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How to approach Scoliosis in the FRCS- Mr Lutchman, Mr Steele and Mr Swamy; Congenital and Infantile Scoliosis- Mr Swamy

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Summary

This on-demand teaching session is designed for medical professionals and it explores the history, examination and diagnosis of scoliosis. Participants will understand the need to ask pertinent questions to make an accurate diagnosis, learn to recognize red flags, and acquire the skills to follow a systematic examination. Participants are also encouraged to attend regional scoliosis clinics or their own clinics to gain additional hands-on experience.

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Description

How to approach Scoliosis in the FRCS- Mr Lutchman, Mr Steele and Mr Swamy

Congenital and Infantile Scoliosis- Mr Swamy

Juvenile and Adolescent - Mr Cook

Spinal cord monitoring - Mrs Grovier - Neurophysiologist

Degenerative Scoliosis- Mr Marjoram

Learning objectives

Learning Objectives:

  1. Identify and explain key questions to ask in a medical history for a patient with scoliosis.
  2. Describe the difference between congenital and non-congenital scoliosis and evaluate which is present in a given patient.
  3. Analyze a patient’s gait and posture to note asymmetry and elevate/dropped shoulder and to help clarify the diagnosis of scoliosis.
  4. Identify red flags from examinations indicating the need for further diagnosis or treatment.
  5. Identify the mode of inheritance for idiopathic scoliosis and explain the concept of variable penetrance.
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Computer generated transcript

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The following transcript was generated automatically from the content and has not been checked or corrected manually.

So Daniel, let me, um, share my screen here. And, um, what I want you to do is, um, look at this picture for me. I try and get that up. Can you see, um, a photograph? Yeah. Ok. So da um, the common scenario is I've been a, I've been asked to talk about this from a, a sort of FRC or perspective. Um, and, um, how, what ST are you, Daniel? ST four. So, is there anybody who's ST, um, I don't know you all, unfortunately, but anybody coming up to the exam, anybody who's ST seven or eight or something take on somebody, Dian Well, Na Bill saying he's happy to be vivid in the, um, yeah, I'm happy to, um, have a go with that. Ok. Nail. So you come on, um, please to meet you. Where are you working now, Bill? Um, I'm working in Royal Lancaster at the moment. Um, Mo Bay University Hospitals. Ok. And have you done any spine surgery? I've done six months in East. Sorry. Yes, I did. Ok. And did you do any deformity there? Um, unfortunately not, it's just basic lumbar decompression. That's right. So, so that's perfect. When you, when have you got the exam? Uh next November like this November as in um in Cardiff. Yes, I'll see you there. So, um so the the scenario um for the Fr CS is, is um either an intermediate case or a short case, um scoliosis does some sometimes come up in adult path but, but they like to bring it for a clinical case. So if I present you in the intermediate case in the exam with er this young man. Um and I said this chap is 15 years old and he's been referred by his GP to, to the orthopedic clinic with a swelling on the left side of his back. Um Tell me the pertinent points in the uh to start with. Um what do you think is the likely diagnosis? Um in this case, he's referred with a swelling on the left side of his back, given the age and the um the presentation I'm thinking of adolescent kind of idiopathic scoliosis. But on my history, I would like to know the um onset of noticing the deformity, the rate of progression. Yeah. So the, the child's um mother noticed it in the summer when they were on holiday at the beach and she noticed an asymmetry of the spine and, and this apparent swelling on the back. So what else you would like? I would like to ask about kind of red flag. So I want to make sure that it's a painless curve. So usually idiopathic um, scoliosis is painless and um, um, kind of, if it's painful, sometimes it's a worrying sign to dig deeper about the pathology of the scoliosis. I would like to know the, the effect on function, participation in sports and school. Um, and I would like to know um, also, um, kind of their expectations and what you are expecting from today's consultation, any respiratory um, problems, any effect on walking distance or running distance um as well. That's, that's not a bad answer. Um But, but it could be much better. It, it, I think you need to be a bit more systematic. So, so let's start with the presenting complaint again. You quite rightly asked, when was it observed? Has it who observes it and has it changed in appearance? Um The next thing