of Natasha Vergy, who joined us to talk about adolescent scoliosis. Please join me in welcoming them. Hello. My name is Natasha Vergy, and I am one of the neurosurgery nurse practitioners at Riley Hospital for Children in Indianapolis, Indiana. Riley is a children's hospital associated with Indiana University Health, and we're a part of the academic health center that is downtown Indianapolis. I feel privileged to be presenting today along with my physician colleague, Dr. Jay, and I thank you for this opportunity. So, today we're going to be talking about spinal deformities, specifically the topics that we'll cover today include various forms of scoliosis, kyphosis and lordosis, spondylolisis, and spondylolisthesis. So, scoliosis can be defined as an abnormal lateral curvature of the spine measuring greater than 10 degrees. Scoliotic curves can be functional or structural in nature. We'll start by talking about functional scoliosis. In picture A, you see postural scoliosis. This is because of poor posture over time. You'll note that on flexion, the scoliosis disappears. In the second picture, you see an example of leg length discrepancy. So, you see the scoliosis when the patient is standing, but when the patient sits down, again, the scoliosis disappears. And then, the last picture is sciatic scoliosis when the underlying cause such as a prolapsed disc is treated, the scoliosis subsequently disappears. So, structural scoliosis is a bony abnormality. It is fixed in nature, so it's not going to disappear with change of position, and it's liable to progress. So, here you can see an example of structural scoliosis. When performing a physical exam on a patient, you ask them to do the Adam's forward bend test with the hands directly between their feet. This allows the examiner to assess for asymmetry. The presence of a hump is a hallmark sign of scoliosis. So, again, you can see in picture A that structural scoliosis is more obvious on flexion. And then, in the picture B, you see a radiograph of progression of scoliosis over a four-year period. You'll note, or it's also important to note that a more rapid progression occurs during the prepubital growth spurt. Again, here's more pictures. You see that the hump is more obvious on flexion, and that you can see in the x-ray that the curve is to the right. So, you can break down structural scoliosis in three different categories, starting with congenital, idiopathic, and then neuromuscular. So, we'll talk about all of those. So, congenital scoliosis refers to spinal deformity caused by a malformed vertebra. It can happen in two ways. There could be failure of segmentation, which is abnormal fusion of the vertebra, or failure of formation. So, the normal shape of the vertebra is disrupted. The error usually occurs during the first six weeks of embryonic development and is estimated to appear in approximately one in 1,000 births. So, these diagrams break down the different defects, failure of segmentation, and failure of formation. This image shows a partial fusion of the vertebrae at T10, 11, and 12, which has resulted in localized scoliosis. So, this slide shows the degree of progression over, or per year, based on the specific congenital spinal anomaly. You'll note that the unilateral bar with contralateral hemivertebrae progresses most rapidly per year. So, physical exam findings, congenital anomalies of the ribs and chest wall often correspond with congenital anomalies of the vertebra. Overlying skin may show a sign of a spinal dystrophism. So, it's important to look for a hemangioma or a dimple to see what else is going on. If you do notice that, then further imaging is indicated. It's also important to perform a multisystem exam on these patients because sometimes other systems can be affected, urologic, and the cardiac systems most often are affected. There can also be fractures and bone softening associated with congenital scoliosis. Typically, congenital scoliosis is mild, but it can progress. And when it does, it progresses rapidly. So, it's important to monitor these patients closely and get imaging and do an exam in like three to six months period. Every time you do see those patients, you want to look at the imaging you get on that day, compare it to the last time they came in, and then also look at the initial imaging so that you're not missing small but important changes. So, now Dr. Jay will talk about treatment. Thank you for allowing me to be here this morning. My name is Andrew Jay. I'm a pediatric neurosurgeon at Riley, and I work with Natasha. We're going to tag team this presentation. Every time Natasha gives me a stern look, I'll stand up and start talking. So, I'll interject some treatment considerations for these different types of scoliosis. So, for congenital scoliosis, it falls under this big category called early-onset scoliosis. These patients are very difficult to treat because they're skeletally mature. They still have a lot of growth left. So, I'm going to be describing three surgical options, three fusion variants, but I'll just start off by saying that these are all suboptimal for the growing child for obvious reasons. I also like to keep presentations kind of interactive, so I may ask you questions. So, please feel free to ask questions