Congenital Scoliosis - T4-T5 in teenager

[robinlbe]

I posted this also in the teenager section, but am posting here in case someone else sees this here and not there.....

Our 14 y.o. son was just diagnosed with scoli last month, and we just saw the spine surgeon for the first time two days ago. He has a 34 degree curve, caused by a congenital hemivertebrae. It is the triangular type, where it didn't separate completely while in utero. Thankfully, he has no pain and no neurological effects from this. His right shoulder, however, is about 4-5 inches higher than his left. I about cried when I saw the xray.

Because the spine is developing at the same time as the kidneys and heart, he will have further tests done on these organs as well. What is most interesting is that at my ultrasound with him at 18 weeks gestation, we were told that one kidney was 1/2 the size of the other, and it was not functioning. However, after much prayer from literally hundreds (if not thousands) of faith-believing people, at the next ultrasound, all appeared normal with the kidney. At birth, our pediatrician felt that his kidneys were normal and nothing further needed to be done.

The spine surgeon we saw is in Louisville, KY at the Leatherman Spine Center. He told us that bracing would do no good at all, and surgery is too risky AND the quickest way to paralyze someone. So we are watching and monitoring his spine. Of course, there is no way to make it better. However, if the curve continues to get worse, we will have to fuse it and stop it in its tracks.

This curve has probably shortened him a little already, hasn't it?
I read that congenital hemivertebraes are 1 in 10,000. Really?!? Have you all found that to be the case?
If you or your child have been diagnosed with congenital, have either of you been diagnosed with kidney and/or heart issues?

Thanks so much in advance for your help, encouragement, advice.....I know I will learn a lot from all of you.

blessings...robin

[leahdragonfly]

Hi Robin,

I am sorry to hear of your son's diagnosis. I know what a shock it comes as.

I am bothered by the comments made by the surgeon you saw, and I would strongly recommend you find another surgeon who is highly skilled with congenital scoliosis. I am quite sure your son has surgical options that aren't expected to result in paralysis. That comment just comes off so callous.

My daughter's scoliosis is entirely different from your son's, but she had really fantastic care at Shriner's St Louis under Dr Scott Luhmann. I would recommend him to anyone. Also, the spine team at Shriner's Philadelphia is one of the very top pediatric spine centers in the world. I am quite sure they will see your son for a second opinion. You can e-mail Janet Cerrone, the spine team PA, at janetcerrone@comcast.net . She can tell you if your son would benefit from visiting Shriners Philadelphia for a second opinion.

I know it may seem shocking the thought of traveling across country for medical care, however, your son's case is very unusual and many scoli surgeons will not be equipped to treat him. You will not regret traveling to St Louis or Philadelphia for care. We travelled from Oregon to Philadelphia for a consult, then later had my daughter's surgery done in St Louis, and it was well worth the thousands of miles we travelled.

In case you are not familiar, Shriners Hospitals are a network of cutting-edge specialty orthopedic hospitals that is some cases also treat children with burns or spinal cord injuries. They provide all care to children 18 and under free of charge, regardless of ability to pay. Families with insurance will be asked to provide their insurance info and the insurance will be billed, but the family will never see a bill or have any amounts due. It is truly amazing. The only requirement is that the child will benefit from their specialized services. You do not have to have a Shriner sponsor.

I would recommend you e-mail Janet Cerrone, and give her a few days or a week to get back to you. I believe she will be able to advise you about getting the most appropriate second available for you son.

Good luck, and please do let us know how things are going.

[Pooka1]

I know it may seem shocking the thought of traveling across country for medical care, however, your son's case is very unusual and many scoli surgeons will not be equipped to treat him.

I completely agree with this and all Gayle's other points.

Congenital scoliosis seems exquisitely rare and you want someone who has treated the most cases. It is conceivable some of the top pediatric surgeons have done less cases than certain other guys. The Shriners guys are a good bet for having seen the most cases in my opinion since they are known for orthopedics as a specialty.

And the comment about paralysis seems to indicate that surgeon may not have done so many cases but of course I don't know that. At this point, if a surgeon made that comment to me, I would ask for the data. We have at least one other child (teenage boy with a thoracolumbar curve) with congenital scoliosis on the group who was fused successfully. No paralysis. I can't think of any other case on the group. Given the prevalence, it's amazing to even have that one testimonial.

Good luck.