you said is you want to know if it's painful. Um So what are the other features that you would ask about that would mean that this is not an idiopathic scoliosis. Have you any pain, what else? Pain, uh blood or bowel problems? Um Any associated weakness. Um um I'd like to ask about, I'd like to exclude kind of neurofibromatosis dysraphism as well. So, any kind of skin or cutaneous features that we have noticed? Um um um And mhm Yeah. So analyzing the uh symptoms, looking for associated uh associating symptoms and looking for kind of red flags as well. So, persistent pain, night pain. Um Yeah. And we also ask things like, have you got any numbness, tingling, pins and needles in your arms or legs? Any neurological symptoms? You quite rightly asked about nocturnal pain and activities on daily living. You asked about progression. What else is crucial if we're thinking that this is idiopathic scoliosis? Is there a genetic element to it or not? Um, family history? Yeah. So I would like to ask about family history, whether there is anybody in the family who has sustained a similar condition? Um, scoliosis could be congenital as well as syndromic. So I'd like to ask about other system involvement if you had any previous visits to pediatrics or um, so this is an otherwise healthy child. It's an otherwise healthy child who's, um, who has no constitutional symptoms. There is a family history, this, um, child's mother had scoliosis and has a maternal aunt who also had scoliosis. It's not associated with any pain, although there's some minor discomfort, it doesn't affect his activities of daily living. And um, and there, you know, there are no red flag symptoms at all in a girl. What specific questions do you want to ask a girl? Time of men, time of menarche? And I would like to confirm that as well on a kind of xrays on the um kind of index. Yeah. Don't jump around. Yeah, we're talking about history. So, so when they ask you something, answer the question. So, um you'd answer by saying in a girl, I'd be specifically interested in the menarche. Um The importance of this is the menarche coincides with the period of rapid growth and therefore the curve is likely to progress during this time. Yeah. Um We, because we'll come and talk about radiographic things, but to jump from a history to an x-ray, just suggest that I sort of kind of disorganized way of thinking about it, you know. So, um um so we've said that we, we mentioned that there's um there might be a family history. What's the mode of inheritance of idiopathic scoliosis? Um I'm not sure, I'm not sure about that. Ok. So the answer is, it's a polygenic mode of inheritance and it demonstrates variable penetrance. What that means is we're not really sure. Um It's polygenic, which means that no one gene association has been identified, but several different types of gene abnormalities have been identified. And so it's polygenic and it shows variable penetrance, which means that although you may have the same genetic makeup, um it doesn't necessarily mean that those genes are switched on. So some patients, for example, we often see kids who are identical twins and one twin may be severely afflicted with a and the other one might have very minor scoliosis. So that means that they probably have variable penetrance. Do you see what I mean? Yeah. Yeah. So that's the key point in the history and you mentioned things like neurofibromatosis, you mentioned family history, how it affects their function. Um Always mention the birth history. Ok. When you have a pediatric um case in the exam also always say I'd like to know whether or not this was a straightforward uh pregnancy with any difficulties or Perin perinatal problems because um I want to exclude uh that this might be a neuromuscular problem. But um in answer to your question, this this child was born after a normal pregnancy and delivery um was is perfectly fit and well has no medical problems. There is a positive family history and there are no neurological symptoms. Um What are the key points ex on examination? Then you've talked about history. What are we looking for when we examine this job? Um on examination, I'm inspecting and looking for kind of um sagittal imbalance and coronal imbalance in looking from the back and from the side. I would like to kind of confirm the um structure scoliosis, the rotation element by asking him to do a forward Adams test. So on bending forward, I would notice a um kind of a rib prominence uh that would confirm kind of um a a structural element of the curve. Yeah. Um Then I would like to assess his gait um as well. Um shoulder levels um kind of um um um look, look at his pelvis as well, pelvis level. Um in um then I would proceed for doing a kind of um neurological examination particularly looking for uh sorry, on an inspection. I also would like to check the axilla for any freckling, just excluding any