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Congenital Limb Deficiencies for Postgraduate Orthopaedic Exams
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Congenital Limb Deficiencies for Postgraduate Orthopaedic Exams
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Language: EN.
Segment:0 .
Good evening, everyone.
My name is KneeKG. And welcome to our webinar this evening, which is on congenital limb deficiencies by John mean John works at the Evelina Children's Hospital. Ernie Foster's exam in November 2020. So I'm sure he's going to make this complex topic very easy for us all to understand coming up for the exams. I'd like to welcome David, who's my colleague, teaching program convenor, for tonight's session.
So just some general housekeeping. We're not too big this evening, but I'll ask you all to stay muted unless you're talking or contributing just to reduce background noise. I'll just share what the program for this evening is. OK, so we're going to have our lecture on congenital limb deficiencies, and if you have any questions, we'd ask you to write them in the chat and we'll ask them after the mock polls because some of those questions might be answered for the interviews.
After we've done the questions of the mcu's, then we will do some hot seat Bible practice with a maximum of three candidates. So if you're interested in the vyver, if you could raise your hand or send a message to myself or David so we can allocate those for you, I do realize that the exams are only two weeks away, so it's very stressful time. But this is the best practice that you can have is to be better to make mistakes in this part than in the actual exam itself.
OK, so. We do have some upcoming courses coming up, so we've got the mock exam course. And we've got dates throughout the year. We've got the case discussion's course at various points as well, and we're also running our first basic sciences for the folks this weekend. You can keep an eye on any relevant courses that are coming up on our website, which is orthopedic Academy.
Co.uk and we are also available on social media. And so at the moment, I'm recording this session, and it will be available on our YouTube channel in a couple of days. So don't panic if you missed any part of it or stop the recording when we start the rivers to protect your identities. And without any further ado, I'm going to hand you over to John for our lecture.
Oh, Thanks a lot, KneeKG. Good evening, everyone. Actually, before I start, I just would like to thank everyone in this fantastic group. I passed my exam in November 2020, and I have to say without the help and the support I got from this group, I wouldn't have passed my exam. And so Thanks.
So everyone helped me to pass my exam for those who don't know me. I'm John and I'm a senior fellow at the London general hospital, and I'm interested in pediatric orthopedics. So today we're going to speak about congenital limb deficiency. So, so basically, this is a tough topic. Actually, it's a very complex cases. The reason we are approaching that topic to do teaching for today is because sometimes it come up in the exam.
So I thought it would be better if we have a resume of the topic and how to answer the questions in the exam. I will try and keep it simple. We will not go into depth in these complex situations. You are not, you know, you don't need to be expert in managing these cases and the exam. OK, so let's just start.
So today. OK, so today, what are we going to do? So first, we will discuss like a general approach to any AIDS case that you met in the exam, either in Dubai or in the clinical. And then we will start our topic by speaking about the embryology. I will speak about the Lempert development.
And then we will discuss congenital femoral deficiency. And then we will discuss what happens in the knee in any case of congenital limb deficiency and what are the things that you should look for? And we will speak about fibular at the last thing in our teaching today. So let's go. Uh, but first, I would like to start with this clinical approach to pediatric cases.
Actually, I use this approach in while I was preparing for the exam and I thought it's useful. So for any case of pediatrics, you start by taking history. So in history, you usually ask about the complaint, the complaint. In other cases, it's usually pain in children. It may not be pain. And if it is pain, the key to you is this pain is significant or knot is to ask if the pain is affecting the patient function or not.
Like, how do you do in and body and their sport participation and all these kind of things? And then you focus on the present history and the present history. You should ask the patient if he had any previous treatment, his condition. And then in the past history, we all know about the prenatal, perinatal postnatal history and the postnatal.
We ask about the developmental milestones generally. And it is very important to ask about the family history in any case, in any pediatric case, especially for those conditions who have a genetic background or usually have a positive family history. And then you should ask if there is other system of like if you have a patient who is a congenital limb deficiency, you need to know if he has congenital heart disease.
Arena, Genesis or anything of this associations, so you should ask the patient if you ask the parents if they are seeing other specialties, if they are seeing the ophthalmology, for example, for any ophthalmological problems, if they are seeing any other speciality endocrinology, pediatric neurology and all these kind of things. And then when it comes to examination for any pediatric case, you should examine the patient from the tip to the toe.
So in other cases, if your, for example, you have a hip case, so you usually discuss examination of the hip. And then after discussing examination of the hip, you mentioned roughly, you mentioned briefly the rest of the general examination, but indeed it's better to have a system in your mind, whatever the case, to try and check on these kind of things. So we're starting from tip to toe. So it mentioned about examination of the head in the head, you look for any abnormal phases that the patient have any syndromic faces.
