Detethering Recovery

[Barb]

C had a detethering in May. He went from walking with afo's short distances (the house and so on) to relying completely on his chair. He also has a cecostomy that for four years worked GREAT and now since May it is completely unreliable. Doctors say things will get better before they get worse but honestly, how long is too long? What has been your experience with this? Getting pretty worried that walking and bowels will never return to 'normal'.

[onedayatatime]

I am 54 and had a detethering 2 years ago. I have always had urinary and bowel incontinence but since the detethering it is much worse. I finally had to have a bladder tack and now use catheters for urination. I fall more now as well. The doctor also told me it could take several years before the nerves recover and things get better.....I'm still waiting. I sure hope Chad does better.

[san antonio]

Hi my son had tethered cored surgery when he was 4 years old before the surgery we thought that he had some control over his bladder and bowl movement, and he was able to use walker for quite good distance.
After the Detethering he lost every thing for good 6 months or so, after that he started to recover rapidly and responding to PT. now he is much better and safer.

[Barb]

That is encouraging!

Hi my son had tethered cored surgery when he was 4 years old before the surgery we thought that he had some control over his bladder and bowl movement, and he was able to use walker for quite good distance.
After the Detethering he lost every thing for good 6 months or so, after that he started to recover rapidly and responding to PT. now he is much better and safer.

[Barb]

I hope that things get better quick for you!

I am 54 and had a detethering 2 years ago. I have always had urinary and bowel incontinence but since the detethering it is much worse. I finally had to have a bladder tack and now use catheters for urination. I fall more now as well. The doctor also told me it could take several years before the nerves recover and things get better.....I'm still waiting. I sure hope Chad does better.

[Spokie]

What exactly is detethering? Why is it done?

[Barb]

Very simply it is the cutting away of scar tissue that has attached to the spinal cord at the lesion site. Over time and growth, the tissue that attaches to the cord literally pulls you backward and can cause decreased bowel/bladder function, leg function and pain (along with a lot of other things I am sure).

It is one of those surgeries that is heavily debated. I know of little kids (4 and 5) that have already had it done a few times and I know of adults who never have. The reason that C had it done was because he started having a lot of back and leg pain. He was 9 and had gone through a big growth spurt.

[Spokie]

I don't understand... can't they do the procedure differently? It's like you're stuck between a rock and a hard place - lose the pain or lose your abilities! I hope they make some breakthrough in this procedure soon. Lola, that should be your first research assignment!!

[lolathechecker]

hmm... its strange that they lose mobility temporarily, but it just goes to show another one of the miraculous ways the human body can heal itself. it probably temporarily damages the nerves having to be cut away near the scar tissue, but it probably only does minimal damage and the nerves have to recover themselves just like when you scrape your knee. it takes several days, but the skin grows back over it and then you're fine. nerves will do the same. it just takes a little longer and every body works at a different pace. some people can fully heal and recover from tonsil removal after only a week, but some people can take up to a month. im definately not an expert, but thats my thoery on everything. i bet C will be doing great after a few months of recovery.

[Dodger67]

No - nerves never grow back - its the only human tissue that cannot regrow.
I am genuinely shocked that you don't know that!
Your neurologist should have explained that on day one!
Thats the whole point why neurologiocal damage is always serious.

Severed or killed neurons never heal. That is why detethering has to be done so urgently and carefully - to avoid permanent damage.

[lolathechecker]

i still havent spoken to a neurologist. and i should have known that because ive read a few books on neurology. my bad.

[Barb]

From what I have learned, peripheral nerve damage can be repaired but not damage to the nerves in the spine and brain. That is why so many of the advances that are being made in the spinal cord injury world will not help people with spina bifida.

Dodger is correct that the timing of the detethering surgery is critical. If you wait too long, the damage may be permanent. However, too frequent detethering can cause massive amounts of scar tissue that creates the same result in the long run.