dysraphism, any kind of sinister cause of scoliosis, like um uh kind of heavy patches or a cafe ole spots. Um Then I'll proceed to do a neurological examination, um specifically looking for the abdominal reflexes. Um You're doing, let's take a step back here and you, you sort of said you're going to look for the patient's Coronal and Sagittal balance. Tell me what you look for when you assess the the coronal balance, what are you looking at? So in, in Coronal, in Coronal, I would like to assess the site of the curve, the um the location of the curve, the magnitude of the curve. Um I would like to ask the patient to, so tell me what that is in this patient. Tell me what uh for this patient, tell me those things. So um this is the um left sided um kind of abi to the left kind of thoracolumbar curve. Agree. Yeah. Um yeah, the shoulder level is slightly dropped on the left side. Um The pelvis looks, looks, looks leveled to me. Um I would like to ask him to do kind of lateral bending movement to see if that curve gets, gets better. Um which, which basically give me an element that, that the patient might be compensating with a nonstructural um kind of curve. OK. So I think again, I think you have all the points. I think you speak fairly knowledgeably about, you know, what you're seeing. But again, I think you would benefit from having a system. Yeah. And, um, I think the, the way I would, I'd encourage all of you to every Friday morning, every time we teach, we say this every Friday morning at the Norfolk and Norwich, we have a scoliosis clinic. I would strongly encourage you if you've not been to come to our clinic before the exam or to go to your regional scoliosis hospital, call them in advance and say I'd like to come and go to one clinic. The system for looking at this is like you say, you look at the child from the front and you look from the back, you watch the child walk and you make a comment on their gait, you get the child to turn away from you. And what I would say is I my system is to start from the top and work to the bottom. So what you would say is looking at the child's head and their position relative to the Pelvis. A plum line dropped from the child's head falls to the left of the midline. So this child has truncal shift to the left. Agree. Yeah, the right shoulder appears elevated compared to the left. Agreed. Yeah, there's a scapular asymmetry and there's a fullness of the loin on the left side with its suggestion of a left-sided loin hump. Looking down, you can see the law increases, the law increase on the right is more marked compared to the law increase on the left. Yeah, I'm going to put my hands on the child's Iliac crest and I feel that the pelvis is level. I will now look at the child's popliteal fossa and they look at the same level. So there's no gross leg length discrepancy. There is no evidence of abnormal cutaneous stigma to overlying the skin specifically, no lumbar nevi or hairy pigmented patches. You see what I mean? I've said the same thing that you've said, but I've approached it in a systematic way and that means that when I'm terrified of the examiner and I'm scared in the exam, I won't forget anything because I'll, I'll follow my system. Uh I'll be nervous and I'll be scared but I'll follow my system and I'll get everything right. You see what I mean? Yeah, noted. Yeah, that applies for your hip. That applies for your knee, for your Colman block test. How you examine the subtalar joint? You go into autopilot because you have a system, what you don't want to do is be thinking in the exam. I know that sounds somewhat paradoxical, but when it comes to clinical assessment, it means you need to go to autopilot and you see what you see, then you look in the axilla, you look at the front of the chest and you're quite right to do a neurological examination. But by then the examiner would have stopped you because most of these kids have, what sort of a neurological abnormality? Sorry. W what's the question? What neurological abnormality are you likely to find in this child? None. None. Indeed. So, there's nothing to find. So, so in the exam, we tend to focus on positive findings. So we want you to look for the things that you can see because the neurological examination is going to be normal, isn't it? Yeah, more than likely. So, um I think you, I think you have the knowledge, I think, I think you have the potential to do well and pass this. But I do think what I would strongly encourage is to have a system for your history, have a system for the exam and then we can talk about, you know, if we get as far as talking about investigations and things you're doing very well. Um This kid had a, this, this x-ray. Um w what does this x-ray show? Uh So this is a uh pa hold spine um um radiograph um showing a scoliotic curve at the lumbar thoracic um region uh with apex to the uh left. Um There is a compensatory kind