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Summary

This on-demand teaching session provides medical professionals with an overview of congenital dislocations ranging from Torretta Logic Dislocations of the Hip to Congenital Knee Hyper Extention. Topics such as the necessary examination techniques, as well as the associated conditions, treatments and prognoses with particular reference to Myelomeningocele Arthrogryposis and Larson Syndrome, are discussed. Attendees can expect to develop their understanding of the types of diagnoses necessary for successful management and treatment of these conditions.

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Learning objectives

Learning Objectives

  1. Describe the signs, symptoms, and causes of congenital dislocations and congenital hyper-extensions of the hip and knee.
  2. Demonstrate knowledge of the diagnosis and management of congenital dislocations and hyper-extensions of the hip and knee.
  3. Differentiate neonatal hip and knee dislocations from other conditions and disabilities.
  4. Explain the biological effects of delayed diagnosis and treatment of congenital dislocations and hyper-extensions of the hip and knee in children.
  5. Evaluate the impact of the UK's health policies on the diagnosis and treatment of congenital dislocations and hyper-extensions of the hip and knee.
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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.

Is that? So I'm going to talk about uh congenital dislocations. These are a different way of looking at this is that this is a joint dislocation. This is a, a joint joint, a congenital joint dislocation. It's actually a form of joint contracture. Next slide, please. The congenital dislocations vary. Okay. You can have a Torretta logic dislocation of the hip. That's a congenital dislocation of the hip where the child is born with a hip that's out, up and out. That's not, that's not your average unstable hip, which is usually where the acetabulum is a little bit dysplastic. This is where the hip is out. But you can see um um the baby looks pretty normal, you know, it's very hard when they're neonate. It's, it's very hard to tell um a leg length discrepancy because as a proportion, the leg length discrepancy will be what a centimeter, half a centimeter um and uh which isn't which, which isn't, which can be hard to detect clinically. They will always have unequal abduction, but they may not be reducible. So then it may be a and in fact, Torretta logic dislocations of the hip, that's congenital dislocations of the hip where the process occurs. Antenatal E is almost always stable. So it's a stable dislocation of the hip. So your sign that is most reliable is unequal abduction. And so you can imagine that babies that are born with bilateral congenital dedications of the hip are those are the ones that slipped the net, the ones that aren't picked up because the United Kingdom in its infinite uh money saving wisdom does not yet have 100% universal ultrasound scheme screening of the newborns. So that's a subtle dislocation of a hit of a joint. By contrast, if you have a look at the baby on the left with congenital hyper extension of the knee, sometimes called congenital dislocation of the knee that is very hard to miss. However, it is usually missed on the antenatal scan. It occurs after the 20 week scan. And even if you see that if the mother has a scan for some reason after 20 weeks or after 32 weeks. Um And you see, but legs at a bent, you can't tell on the scan that they're bent backwards, which is what, what is, what is happening here. Um You, you just assume it's a baby with its knees bent and um and it's only when the baby is born when people are absolutely upset, horrified and hysterical. And who do they call the orthopedic registrar on call? Next slide, please. So let's have a quick chat about teratological hip dislocation. I couldn't help myself. I got two over excited a moment ago and I started talking about it too early. So this what I was trying to say is that these can be missed if there bilateral, they can be missed because in the United Kingdom, in its infinite money saving wisdom, um Babies, newborns are usually examined by midwives these days, you only have to have examine 30 babies to become competent as a midwife as I discovered um in Cambridge to start examining babies hips at birth. And when I did a little audit on midwives, um um the quality and the nature of the neonatal hip examination was alarming at times. Um My swipe card doesn't seem to work anymore on that ward. I can't get in the midwives did something they haven't invited me back to um um completely audit anyway. So um so these babies get missed and the hips are out. They are not unstable. Usually an early diagnosis I'm afraid can change the prognosis of a child with a Torretta logic, hip dislocation, closed techniques are likely to fail. Um So you do have to do an open reduction and the quicker you do an open reduction on this particular type of hip, the better, the very memorable child that I had was four years old when um when this was discovered, they had always, they've been late walking, they'd always been had an odd gate by a waddling sort of John wane, wide legged gait. When this child got off the floor, he would get off in a really