[Pooka1]

robinlbe,

By coincidence, Dr. Hey blogged about a very complex congenital case he handled recently. Note how he consulted with other surgeons. I suggest you want a surgeon who will do that also for your son.

http://drlloydhey.blogspot.com/2011/...75-degree.html

Last week we straightened up Emory, who is 12 who had complex congenital scoliosis measuring about degrees at Wakemed. Due to complexity of her deformity, I did extensive preop planning, including 3 dimenensional analysis of her cervical and thoracic spine using special software. I actually presented her case at Boston Children’s Hospital special Fellow Reunion Conference right before the Grice Dinner and Lectures. Many thanks to Dr. John Emans, and others who helped give their input!

Intra-operative O-Arm Navigation and 3D analysis was also performed. I took the preop 3D analysis I did for each pedicle level, and actually arranged them into a PowerPoint presentation which I then printed and posted on the wall in the operating room! That was very helpful. Surgery was T1-T12 instrumentation performed along with several osteotomies. WakeMed OR team did great.

Her surgery went well, with 3 night stay at WakeMed Raleigh Children’s Hospital, going home on Saturday doing well.
This surgery helped with her severe shoulder obliquity caused by the severe scoliosis.

[robinlbe]

I knew I had come to the right place!!
Ladies (I'm assuming, ladies, anyway...you sound like VERY informed moms to me!!!!), thank you for taking so much time to "talk" to me and give me so much advice and encouragement. I had been reading through the forums for a couple of weeks before our doctor visit, and before I had been given permission to post, so I had read about the Shriners' hospitals and all they could do. We live only about four hours or so from St. Louis, so that's not terrible.

I do think I'd like a second opinion. Even though my son's scoli isn't terribly noticeable (because he is so active, and he doesn't wear tight shirts), I am so scared it's going to get worse. I guess I was just relieved the spine doc wasn't knife-happy. My son is in ROTC, and is running his mile, doing his push-ups, pull-ups, sit-ups, etc....he's always been my active child - riding his bike w/o training wheels at age 4, doing cartwheels, flips, etc, my skateboarder, my true dare devil!!! He is not supposed to stop doing all of this. I want him straight, of course - but I'm so scared that surgery will cause him to stop doing this stuff.

AND to be honest, bone surgeries of any kind scare the daylights out of me- in the last two years, I have watched my mom have a back surgery and a hip-replacement surgery to get WORSE in her condition!!!!! Of course, I know this is not the same thing.....I know. But it scares me something awful. I would be willing to take a chance on myself - because I'm old. But I'm scared of doing so with my 14 y.o.

Does that make sense?

And yet, I know that YOU each have gone through this very thing........I know.
I honestly did not know we were getting into something so serious.
I actually was hoping my chiropractor would be able to help, but when she saw the written report about his xrays, and she saw the "hemivertebrae" on there, she wouldn't touch him. It wasn't until we saw the xrays ourselves this week that we realized what we were dealing with.

Yes, I do think you all are right. We need to get the data of how many wedged hemi's this doctor has dealt with. We also need a 2nd opinion.
At this point, my son has the rest of his life - hopefully another 70+ years to go.

Thanks again for everything you've shared....
blessings...
robin

[titaniumed]

Hi Robin

We do have quite a few knowledgeable mom’s here! What would we do without them? (smiley face)

I will ditto Gayle’s post (again)....You son is not a standard or common type of scoliosis and needs to be seen by some of the experts....Experts like those at USCF, HSS,TCSC, or any other institution that deals with hemi’s or oddball vertebral shapes. Shriners also deserves HIGH marks. These types of hospitals have the best of the best brainstorm and discuss cases like this. Its nice to know that many guru’s will be looking at your sons x-rays.....

I’m so sorry about this. If he were my son, I wouldn’t dwell on this at all. I don’t think that they will make a quick surgical decision, but will probably want to watch him like a hawk for now. His hemi is up high.

If he has any x-rays taken, get digital copies burned to disc for your records. This goes for CT’s and MRI’s also. They are your property.

Welcome to the forum.
Ed

[rohrer01]

I could be wrong, but I think "fierceliketiger", who is now in her 20's also had a hemivertebra. If I remember correctly, she had hers removed, although I don't think it was really high up. Her problem was broken rods and she's been referred to a different doctor in a different state. You might benefit from looking her up and reading through her posts or private messaging her and ask if that was the case and how the initial surgery went as far as removal of the vertebra. But again, I could have misunderstood.

[titaniumed]

Rohrer

You have a good memory. Her hemi was at L4.

She was revised at Twin, said she was doing ok a month later, and stopped posting. I dont think she is online or she would post.

Maybe she will surprise us and say hi. I hope she is doing ok.
Ed

[robinlbe]

I've shared this info. with my husband....and about the Shriners hospitals with my son.
I do think we will get the 2nd opinion.

We need to get the xrays on our own disk/flashdrive or something. Right now, they're at the hospital.
We'd like to avoid surgery, if at all possible. As long as our son is not in pain, and the curve is not causing him problems...and is not progressing. Of course, we won't know *that* until the next visit (in February) with the next round of xrays.