of thoracic curve as well. Uh approximately um apex to the right. Um I believe the thro lumbar uh could have a um a co angle of about 50 to 60 the proximal curve may have a less degree of that look at around 30 40. Yeah, I, I think, I think that's very good. I think you certainly would pass on, on your comments. There's an obvious scoliosis and we're looking at the spine from behind. So there's like you said, the Thoracolumbar scoliosis is convex to the left and the um, proximal curve is convex to the right. Um You can see the cardiac shadow. So we know we're looking at the spine from behind. Um And, and what I would say, you know, to embellish things is again, um that's the obvious abnormality. But the other thing I'm looking on the xrays, like you commented before, I'm looking at the child's Risa sign, which looks to be Risa. How much, what do you think Risa? Do you think it is beyond five is completely, it's hard to tell, isn't it? It's either one or five. I think this, this child was skele Teme. So I think you're probably right. It's, you know, almost five and you look at the and you can look at the clavicula, you can see the clavicles are level level. Yeah, you can see that the pelvis looks roughly level. There's a chest wall asymmetry, but I think your comments about the Cova are, are well made. Uh So, um yeah, if you get the time come down to our clinic and um we'll be happy to show you around. Um what I wanted to show you the last thing before we get Mr Swami on hopefully, is this the reason you get a rib hump is because the spine is twisted, isn't it? So, in idiopathic scoliosis, uh, the spine is rotated. Um, and you can see here on this picture that the, the, the convex pedicle is of a normal caliber, but the concave cave pedicle is often high plastic and so the spine is twisted, the rib hump, deformity becomes evident, er, and the spine is rotated, which is why you get a rib or loin hunt deformity. Um, so I'm gonna stop sharing. Um, really, er, the key is to get into your clinics, er, see as much as you can. Um, and like I said, we're very happy to entertain any of you who want to come to our clinic. It's a very common thing in the exam. You need to know the history, have an eye system, the examination, eye system and then talk about the xrays. But nave, I thought it was good. Thought it was good but it, it shows that, um, you've done a lot of reading but it also shows that you're not speaking from first hand experience. Yeah, definitely. I'm just trying to put the Bozz, the Bozz pieces together. Yeah. So you've got the knowledge so consolidated, you say you're up north, where is your nearest scoliosis unit? Nabil. Um, I believe this is going to be Sheffield or Birmingham. They Sheffield guys are phenomenal. Mr Well, and Mr Cole, lovely guys. You call them up and say I've got the exam coming up. Um, could I please come into one of your clinics? The problem is if you're not local, are you on that train program? I not, I'm not training unfortunately. But, um, you know, I don't know where you live but if you want, if you want to um, drop us an email or speak to seb, we're very happy to have people come into our clinics. Um It's the only way to learn, um you have the knowledge but to put that together and to convey it um requires a little bit of practice, but, but you did very well. Uh S so Mr Lum, we got Mr Swamis uh ready now. So, um I'm gonna share the slides of mine and er Mr Swami will give us presentation. Um One moment. Can you hear me, sir? Uh I can hear you. Yeah, I'm just going to try and load up your, well, your little slides. Now, one moment it's slightly different way we are doing teaching today. Um Mister Steele has asked me to do talk on about congenital scoliosis first and um I believe later, Mister Cooke and Mister Margin will be talking a lot more about idiopathic and um degenerative scoliosis as well. So, uh I won't be quite, I won't be exhausted. Yeah. Ok. So, uh just, just to pick on nave again. Sorry, navel. Uh You're the only name that I can remember. My pleasure, my pleasure. I'm happy to be. So, what do you think? What is scoliosis? So, scoliosis is a uh three dimensional deformity that includes rotational as well as um key four kind of key. Um It could be congenital, it could be idiopathic, it could be neuromuscular. So, stop that. So, uh so what you say it could be kyphoscoliosis as well. Is that a, is that a complete definition? Is that a proper definition of scoliosis? Um I would say by definition of scoliosis is a, a cob angle more than 10 on, on a, on a pa kind of eric radiograph. And why do you use the word pa radiograph? Uh Because when we talk about scoliosis, this is an old concept, isn't it? We are, are you going to when what scenarios and investigations do you use pa and lateral or a P? Is it a two dimensional pictures that you use the word for or three dimensional pictures? Yeah, it's