back. So, an in situ fusion is where you do a fusion with no intention to correct the deformity at all. You're just hoping to fuse them in place to prevent progression. It usually involves very long segment fusion, so basically the entire spine. A hemiepiphysiodesis is doing a fusion on one side of the spine, so on the convexity of the spine. So, if you can imagine, if you tether the convexity and leave the concavity alone, over time you're hoping that the spine grows out of that concave side and straightens up by itself. So, what we're taking advantage of is crankshaft deformity, which we'll talk about later on in the presentation as well. And the final way to treat these deformities is with hemivertebrate resection. So, Natasha described congenital scoliosis as a structural abnormality of the spine. So, many times it's a spine that the vertebrae are supposed to look like rectangle building blocks, but in these cases they look like triangular wedges. So, the idea is to drill out that triangular wedge and do a fusion, put in screws and rods and straighten out the spine once you've done that osteotomy. Can someone tell me why doing a fusion in a baby is not a good idea? Back in the room. Yeah, perfect. So, one big reason, we're trying to correct a cosmetic problem, right? And by fusing, you basically keep the body baby size while everything else continues to grow. So, I think we've worsened the problem, right? So, what's the other problem of causing a fusion? So, when you fuse the rib cage, what else happens? Yeah, it's really good. So, problems with mobility afterwards. Perfect. So, that's the big thing. So, when you fuse a rib cage, you cause restrictive lung disease. When you cause restrictive lung disease, you can cause pulmonary hypertension, which then leads to right heart failure, which then leads to death. So, then this, by doing a fusion in an immature child, we can actually cause the child to die. So, it's a very serious thing that we're undertaking. We'll talk about all this later on in the presentation as well. Back to Natasha. Okay, so we're going to move on to idiopathic scoliosis. Idiopathic scoliosis makes up 80% of all cases of scoliosis. It can also be divided into three groups by age, infantile, juvenile, and adolescent. Adolescent is the most common. But we'll start with a quick overview first. So, infantile scoliosis is more common in males, and most times the convexity is towards the left. With juvenile scoliosis, this is pretty uncommon, although it may be underrepresented because it's not detected until they're actually an adolescent. If you do see juvenile scoliosis when the patient is less than 11 years old, it's important to do a more thorough workup because it's so uncommon. And then, lastly, with adolescent scoliosis, it's more common in females, and usually the curve is to the right. So, breaking down infantile scoliosis a little more, it is still rare. It's seen between ages zero and three. Males are more often affected. And this often resolves spontaneously. But when it does progress, it can cause a high incidence of cardiopulmonary dysfunction. So, diagnosis is based on the patient's age, the absence of any other spinal cord abnormality, the location of the curve, which is typically in between the shoulder blades, and then the left-sided apex. So, for prognosis, the severity of the curve kind of depends on how the patient will do. If the curve is equal to or less than 20 degrees, it's typically nonprogressive and will resolve on its own. If it's greater than 30 degrees, these tend to worsen without treatment. So, the degree is determined by identifying the apical vertebrae and measuring the difference in the angle of each rib attaching to the vertebrae. So, now Dr. J will talk about treatment. So, again, infantile idiopathic scoliosis falls under early onset scoliosis, as well as juvenile idiopathic scoliosis, which Natasha will talk about in a few minutes. So, it's much better. So, we said that fusion techniques are not very optimal for this patient population because they continue to grow. You want to keep up with their growth. So, it's much better to treat them with casting and bracing over time until they reach skeletal maturity. But you can imagine that that's very labor intensive, trying to keep up with the child's growth, changing out casts, changing out braces over time. But I'll still make the point that that's better than doing a fusion surgery. Even though I'm a pediatric spine surgeon and love doing surgery, this is not the right thing to do for these patients. In situ fusion has been described as an option. Maybe a more promising technique are growing rod techniques. So, basically fusion-less spine surgery where you try to keep up with a child's growth. And we'll talk about that in a few slides as well. Okay, so now we'll move on to juvenile idiopathic scoliosis. This presents between ages 4 and 10. Again, it's pretty uncommon. If you see this when the patient is younger, so 4 to 6 years of age, the male to female ratio is pretty equal. Whereas as the patient gets older, it looks more like an adolescent picture. So, kind of like with infantile scoliosis, if the degree is more than 30, then it's probably going to progress and be worse without treatment. So, typically