I feel like being an ostrich right now, quite frankly!

In the last two years, we have dealt with my own breast cancer, my mom's two surgeries (with her contracting MRSA and nearly dying with the first), my daughter passing out and ending up with severe concussions twice, two high school graduations (and kids going off to college), and my teaching jobs being reassigned three years in a row......I've had quite enough, thank you very much...

Oh, and not to mention I turned my ankle - which ended up as three fractures there, and a total of three casts!!! And then just three months ago, I tore the tendon in my right ring finger (which doesn't sound like a biggie, except that I was in a splint for eight weeks 24/7, and then it took another month to get out of it altogether!!)
.....yes!!! All of those things since November, 2009..... yippee....Life is a roller coaster, if nothing else!

Did I mention "stress" was my middle name right now??? ha!
anyway....thank you for being here for me in this newest test and trial in my faith.
God is faithful and He will carry us through this, too....
I'm just holding on for this ride, too....

blessings...
robin

[Pooka1]

In the last two years, we have dealt with my own breast cancer, my mom's two surgeries (with her contracting MRSA and nearly dying with the first), my daughter passing out and ending up with severe concussions twice, two high school graduations (and kids going off to college), and my teaching jobs being reassigned three years in a row......I've had quite enough, thank you very much...

Oh, and not to mention I turned my ankle - which ended up as three fractures there, and a total of three casts!!! And then just three months ago, I tore the tendon in my right ring finger (which doesn't sound like a biggie, except that I was in a splint for eight weeks 24/7, and then it took another month to get out of it altogether!!)
.....yes!!! All of those things since November, 2009..... yippee....Life is a roller coaster, if nothing else!

Okay enough "fun" for you. Get off the train and let someone else on. :-)

That is quite the list! I think life has been very unfair to your family.

[Mustang]

Hi there,

I've only ever lurked on this forum, but this thread caught my eye so I had to respond.

I'm 30 years old and from the UK, and was born with Spinal Dysraphism (Spina Bifida) and Diastematomyelia (split cord malformation, with a bone spur through the cord, causing tethering) with bony malformations of my lumbar spine. No one is absolutely sure how many lumbar verts I have, because they are so hard to identify, but it was assumed that my hemivertebra was at L3. This was a strange-shaped vert, but the wedge was mostly posterior, causing lumbar kyphosis. meThis was identified at birth, but as my scoliosis was mild then, they decided to monitor me regularly to see how things would pan out. I had neurosurgery on my spinal cord when I was 3 years old, to remove the bony spur and release the tethering (initially successful, but the spur grew back), but the hemivertebra was left alone.

Things were fine with my back for most of my childhood - my curves were at 40 degrees when I was 15, so not too bad. However, just a few months after that last xray in 1997, I developed severe pain in my lower back, right buttock and leg and right leg spasticity quite suddenly. I was admitted to hospital on two occasions to have a series of scans and tests in order to find out what was wrong. After a CT myelogram, they discovered that the hemivertebra was actually compressing my spinal cord and nerve roots. My surgeons (ortho and neuro) discussed what could be done for me surgically, and eventually decided on decompression and fusion L2-L4. They warned me it was a risky surgery, and I wouldn't come out of it with any correction because they can't stretch someone with a tethered cord as the risks of paralysis are too high. But, if I didn't have the surgery, I was looking at progressive paralysis anyway, so that combined with the pain I was living in made me choose to have the surgery. So, aged 17 I underwent a 14 hour operation, during which they carefully excised the hemivertebra posteriorly (they had discussed doing it anterior and posterior in a two stage procedure, but once they'd opened me up they decided to do it all in one sitting) using the Wiltse approach (an incision either side of the midline), packed the spine with bone graft then used pedicle screws for stability.

I won't lie, my recovery was pretty rough. I had a lot of post op pain, nerve irritation and a contracture of my left hip (caused by the length of time in traction on the operating table). While the operation thankfully didn't paralyse me, I did come out of it with nerve root motor damage in my right leg, meaning my foot has no voluntary function and some of the major muscles are permanently damaged. The spasticity also worsened, but was not as painful as before the op. I couldn't walk because of this, and it took months of tough physio to get me up and walking with sticks. However, the operation was successful in that it sorted out my back pain, and I considered losing some function as a fair 'trade off', and I don't regret having the surgery for one second. Do remember that I have spinal cord damage from birth though, so my experience isn't necessarily the norm!