Here is some good basic info on the procedure from the SBAA (http://www.spinabifidaassociation.org/site/c.liKWL7PLLrF/b.2642343/k.8D2D/Fact_Sheets.htm)
Spinal cord tethering is a common cause of deterioration in a child with Spina Bifida. Although the exact frequency with which it occurs isn't entirely known, it is estimated that from 20-50% of children with Spina Bifida will, at some time, require surgery to untether the spinal cord, making this operation the second most common operation (behind shunt operations) in these children. This article will address some of the common questions that have been raised about spinal cord tethering.
What is spinal cord tethering, and why is it bad?
During the early stages of a pregnancy, the spinal cord of the fetus extends from the brain all the way down to the coccygeal (tailbone) region of the spine. As the pregnancy progresses, the bony spine grows faster than the spinal cord, so the end of the spinal cord appears to rise, or ascend, relative to the adjacent bony spine. By the time a child is born, the spinal cord is normally located opposite the disc between the first and second lumbar vertebrae, in about the upper part of the lower back. In a baby with Spina Bifida, the spinal cord is still attached to the surrounding skin, and is prevented from ascending normally; the spinal cord at birth is therefore low-lying, or tethered. Although the myelomeningocele is separated from the skin and closed at birth, the spinal cord, which has grown in this position, stays in roughly the same location after the closure, and usually quickly scars to the site of the surgical closure. As the child (and the bony spine) continues to grow, the spinal cord can become stretched; this damages the spinal cord both by directly stretching it, and by interfering with the blood supply to the spinal cord. The result can be progressive neurological, urological, or orthopedic deterioration.
What are the symptoms and signs of spinal cord tethering?
Children with spinal cord tethering may develop many different symptoms and signs. Conversely, many of the symptoms and signs of tethering can be caused by other problems, and the neurosurgeon needs to sort out what are the likely causes of the signs and symptoms in each case. Back pain, typically brought on or worsened by activity and relieved with rest, can be a sign of tethering. Sometimes the back pain is also associated with leg pain, even in areas that are numb. Changes in leg strength, or deterioration in gait (walking) can be signs of tethering. Manual Muscle Testing (MMT) of muscle strength is usually performed by physical therapists experienced in performing these tests, and can detect muscle weakness; these tests should ideally be performed on at least an annual basis, or whenever there is a change, to document changes before they become severe. The MMT has been shown to be a very reliable test of muscle strength if performed by experienced therapists. Progressive or repeated muscle contractures or orthopedic deformities of the legs, and scoliosis, may be signs of tethering as well. Finally, changes in bowel or bladder function can be signs of tethering; urodynamic studies, which provide an objective test of bladder function, can be very helpful in determining whether the changes are significant, and can sometimes detect bladder changes before they become apparent clinically.
How is a tethered cord diagnosed?
If a child with myelomeningocele and shunted hydrocephalus presents with clinical worsening, the first issue is to determine whether or not the shunt is working, as shunt malfunction can cause any of the signs or symptoms discussed above – one should therefore always check the shunt first! Accordingly, the first test is usually a Computed Tomography (CT) or Magnetic Resonance Imaging (MRI) scan of the brain. Although an increase in the size of the ventricles of the brain (that contain the cerebrospinal fluid) suggests a shunt malfunction, it is important to know that as many as 10-15% of children with shunt malfunction may have little or no change in the size of the ventricles. In some cases, the shunt is tapped (by inserting a needle through the skin into a ‘tapping chamber' for this purpose) and the flow of cerebrospinal fluid from the shunt, and the shunt pressures, are measured as another means of assessing shunt function. If there is any question about shunt function, the neurosurgeon may explore or revise the shunt at surgery before considering an untethering operation.
Once the shunt is found to be working, an MRI of the spine is performed. It is important to know that virtually every child with Spina Bifida has evidence of tethering on the MRI for the reasons discussed above; untethering is therefore generally only performed if there are clinical signs or symptoms of deterioration. The MRI is obtained both to show the neurosurgeon the anatomy of the tethering, and to exclude other abnormalities such as a syringomyelia - or syrinx for short (a fluid filled cavity within the spinal cord); diastematomyelia, or split cord malformation (in which the spinal cord is split into two halves over a part of its length, with a bony spur between the two halves of the spinal cord); or a dermoid cyst (in which a small tag of skin that was enclosed within the area around or within the spinal cord). Although MRI is the imaging study most commonly used, additional studies may include spine X-rays or CT scans of the spine, to look for various other bony abnormalities, or to follow the progress of scoliosis. Again, other functional studies may be done, including MMT and urodynamics, both to compare with previous studies to document a change, and to give a baseline against which to compare after the surgery.
When and how is surgery performed?