you observe patients between or less than 20 degrees. You can think about bracing between 25 and 30 degrees and then surgery options for patients that are more than 30 degrees. So, these are some of the casting and bracing options for these early onset scoliosis patients. So, you'll see here is a cast. It's actually a plaster cast that's put on. It needs to be episodically changed over time. This is a Boston brace and this is a Milwaukee brace with a metal bar to try to keep the deformity from progressing. We talked about in situ fusion and that's a common theme throughout. But again, it's not a great option. We talked about in the congenital scoliosis where you do a fusion on one side, you try to take advantage of crankshaft deformity. But a lot of times when you perform a fusion in a growing child, it's unpredictable what the spinal do because it's a 3D deformity. It can actually worsen the deformity over time by doing a fusion in a skeletally immature child. So, growing rod constructs. So, these are very interesting. Who's heard of VEPTR before? In the back. Do you want? Okay. Perfect. There's probably a lot of overlap between pediatric orthopedics and pediatric neurosurgery, so you probably see some of those patients come over from the pediatric orthopedic side. So VEPTR stands for Vertically Expandable Prosthetic Titanium Rib, and basically was described by Dr. Bob Campbell, an orthopedic surgeon in San Antonio, Texas. He originally described this to treat respiratory insufficiency. So for these children that have very small rib cages, he described a rib-to-rib device to slowly expand the rib cage, the rib space over time to try to not get restrictive lung disease and then heart complications afterwards. People started extrapolating and saying, well, if it works for respiratory insufficiency, why not do rib-to-spine and see if that can treat early-onset scoliosis or put the spine-to-spine or spine-to-ilium. So a bunch of different combinations have been tried, and so it's now one of the devices in the armatarium of a pediatric spine surgeon to treat early-onset scoliosis. And it still doesn't get away from the fact that every six to nine months, the patient needs to go back to the operating room to have these devices expanded so you can keep up with the child's growth. And so VEPTR kind of spun off from the traditional growing rods, which are basically putting in two rods like that, and they're kind of joined by a domino connector. And then every six to nine months, you make a small incision right over the domino connector, and then you just distract the patient over time. And then when it finally grows off of that domino connector, it's time to put in a new rod with greater length so you can keep up with growth. Another type of growing rod construct is called a Shilla procedure. So the Shilla procedure was described by Dr. Richard McCarthy, an orthopedic surgeon in Arkansas. Shilla is actually the name of a hotel in Korea, and his story is that one night he fell asleep and had a dream about how to treat these early onset scoliosis kids because they are a nightmare to treat. And what he came up with is this technique where you do a fusion at the apex, a very limited fusion at the apex of the deformity and at the ends of this construct, you put in screws where the rod lays into the head of the screw but you don't tighten it all the way down so that over time, the rods act like a railroad track to allow the spine to grow so you're directionalizing spine growth. It's a relatively new procedure, not a lot of long-term data is out there but it seems pretty promising and even more recent has been percutaneous magnetic expansion of rods over time. You guys have any questions about these growing rod constructs? Pretty interesting, a lot of research being done. That's a great question. So, I think bracing is pretty effective but we'll talk about what does that mean. How long do you need to wear the brace for? How many hours in a day? And like I said, it's very difficult for kids to stay compliant with the current recommendations which is to be in a brace for 18 or more hours during the day. But we'll go over more of that later on in the presentation. There's a prospective randomized trial that was published in New England Journal of Medicine called the Brace Trial. And it talks exactly about what you're asking. So next we'll talk about adolescent idiopathic scoliosis or AIS. This is scoliosis between the ages of 10 and 18 years of age. And it has an unknown cause. It's typically seen in the female population. The prevalence ranges from 0.5 to 5 per 100 adolescents. The good news is only 5% of patients diagnosed with a curve greater than 10 degrees progress to a curve greater than 30 degrees. Although the cause is unknown, there are ways to determine the risk of curve progression. The scoliosis score is the first and only genetic test to predict the likelihood of curve progression in AIS. It is utilized by a saliva sample. That sample is analyzed and compared to genetic markers that are linked to the risk of curve progression. And then it indicates low, intermediate, or high likelihood. 