Congenital scoliosis is a sideways curvature of the spine that is caused by a defect that was present at birth. It occurs in only 1 in 10,000 newborns and is much less common than the type of scoliosis that begins in adolescence. Even though congenital scoliosis is present at birth, it is sometimes impossible to see any spine problems until a child reaches adolescence.
Dr. Gupta joins the show to explain more about congenital scoliosis and when to refer to a specialist.
Treating Children With Congenital Scoliosis
Featured Speaker:
Learn more about Munish Gupta, MD
Munish Gupta, MD
Munish Gupta, MD specializes in adult and pediatric spine surgery, with a focus on complex spinal reconstructive surgery for treatment of all spinal deformities of the lumbar, thoracic and cervical regions. He has over 23 years of experience in treating spinal deformities such as scoliosis, kyphosis, spondylolisthesis and Flatback syndrome. Dr. Gupta also specializes in spinal revision surgery and spinal tumors.Learn more about Munish Gupta, MD
Transcription:
Treating Children With Congenital Scoliosis
Melanie Cole (Host): Welcome. June is Scoliosis Awareness Month, and in that vein, we’re talking with Dr. Munish Gupta. He’s a Washington University Pediatric Orthopedic Spine Surgeon at St. Louis Children’s Hospital. Dr. Gupta, welcome back, always a pleasure. You’re such a great guest. Tell us what’s congenital scoliosis? Does this run in families?
Dr. Munish Gupta (Guest): So congenital scoliosis, people mistaken as something that happens at birth and it progresses, but actually it’s an anomaly of the spine itself. So the spinal column is not formed properly. It’s very unlike some of the other syndromes where they have congenital myopathy or so and it’s a muscle problem, but it’s actually a problem with the building blocks of the spine, so the spinal column is not formed properly and it’s from an embryonic stage. It happens in the first trimester, like the first 6 weeks. Something happens where the spinal column is not formed properly. That’s what congenital scoliosis is, and the scoliosis is side to side curvature as you know, but you can put this all together as congenital spine deformity. So depending on where the malformation is in the spinal column or the building blocks of the spine, you can have kyphosis or scoliosis.
Host: How and when is it diagnosed? Tell us a little bit about diagnosis and some of the signs and symptoms that pediatricians should be looking out for?
Dr. Gupta: So you’ll get a patient that’s quite young and maybe developing a rib hump or a curvature of their spine, and of course it could be other things, but one of the things it could be is congenital scoliosis, and if you do get an x-ray and you see that the vertebral bodies are not formed properly, then you have to think about what other things that could go wrong at the same time. So all the systems are forming at that time, so you want to check for urologic abnormalities or cardiac abnormalities because 25% of the time these patients will have some sort of urologic conditions and 10% of the time they might have a heart problem. So you might want to think of getting an ultrasound of the kidneys or the heart to make sure that there’s no other problem that happened at the same time when the fetus was developing and the other thing is you want to make sure there’s no spinal cord anomalies that happened with congenital scoliosis at the same time.
Host: What ill effects can result from that spinal deformity and scoliosis? What happens as a child grows and matures?
Dr. Gupta: So it depends on the anomaly itself. So some anomalies are kind of benign so there are different kinds of anomalies. One is when the vertebral body is not quite fully formed where that’s called failure formation, or if it’s formed fully but it’s connected to the other one so it doesn’t grow properly, so it grows sideways because it has asymmetric growth, that’s called failure segmentation, or they can be very mixed. So there are some anomalies where they have a failure segmentation on one side where it doesn’t grow and it has failure formation on the other side where it has extra growth plates. So it’s all about the asymmetry of growth, so in those kind of problems, the anomaly makes the deformity grows fast, and if that happens then you can get a severe scoliosis in a relatively young child.
Host: Tell us a little bit about treatment options. Once you’ve detected what’s going on, what happens next Dr. Gupta?
Dr. Gupta: So once you diagnose the patient with congenital spine deformity or scoliosis and you’ve evaluated what this is, then you probably should refer to a spine surgeon just to make sure that you’re not missing something, and once the spine surgeon sees these patients, you can sort of follow them or observe them. It doesn’t mean neglect. It means observation, you follow them every 6 months or a year depending on the anomaly because some anomalies are more deforming than others, and then you can follow them with x-rays or exam and if they get worse, then depending on the size of the anomaly and how many anomalies you have, if it’s a very small anomaly, very localized you can sort of fuse the spine there, kind of spot weld it so it doesn’t deforme as much or you can watch them and see how it grows.