After all of the diagnostic studies have been performed, the neurosurgeon may want to untether the spinal cord. The decision to untether requires some clinical judgment on the part of the neurosurgeon, who must take into account both the patient's symptoms and signs and the results of the pre-operative studies. Unfortunately, since the MRI almost always shows tethering radiographically, the decision usually relies on the neurosurgeon's judgment as to what is causing the patient's symptoms and signs. A child with mild back pain who is otherwise stable might reasonably be watched or managed without surgery as long as they remain stable and the pain is manageable. On the other hand, progressive or severe pain, loss of muscle function or deterioration in gait, or changes in bladder or bowel function usually require an operation to prevent further deterioration. Timing of surgery is important, as the longer deterioration is allowed to continue, the less likely function will return to its baseline with surgery; the timing depends upon the magnitude and rapidity of the changes.
The untethering procedure usually involves opening the scar from the prior closure; occasionally, an incision may be made perpendicular to the original scar, particularly if the original closure was horizontal on the back. The scar is dissected down to the covering (dura) over the myelomeningocele; often the dissection includes the more normal covering just above the scarred area to obtain landmarks and orientation. Sometimes a small portion of the bony vertebrae (the laminae) are removed to obtain better exposure or to decompress the spinal cord. The dura is then opened, and the spinal cord and myelomeningocele are gently dissected away from the scarred attachments to the surrounding dura. There are many methods for doing this including scissors, scalpels, and various lasers; one way is not necessarily better than the others, and the surgeon usually has his or her own preference based upon their experience. Once the myelomeningocele is freed from all of its scarred attachments, the dura and the wound are closed.
Recovery in the hospital is generally about 2-5 days; some surgeons require that the child remain flat in bed for a couple of days to minimize the risk of spinal fluid leakage from the wound. Pain is usually not severe, as the child usually has some degree of numbness in that area anyway. The child is usually back to fairly normal activities within a few weeks. Recovery of lost muscle and bladder function is variable, and again depends upon both the degree and length of the pre-operative losses. Although we hope for improvements, it is important to understand that untethering is designed primarily to prevent further deterioration, rather than to improve deterioration that has already occurred.
What are the complications of untethering?
Untethering is generally a very safe procedure in experienced hands; however, the scar can make dissection difficult and the abnormal anatomy can be confusing at times, even to the experienced neurosurgeon. Complications are few, but include 1) infection, 2) bleeding, 3) damage to the spinal cord and myelomeningocele resulting in worsening muscle, bladder, or bowel function. The combined complication rate of surgery is usually only 1-2%. Although some have suggested that shunt malfunction may occur secondary to untethering surgery, it is probably more likely that an occult or unrecognized shunt malfunction was the original cause of the deterioration in the first place.
Is repeat untethering necessary?
Symptomatic tethering can occur at any time in the child's life, although the most common time is in the early pre-adolescent period (7-12 years) and extending into mid-adolescence. Symptoms from tethering can often occur during periods of growth, as might be expected, or during growth hormone treatments for short stature. Since all children grow, it is puzzling as to why some children develop symptoms and signs of tethering, while others don 't; perhaps some children's spinal cords are more lax early in life, or tolerate a greater degree of stretching than others.
Although most children require only one untethering procedure, a minority (perhaps 10-20%) require repeated untethering operations as the child continues to grow. Those who undergo untethering very early in life (as toddlers or young children) may more frequently require additional untethering procedures later, as they continue to grow. Fortunately, once the child stops growing, and their ‘adult, height is reached, clinical deterioration from tethering becomes much less frequent (although it can still occur).
Can anything be done to prevent tethering?
Many techniques have been tried to prevent or minimize tethering, but none has met with unqualified success in long term studies. Surgeons have placed grafts of various substances such as Dacron, Teflon, and other materials around the myelomeningocele hoping to prevent scarring to the surrounding dura. Some of these have actually produced more scarring. Others have cut off the myelomeningocele (cordectomy) if the child has no leg function, hoping to eliminate the scar from the myelomeningocele (however, this technique carries a risk of worsening bladder function and obviously eliminates any hope of recovering any functions if something in the future were to allow this). Most neurosurgeons now bring the edges of the flat myelomeningocele ‘placode' together during the initial closure, and sew the edges together to re-create the spinal cord in order to minimize formation of scar. Although this makes subsequent untethering easier and less risky should it need to be done, neither this nor any other technique have been proven to reduce the frequency of subsequent tethering in the long run. Research continues into this important area.
In conclusion, although the tethered cord is a common condition requiring surgery, it is also very treatable. Modern microsurgical techniques and the availability of such techniques as the operating microscope and lasers have made this a relatively routine surgical procedure in the hands of an experienced neurosurgeon. With close observation, it should be possible to diagnose this condition early and untether the cord before progressive and permanent damage occurs.