30% of patients with AIS also have a family history of someone in their family with scoliosis. So AIS is generally not associated with any pain or neurological deficits. The curve usually doesn't put any pressure on other organs such as the heart or lungs. Any patient that has a left-sided curve has a high incidence of intraspinal pathology like a syrinx or a tumor. So that would warrant further evaluation with imaging. So depending on the degree of the curve, the scoliosis may be barely seen, or it could be very significant. When you're performing the physical exam, you can look for shoulder height asymmetry, truncal shift, pelvic tilt, and rib hump deformity. When the patient is viewed from a lateral position, they typically look normal. Other things to do during the physical exam include just observing the patient in their environment, assessing their posture, do a thorough skin examination looking for café au lait spots or any other sign that it might not be adolescent scoliosis. And then check the lower extremities, look for muscle bulk and tone, look for leg length discrepancy, perform a thorough neuro exam, and then make sure to look at the patient when they're sitting, standing, and also walking. So this next slide shows those physical exam findings that we just talked about. In the first picture, you see the scoliosis shoulder height asymmetry. In the second picture, you can see truncal shift and the pelvic tilt. And then in the third picture, you see the rib hump deformity. And then in the final picture, you see that when you're looking at the patient from the side that they look pretty normal. So now Dr. J will talk about imaging and treatment. So the way to evaluate AIS is with full spine x-rays, scoliosis x-rays. You want to get a look not only locally where the deformity is but a global view of what the balance is for the patient. You use the Cobb method of measuring scoliosis. And that basically is just picking out the first disc space that's wedged and the last disc space that's wedged and using that as the limits of your deformity. I'll say that the inter-observer error for this is very high. So what you pick as a rostral level and a caudal level versus what I pick versus what a radiology picks is very different. So I think it's very important as you follow a patient along in clinic, you do these measurements yourself because you know how you did the measurements. Bending x-rays could be helpful. And these are usually used pre-op to get an idea of how flexible or rigid a curve is. And it might help you plan out whether you need to do osteotomies. Osteotomies are cuts in the bone to kind of loosen up the deformity so that you can actually get some correction. When you follow a child that you've initiated brace therapy, it's important to get the same x-rays going forward. So on future x-rays, they should continue to be in the brace so you can see how effective the brace therapy is. So kind of common sense. You also, when you look at these x-rays over time, you want to get an idea of how close to skeletal maturity are they because that tells you if they're about ready to go for surgery or not. In a teenage girl, it's pretty easy. Once they start their menstrual cycles, there's about one or two years of growth remaining. So when they start their menstrual cycles, that's when you can start planning, okay, we can start talking about surgery now and get something on the books. The other way to assess skeletal maturity, the most common way is the Risser sign. So the Risser sign is basically the secondary ossification of the iliac crest. I don't know how well this projects, but there's a secondary ossification center that caps the iliac crest. You can actually see the second layer of calcification over the crest. It usually starts from lateral and goes medial. So here's a cartoon that might be a little clearer. So Risser grade 1 is very lateral. Risser grade 4 to 5 is when it completely covers the iliac crest medially. So Risser grade 4 or 5, that's when you consider a patient skeletally mature. Natasha mentioned some red flags. So these are the red flags. So if you have adolescent idiopathic scoliosis in a young child, so less than 10 years of age, I think that should raise concerns. If it's a male, that should raise concerns. Usually adolescent idiopathic scoliosis happens in teenage girls. That's the norm. If there's a kyphotic deformity associated with it, that's a red flag. If there's pain associated with it, that's a red flag. If it's rapidly progressive, that's a red flag. Or if there's neurologic deficits associated with it, that's a red flag. In those cases, further workup is necessary. That should include an MRI. So does anyone want to answer that question? What neurological…back of the room. Perfect. Very good. What else, right? What's associated with the syrinx? What else could be associated with the syrinx? Awesome. Did you have anything else to add? Okay. Perfect. And spinal cord tumor also, right, that can be associated with scoliosis as well. So, those are the things that we'd be looking out for. This is not meant for you to digest in any way at all. This is just to show you that adolescent idiopathic scoliosis is very complicated, and there's many different classification schemes out there. This happens to be called the Lenke classification. The way you can think about it is pretty straightforward, though. I think