Does you have or have any abnormal shape of the head like plager? Carefully microcephaly? You look at the eye. Is there any evidence that blows clearer that can point to a surgeon slap perfecta? You look at the development of the teeth and all these things. And then in every pediatric case, you should check the spine and need quick neurological examination.
And don't forget to examine the upper limb and end the lower limb. You should look for any evidence of coronal or sagittal plane deformity. You look for any rotational abnormality, you look for any length discrepancy. So to resume that, it's in every pediatric case, you should say I will look for the face. I look for any abnormal flashes you would look for.
I need to examine the spine. I would look for any neurological deficit. I would. I have to examine the upper limb. I examined the lower limb by you look for any such terraplane deformity and irritation of abnormality. Any length discrepancy. Regarding investigations, it's according to each condition, sometimes in the X-ray city, the MRI is, but my father is in pediatric cases, always ask for more views because as we mentioned earlier, if you are seeing a patient to have a lower limb deficiency, so he may have a associated condition in the upper limit, but you may have a condition at this point and whenever you are also expecting something a little that, please ask for more investigations.
And if you are, if you have a case like, for example, this abnormality the ankle, then you have to check the whole leg. OK hey, next, you're going to speak about embryology. I have proclaimed please. OK, so let's start.
So we all know that embryology is just the heartbeat. You know, it's like a dry topic, and I usually find it difficult. So what are you trying to do today? I try and make it as simple as I can. And so usually the Lempert and very Genesis stars between the ages of 4 to eight weeks. And it starts at an outgrowth of mesoderm into the overlying ectoderm.
So this is the embryo. So this is the metal dog and the Black line that is covering it at the ectoderm. Ok? the mesoderm, the outgrowth and form. The Lempert is formed of two types two layers of mesoderm. The first is somatic mesoderm that will give rise to the muscle tissue later on, and the lateral Mason mesoderm that will give rise to the bone and cartilage.
So when we describe the development of the Lempert, we usually. Describe it in 3 axes, and each axis has a different molecular pathway, and to be honest, this I know that it's a bit difficult and it's a bit difficult to memorize the name of the genes and the axes and everything, but it does come up in the exam and does come up as an empty key in the first part.
And it can be asked, you know, in the second part and the 5 and the basic science table. OK, so the first asks, is it the proximal to distal axis, so the proximal to distal axis growth of the limb, but is organized by the epicondyle ectoderm ridge? So this ethical ectoderm ridge is a thickening of the ectoderm overlying the mesoderm of the limb, but it secretes fibroblastic growth factor.
And this fibroblast, the fibroblastic growth factor, stimulate the underlying mesodermal cells to differentiate into specific types of cells that give the Lempert its shape. Eventually, the effect of removal of the epicondyle dermal rate is growth the rest of the Lempert, which will present later as a congenital amputation of the limb or transverse deficiency.
Uh, so we can see on this photograph this is an example of a congenital amputation or transverse deficiency of the limb part, and it resulted from removal of the epicondyle ectoderm or rage or damage to the epicondyle ectoderm ridge. The other reason? Uh, if you have a typekit plantation of the epicondyle ectoderm road rage, it'll cause extra them.
It's not a common kind of thing to see. Uh, here you can see an example of that, where you can see the two feet are growing in one of them is because the power of the cells of the epicondyle to the marriage are implanted far away from where it should be. So the typical ectoderm marriage after a while will have entered Interdigital necrosis, and this will result in the separation of the fingers or the toes.
If this fails to happen, this will result in syntactically. Here's an example. It's a photo of the Fort where you can see there is syntactically in the second and third toes. So basically in the exam and the basic science table, we can't get any of these pictures. So if you see any of these pictures, don't panic because you will not be asked about this specific condition.
But all the discussion will go into where the limbo develops. And I have to say that this picture is a transverse deficiency of the limb actually is a picture that I had on my exam. So Uh, now we speak about the second access in the Andrew posterior axis. So if you look at the picture on the right hand side of the screen, so this is the embryo.
And this is the limb. But and this square is just a magnification of the limb pop. So let me. So from what we can see here, the limb pod has a both and posterior border. So it's a quite confusing because this empty border will give rise to the radial side of the forearm lateral and this posterior border will give rise to the ulnar nerve border of the forearm.
So these this entry procedure access development is regulated by the zone of fertilizing activity. So cells cells on the posterior aspect of the Lempert get thickened and active and regulate the differentiation and to enter and posterior axis. So this is regulated by the sonic hedgehog gene and the Indian hedgehog gene to make things more complex.
