn't seen her scans or her - just said that from to our talk over the phone)
    - she was decompressed for Chiari I Jan 99 - all of her "brain" symptoms were alleviated, most of them almost immediately
    - alerted most specialists we had seen previously - most didn't respond - few were interested
    - all docs who disagreed with Chiari I diagnosis were of that opinion not because of the fear of inducing scar tissue or a tethered cord (she had already been diagnosed with this), but because of the lack of significant herniated cerebellar tonsils (she had no herniation of her cerebellar tonsils into the spinal canal - I have some MRI scans online somewhere if you're interested)
    - during decompression surgery, it was discovered that her tonsils were actually "short nubs protruding into the 4th ventricle" - this was/is not visible on her MRI films
    - leg, back, bladder and bowel control problems persisted, so we continued trying to get answers for this
    - on a couple of occasions in 1999, after the Chiari decompression, our daughter had bladder control - she actually went to the toilet on her own several times (the first time it happened for one whole day) - a few months later, it happened sporadically over a period of a week or two - then it stoppped and she was completely incontinent again - very strange!
    - leg pains also seemed to do a yo-yo effect within the first few months after the Chiari surgery, then stabilized (still had leg pains though)
    - fall 2000, she had flexion/extension spine MRI in Hartford at the request of the neurosurgeon in Chicago - we're from Canada and since no one here wanted to do anything about it, not to mention it would take months to get one done anyway, as well as the docs here stated they didn't know anything about flexion/extension (I still don't believe this), and since we had medical contacts in Hartford, she had the MRI done there at a better price for us than in Chicago - MRI indicated that the conus did not move from flexion to extension - although this did not prove definitively that the cord was tethered, considering her symptoms and worsening of the pain, a 2nd tethered cord surgery was recommended - checked with local neurosurgeons but they didn't agree
    - she had her 2nd tethered cord surgery June 2001 and, although problems were found and fixed as best as possible, no symptoms were alleviated
    - 2 months ago, saw neurosurgeon who did first tethered cord surgery - he asked her to bend down and touch her toes (while standing up), but she could not go down far - after he insisted that she try harder, she collapsed on the floor and cried due to the pain in her knees.


    Yesterday, we visited a neurologist to follow-up on her existing problems. Although she prescribed some pain med, she doesn't think her leg pain is neurologically-based. Her reason is that her leg pains only occur behind both knees. She's had knee scans and exams and nothing abnormal seen or felt. If only one knee was affected, I might believe that the problem is not a neurological one. However, since she always feels pain behind both knees and considering her spine problems, I tend to think her problem is neurological and she is experiencing deferred pain.

    I often have to carry her when she begins to feel pain and can no longer stand. I often carry her on my shoulders. When I do this and eventually put her back down, she always has to immediately squat (like a baseball catcher) for comfort - the pain is behind both knees. She will be down for a certain amount of time before being able to stand again. There's something very weird about positional factors. When she had her flexion/extension MRI, she was put under and the radiologists could somehow tell she was in alot of pain when her back was flexed (fetal position). They would administer pain med while she was under. Then there is the strange factor of her only being able to evacuate bowel movements while kneeling down.

    To answer your questions...

    Walking: She often tip-toed prior to her first tethered cord surgery back in Sep97. This seemed to be the only symptom which resolved. It's hard to tell now - she does sometimes tip-toe but she insists she just likes doing it. It's definitely not as frequent as it used to be.

    Pain: My wife and I are somewhat of the opinion that her kneepit pains are neurologically-based. The fact of nerves being compressed in the lumbar spine would seem to indicate this is affecting something, although I suppose it could simply be affecting bladder and bowel control. As well, when she sits on my shoulders and I put her down, she always squats due to pain behind her knees (suggesting a lumbosacral problem perhaps?). However, you seem to be in agreement with the neurologist we saw yesterday that since her pain is localized, it doesn't suggest a neurological problem. She thought it would most likely be an orthopedic problem - does this sound correct to you as well? You seem to think that if it was neurological, the pain would also be felt from her thighs downward. Problem is we've seen one or two orthopedists, she's had Xrays and ultrasounds of her knees and thighs and Xrays of hips and full-spine and nothing abnormal - the ortho docs think it's a neurological problem. We're now hoping to get MRI scan of the hips, thighs and knees to get a clearer picture, but I know it will be a battle trying to get this done here.

    Incontinence: She has undergone urodynamics studies.

    She has been hyperreflexive for the longest time. Many docs think that only hyporeflexive patients suggest spine/neurological problems, but there are specialists out there who say otherwise. I think I may have read this somewhere too. Another thing worth mentioning is that she had a small Baker's Cyst behind her left knee, which was deemed insignifant - this seems to be the only thing which resolved after her 2nd tethered cord surgery this past June as the doc can no longer feel it and was not seen in her knee ultrasound. Spine Xrays prior to her 2nd tethered cord surgery showed "mild" scoliosis, kyphosis and lordosis. We were told that the laminectomy from the first tethered cord surgery could have contributed to this as could the tethering itself - since it was discovered in her 2nd surgery this past June that the cord was indeed tethered with lots of tension, I'm speculating that the "mild" scoliosis/kyphosis/lordosis could have been a result of the tension on the cord. As far as I can tell, her leg pains are only behind the knees. I know that over a year ago, her pediatrician told her to squat (sitting down, not like a baseball catcher) and she complained of mild pain/burning in her thighs - but this is atypical.

    We have no other siblings and she is doing well socially and in school. There is the problem now of the worsening pains affecting her walking which may end up affecting her in other ways. Once more, if you can recommend any tests (e.g., Cine MRI of spine, MRI or CT of lower body), specialists or institutions, we're willing to consider them - even travel outside the country if need be. Although her tethered cord/nerve compression problems in the lumbar spine sort of indicate the reason for her remaining problems, we're not 100% convinced of this yet. There may yet be something else affecting some or all of her remaining problems which we'd like explored to the fullest.

    Thanks very much (ejfs@sympatico.ca).

  7. #7

    more info from daughter on leg pain

    Well, my daughter told us something yesterday. When she feels pain behind her knees, she does have a strange feeling down her thighs to the knees but no lower. She didn't feel it was a big deal because it is nothing compared to the pain she feels behind her knees. It's apparently a constant feeling in her thighs but she doesn't really know how to decsribe the feeling. I'm guessing sciatica here. Would you concur? She also cannot sit with her legs crossed as she feels the same pain. She cannot sit down for long periods of time and is constantly changing positions.

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