if you just think about it in the Cobb angle, that should give you some clarity. So, less than 10 degrees, you don't even call it scoliosis. Between 10 and 20 to 25 degrees, it's a mild case of scoliosis. In those cases, no therapy needs to be initiated. You just continue to follow them closely with serial x-rays. Between 20 to 25 degrees and 40 degrees, that's moderate scoliosis. That's when brace therapy should be initiated. And then beyond 40 to 45 degrees, it's severe scoliosis, and that's when surgery is indicated. So, a little more straightforward way of thinking about scoliosis. So, we were talking about bracing before. So, brace therapy between 20 to 25 degrees and 40 degrees can be very effective. You can actually treat a child with adolescent idiopathic scoliosis with a brace alone, but it requires being compliant with brace wear. So, it used to be that we recommended braces be worn 23 hours a day, and they would basically take the brace off for one hour just to shower, just to clean underneath. But they would sleep in the brace, they would go to school in the brace, everything was in the brace. Then people thought that that was cruel and then went to nocturnal bracing only. So, basically just during the 12 hours at home or at night while they were sleeping, but you could have the brace off when you're at school and having other social interactions. People saw less efficacy with that sort of approach. So, this is when the brace trial came out, and it was published in New England Journal Prospective Randomized Study. The investigators used 18 hours as kind of a subjective cutoff. So, they looked at children who wore the brace less than 18 hours and those that wore it greater than 18 hours. And it was much more efficacious in the kids that had the brace wear for more than 18 hours. So, now the recommendations have swung back to saying you try to wear it at least 18 hours a day. And if we can go back to 23 hours a day, probably the results would be even better. This is the typical type of surgery that we would do today. Usually it's a posterior approach. Usually you try to get some sort of fixation at every level to encompass that deformity, to get control of that deformity. There's also anterior approaches, which people have advocated. It's a much shorter segment of fusion you can see, and you can get just a nice cosmetic result. But the problem with anterior approaches are the approach morbidity. So, to go through someone's chest to do the surgery or through the retroperitoneal space, it's pretty morbid. So, I think the mainstay for adolescent idiopathic scoliosis surgical treatment is posterior. And this is just a contrast. This is what our specialty in spine was doing a long, long time ago when Dr. Paul Harrington first described instrumentation. I don't know if anyone was around during that and still see Harrington rods. Right, exactly. So, pretty limited. So, you could see that there were only two points of fixation, rostral and caudal. Usually only one rod on the concave side, and basically used distraction to try to straighten out the spine as much as possible. But you corrected things in the coronal plane, and you completely ignored the sagittal plane. So, when you looked at them on a lateral X-ray, they had a very flat back, which caused problems later on in life, too. So, now we'll talk about neuromuscular scoliosis. There are two forms of neuromuscular scoliosis. There's neuropathic that results from abnormal nervous innervation to the axial musculature, and then the myopathic type, which is a primary muscle disorder affecting the musculature adjacent to the spine. So, this box shows the conditions associated with both neuropathic and myopathic. So, with neuropathic, conditions include poliomyelitis, cerebral palsy, and myelomeningocele. Cerebral palsy is the most common condition associated with neuromuscular scoliosis. And then an example of myopathic is muscular dystrophies. So, as neurosurgery providers, we often care for people with myelomeningocele. Patients with myelomeningocele can have various spinal deformities, as listed on this slide. Spinal deformities is the most common occurring orthopedic problem for people with myelomeningocele. Scoliosis accounts for more than 50% of the spinal anomalies that these patients have, and it is described as a long, C-shaped curve. So, this table demonstrates the prevalence of each deformity in patients with myelomeningocele. And you can see that scoliosis, kyphosis, and lordosis are often diagnosed when they're older, 10 years of age or older. So, these curves can arise at any level and are often associated with vertebral body abnormalities, such as defects of segmentation and formation, as well as mixed defects, making this patient population also very difficult to treat. This table shows the incidence of scoliosis by neurological level and bony defect. And you'll see that above T12, the incidence is much higher. So, patients with myelomeningocele can also suffer from kyphosis. A kyphosis deformity can be defined as a roundback deformity, a sharp forward angulation in the thoracolumbar region. Another condition that is associated with neuromuscular scoliosis is neurofibromatosis. The nondysplastic type has more of an idiopathic scoliosis