So the sonic hedgehog gene also contribute to proximal to distal limb, but development through a feedback loop to the epicondyle ectoderm ridge, so you can see that on the right hand side of the screen. So this is the typical ectoderm ridge. They said the zone of polarizing activity. On the posterior boarder of the. So the ethical ectoderm rich regulate proximal to distal growth through secretion of the fibroblastic growth factor.
You can see that the zone of paralyzing activity secrete or activate the sonic hedgehog gene, which regulate the development of the limb body and the Anthropocene axis, and this regulate again the proximal to distal development through a negative feedback loop. Transplantation of the zone of paralyzing activity from posterior to anterior aspect will cause myriad duplication of the ulnar aspect of the hand.
I know that it's a bit tough, but we have to go through it. Let's continue. So this is the last access its dorsal to ventral axis. So growth through the dorsal and ventral aspect or differentiation into dorsal and ventral aspect is regulated by the wingless type signaling pathway, and it comes from the dorsal ectoderm.
Uh, so they signaling pathway induce the underlying mesoderm to develop characteristics of the docile structures and because the activity of this pathway is blocked on the ventral aspect. So this will allow the ventral mesoderm to differentiate into ventral structures, say the w.a. pathway also contribute to regulation of the S gene.
And this reflects the complex interaction and coordination among the 3D pathways responsible for limb development. So and the pictures on the bottom of the screen, we can see that this is the dorsal aspect where we have the w.a. gene active and this is typical ectoderm ridge where we have the fibroblastic growth factor. And they said the about where we have the SH gene.
And if you and you can draw the example drawing, look at this one, it can help you memorize these kind of things. So I hope that no one of you guys now is covering his AIIS regarding the rest of the teaching today. We're going to think that are more clinical and you'll find it more interesting. So for congenital limb deficiency, we will start by congenital femoral deficiency, so congenital femoral deficiency is the new term, and it now replaced proximal femoral focal deficiency because it includes a wider spectrum of pathology.
So demographics of congenital femoral deficiency, so the incidence it's a bit higher, so it's one case in every 50,000 people, the male to female ratio is one two. It's usually unilateral in 85% to 90% of the cases, so it's rarely bilateral when unilateral, the right femur is usually most commonly affected.
It can be diagnosed by in utero ultrasounds, where they can measure the length of the femur to reflect on the gestational age. So in case of discrepancy between two sides or in case the discrepancy of both femoral regarding the rest of the milestones are measured in utero ultrasound, we can make an earlier diagnosis. So as we have mentioned earlier, the congenital femoral deficiency is a spectrum of disease.
So it starts from having a normal but slightly short femur to having FEMA with just abnormal Nick shaft and go to FEMA with severe available. And sometimes the second step in the spectrum is having through the proximal femur or at the end of the spectrum, you have a complete absence of the proximal femur and this is called proximal femoral focal deficiency.
So the term congenital femoral deficiency reflects better the whole spectrum of disease. So let's start. So are you going to mention about the non-federal structures? Sorry, just listening. The non-federal structures that are also involved in congenital femoral deficiency.
So whenever you have a case of congenital femoral deficiency, you have to look at the limb from starting from the acetabulum down to the toes, because as we are mentioned earlier, lots of congenital deformities can be associated together. So your first look at the acetabulum. So the acetabulum is usually dysplastic. And if it is dysplastic, so it's usually the established dysplasia is usually progressive, which means that with time it gets worse.
And this is contrary to what we see in the media age where with growth, the acetabulum grow and become deeper and remodel into a better acetabular index. And then if you look at the thigh, usually you have muscle hyperplasia. Some of the muscles are absent, some of the muscles are hypoplastic, some of the muscles are contracted, and you have to assert that on a case by case basis. The other thing is the blood vessels are neurovascular.
Bundle is not in its usual anatomical landmarks, so it can be displaced immediately, laterally. So whenever you do surgery, you should be aware of that. If we come down to the knee so the knee can have a spectrum of disorders in cases of congenital femoral deficiencies, the patient may have hypoplastic lateral femoral condyle, and this will present to his general Falcon. The patient may have patella hyperplasia or may have cruciate ligament dysplasia or absence of the cruciate.
If we look at the tibia and fibula, we look for any tibia and fibula. And on the ankle, we should look for any evidence of partial coalition in case of any of coalition. The patient may have a Bowl. And so the joint where the top of the tails is acting like the Bowl and the rest of the ankle or the Mortis is acting like a socket. And the reason of having ball and socket joint because the subtalar joint is fused, so the ankle joint will take over and do the function of all the joints of the foot, sir, to take the shape of the ball and socket joint to give a wide range of movement and illustrate the kind of thing.
Then we have to look at the foot and the foot. You look for any absent trace, you look for any evidence of syntactically. And then as we have mentioned earlier, don't forget to look at the other side. Don't forget to look at the upper limb, look for any ulnar nerve deficiency, which is the most commonly associated deformity in the upper limb.