Anyway, I just wanted to tell my story to show that they can and do remove hemivertebrae in older children and adults. While it is undoubtedly a complex surgery, certainly more so than a standard fusion/correction of idiopathic scoliosis, it's by no means a given that you'll end up paralysed, especially if there's no underlying cord involvement. I would advise you to get a second opinion from another surgeon for your son rather than just going by what one surgeon has said - surgeons can and do disagree frequently, but the important thing is that you feel confidence in the surgeon's abilities.

[robinlbe]

Mustang, Thanks for sharing your experiences!
I feel I have NOTHING to complain about after all you've been through....I realize that was not the purpose of your posting, but it did give me a great perspective AND it helped me to know that we might have other options.

I don't know if removing Samuel's hemivertebrae would be helpful to him or not.....how would one decide? It's not impeding his spinal cord, apparently.
It's just jacking up his spine at an incline, causing the scoli. As he's growing, it's getting worse (I'm guessing). He's 5'7" now, and probably has another 5-6" to go, as I am 5'11" and my husband is 6'1". My oldest son (age 20) is 6'3", and my daugher (who was my 23.5 weeker preemier, who weighed one pound, four ounces at birth) is 5'6". So we're on the taller side

So if the hemivert. is removed, would that just keep the curve from getting worse?

I'm glad to hear of the successful hemi removals.
That is helpful.

Beyond that, we are checking out the other things that go along with same-time development as the vertebrae separation - the kidneys and heart. Our doctor was able to get appointments for Thursday - which is in three days. Once we get those results, then I'll check further into the Shriners and see if they'd be willing to see our son.

Thank you, again, for all of your advice, encouragement, sharing, and more!!
Blessings...robin

[Mustang]

Hi Robin,

I'm glad you found my post helpful, i'd hoped that you'd be able to glean something from it in order to help you with your own quest to get your son sorted

I forgot to mention that that luckily I don't have any issues with my heart and my kidneys were fine as a child. I did develop some dilatation of the collecting tubes within the kidneys as I got older, but that was because I have a neurogenic bladder and suffered with severe bladder spasms as well as not voiding properly for many years, but i'm happy to say that i've managed to reverse this damage by self-catheterising. I hope the tests show that your son's heart and kidneys are in tip top shape

Has your son had an MRI scan yet? If he hasn't I imagine he'll have one soon enough. It's encouraging that he doesn't have any signs of neurological involvement, but the MRI will conclusively show whether or not there's any underlying spinal cord lesion or tethering.

I'm surprised at the extent of his shoulder obliquity (4-5" difference) if the curve really is only 34 degrees - if you've got any photos or xrays i'd love to see them (don't worry if you'd rather not, i'm just always interested in seeing these things). I guess it's because of how high up the hemi is - I believe high thoracic hemeverts are much less common than lumbar. Anyway, I think the fact that the curve is not yet in surgical territory (at least 40-50 degrees), he has't finished growing and there aren't any other indications for surgery (neurological compression, pain or threat to other organs like heart and lungs) means that surgeons would be unlikely to offer surgery just yet. I've read that hemivert excision anywhere above the level of the conus medullaris (tip of the cord, usually lying at around L1) carries a higher risk of neurological damage. Even though my hemivert was L3, my cord is tethered at L5-S1, so actually I think the risks of damage for me were about the same as for a thoracic excision. Anyway, so personally I think the watching and waiting approach is probably best right now. Presumably he's being xrayed and the curve measured every 3-6 months?

Oh, just one more thing - other articles i've read suggests that fusion/correction of a curve caused by hemivertebra is said to be more successful (i.e better correction) if the hemivertebra is excised first, rather than fusing around it (which also increases the rate of pseudarthrosis).

Glad you're going for the second opinion! I'm in the UK so can't comment on any doctors in the US, but there's enough US-based people here to know more about that. I hope you get on ok with it

[Mustang]

Oh, and here's a couple of pics of my spine, xray and CT, in case you're interested

http://i390.photobucket.com/albums/o...py/image-1.jpg

http://i390.photobucket.com/albums/o...ppy/image9.jpg

[rohrer01]

Wow, Mustang. Being born with all those problems and seeing your spine, especially the side view, I'm surprised that you were ever able to walk. I have a friend who was born with a meningiocele (sp?) spina bifida and he's never been able to walk. Thankfully he wasn't mentally impaired as that sometimes goes along with it. It's quite obvious that you are not mentally impaired, also. Don't take this the wrong way, please. You are one really fortunate lady that you can walk and are not mentally impaired. Me and my son both have spina bifida occulta. His is worse than mine. Neither one of us have any side effects from it what-so-ever. It was just a finding when we had x-rays for other reasons. Even though all of us on the forum either have scoliosis or have a loved one with it, I think we all have things to be thankful for. Thank you for sharing.