a two dimensional xrays are two dimensional. He he by definition scoliosis, a two dimensional problem or a three dimensional problem. It's three dimensional problem. OK. So going back to that uh could do you wanna redefine your the definition? So it's a deformity that affects the spine in both sagittal coronal and axial planes. OK. Uh I'll, I'll go back to you again. Uh If it, what are the normal curves as you see in the Saal plane and the sagittal plane, I see a lumbar curve and a thoracic um a lumbar Lordotic curve which is about 10 to 40 degrees and aortic thoracic curve which is around less than 40. It's very important going back to work to reflect, not just for you, for all everyone of you to what Mr Lachman was highlighting on uh you just, you should try to learn the habit of just answering the question. Uh Try not to keep talking and adding more. Uh uh you know. Uh sure. So, so basically, uh just going back again, uh it said, could you go to the next slide, please? As I said, I should have uh uh uh we should, we could have done that to listen to the first. But uh it, it's very important when we talk about uh any deformities or anything in particular to understand what we really are talking about. Uh That's why uh even in congenital, I just put this first slide. So when we describe any three dimensional objects, ie human beings, we always talk about three dimensional plates. So we talk about the coronal plane where you're looking at something from the front, you talk about the sagittal plane where you're looking at it from the side ie a two dimensional equivalent of a lateral. OK. And we talk about axial or horizontal planes where we cut right in the middle and look from the top. So by definition, if you don't get yourself muddled with kyphosis, lordosis and everything. So by definition, uh scoliosis is a coronal plane deformity. OK. So that means you're looking from the front to the back, we get the pa x-ray as against an A P because of the soft tissues. Uh But, but by definition, uh deformity of the coronal plane of, as you said, more than 10 degrees is scoliosis. So keep your definition simple. Uh but also uh you need to be very specific when we ask about definitions. OK. And of course, remember the rotational problems, OK. But the the curvatures that you'll see from the side, which is the sagittal plane are the kyphosis and the lordosis. And we know that we have normal kyphosis and normal lordosis. So we do not call them per s has deformities. We either refer to them as excessive or loss of. OK. OK. So next slide, please. So again, uh as you mentioned, there are different reasons why we develop scoliosis. And m was talking briefly about uh the uh pathogenesis of idiopathic scoliosis, but we also have scoliosis due to a congenital problem, uh or uh neuromuscular problems or degenerative problems. Uh Next slide please. So we'll only focus on congenital scoliosis. Uh You will have a lot of teaching on other forms of scoliosis later. So, congenital scoliosis basically represents uh a spinal malformation due to the defects of formation segmentation or mixed ones. So, essentially, you have a structural problem within the skeleton. That is the reason why you're developing scoliosis. And it, it generally is characterized by longitudinal or a rotational imbalance. When I say longitudinal, I'm talking about coronal plane uh problems and rotational imbalance, as we mentioned is usually three dimensional, but it is predominantly in the axial plane. OK. Uh Secondary to congenital malformations can uh it can be because of problems within the vertebra or it could be because of problems within the pelvis itself. OK. Uh Next slide, please. So uh again, uh going back to what you were mentioning, our understanding of scoliosis has changed quite a lot. Uh since the advent of three dimensional imaging IE CT scans and MRI scans because historically, all most of our understanding of the human body was two dimensional as well. So init the initial classification of uh congenital uh we've lost your slight. Uh What the next one? Yeah, I is that the right slide, Mr Swan. Uh No, the one there was in one in between. Have you not got it? Oh yeah, that's one. Yeah. So uh the initial uh classification of congenital scoliosis uh was done by RB. And uh he based these classifications uh based on x-rays but uh with the 3D uh imaging do CT scan, uh we have uh a much more knowledge of uh the three dimensional anatomy and now we have a greater understanding of the embryology as well and the pathogenesis as well of congenital sclerosis. Next slide, please. Uh So as we mentioned, scoliosis, uh is an imbalance in uh results an imbalance in the longitudinal growth uh due to defects in the uh formation segmentation. And it can be a combination of things. I've just taken this slide directly from uh uh Miller because this is the, that's the book we all read. Uh We'll