picture, whereas the dysplastic type can cause severe kyphosis and scoliosis. So, here is some imaging. In the first picture and picture A, you see a congenital curve. The second picture is a paralytic non or a long C-shaped curve. This one's actually from polio, following polio. And then the C, picture C is a patient with neurofibromatosis who has a sharp curve, which is not uncommon for this population. And you can also see the cath ALA spots on the patient's back. So, for patients with neuromuscular deformities, you're not just assessing the curve, you're looking at the clinical indications for surgery. Decreased pulmonary function, difficulty walking in a patient that previously was ambulating, difficulty sitting in a patient who's wheelchair bound, a patient who typically cats independently that can no longer do that, a patient with intractable back pain or a patient who now has pressure ulcers because of their curves. Those are all indications for surgical treatment. So, at Riley, once a patient with neuromuscular scoliosis is identified as a candidate for surgery, they are directed to the preoperative clinic where they'll obtain official clearance. So, the preop clinic is made up of a multidisciplinary team, including neurosurgery or orthopedics, developmental pediatrics, anesthesia, pulmonology, physical therapy, and care management. This is a great clinic, and it's often utilized for patients who have questions. Prior to surgery, they get a really big work-up from developmental pediatrics, making sure they're nutritionally ready for surgery, from a respiratory function that they're doing okay and they're at their baseline, anesthesia meets the patient. So, it's really nice for families to be able to meet all of these people prior to their big surgery. And they even meet the physical therapist and case management person that are on the floor postoperatively with the patient. So, the physical therapist oftentimes works with the patient and family on that clinic day and they'll go over techniques on how they're going to need to turn and transfer from after their surgery, because it's obviously different. So, treatment options for patients with neuromuscular scoliosis. Observation sometimes is the right choice. It could be appropriate if the risks of the surgery outweigh the benefits. Bracing isn't the best option because of their body habitus and it's just a very difficult population to brace. And then surgery is definitely an option that Dr. J will talk about. So, I'll just stress that, you know, actually this neuromuscular scoliosis patient population is probably the area where pediatric neurosurgeons can have the biggest impact. I think, you know, for the adolescent idiopathic scoliosis patients, it's really difficult to get referral patterns to change from orthopedics in that group. I think, you know, AIS represents bread and butter for pediatric orthopedics. But for the neuromuscular patient population, if you just think about it, you know, as a pediatric provider, these are the patients that we see every day. I mean, these are the kids with cerebral palsy, spina bifida with hydrocephalus, tethered cord problems, baclofen problems. So, these are patients that we run into every day and why not us as far as taking care of the scoliosis. So, as far as our surgical treatment, there's many ways to fixate the spine. There's the most common pedicle screws, there are hooks, there's wiring. What I'll show you is another way to fixate the spine using sublaminar polyester bands. So, here we're just creating space underneath the lamina by resecting ligamentum flabum. Once that's exposed, we have the epidural space available to us and we carefully pass this band underneath the lamina from caudal to rostral. We're pulling the band through very carefully and we do this at every level at the apex. And we tie this into straight rods and then there's a tensioner for the polyester bands where we basically translate the spine over to the shape of the rods, which is straight. So, we're basically pulling the spine straight. I think that this is technically pretty straightforward. I mean, you don't need to be a great technical surgeon to do this, but I'll also say that the risk is very high in doing this because every pass of the band is right above the spinal cord. So, the risk of spinal cord injury is much higher than pedicle screws. Pedicle screws, you're trying to obviously avoid the spinal canal and the spinal cord. Here, every pass, you're going into the spinal canal right on top of the spinal cord. So, my heart stops every few seconds when we're passing these bands for sure. So, next we'll talk about kyphosis. In a patient with kyphosis, the upper part of the spine curves more than normal causing a rounded or a hunchback appearance. So, kyphosis can be broken down by age group. Within each age group, you'll see different causes. So, these images show the clinical appearance and radiographs of someone with kyphosis. Here's an example of a patient with childhood kyphosis caused by tuberculosis. This can occur as an abscess in the epidural space creating pressure on the spinal cord. And it would typically present with back pain and have a gibbous formation. Another form of childhood kyphosis is congenital. There are two