laborious old man sort of way, pulling his hands onto his thighs as he got up. And um what sign is that? Shout out please that I'm describing when you can't get up off the floor quickly, you um pull yourself up gradually putting your hands onto your knees, onto your thighs as you get up. Very good iggy. What, what does that mean? Gowers sign? Uh a proximal myopathy. So, oh my gosh, you are so good. Absolutely right. It means proximal muscle weakness, which is why people of my age get it. Well, the the obesity doesn't help but you your, your, your proximal muscles get a bit weaker and so you can't get up off the floor in a quick fluid motion anymore. Most toddlers are on and off, on and up, up and down off the floor very quickly. So these Children do stand out, but his a proximal muscle weakness was not due to Duchennes muscular dystrophy, which is what that sign is classic for. It's because he had bilateral dislocated hips. He was seen by pediatricians, he'd been saying, and it was the neurologist at Addenbrooke's had already done muscle biopsies under general anaesthetic, had done nerve conduction studies on this four year old. They then did a spine X ray and at the bottom of the spine X ray, these two dislocated hips was discovered and they called me and, um, and the parents or that they were the most grateful parents I think I've ever had because a child with dislocated hips to a parent. In retrospect, I understand it now, is such a fixable problem compared to a child with a progressive, um, ultimately fatal, more muscular dystrophy. So, by comparison that they, they thought they'd won the lottery when they met me, not a few, a year later, when, after to, to, you know, when all the hip surgery, when the reality of a hip surgery comes under. But anyway, I'm I'm digressing and I'm being an old person just ruminating about old cases. So early diagnosis helps, but early diagnosis can be tricky. Almost always. There are almost always, there are associated conditions. Um and just make this your chant now, right? Make this your Buddhist Hindu meditation. All chant myelomeningocele arthrogryposis. Iss Larson syndrome. Repeat it to yourselves. Myeloma, ninjas, ill arthrogryposis iss Larson syndrome. Because when you see congenital contractures causing dislocations, you think myeloma ninjas, ill arthrogryposis Larson syndrome. Next slide, please. Congenital knee hyperextension is unbelievable. When you first see it. When the baby's ear comes out by their knee, why their, their knee comes out by their foot comes out by the baby's ear. Um And it's um can you see the little tiny x rays the far left of my screen? That's why it's called a knee dislocation. Um um in the more severe cases the the the knee is desiccated. It is associated with which conditions. Yes, myelomeningocele arthrogryposis and Larson syndrome. Those Children that have congenital knee hyperextension associated with those conditions are often bilateral and often have a poorer prognosis. Please look for hip and foot deformities there often missed because this presentation is so dramatic. The incidence is actually less. Well, it's about between about one in 10,000 which in a hospital, Adam Brookes has 5500 babies born there a year. So we will see this once every couple of years next slide, please. So the management that, well, first of all, make a diagnosis and then you consider the problem. What does that baby have? They have a quadriceps, shortening or contracture, they have a tight anterior capsule of the knee in the more severe cases like the X ray I showed you on the previous screen, there's anterior displacement of the hamstrings and there's cruciate and collateral ligament. Well, it will be lax when you reduce the knee, but at the beginning, it's taught and tight, but in the wrong position, treatment is start splinting as early as possible. Um, and um, initially the splints will make our don't make much difference. They, you know, every they, they, they, they take the knee from 40 degrees of hyperextension to 35 degrees of hyperextension. Then the next week you'll suddenly have another 15 degrees all at once, then you'll slow down again and it takes a while. Um, and, but it should be normal by three months if it is not getting normal. If you have 30 degrees or more of a fixed flexion at that knee, sorry, fixed hyper extension of the knee, then you um, sorry, let me start again. If you don't get further than 30 degrees of flexion by three months, you really need to be thinking about surgery and surgery is soft tissue if you get do it early enough things like quadriceps, um lengthening either percutaneously or through a former formal privy on why lengthening. And occasionally you have to do patella distal ization, um femoral osteotomy but not often. Usually splinting works. Occasionally you need to do some quadriceps lengthening. Which Children do you think you might have to do quadriceps? Lengthening on? Consider that chant that I've just taught. You just shout out anybody who would you have a low index for doing surgery? Arthur gripe roasting, well done. So I see you're, you're, you're the man now. I don't know if our mood is still here. Um He wanted to do weekly visits on centralization of the radio club hand. Um and I slightly poo pooed it ahmed. This is where you would do three daily visits, perhaps not