reflect on that a little bit later as well. Next slide, please. So when we talk about longitudinal imbalance, uh as I mentioned, uh we talk about deviation in the coronal plane. Uh So the, the vertebral body with instead of being a short cylinder can be trapezoidal or we can have what we call a hemi and the hemi vertebrae itself uh can be fully segmented or it can be partially segmented or it may be unsegmented. And what happens is if it, if I always talk about uh I tell patients when I see them as when you have, when we, we, we have, we all have Children, but some of you may not have Children. But when we are, we, we were young, we, one of the common things we ask uh Children to do is stack up a cube, you know, one cube or the other. But if I give a vege in the middle or if I don't give you a, a short cylinder and if I give you something slightly different, then obviously the tower will start to leave. And that is uh how the congenital scoliosis develops. And uh if you have a adjacent or successive uh hemi vertebra, then you can have a short arch scoliosis. And uh if when we pick up these uh short a uh hemi vertebras, uh at birth, they do tend to have a very high degree of progression. But when they are uh intermittent uh and but if there is a and if they're unilateral, then they can give us a long arch scoliosis. And sometimes this congenital scoliosis. If, if you have a very long arch can also mimic a neuromuscular scoliosis. Next slide, please. There's no slight uh there. Uh So OK. Yeah. So with, with the rotational imbalance, uh this is uh mainly because of the way that the embryology happens and the way that the skeleton develops, uh There, there is an effect of traction. Uh There can be osseous bridging, there is effect of pushing. Uh especially you can have significantly big uh transverse processes and it's usually a combination of uh traction and pushing. And uh if the congenital abnormalities involve the sacrum as well, then that can lead to saccular agenesis and pelvic malposition as well. Next slide, please. So, uh if you, when we with, in terms of the classification uh itself, we also talk about whether the scoliosis is compensated or uncompensated. And this is something we always talk about whether we are talking about congenital scoliosis, neuromuscular or idiopathic scoliosis. Uh If, if you have uh symmetric hemi vertebra in, in about four or five segments. Then uh so I think the slides keep uh flickering. Uh then uh yeah. So then you can, I, I, if you have a compensated uh symmetrical uh uh deformity, then uh not all deformities do require surgery. But if the hemi vertebra are uh uh quite uh distant and if there is more than six vertebra involved, then they can, they almost always require surgical correction. Next one please. And uh uh this is again, something you may all need to revise on from miller uh defects of segmentation. It can be unilateral defect leading to a longitudinal bar. It can be a bilateral defect leading to a vertebral block or it could be a combination anomalies. So, heb on one side and a bar on the opposite side or a hemi vertebral block and longitudinal. You, you have a combination of defects that can develop next slide, please. So there, there is no real uh understanding of the pathophysiology, but there are lots of postulates and uh theories that have been uh uh put forth. Uh One of the uh postulates that they have is uh there is uh you know, if, when patients have uh when, if, if uh mothers have fetal hypoxia or uh or gestational diabetes or if the fetus is uh is exposed to any form of fetal hypoxia uh and, and epileptic medications, uh moms having prolonged periods of uh fever. So, all these have been known to be associated with uh congenital malformations and there are some genetic defects, uh susceptibility to genetic defects as well. I think mis lodgment was uh leading uh to certain genetic uh uh changes that have been postulated. Next slide, please. So when, when a patient comes in to the clinic and with the suspicion of the congenital scoliosis, uh what are the things that you really look for? Um Nabil again? Uh Is it Nabil? Yeah, I'm here. Ok. What, so a patient comes in you, you, we, we spoke earlier about uh uh idiopathic scoliosis. But what are, what are the things that you would like to look for in, in more the neuromuscular or congenital type scoliosis? Um I'm looking for kind of skin stigmata, dystrophy, cutaneous manifestations, uh kind of a dystrophic curve of a neurofibromatosis. I'm looking for a sharp angle curve. I'm looking for neurology. Um because this is common with the uh with um with congenital and they are high likely to progress as well. That's right. Yeah. So, so when, when there is a suspicion of a neuromuscular or a congenital scoliosis, uh unlike uh idiopathic scoliosis, you are, uh you should really be looking a lot closely uh for any other associated