[Barb]

No, it isnt your bad. We are all learning here! I learn something new about sb everyday and I think know it all (just ask me, rofl)

i still havent spoken to a neurologist. and i should have known that because ive read a few books on neurology. my bad.

[Barb]

Unfortunately, since the MRI almost always shows tethering radiographically, the decision usually relies on the neurosurgeon's judgment as to what is causing the patient's symptoms and signs. A child with mild back pain who is otherwise stable might reasonably be watched or managed without surgery as long as they remain stable and the pain is manageable. On the other hand, progressive or severe pain, loss of muscle function or deterioration in gait, or changes in bladder or bowel function usually require an operation to prevent further deterioration. Timing of surgery is important, as the longer deterioration is allowed to continue, the less likely function will return to its baseline with surgery; the timing depends upon the magnitude and rapidity of the changes.

I wanted to comment on this section of the article. This is exactly what C experienced. We watched and managed the pain which was mild for about a year. Then suddenly, it became very painful and within a week we had the surgery done. It was very obvious to us that it was time, NOW, When the NS performed the surgery he said he was shocked that the cord "snapped back like a rubber band". How is that for parental guilt (although I know he did not mean it that way as he has always agreed with us on the timing issue of this). So, maybe we waited too long, who knows? I know it can take up to a year, so if by May he isn't fully 'recovered' then we will know that this is the way his function will probably remain.

[Dodger67]

I dodged 2 bullets with my SB - I don't have hydrocephalus or tethering.

However in my late 30's an additional but completely unrelated congenital defect was discovered (rather dramatically so!). I had a malformed heart valve - to be specific a bicuspid aortal valve. It shows no symptoms at all until the valve suddenly fails and starts leaking, usually some time in your 30's. So I had open heart surgery when I was 38 and now I have a ceramic heart valve, it works perfectly. The only residual problem I have is mild hypertension which is well controlled with medication.

[angel]

My son had a tethered cord and had it released when he was 2 years old. I was told when he was a baby the signs were lack of movement in his extremities. With him it was no where near the case. His digestive system messed up. He didn't have a bowel movement for 30 days. Yeah that wasn't a typo 30 DAYS!!!!
I took him to his doctor after the first couple of days with out a movement and they told me to give him milk of magnesia and he would go. We were in the doctor's office every other day after that they kept adding stuff for me to do mineral oil, supositories, enemas, By the 20th day we were up to 3oz of mineral oil a day 3 oz of milk of magnesia a day, 3 supositories and 3 enema's a day and still NOTHING HAPPENED. I begged and begged telling them something was terribly wrong. But i was just an over reacting mother. By the 25th day he stopped eating and wouldn't do anything but lay in the floor and cry. I forced them to hospitalize him i wasn't leaving that hospital till someone did SOMETHING!! They did the same thing in the hospital that i did at home all the while i was telling them it just didn't work. On the 29th day he started having bowel movements come out of his mouth. I swore to everyone there i was going to sue the S*$#@ out of them if my baby died!!!
They did an X ray and found that he was impacted all the way up his esophogus. His intestines were full his stomach and it was going up his esophagus. They immediatly took him into surgery and removed it which afterward they told me he would never have been able to get that out even if he were an adult!!!!
We went to everyone we could find that had something to do with gastrointerology and they all said nothing was wrong with him. There was obviously something wrong!! We went for his yearly MRI a couple of weeks later where they said his cord was stretching and had to be releived immediatly and he thought that is what caused the bowel problem. He had the surgery done and was home running around 4 days later. The bowel is still something we struggle with since that happened things never returned to normal for him in that area.
As far as his recovery from the tethered cord release the first couple of days were rough for him but on the 4th day it was like nothing ever happened to him.

[Barb]

That is awful. C has had that before where he is so impacted that he throws up. The cecostomy fixe that issue for him but OMG, how could they let that go 30 DAYS!!!

[lolathechecker]

from what i have read and seen on different websites, it looks to me like there are no by the book symptoms of tethered cord. which bothers me, because i like to know what i need to watch for. apparently it can be any out of the ordinary random symptom. i have even read some way off symtoms that kids had that ended up being shunt malfunction.

[mommy2cnj]

i have even read some way off symtoms that kids had that ended up being shunt malfunction.

Count my daughter in on that one. We were looking at the possibility of tethered cord (and OMG....the NS didn't want to treat my daughter. Said we needed to go to the hospital we had moved from......a nightmare til the myelo nurse said look, they have their care established here, their insurance is for here, you CAN'T turn these people down). It took over a year and we still hadn't gotten a yes or no answer.