basic types. Type 1 is failure of formation and type 2 is failure of segmentation. There also can be a combination of both. So, here are pictures of type 1, failure of formation and then type 2, failure of segmentation. So, type 1 typically progresses more rapidly with the skeleton in the first year of life growing, so progression is much more likely. With type 2, that has a slower rate of progression and it may not be visible until the patient is older, like in adolescence. So, in this picture, you see a child that has an abrupt congenital kyphosis located in thoracic spine. The MRI shows a posteriorly located hemivertebrae which is causing impingement on the spinal cord. So, just like with congenital scoliosis, with congenital kyphosis, it's important to look for other defects in other organ systems. Dr. J will now discuss treatment. So, I think there's a lot of similarities between kyphosis and scoliosis. Kyphosis being a coronal deformity and kyphosis being a sagittal deformity. So a lot of the treatment options are very much similar. So here, again, we talk about doing nothing and just observing for mild cases versus thinking about surgery. And when you think about surgery, a lot of times these deformities are rigid, so you need to incorporate osteotomies or cutting the bone to loosen the deformity so you can get any degree of correction. And a lot of these osteotomies are described, Smith-Peterson-Ponte, PSO, pedicle subtraction osteotomy and vertebral columnar section involves cutting a wedge into the bone so that you can bring the spine back into a more straight position. So one type of adolescent scoliosis condition is called Schurman's. This is caused by an increased growth of muscular activity. The vertebrae in the thoracic spine may give slightly and become more wedge-shaped, causing an exaggerated kyphosis. So this is typically seen in males, and it usually presents between ages 12 and 16. When you're looking at the patient from the side, having the patient bend forward, you'll see a sharp angular kyphosis. So the deformity with Schurman's is not correctable with changes in posture, unlike postural kyphosis. So sometimes this is underdiagnosed because parents will chalk up the kyphosis to posture problems. So here's a list of differential diagnosis for Schurman's. So complications with Schurman's is typically rare. Neurologic complications could be spastic paresis in the limb or spinal cord compression. Cardiopulmonary dysfunction is seen in severe thoracic deformity. So now Dr. Jay will talk about imaging and treatment. Great. So Schurman's kyphosis is the adolescent idiopathic scoliosis equivalent in the kyphosis world. So the workup is going to be similar. So full spine scoliosis x-rays to get a good view on what global sagittal balance is. Use a method of Cobb to measure the severity of the deformity. It typically involves a thoracic spine. So the thoracic kyphosis is normally between 20 and 40 degrees. So anything beyond 40 degrees is considered abnormal. This is the method of Cobb, just using it from the sagittal plane. Here's where you see a lot of wedge-shaped vertebrae. Also the end plates are very ratty, like they're under a lot of biomechanical stress. Here's a good example of a pretty significant case of Schurman's kyphosis, so leading to a hunchback deformity. And again, this is showing the wedge-shaped vertebrae. So a lot of times, unlike adolescent idiopathic scoliosis, Schurman's kyphosis is associated with pain. So usually you get pain, not where the deformity is, but in the low back. And the other common characteristic that you see in these kids are they're very skinny. Can anyone think of why that happens? Why do you get low back pain instead of pain right over the deformity? And why are they very skinny? Anyone want to take a guess? That's a good guess. The main reason is because of the kyphosis, their posture is pitched forward, so their head is pitched forward. And what the body wants to do is bring the head over the pelvis again. So your lower back is now working overtime to pull your head back so that it lines up over the pelvis. So that's why you get the low back pain. And because the muscles, the paraspinous muscles, are working overtime, you're in a very high metabolic state. So these kids are actually very skinny when you see them. Bracing can be effective. It's really just used to slow the progression of the kyphosis over time. Surgery is indicated for cases over 85 degrees. It used to be a little more lenient, but we give a little more time for conservative treatments nowadays. And then incorporating osteotomies again if surgery is contemplated. And this is a very typical case of Sherman's kyphosis. So not only the deformity, draw your attention to that, but also just how skinny this kid is. So that's pretty classic. So here's a list of causes of kyphosis in the elderly population, degenerative osteoporosis, postmenopausal, and senile. So here's a very severe case of kyphosis. In a case severe as this, some preexisting deformity or longstanding metabolic bone disease was probably present. But this is a really good example to see how serious and how dysmorphic kyphosis can be. Next we'll talk about spondylolisis, which is a break in the vertebrae, typically in the region