malformations. And uh uh a nutritional assessment is extremely important. A lot of these patients can uh may need uh a chance to uh support their nutrition and uh you will notice uh associated you know, stigmata on the skin, as you can see here, hypertrichosis and some uh dermal sinuses. Uh and they're usually uh associated with congenital scoliosis. Next slide, please. Uh This is an example. And the other thing that you there are a lot of associations are uh not just spinal uh malformations, but also they can be associated with other skeletal malformations. And this is an example of a child with bilateral hemimelia uh with a significant uh bilateral vertebral blocks. And uh also hemi so just do not examine the spine itself, just try to examine the whole of the uh the child and, and definitely the limbs as well. Ok. Next slide please. Uh the congenital scoliosis. Uh Sometimes we unlike what many people think are very, very closely associated with uh uh several other organic uh malformations and neurologic malformations are associated as uh as high as 35% of them can have some form of neurological malformations. And uh the most commonly encountered uh dys my uh chiari malformations, intradural lipoma, tether cords. These are extremely common. And uh hence, we, we do tend to have a very, uh you know, low threshold to get uh extensive investigations. And that is why the congenital uh treating congenital scoliosis. Although uh from a technical skeletal point of view is not uh any greater challenge. Uh But uh the associated malformations makes it a hugely different beast. Uh urological anomalies are very, very common. Uh The reported uh rate in the literature is as high as 20% horseshoe kidneys, reflex hypospadias. And you can see an example of a joint inguinal hernia and you can clearly appreciate that the child probably has got a Coronal spinal deformity. Next slide, please. Uh heart uh problems, uh congenital, heart malformations, phal tetralogy. Can anybody tell me what are the components of phal tetralogy? The be come on sit. Uh there's a aortic transposition and uh a ventricular septal defect. I think I can't remember the other one. You haven't been quick enough to Google. It, isn't it? No, the, the the this is a, this is an exam. Uh You need to be very aware of uh uh you know, significant uh congenital uh malformations within the uh cardiac and hence, uh we do have uh pediatric colleagues very heavily involved in managing these uh patients and uh other skeletal abnormalities. It's extremely common C til is uh quite a common association with uh uh congenital scoliosis. And we also, we already looked at the patient with uh hemimelia. OK. Next pa next slide. So what happens? What is the natural history when we talk about when we are talking, when we, when you see patients in the clinic, one of the things that we patients can ask you or one of the things we have to postulate and understand is what happens if you do not treat them or what is the natural history of uh congenital scoliosis. And I'm sure Mr Cope will tell you about natural history of idiopathic scoliosis uh later on. So, the as all curves progression rate uh depends on the type of anomaly patients age and uh the place of the curve. And this is quite typical with the most scoliotic curves, but the highest progression uh seems to be in those with the mixed defects because uh as we've uh seen, uh this is an example of a mixed defect where you can see uh segmented hemi and uh at multiple levels. And uh what is interesting is uh the progression seems to be really bad if uh if a child has been uh known, if, if an investigation picks up a uh congenital uh defect within the spine uh at birth. And uh the normally when we talk about idiopathic scoliosis, we talk about the progression rate of about uh 1 to 3 degrees per year. But in, in a congenital scoliosis, it can be as high as up to 10 degrees a year. So they can progress very rapidly and they normally have a bimodal uh progression. Most of it happens within the first one, first five years or uh when they have peak growth um in their adolescence, uh also the soy thoracic uh and uh the lumbosacral uh area seems to be more uh common for uh rapid progression. Next slide, please. So the, the treatment as always, what are the ways you can treat uh congenital scoliosis. Uh Most of uh congenital scoliosis will require some form of intervention. Uh And depending on what is the age of presentation, uh and what form of treatment and what's the, the curve characteristics, we sometimes have to intervene as young as 1 to 4 years of age. And uh as I mentioned, if it's present at birth, then it's usually an indication of a worse prognosis and uh uh other, otherwise we always try to intervene uh for those curves which are more than 40 degrees. Uh uh the books say 40 degrees. But uh more realistically, we do assess the patient as a whole and skeletal