Host: Now as far as all the different treatment options, is there a way to brace option? Is this something that you tell parents that they wait to brace? You do this right away? Tell us a little bit about how that all works and how you work with other providers and with the parents?
Dr. Gupta: Yeah so I think, one thing that’s really important to know is that congenital scoliosis or spine deformity in general is not very amenable to bracing, so most of the time you observe, and if it gets severe enough – you have to exercise some of the surgical options. One surgical option is you put a groin construct to make the spine grow as the child grows until they reach maturity and you fuse, or you can do a limited fusion of the very abnormal segments, so the rest of the spine doesn’t get deformed and it’s not affected by it so they get a pretty much straight spine and they grow together with a straight spine. Or finally, if they’re old enough, like in their adolescence and you find a congenital anomaly, you can actually go right to surgery if it’s progressive.
Host: Then tell us a little bit about the surgery and what’s the follow up care for children like that have congenital scoliosis?
Dr. Gupta: So the first thing after we see them, we of course evaluate their deformities, make sure that the spinal cord doesn’t have anomalies, make sure their GU system and their heart is all okay. Then we follow them every 6 months or so, depending on how fast they’re growing, and then if they start to get worse in terms of progression of the curvature or the anomaly, then we think of surgical treatment.
Host: Do all children have this risk of progression? Does it happen for the most part to most children diagnosed with this?
Dr. Gupta: No, that’s the beauty of it. Not everybody with congenital spine anomalies will need surgery. It depends on what malformations that they have and their different way these progress. So small hemivertebrae that has fully segmented area, that means that has extra growth plates, that’s more likely to progress. A semi-segmented one which has only half the growth plates will progress less, and the non-segmented will have no extra growth plates, will not progress, or the ones that the two vertebrae are just sealed together, we call them a block vertebrae, they don’t progress because they keep the spine straight. They just expand and grow in the same direction. So it all depends on the malformation the child has. So really if you analyze the deformity and then you follow the growth, it tells you how it’s going to be.
Host: Wow, isn’t that amazing. So what would you like pediatricians, other providers to know about congenital scoliosis, and when you feel it’s important to refer? Is earlier better?
Dr. Gupta: In general yes. So when you see a child with a deformity that’s already showing on their physical exam and you get an x-ray and you find that it’s a congenital deformity of the spine, then I think it’s an appropriate time to refer and at the same time, think about what I said about associated anomalies because as these systems are developing at the same time, they can also have malformations like the GU, the cardiac, and the spinal cord. So think in those terms, getting an ultrasound of the heart, kidneys, and maybe an MRI of the spinal cord may be a good idea, so think about those terms, and I would say that the thing to tell parents is look don’t panic, we know what this is, we’re going to get you to see an expert and then we’ll go from there. It doesn’t mean that you automatically need surgery, we need to evaluate what you have first.
Host: So important and what can that physician expect from your team after referral in so far as communication with the referring physician and your team approach?
Dr. Gupta: So at St. Louis Children’s I think we have a pretty good team around us in the clinic, so when you come in to see the doctor, you’re going to see the nurses and everybody else, and the first thing we do is we do a long history and physical, make sure there’s nothing else wrong. Of course do the physical exam as well as x-rays to determine what you have, and then we decide on any kind of x-ray exam you may need such as, what I mentioned before, ultrasounds of the kidneys, the heart, MRI of the spinal cord, and then we give you a pretty good assessment of what is going to happen and what the follow up is going to be like so the parents first can be relieved and the number one question the mom and dad want to know is did they do something wrong, and the answer to that is they did not do something wrong. This is not hereditary as far as we know, and it just happens. So nobody did anything wrong.
Host: That’s really important information and I’m so glad that you cleared that up for the listeners. So important. Do you have anything else that you’d like to add about congenital scoliosis.
Dr. Gupta: The only thing I’d like to say is that people confuse congenital as being some of the other diseases and it’s really just a malformation of the vertebral column. It’s a physical malformation. Just imagine if there was a building, and there was a brick that was placed obliquely in the middle of the building and makes the building curved, that’s what you should think about with congenital scoliosis and that’s really what it is, very basically, and you just follow it. If it gets worse, you fix it, and if it doesn’t you leave it alone.