And you look for any other deformities in the upper limb like syndesmosis between the radius and the ulnar, radial Amelia. You look for any finger abnormalities, you look to examine the shoulder. You should examine all to the spine and look for any evidence of spine disease like congenital scoliosis.
So you have to look to the limb as a whole, and by looking to the limb as a whole, then you will complete the puzzle and reach the diagnosis at the end. OK, so. Uh, we were speaking now about how to approach a case, so whenever you have a case of congenital femoral deficiency, then you start with history taking, as we have mentioned earlier, you start with examination.
So if you keep the other deformities that we have, we have mentioned here. So this will be the phases of the clinical examination will be the pages of the investigation that we will go for later on. So an examination? Don't forget to examine for limitless discrepancy. So these patients have discrepancy. So you should assess how much discrepancy they have and roughly can.
You can say I can see that the severely short and the ankle joint is below or above the level of the knee joint on the other limb. But then you have to examine the hip and see if there is any contractions in the hip. If there is any flexion flexion deformity examined for the range of movement and then examined the knee, you look for any contractions, you look for any patellofemoral disorder, you look for any limb deficiency, you look for any deformity and then you examine the ankle and again, you look for any contracture deformity.
Seven the range of movement and it's very important to examine stability of the ankle as well. So we'll come back to each joint. So for the hip, look for any contraction like external rotation contractor. Fix it flexion deformity do the Thomas test as they do in adults. Look for any iliotibial band contracture do the egawa test.
If there is decreased abduction, this could be due to savara for the need, look for any fix flexion deformity, look for any program that will be associated with lateral femoral hyperplasia. Look for any ligament plaques to examine the patellofemoral joint. To look for any hypoplastic patella, look for any patellofemoral tracking.
These patients may have small patella and may have congenital patella dislocation. Examine the knee. Look for any meniscal clunk. They may have described meniscus and the ankle. Look for any evidence of iguanas. You have to commend if the ankle isn't vigorous or focused if the ankle looks normal. You should comment on any stability is unstable in focus or favors.
The ankle itself may look normal, but may have decreased or flexion due to abnormality on the distal tibial articular surface. Look at the level of the lateral medial. It may be more proximal than the mesial medial or at the same level, which is again, is no is abnormal. And again, you look at the foot. Look for any absent race. You look for any syntactically.
And don't forget the other things that we mentioned earlier, so look for the other limb. Look for the upper limb. Look for the spine, though neurological examination.
And then for imaging. We should have a full and robust your standing leg with the particular pointing forward. And if there is a limitless discrepancy, you can use a block to better balance the pelvis and will help with this full length X-ray to be most accurate. We can get a full limb lateral X-ray and maximum extension to look for any flexion deformities.
You can get a pelvis entrepris, 2x ray and supine position and check the acetabular index. You look for ossification of the femoral head. So the acetabular index, as we can see on the right hand screen, we can see it's measured between the helgeland line and a line along the roof of the acetabulum. It's normally around 20 degrees.
So you can ask for an MRI scan, and the role of MRI scan is to look for any proximal femoral femur that is not it's not ossified. So if the proximal femur does not show up in the x-ray, this means that it could be a cartilaginous, are not classified as yet or could be deficient. So the MRI will help with that. And look for any. You can ask for.
MRI is the need to look for any congenital ligament deficiency. Cities can has a lower role, actually, but you can. You can ask for MRI scan in older kids to look for it to assess the acetabulum and the pelvis. OK, so classification. Classification is a bit difficult.
Don't worry if you can't memorize it fully, we will go through it. But yeah, again, I know it's a bit complex, but it's got the treatment, so it would be better if you have just an idea about it. So these are historical classifications. They are not used anymore. So first, the etkin classification and then in 1959.
So it's basically classify the content of deficiency into those with good acetabulum. And these are a or B and those with poor Statlander or C and d, and this is so classified into a or b, according the appearance of the proximal FEMA. Uh, perhaps in 1983 made it a bit more complex, but again, it does not dictate which treatment you're going to give the patient.
So again, it's not in use right now. Yeah, so all these classifications are not satisfactory. The mostly used classification right now is the paleo classification, and it's done in the 1998. So for type one, so type 1 as intact femur and the hip and the knee are normal and by. OK and for type one, it is so classified into one a where the patient will have normal certification of the proximal femur.
But the abnormality in one egg could be in the neck shaft angle could be the whole femur slightly short, the neck shaft angle could be inverse, could be in retro virgin and in time be. The femur is well formed, but there is delayed or suffocation of the proximal femur, and the late ossification could be either only in the neck or could extend to the softer kanturk area or could be combined Nick and slap to contact types.