to the surgeon but to the physiotherapist. Um frequent changes of the Splint a unnecessary because it's a neonate. And secondly, because, and the splint slips. And secondly, actually it does make a difference. Next slide, please So congenital dislocation of the patellar. This can be a little bit tricky to diagnose. So, the child above is um the sort of Children I've seen actually my latest child is a child with Down Syndrome who's a Afghan refugee living in a hotel near Chelmsford. And um they have had this dislike they patella bilateral patellar dislocation since birth. The child is now nine. Um the father has really hooked onto the fact that I said that I would not operate until they've been re housed and could be um could be mobilized safely and um could be mobilized properly with a physiotherapist and keeps, keeps coming for appointment so that I can write another letter to the home office and to the housing department. But it takes a while till you see that classic general val gum fixed flexion deformity. These can be hard to diagnose because um um um that they, you know, they don't delay walking by very much. Um They're often part of other conditions. What other conditions could they be part of? Shout, please? Arthrogryposis. Iss Yeah, what else? Maya Langham Miley. Very good License syndrome. Last and excellent and Down Syndrome as I mentioned as well and Cornelia de Lange as well. So, um so, uh but yes, you look for those conditions is very rare to see this without those conditions, I must say. Um But when does the patella Aasif I when can you make that diagnosis with an X ray for lovely iggy, lovely, three or four. So actually, if you see a child before and you think this might be the case that they can't fully extend their knee, they've got a fixed flexion, they look like they might have a general valgus deformity. So, remember you're not meant to have a general valgus deformity before the age of four. So, um, well, you can at the age of four. So let's say a little bit earlier than that. Then you need an ultrasound scan or an MRI to confirm your diagnosis. There's no point sending them for an X ray. Next slide, please. So, treatment treatment. Well, just think about the condition, a congenital dislocation of the patella, it's lateral, it's, it's got to be tight if it's congenital because it would have happened antenatal. E so you need to do the mother of all lateral release is you have to release the whole of the vastus lateralis basically from the intermuscular septum. And sometimes you have to release a bit of the vascular vastus medialis from the vastus lateralis. You need to do a huge medial briefing and you do a quadriceps, lengthening. Um the um A Z lengthening or A V A N Y lengthening and the patella tendon shortening usually by taking by splitting the patella tendon, it's called a trouser um movement. You split the patella tendon and you move the lateral portion over the medial side and attach it immediately. Sometimes you have to do a distal femoral osteotomy, particularly there's a severe general Algom osteotomy. But okay, you've not, you've, you've probably not seen it. It is a really fun operation to do, may I say? Um and it, but it makes sense, doesn't it? Everything that's written there, I hope is intuitive. Next slide, please. So, let's move down and we've done the hip, we've done the knee, we've done the patellar congenital vertical Taylor's is the more common dislocation that you'll see. Congenital congenital vertical Taylor's after all is really a dorsal dislocation of the talonavicular joint. It is rare but you will see it, it is you bilateral in most cases, but it says 50% of cases, but in my experience is bilateral. In most cases. Next slide, please also quite dramatic, this very dramatic rocker bottom foot. Um um that you see with no, with the dorsum of the foot, sort of attached or not, not physically attached but sort of uh adherent adjacent to the anterior tibia. Um The treatment um um Well, the first thing you do is to look out for the associated conditions. Almost all of these have an associated condition. What are they Lawson syndrome? And yeah, great prices. Yeah, arthrogryposis. What else? South Aston. That was the last one and oh, I got, got the mantra um while I'm the ninjas. Yes, well done, well done, well done. Um Yeah, and then you can add in um some neuromuscular conditions cerebral palsy, you can add in um uh diastematomyelia, which is a type of, it's not, it's like it's like a spinal bifida um that are particularly common for CVT congenital vertical Taylor's. Um uh you do a stretching technique, basically a preoperative stretching. It happens to be called the reverse Ponsetto technique. And then you will have to do some type of achilles tendon lengthening. You might have to do apparently as long as lengthening, but you then do a pinning of the talonavicular joint. You reduce it usually open and you pin it next slide, please. Larson syndrome. The rarer one, it's apologetic disorder of connective tissue. It's poly genic, it's also got quite a wide sort of um presentation. Um The phenotype can be is quite broad. So the lady on top has Larson syndrome, but you know, you wouldn't know very easily whereas the boy at the bottom has very, very classical Larson syndrome that, that the frontal bossing, the depressed nasal