Host: Great information Dr. Gupta, thank you so much. What a great guest and educator you are. Thank you for joining us again. That wraps up this episode of Radio Rounds with St. Louis Children’s Hospital. To consult with a specialist or to learn more about services offered, please call the Children’s Direct Physician Access Line at 1-800-678-HELP and head on over to our website at stlouischildrens.org for more information and to get connected with one of our providers. If you as a provider found this podcast informative, please share on your social media and be sure to check out all the other fascinating podcasts in our library. I’m Melanie Cole.
Treating Children With Congenital Scoliosis
Melanie Cole (Host): Welcome. June is Scoliosis Awareness Month, and in that vein, we’re talking with Dr. Munish Gupta. He’s a Washington University Pediatric Orthopedic Spine Surgeon at St. Louis Children’s Hospital. Dr. Gupta, welcome back, always a pleasure. You’re such a great guest. Tell us what’s congenital scoliosis? Does this run in families?
Dr. Munish Gupta (Guest): So congenital scoliosis, people mistaken as something that happens at birth and it progresses, but actually it’s an anomaly of the spine itself. So the spinal column is not formed properly. It’s very unlike some of the other syndromes where they have congenital myopathy or so and it’s a muscle problem, but it’s actually a problem with the building blocks of the spine, so the spinal column is not formed properly and it’s from an embryonic stage. It happens in the first trimester, like the first 6 weeks. Something happens where the spinal column is not formed properly. That’s what congenital scoliosis is, and the scoliosis is side to side curvature as you know, but you can put this all together as congenital spine deformity. So depending on where the malformation is in the spinal column or the building blocks of the spine, you can have kyphosis or scoliosis.
Host: How and when is it diagnosed? Tell us a little bit about diagnosis and some of the signs and symptoms that pediatricians should be looking out for?
Dr. Gupta: So you’ll get a patient that’s quite young and maybe developing a rib hump or a curvature of their spine, and of course it could be other things, but one of the things it could be is congenital scoliosis, and if you do get an x-ray and you see that the vertebral bodies are not formed properly, then you have to think about what other things that could go wrong at the same time. So all the systems are forming at that time, so you want to check for urologic abnormalities or cardiac abnormalities because 25% of the time these patients will have some sort of urologic conditions and 10% of the time they might have a heart problem. So you might want to think of getting an ultrasound of the kidneys or the heart to make sure that there’s no other problem that happened at the same time when the fetus was developing and the other thing is you want to make sure there’s no spinal cord anomalies that happened with congenital scoliosis at the same time.
Host: What ill effects can result from that spinal deformity and scoliosis? What happens as a child grows and matures?
Dr. Gupta: So it depends on the anomaly itself. So some anomalies are kind of benign so there are different kinds of anomalies. One is when the vertebral body is not quite fully formed where that’s called failure formation, or if it’s formed fully but it’s connected to the other one so it doesn’t grow properly, so it grows sideways because it has asymmetric growth, that’s called failure segmentation, or they can be very mixed. So there are some anomalies where they have a failure segmentation on one side where it doesn’t grow and it has failure formation on the other side where it has extra growth plates. So it’s all about the asymmetry of growth, so in those kind of problems, the anomaly makes the deformity grows fast, and if that happens then you can get a severe scoliosis in a relatively young child.
Host: Tell us a little bit about treatment options. Once you’ve detected what’s going on, what happens next Dr. Gupta?
Dr. Gupta: So once you diagnose the patient with congenital spine deformity or scoliosis and you’ve evaluated what this is, then you probably should refer to a spine surgeon just to make sure that you’re not missing something, and once the spine surgeon sees these patients, you can sort of follow them or observe them. It doesn’t mean neglect. It means observation, you follow them every 6 months or a year depending on the anomaly because some anomalies are more deforming than others, and then you can follow them with x-rays or exam and if they get worse, then depending on the size of the anomaly and how many anomalies you have, if it’s a very small anomaly, very localized you can sort of fuse the spine there, kind of spot weld it so it doesn’t deforme as much or you can watch them and see how it grows.
Host: Now as far as all the different treatment options, is there a way to brace option? Is this something that you tell parents that they wait to brace? You do this right away? Tell us a little bit about how that all works and how you work with other providers and with the parents?