So this is type 1 a, as we can see in this picture, so the femur is normally formed, but it's slightly shorter than it should be. And type 1 be the proximal femur is still cartilaginous with the late ossification. Say and then type two, typekit has mobile, so the arthritis. And say the greater Toronto hypothesis is usually present and then he is usually more white.
So just to differentiate between one B and type 2. So in one B. The proximal femur is formed by still cartilaginous and there is delayed acidification of the proximal femur. But in type 2 a, there is missing part in the proximal femur, and this missing part is called pseudo arthritis, where these two parts of the femur are connected with fibrous tissue with no cartilage, no bone connection between these two.
And again, to type 2 is classified into type two, where the femoral head is mobile and the acetabulum type 2b, where the femoral head partially fused to the acetabulum and type c, where the femoral head and the sternum are completely fused or could be absent. Don't worry about the sub classification, but now we know that type 2 is a type where the proximal femur has to do astrologist and part of the femur.
These two parts of the female are not connected. Type 3 is a severe type where the patient will have their physical deficiency of the femur, but the greater the greater Toronto process is absent. So this how to differentiate type III from type 2. So again, it's classified into three areas where the best advice is there and the emotion is more than 45 degrees or typekit, where the distal Fischer said there.
But the emotion is less than 45 degree, and typekit is complete deficiency of distant femur or fusion of the distal female remnant to the tibia. Again, I would say, don't, don't, don't make things complex, guys. You don't have to memorize the subtypes of type III and type 4. Is this deficiency of the femur? But the proximal end is normal, as we can see on this photograph here.
So we speak generally about the principles of surgical treatment. They so. The step one and treatment, so if we say we have a short limp, so in order to lengthen the limb, you should have a stable hip and stable knee because if you don't have a stable joint above unstable joint below, you can't lengthen the femur. If you lengthen the femur while there is like so arthritis in the proximal femur.
So the lymph will not, will not grow, will not increase in length. But what will happen is the proximal femur will continue to migrate approximately at the site of the pseudo. So step one in management will be to stabilize the hip and stabilize the knee in case they are unstable. If they are stable, then you will skip step one and go to step three.
So how? How do you stabilize the hip? So at the age two to three years, you should go for operations to stabilize the hip, so the principles of management will be to release any contraction if there is any contraction of the flexes. The doctors, the elite band, you have to lengthen those. If there is any proximal femoral deformity, you have to do proximal femoral osteotomy to correct the errors, the retro virgin, the external rotation.
If there is acetabular dysplasia, you have to do pelvic osteotomy to correct the acetabular dysplasia. And if you do all these steps, this is called super hip procedure. Uh, so this is an example of a super hip procedure where we proximal femoral osteotomy is done together with a pelvic austerity to correct the acetabular dysplasia, and the osteotomy is fixed with a screw along the neck.
So the aim of the screw is to encourage the ossification of the proximal femur. There is whole cartilaginous at that age, and they put a rush spin in the Russian pan inside, interim medullary and augment this with a figure of eight tension band wire. However this kind of operation ended up with complications. So the most common complications was recurrent various or the proximal femur and delayed ossification of the proximal femur.
And since these operations, these kind of complications are reported frequently. The surgery developed into this new techniques of surgery. So to avoid the recurrent virus, we shifted to using fixed angle plate either pediatric DHS or plate plate or proximal femoral looking plate, and to enhance calcification of the proximal femur. You can add bone morphogenesis routine and to the proximal femur through a separate drill hole on top of the plate, as we can see here.
When you mentioned, so this is an example, so you can see on the left hand side, the proximal femur is not ossified, but with MRI scan was confirmed that the proximal femur is still there and it's cartilage. And so and the neck shaft angle isn't severe virus. So and then we can see here.
That the various is corrected and it's fixed with a fixed angle blade plate and one more photogenic protein was added through a separate drill hole on top of the blade plate. And we can see on the right here how the proximal femur. Now was defined and maintained the neck shaft angle. So when you be careful when you mentioned about the more photogenic routine in the exam because it's not FDA approved for use in children, so it's used in children and off-label fashion, and it's used in other indications like you are through the stevia and memorabilia.
The reason why is not. It's not FDA approved for use in children because it has a theoretical risk of oncogenes, particularly in pediatric cases. They believe that it induces the mesenchymal cells to transform into cells with malignant potential. However, this is a theoretical risk is not approved by a clinical study. And as we all know, it's used in adults for spinal fusion to decrease the risk of pseudo arthritis.
And there is an evidence I quoted the reference here, where they proved that it doesn't cause any increased risk of cancer. So we summarized how you stabilize the hip and we will figure out how you stabilize the knee. So basically the most common associated ligament deficiency is cruciate ligament deficiency. But even in those who have cruciate ligament deficiency, they tend not to have clinical intricacy and stability.