Last Jan., J had flu-like symptoms. Myelo thought it was perhaps flu and wanted her urine checked. I didn't feel comfy with that so I took her to our home Dr. He wanted her admitted, if for nothing else, to have a complete series of tests ran.....urine, blood, x-rays, CT, etc. Thank God I chose to call him. (Still scary at times thinking about it) J's shunt was clogged so she was transported to another hospital.

We got there and they ran daily CTs/shunt series x-rays. Finally did surgery; said her shunt tubing had much calcium-buildup on it. Sometimes they do that when bodies reject them (erm...WHAT??!!). The tubing had come apart. This can cause the shunt malfunction symptoms, but it doesn't happen right away, due to the fluid still being able to empty, but only at a slower rate.....emptying into the body rather than down the remaining shunt tubing.

Something was still wrong. She also was having seizure after seizure, all of a sudden. She'd been dx'd with seizure disorder in '01, yet didn't have more than 1-2 per year; now it had turned into sometimes more than 1 a day......6-12/month. That's not a lot when considering others have many more; that is a lot when you jump to that from 1-2/year.

She went into a seizure a few nights after her tube replacement surgery. Her bp skyrocketed 200/180-something. Here I'm thinking OMG.....she should be dead at that rate and WHY aren't these people doing something? They gave her her seizure-stopping med. She stopped, although she was basically comatose for an hour and half after. The very next night, she did the same thing....same bp/seizure-stopping med, comatose. They said due to the uncertainty that her airway be open, we're putting her on a vent. (That's extremely scary for anyone.)

They finally came to me the next morning and said we're taking her into surgery.....CT shows shunt is clogged. (OK, isn't this the original reason she was transported?!) I had to literally call the director of the children's hospital to say, something is wrong with this baby and if you/your staff does not do what need be done for things to get better, should something happen, you will be held and ARE liable. Was a nightmare!

The Dr. (who remember, didn't want to originally see us), came into me after surgery. Said she has slit ventricles, the right ventricle where the shunt was at has a lot of tissue in it and one little move of the shunt and it could clog so therefore, we added a T ..... basically added a tubing to the left ventricle as well (a complex shunt it's now called). Therefore, if the right side stops draining, the left side will still kick in. He got up, shook my hand, smiled and said "OH, and I told them to take that machine off her....she doesn't need it anymore!" Then walked out. Needless to say, all the nurses (from clinic to the floor nurses at the time) are astounded at how well I am respected by this man after all of this.

One little J girl has *knock on wood* not had any type of seizure in just over a year now. Her shunt's been working fine.

And one more fine point? She was getting into much trouble due to "behavior" at school prior to the surgery......that's changed as well!

Oh....and one more thing? It wasn't tethered cord. :)

[angel]

That is awful. C has had that before where he is so impacted that he throws up. The cecostomy fixe that issue for him but OMG, how could they let that go 30 DAYS!!!

My son still has these issues as i said before. He takes miralax for it and it works great. We can't miss a dose though if we do he pays dearly for it. During his stay in the hospital after his spinal fusion i kept insisting that they give it to him. They wouldn't (i can't remember the reason) and it took us a month to get him right again. They said they would not do a colostomy for him until the problem became uncontrolable any other way.
I don't know why they let it go so long to everyone it was obvious the kid was in pain. His little belly was so big he looked pregnant. I am just glad that ordeal is over. Now i know about a hundred different ways to make him go to keep him out of the hospital.

[Barb]

Just to clarify that a cecostomy is different from a colostomy.

[angel]

what is the difference?

[kali]

I am also wondering what is the difference between a spinal fusion and detethering?

[angel]

I will try to explain this as best i can. A spinal fusion is where they actually fuse parts of the vertibra together to make them more stable and stronger. A tethered cord release is where they detatch the spinal cord that is attatched to the bone.

[kali]

thanks angel , is it the same bone that is being operated on? my son hasnt needed such operations, hope he never does, but I have to be knowledgable about these things. the main sympton is back pain? by the way I think our sons are arround the same age, mine is 9 years old

[angel]

My son's cord was tethered at the bottom (i forget which vertibra the spinal cord stops at but i believe it is in the lumbar area) and his fusion was done to his neck.

[Barb]

Cecostomy- a g tube like button is placed in the cecum (intestines). Daily or every other day you flush the intestines out with water. Feces exits normally and you do the whole thing on the toilet. Really no different than the way everyone goes but you force it out with the water.

Colostomy-feces continually collects in a bag that is carried around on the body.