Dr. Gupta: Yeah so I think, one thing that’s really important to know is that congenital scoliosis or spine deformity in general is not very amenable to bracing, so most of the time you observe, and if it gets severe enough – you have to exercise some of the surgical options. One surgical option is you put a groin construct to make the spine grow as the child grows until they reach maturity and you fuse, or you can do a limited fusion of the very abnormal segments, so the rest of the spine doesn’t get deformed and it’s not affected by it so they get a pretty much straight spine and they grow together with a straight spine. Or finally, if they’re old enough, like in their adolescence and you find a congenital anomaly, you can actually go right to surgery if it’s progressive.
Host: Then tell us a little bit about the surgery and what’s the follow up care for children like that have congenital scoliosis?
Dr. Gupta: So the first thing after we see them, we of course evaluate their deformities, make sure that the spinal cord doesn’t have anomalies, make sure their GU system and their heart is all okay. Then we follow them every 6 months or so, depending on how fast they’re growing, and then if they start to get worse in terms of progression of the curvature or the anomaly, then we think of surgical treatment.
Host: Do all children have this risk of progression? Does it happen for the most part to most children diagnosed with this?
Dr. Gupta: No, that’s the beauty of it. Not everybody with congenital spine anomalies will need surgery. It depends on what malformations that they have and their different way these progress. So small hemivertebrae that has fully segmented area, that means that has extra growth plates, that’s more likely to progress. A semi-segmented one which has only half the growth plates will progress less, and the non-segmented will have no extra growth plates, will not progress, or the ones that the two vertebrae are just sealed together, we call them a block vertebrae, they don’t progress because they keep the spine straight. They just expand and grow in the same direction. So it all depends on the malformation the child has. So really if you analyze the deformity and then you follow the growth, it tells you how it’s going to be.
Host: Wow, isn’t that amazing. So what would you like pediatricians, other providers to know about congenital scoliosis, and when you feel it’s important to refer? Is earlier better?
Dr. Gupta: In general yes. So when you see a child with a deformity that’s already showing on their physical exam and you get an x-ray and you find that it’s a congenital deformity of the spine, then I think it’s an appropriate time to refer and at the same time, think about what I said about associated anomalies because as these systems are developing at the same time, they can also have malformations like the GU, the cardiac, and the spinal cord. So think in those terms, getting an ultrasound of the heart, kidneys, and maybe an MRI of the spinal cord may be a good idea, so think about those terms, and I would say that the thing to tell parents is look don’t panic, we know what this is, we’re going to get you to see an expert and then we’ll go from there. It doesn’t mean that you automatically need surgery, we need to evaluate what you have first.
Host: So important and what can that physician expect from your team after referral in so far as communication with the referring physician and your team approach?
Dr. Gupta: So at St. Louis Children’s I think we have a pretty good team around us in the clinic, so when you come in to see the doctor, you’re going to see the nurses and everybody else, and the first thing we do is we do a long history and physical, make sure there’s nothing else wrong. Of course do the physical exam as well as x-rays to determine what you have, and then we decide on any kind of x-ray exam you may need such as, what I mentioned before, ultrasounds of the kidneys, the heart, MRI of the spinal cord, and then we give you a pretty good assessment of what is going to happen and what the follow up is going to be like so the parents first can be relieved and the number one question the mom and dad want to know is did they do something wrong, and the answer to that is they did not do something wrong. This is not hereditary as far as we know, and it just happens. So nobody did anything wrong.
Host: That’s really important information and I’m so glad that you cleared that up for the listeners. So important. Do you have anything else that you’d like to add about congenital scoliosis.
Dr. Gupta: The only thing I’d like to say is that people confuse congenital as being some of the other diseases and it’s really just a malformation of the vertebral column. It’s a physical malformation. Just imagine if there was a building, and there was a brick that was placed obliquely in the middle of the building and makes the building curved, that’s what you should think about with congenital scoliosis and that’s really what it is, very basically, and you just follow it. If it gets worse, you fix it, and if it doesn’t you leave it alone.
Host: Great information Dr. Gupta, thank you so much. What a great guest and educator you are. Thank you for joining us again. That wraps up this episode of Radio Rounds with St. Louis Children’s Hospital. To consult with a specialist or to learn more about services offered, please call the Children’s Direct Physician Access Line at 1-800-678-HELP and head on over to our website at stlouischildrens.org for more information and to get connected with one of our providers. If you as a provider found this podcast informative, please share on your social media and be sure to check out all the other fascinating podcasts in our library. I’m Melanie Cole.