So even if there is isolated entrepreneurial instability in the knee, it's not necessarily an indication to the knee ligament reconstructive surgery in children with congenital femoral deficiency. The surgery can be done at the adolescents if it is symptomatic. And so if you have severe instability where there is no endpoint, then you can argue that you can do extra articular like Macintosh procedure to reconstruct the anterior cruciate ligament and during lengthening, you should be careful because when you lengthen the femur, if there is an associated cruciate ligament deficiency, this can cause progressive subluxation of the knee.
So you have to spend the knee and add the ring of the external fixator and at the proximal tibia and hinges at the knee level. So he's an example, so we can see here on the X-ray that the female on the right side is shorter than the female on the left side. OK, so this was lengthened by an external fixator over nail, and we can see that there is a ring spanning the knee to avoid progressive subluxation of the knee, and we can see the clinical photograph here where this ring at the knee below the knee level is attached to the main external fixator with hinges, which will allow the patient to do a range of movement and avoid knee stiffness.
OK, now we move to step two in the management, so we mentioned that step one will be stabilizing, help stabilize the knee and then step two will be serious lengthening of the femur and the tibia if needed. So the first step to plan you're lengthening is to predict the total ambulance discrepancy at skeletal maturity.
And the easiest way to do that nowadays is via the multiplier method. It's a smartphone mobile app that every person can download and use. So you should aim for around two to three lengthening and and each lengthening the lens in the FEMA about 5 to 8 centimeters. Don't worry about these numbers if you can't memorize. So the first lengthening is usually around the age three to four years.
And if you apply the rule of four, then you can do one episode of lengthening every four years. And so in those who are young, you can use external fixator, and in those who are approaching skeletal maturity, you can use the magnet nail. Uh, at the age of 12 to 15 years, consider a facilities of the longer limb if there is only a small amount of residual resemblance discrepancy.
And when you lengthen the femur, the complication that it could happen is an ifixit flexion deformity. This is because stretching of the bone and stretching of the soft tissue can cause flexion. Deformity of the knee should be careful about that or when after you remove the external fixator, the patient may have bending of the regenerate at the side of the lengthening because the original is usually is not as strong as the rest of the FEMA.
Or sometimes they may have free fracture at the side of the regenerate to avoid that. The patient should have excessive physiotherapy and be encouraged to move the knee while doing the lengthening and keep an eye on the new range of movement to avoid flexion deformity and to avoid bending of the femur or fracture. It's better either to lengthen over Mayo or to put an interim dollar in the femur after he finished lengthening.
So from what we can see here, so this is a congenitally short femur that was lengthened with an external fixator and then the patient presented with a fracture. The fracture could happen either at the bedside or at the side of the regenerate. So this is an example, so you can see here there's a congenital femoral deficiency that is told through this in the proximal femur, and this is a long legacy.
And we can see here after doing the Super hip procedure. So this would work through this healed and the deformity in the femur corrected with a good neck shaft angle and good acetabular index. And then the patient had his first episode of lengthening. We can see that the ambulance discovered the femur is longer than this picture. And this is the last episode of lengthening, and we can see that the external fixator across the knee with the ring below the knee level to avoid knee suffocation.
So step three, as we mentioned earlier, it's either him thesis or complete with his thesis. So you can't do contralateral thesis around the knee as an adjunct to equalize the balance discrepancy or if laterally, you can do him if it's your thesis, if the patient has got a bogus deformity of the knee. So this is another example, this patient had a super hip procedure and then had lengthening those external fixator.
And we can see here a ring across the knee to avoid subluxation at the knee. And after removal of the external fixator and entering the rod was inserted to avoid fracture at the regenerate site. And this is the last episode of lengthening, and it's done by a magnet needle and became this patient developed or had a fungus needed for empezo and the same X-ray.
You can see there is 8 plate on the medial side of the femur to correct any bogus deformity. So what happened is the hip is an unreconstructed, all like complete deficiency of the proximal femur. What are the options of management? This is a very complex situation like we see in Paley type three, when a person that can be done for these cases is rotational velocity, so rotational capacity is you rotate the whole limb 180 degrees, so the knee joint will act as a hip.
The ankle joint will act as a knee and then you can attach a prosthesis started from the ankle Downward. Let's see an example for that, so here we see. So you can see that now the whole limpets rotated 180 degree and the fibula is on the medial side now and the femur is fused to the alien. So this is an X-ray of a Paisley type III congenital femoral deficiency, and coincidentally, this patient has got multiple hereditary X-ray sources.