bridge, the low set is the cervical um kyphosis scoliosis. Um uh Not to mention the knee joint contractors, all sorts of joints are contracted here and they can be dislocated as well. Can you see the elbow there? So the elbow probably has a radial head dislocation but it's in fixed flexion. Um but it might be a dislocation of the elbow joint itself. Um Bilateral knee dislocations is quite a common finding. Um And but there will be multiple joints involved. Um and in the more severe cases, um this um cervical spine and cardiac anomalies are involved as well. Next slide please. So it's a mutation in college in the chrome third chromosome in the 21 region. It's a region that um codes for, but there are various mutations that can occur here. There's one um at the collagen seven mutation. Um and um there's many mutations of the Philomene be of the situs skeleton which is involved um um in many structures, particularly in chondrocytes at the Fyssas, which gives um the short stature of little clinical photo I showed a moment ago. So, Philemon be collagen seven chromosomal disorder, palla genic and with a broad phenotype or a varied phenotype. Next slide, please. Arthrogryposis, arthrogryposis. Um There are over 400 genes for arthrogryposis. It's basically fixed contractors of many joints. Um um um when their arm, when there's more than one joint or joints in them, all four limbs, it's called arthrogryposis, multiplex congenital. Er, it's almost always arthrogryposis, multiplex, congenital. So that's often just that's the name, but there are slightly different types. There's um you plasia, distal arthrogryposis and Pterygium syndrome. Um, you know, plasia is the baby at the top. It's the most common type. It's um all joints involved also multiple joints involved, um including the proximal joints that um you plasia has that can you see the shoulders there, they internally rotated there. Um, they can't, well, it could is any clinical photograph but they can't have duct very well and they're internally rotated, the knees can either be flexed or hyper extended and the feet can be club feet or vertical Taylor's. Um, and, um, but, and that's your, um, yo plasia. The distal arthrogryposis is the baby below. They can be hard to detect. Often they come to the Club foot clinic and it takes, you know, it takes you to taking a, examining the child, you know, really having a look to see if the hands are involved as well. If it's bilateral club feet, how much range of movement does the, do the knees have? Um But that's distal arthrogryposis and pterygium syndrome is characterized by these sorts of folds of skin, these back wing type folds of skin around the joints, particularly the knee. Um Next slide or somebody um uh Dr Addy M B B S medicine with honors at Hull um is um uh telling me that are Philomene see variants associated with hypertrophic cardiomyopathies. I don't know. You're, you're flattering me with asking me that question. Should I just say yes? Yeah, type three to that question. It's I, I honestly don't know Dr Addy M B B S, I'm sorry. Um Okay. Uh That's uh I shouldn't read the chat actually. That's quite an off putting question. Okay. But, but a good question, but I don't know the answer. I'm sorry. Um And I need to talk quickly about congenital radial head dislocation it is rare. It is usually a symptomatic. Um, and can we missed because it's not functionally functionally. It can be, um, Children can function quite well with it. Um, there's a reduced extension of the elbow and there's reduced Super nation. Uh, next slide, please. But the classic, um, finding because sometimes it's hard to work out if it's, um, Children injure themselves, you know, and, um, um, Children fall off trampolines and things and you don't know whether this X ray is suspicious. There's uh don't, don't apologize, Dr Addy. Mm BBS. Um You don't need to apologize for asking that question. It's um just cause I don't know. Um I, I should be the one apologising to you. Um So sometimes you have a child with an injured elbow and you're asking yourself, is this a congenital dislocation or is this acute or is this four weeks old, three weeks old, you know, a missed injury? Well, in a congenital dislocation, the dislocation is posterior. Um but that can be with trauma as well. Um And um but the key is that on an MRI scan or an arthrogram, the radial head is con um not concave. So I wrote that wrong um convex and the capital um is hypoplastic. So that's, that's the difference on Arthur Graham and an MRI scan. The other difference is that it's bilateral. In most cases, there is really no treatment, you can't improve their range of movement. There function is usually very good. Um um and I would advise um just, just keeping them under review, trying to get them veered off surgery. There are cases of people having radial head excisions in adulthood, um, and elbow replacements in adulthood. But actually, um these are the odds, sporadic case reports and it's hard to tell why the procedure was performed. Next slide, please. Um I think that is it, I was just rattling through really. Um So, so, so the conclusion is that with congenital dislocations, they can be alarming, but they can be deceptively mild. Um they usually occur antenatal e they always have a genetic cause just because we don't know it yet, doesn't mean