maturity and we can defer it for as long. Uh Then we try to do that. Uh The other reason for very early intervention is of course, congenital malformations. Uh A lot of these congenital scoliosis can be associated with the thoracic and rib pump deformities. And uh uh th that may lead to thoracic insufficiency as well. And those are the times when we have to intervene early. Next slide, please. So the, the means of intervention uh in very young patients, uh we can do uh what we call is an in situ fusion uh or uh just fix it as it is and let the rest of the skeleton catch up. Uh I've lost a slight uh set, then we, we also can do uh hemi epiphysiodesis. If you've uh if you've all worked in pediatric surgery, uh pediatric orthopedic surgery, you all would have come across hemi epiphysiodesis, commonly performed around the tibia. Uh So we do do these hemi epiphysiodesis procedures within the spine as well or we can uh excise the hemi vertebra excision is also one of the procedures we can follow. It all depends on the curve characteristics, the nature of the uh congenital, uh scoliosis and also the age of the patient. Next, please. So the in terms of definitive surgery or uh with instrumentation and fusion, there are two predominant modalities that we use. Uh One of the, the first of the predominant modality is uh the use of growth rots. And there are conventional growth rots. Uh The the picture in the middle uh is uh what we call is a conventional growth rod where we have overlapping rods and at every regular intervals. And as the child uh is growing, we have to open up the segments and we have to distract to unload the skeletal maturity. The, the x-ray on the left is that of a magic rod. So it is the magnetic rod where you have the proximal and distal anchors. And uh the pictures on the right uh shows you um the ways of achieving lengthening. We have an external device which uh is connected and when you activate the device, it elongates the magnet over a period of time. Uh So these are some of the conventional and the more recent uh techniques that we have used. The magic rod has um have been investigated quite a lot because of cobalt uh problems with metals. And at the current time, we do not use magic drugs. Apart from very selectively, a few, few surgeons are still using the next slide, please. So this is uh something that uh has been asked in your exams uh where called expandable prosthetic titanium brick. So effectively, this is a procedure that is used uh when we need to correct uh curves and also expand the thorax by doing a thro uh it's an extremely morbid procedure. Uh Not all of us do it and there are lots of funding issues. Uh We have to get special funding and it's only done in a few centers in this country and around the world. Uh Next slide, please. So in, in the principals uh uh strategies in terms of treating congenital scoliosis is uh slightly different to those uh that we use for adolescent idiopathic and, and the, the primary aim for any scoliosis uh treatment. Uh When you, when you're asked about what is the primary aim of surgery is to prevent, is to stop the progression of the deformity. And generally, as a rule, we, we do not want to do any heroic surgeries. We do not want to have a perfect straight spine. The primary aim is to prevent it from getting worse and if we can do it as safely as possible, and try to get as decent correction as possible. That is our aim. And uh uh the difficult challenges in treating congenital scoliosis is of course uh understanding the growth uh and understanding the differential group between the cavity and the convexity and uh what particular procedures we need to choose. And also um understanding that uh when do we intervene, the biggest challenge for us is when do we intervene? Uh at what age do we intervene and what other uh procedures need to be done? Sometimes we have to adapt hi tractions and things like that. Next slide, please. I think I'll stop with this because it is quite uh exhaustive, neuromuscular scoliosis. The principles next slide, these are are very similar. Next slide. Uh As we have mentioned, congenital scoliosis is associated with uh as high as 35% of patients can be uh can have a neuromuscular component as well. Uh And it's quite a big topic by itself. So I'll stop with this. Any questions. Mm uh No questions in the chart. I think uh uh congenital scoliosis is not something that uh Loman will be able to confirm that. Uh it's not something that is routinely seen uh as an exam case. But uh you can uh you do have a lot of other skeletal malformations and they can uh occasionally there can be stable patients who may be brought into the exam. Uh But uh again, come to our clinics, uh feel free seeing a few patients will be a bit more uh realistic than uh having a lot of book knowledge. That's great. Thank you very much, Mister Swan. Now I'll pause the recording.