of the pars interarticularis. This is typically caused by stress or fatigue fracture. There are five categories, including dysplastic, isthmic, degenerative, traumatic, and pathologic. Most commonly in the pediatric population, we see dysplastic and isthmic. Dysplastic arise in congenital abnormalities that are associated with other osseous abnormalities, including maloriented or hypoplastic facet joints. With isthmic, there is a deficiency in the pars interarticularis without associated facet joint or other osseous abnormalities. It is usually associated with repeated traumatic stress to the spine. So I'm going to turn it over to Dr. Jay to conclude our presentation. So just a few more slides left, and Natasha and I know that we are standing between you and lunch. So we'll make sure we finish on time. So spondylolisis, as Natasha was saying, is a defect in the pars interarticularis. Pars, if you want to imagine, they occur there on each side of the spine. They're like shingles that hold the spine together. So the top pars hanging on to the bottom pars provides stability. So when you get a break in that pars, you can have instability. A lot of times, this is micro instability. It might not even show up on imaging, on flexion extension imaging. But the micro instability is enough to cause pain. Classically, you describe this finding on oblique x-rays. So the Scotty Dog is this. So just use your imagination. So this is the nose of the Scottish Terrier. This is the ears. Here's the body. Here's the front legs, back legs, and a very short tail. So when you get a break in the neck of the Scotty Dog, which is very sad, that is a pars defect or spondylolisis. So spondylolisis is just one reason for spondylolisthesis. And it typically presents with back pain. There could be a radiculopathy if the nerve that is underneath that pars is crunched. So it's like a guillotine effect causing leg pain. Cauda equina syndrome, which is a neurosurgical emergency, is very uncommon and almost unheard of. Typically, high grade spondylolisthesis, so grade 2 or above, that's an absolute indication for surgery. And the classification, I skipped over very quickly, but it's the Myrdang classification, which basically just measures what percentage of slippage you have. So between 0% and 25% is grade 1, between 25% and 50% is grade 2, 50% and 75% is grade 3, 75% and 100% is grade 4, and a complete dislocation of the spine is called spondyloptosis. For grade 1 spondylolisthesis, the treatment algorithm is conservative treatment. So you try to treat these patients through for about three months. It may include brace therapy. It should include physical therapy. What you're hoping for is a stable nonunion. So basically to have, even if you can't get bone to grow across that gap, to at least get strong scar tissue to hold the fragments of bone together. And a lot of times that relieves symptoms. And at the end of the three months, you try and let the patient go back to normal activities to see if they tolerate it. If not, then surgery becomes a treatment of last resort. And surgery is usually posterior. It usually involves a single level, just one level above, one level below, just across that PARS defect. There's other more exotic ways to treat spondylolisthesis, including motion sparing methods, but they're not as popular as just the straightforward single-level fusion. Some of the complications in general that you see with this complex spine surgery, spinal cord injury, blood loss, and we try to, a lot of institutions should have blood conservation, blood salvage protocols, including the use of TXA and CellSaver. Another more common, thankfully those aren't very common, more common complication is infection. So I would encourage you, so this is a whole other talk about quality improvement in pediatric neurosurgery, maybe neurosurgery in general, but I would encourage you if you guys are looking for a QI project to do, a very easy one is to put together a spine infection prevention protocol. And basically, you know, if you ask me what's the most important step in this protocol that prevents infection, I'll tell you I don't know. The most important thing is just to do things the same way. And that's a very basic QI principle, that if you standardize care, outcomes will improve, and by the way, costs will decrease as well. So if you're able to decrease the number of infections, you decrease readmissions, re-operations, so on and so forth. So that'd be a real cost savings. The challenge is to get a bunch of surgeons who do things different ways to all agree to do things in one way. That's probably the biggest challenge in putting together something like this. At our institution, we decreased an overall infection rate between orthopedics and neurosurgery of about 78%, 7% to 8% to less than 2%. So that's pretty dramatic. I think this is our last slide. So on behalf of Natasha and I, thank you very much for paying attention and hanging in there just before lunch. If you have any other questions, please feel free to ask us or approach us afterwards. We'll hang around for a few minutes. Thank you.

spinal deformities

scoliosis

kyphosis

spondylolysis

spondylolisthesis

diagnostic methods

treatment options

adolescent spinal deformities

management