it, there's no genetic cause and it's looking for concomitant problems, connect concomitant, connective tissue disorders in particular, keeping your eyes open or if you feel that you can't do it, then um making sure that they're referred to somebody like a geneticist who can, who will be thinking more globally. Any questions if we're on call and we're the registrar who gets called about baby born with congenital need dislocations. Um A few questions. Do we get an X ray? Do we reduce it? Do we give them G A like do we put them straight into some sort of? So, um so, so, so you first congratulate the parents and tell them it's fixable. Um um And um you examine the child, you need to look at the concomitant deformities and concomitant conditions. And um, you'll look very, very smart if in the notes or by direct discussion with the neonatologist or the pediatrician, you mentioned that there could be cardiac anomalies, etcetera. Um, looking for Larson syndrome, you'll need to examine the other joints. They're not always obvious if there's a contractor at the elbow joint, some and it's fixed an extension. Well, of course, any fool can see that, but actually, sometimes it can't extend fully. It can only extend to 30 degrees and there's a fixed flexion of 30 degrees bilaterally. Or you can imagine how that could be missed. But you, as a registrar are going to be examining every single joint and you're not going to miss that, you're going to market and you're going to come back and examine it again or someone else is going to examine it again a couple of weeks later, a week or so later. Um, that night, um, your emphasis should be on congratulating the parent. Um, just gently, you see how, how much you can move the knee and make a note of it. I can't move it, I can move it five degrees, 10 degrees, whatever. And then they should have, there should be, if there's any, if there's a, a birth unit, babies being delivered, there should be a physiotherapist attached to that unit and that physiotherapist when they come on can start splinting the knee. Yeah. Um, and, and, um, but do start or do imply do a sense of urgency about knee splint ege because remember the incidence is one in 10,000, 1 in 15,000 that physiotherapist may never have seen one before. So you have to imply a sense of urgency which is valid. Um um The quicker we start splinting the quicker and it's cereal splinting. Remember, so every, every splint brings in a tiny bit more flexion. The quicker that started, the quicker that this can be rectified. Would you X ray? Um, not in the night, not in the middle of the night. No, just, um, but yes, I would. It confirms the diagnosis for you, doesn't? It, it gives you a baseline. It, um, it tells you where you were. Um, no, I would, but it's not, I wouldn't delay splinted to X ray, for example. Um I wouldn't, I wouldn't at this age in my life, I don't create, I don't look for excuses to increase my systolic BP. I am not, I'm really, I'm not, I'm seriously, I'm not bothering radiographers in the middle of the night and having around with them to go to a neonatal unit to X ray. A baby. The like of which they've never seen before. That can wait till more senior people. Come on the next day. Can I ask about radiohead dislocations? It's quite a very classic exam question. Oh my God. It's, and it's also, it kills you when they present is because MS spontaneous are not that uncommon, you know. Um So is this, is this congenital or is this related to last year's elbow injury? Yeah, we got, we got asked that, I think in the last years mark, uh how, how common is it and how practical offer offer like reconstruction of the annual ligament? I think I got to reconstruction options by all means off of that. But you've got to confirm, it's, you have to confirm it's post traumatic. Okay. Yeah, it's, is it post traumatic and does the arthrogram and the radial head? Um MRI scan show that the end of the radial head is concave. Now, if you've had a traumatic L A radial head dislocation, first of all, it's usually anterior, okay, not always, but usually. Um um so that should give you a clue that this is a post traumatic um injury. But even those can be very long standing and if it's been, you know, in a small, in a, in a young child a year or so, and suddenly they're radial head starts, that grows a bit and it no longer is concave. So, and that can be very, very hard um to reduce where as soon as it starts to go lose its concavity, you know, it's been longstanding. Um And um and you know, okay, you've decided it's not, if it's unilateral in particular, it's more likely to be traumatic if it's um especially if it's anterior and unilateral. So you now know, it's post traumatic, but are you still going to reduce it? Are you still going to reconstruct annular ligament? Personally, in my hands, reconstructing the annual ligament is a fantastic and fun operation, right? I can't tell you, um but almost sensual joy. I feel when I see the child extend their fingers and wrists in recovery after I have been doing an annual ligament repair. But can I also show you the depths of despair? I feel six months later when the radial head is coming out again? Okay. And that has been my experience and it's, um, and, um, and I now think that you have to, if you're dealing with a long standing missed post traumatic anti.