So they said just to zuma, to the pelvis. And this work has been done to him, so this is a rotation policy, we can see that the whole TVA is rotated 180 degrees. The female is fused to the Alien and a processes that attach it to the land. So here's the post picture, you can see the earlier femoral fusion.
So this is a clinical photograph after this operation, you can see that the patient, the knee joint access, the hip and flexion of the knee will be like flexion of the hip, but he will not have any range of reduction or reduction. And the ankle joint will act as a knee and then will attach oversees a prosthesis. The other option is to do pelvic support osteotomy, so pelvic support osteotomy is when you have deficiency of the femur, so you do an osteotomy at the level of the skin and move the proximal femur to be touching the skin, say, and then it's a complex operation.
I don't want to go into much detail, but it will allow the patient to do is kill weight bearing. Briefly, you mentioned about the knee in congenital limb deficiency, what you look for in the knee. So you look for any kind of deformity like Volker's deformity is the most common and the reason or the cause of the fungus deformity is when you have a hypoplastic lateral femoral epicondyle on the distal femur.
Or it could have a proximal tibial origin as well when the latter part of the proximal of the proximal tibia is delayed notification. We can see on this X-ray on the right how to measure the height of the lateral femoral epicondyle, and it's usually less if it is compared to the medial epicondyle, and it's less if it is compared to the lateral femoral condyle of the other intact limb.
Look for any knee instability the patient may have hyperplasia or at plaisir of the cruciate ligament, either the anterior or the anterior and posterior. Some of these patients may have fixed it. Flexion deformity look for any patellofemoral joint deformity like patella hyperplasia, lateral dislocation or trochlear dysplasia. I mentioned briefly about the cruciate ligaments dysplasia. Say this is a radiographic classification of cruciate ligament dysplasia.
You don't have to memorize the classification, but if we mentioned it briefly, then you can. You can know how to look on the X-ray. So in type one, the patient will have hypoplastic lateral tibial spine and normal medial tibial spine. And if this is the case, so this patient will have hyperplasia of the anterior cruciate ligament and normal posterior cruciate ligament. So in type t, the patient will have complete absence of the lateral tibial point and hypoplastic medial tibial spine.
So this patient in type 2 will have complete absence of the ACL and hypoplastic ACL in typekit three, where both the tibial spine are not there. So they are completely deficient and we can see that the femoral the femoral node is not developed as well. So this patient will have complete deficiency of the ACL and the PCL.
Yeah, so this is the classic matter, so it's difficult to apply this classification before the age of six years, and the reason behind that is most of the proximal will be cartilaginous at the age. Uh, yeah, so then we were speaking about media. So fibular Amelia is a condition where part or all of the fibula bone is either hypoplastic or dysplastic or is not there at all, and it could be associated with hyperplasia and dysplasia of the tibia.
And hyperplasia dysplasia or a plaisir of part or all of the foot? OK, so the incidence is quite rare as well, so it happens. The incidence ranges from 1 in 135,000 to 1 in 50,000. Uh, the majority of the cases are unilateral. It's usually negative family history. And in patients who have bilateral fibula Romania, it's usually autosomal dominant.
So let's look at what problem these patients will have, and then we will think about how we sort this out surgically. So the problems in patients with was fibula humiliate that they may have limb discrepancy, they will have foot and ankle deformities. And this is the most important. And this will be the focus on the treatment that we have. Foot and ankle deficiencies as well. They will have tibial deformity.
They will have journey home and knee instability. So regarding the limitless discrepancy it could be femoral, the patient could have associated congenital femoral deficiency, could be TVO could be coming from the foot. We discussed before you look for any knee deformity, any instability, the tibial deformity is usually enter and remedial bombing and the reason behind the intermediate bombing, and that the remnants of the fibula will act as a tether and prevent the tibia from growing normally.
So if the data at the site of the fibula. So it will be on the posterior lateral aspect of the tibia. So this will cut the tibia to grow into intra medial bearing. You may have a skin dimple at the apex of the tibial vowing. Like, in this case, so we can see the trivial spying and immediately there is a temple at the apex of the TV and deformity and the ankle is in vulgar deformity.
The ankle is usually unstable, and this is the most challenging part in the management of mania, so we can see on this photograph how the patient is standing with the ankle is in severe vulgar. And this is another photograph. And the reason behind the new focus is the fibula is not there, so they lose the lateral butters to the ankle, so the tail has nothing to support on the lateral side, so drifts into Volcker's classification again will mention it briefly.
So this is the pain classification that is most commonly used nowadays. So in type one, fibula at the ankle joint is stable and the fibula is just slightly shorter than the other side. It's usually mild time it may not be diagnosed, and the balance, discrepancy and maturity is less than 5 centimeters in type 2. They tend to have ankle Volker's, but it's dynamic so you can correct it passively.
The fibula is usually shorter than the tibia, and they usually have a bone and socket joint ankle joint, and they have limited dorsiflexion. And type three, there is a fixed angle equine fungus, so it is subdivided into three a wear. So though, three a where it's the anchor type. So the origin of the Vargas is coming from the ankle, the distal tibial a purposes is a broken item and Volker's or type 3b, where the origin of the fungus is in the subtalar joint.
So there is some coalition that is more United and various and equines and typekit 3c is confined ankle and subtalar type for the ankle deformity is an equine virus. The coalition was United in various position and there is associated distal tibial deformity and differential diagnosis of this case will be clubfoot.
Surgical treatment. So as you have mentioned before, a major the expected limitless discrepancy and maturity, and we lengthened the tibia. Uh, below centimeters more than 5 centimeters in young children can cause growth inhibition. So in the fema, you can learn some more than you can listen on the TV.
And again, we can apply the four year rule. So you do two to three lens openings and you start at the age of 4 and you lengthen your spirit, you're lengthening episode four year apart. Consider it prejudices of the long limp around adolescence. If there is associated congenital femoral deficiency, you can plan femoral lengthening either simultaneously or in a separate sessions. Uh, you have to do a surgical plan and then you decide according to the classification.
So in type one, the only problem is slight shortening of the lower limb, so you can plan for it, just tibia or fibula lengthening. You don't have to do any food fixation and the patient doesn't need any kind of food surgery. So in type typekit, that is ankle instability. And because of the ankle stability, as we have mentioned earlier, the fibula is short. This X-ray that is on the screen is a normal ankle X in a child where deficiencies of the distal fibula is at the level of the ankle joint.
OK we can see here this is the X-ray of a case of Romania, where the prices of the distal tibia is at the level of the distal tibial voices. So in typekit. So the fibula is short. So the way to stabilize the ankle is to shorten the tibia. So you relatively lengthen the fibula, so you do an operation culture, the operation where you do shortening realignment osteotomy of the distant tibia to correct the Vogel's and stabilize the ankle after this operation or together with the tibia, can be lengthened.
So this is how we do this operation, so you do immediate incision, t-shaped incision in the Persian. And then you do a small incision at the syndesmosis level, you separate the two from the fibula. And then you do the osteotomy, it's a pipeliner osteotomy to correct the deformity and the distal tibia.
And then after you do that, you shorten the tibia and you preliminary you fix it with wires. And the next is to be stabilized, the syndesmosis was a tightrope. And here an example, so you can see this is the three city before the operation and these are the X-rays. We can see that the five lights, it's short and the ankle is drifting into August, and this is the operatively image with the osteotomy and the shortening of the TV.
And this is after fixation. We can see after fixation that now the forces of the distal fibula is at the level of the ankle joint. In typekit, 3 and four, we do the Super ankle procedure, and super is not it's an acronym for systemic utilitarian procedure for extremity reconstruction. So in part 3 and four, lengthening is often combined with the soccer ankle procedure, so you do both at the same session.
So the principle of it is it's quite similar to the management of type t. So here we can see the deformity of the distant tibia. So the lateral distal tibial angle is less than 90 degree, and there is deformity of the anterior of the end of the anterolateral distal tibial angle. It's more than 90 degree, and this act as a mechanical block for the ankle, those reflection.
So to do this, to do this operation, we do two long incisions on the lateral aspect of the leg, one proximal and 1 distal. And the first step in the operation is to remove the fibula and leg. Any remnants of the fibula should be removed because these will act as a tether for the growth of the tibia and cause tibial deformity.
In order to do that, you have to dissect and protect the common ulnar nerve and superficial peroneal nerve. And then after you do that, he released a capsule of the ankle joint. And then you do osteotomy of the turtle coalition, so you correct the alignment of the calkin to the details, as we can see here.
To do that, you have to rotate the continuum and translate on the same time and then you fix the tail details in the ankle joint. And then after that, you do shortening realignment osteotomy to the distant TV. And this will be the last. They define a picture of what we have done, so it's a subtler osteotomy, distal tibial osteotomy and preliminary fixation.
And then you add a frame on the top and you put this ring here to allow the patient to do weight bearing. And if you plan to do lengthening on the same session, you can do a proximal osteotomy and you can lengthen on the same operation. So here's an example. So we can see here that the ankle isn't severe, but there is a skin temple at the apex of the tibial deformity.
It's untrue medial bearing of the tibia we can see on this X-ray ray. The femur looks pretty normal as compared to the other side. You can see the ventromedial bearing of the tibia the severe focus of the ankle. We can see on the lateral view here that the whole bones of the ankle are fused into one single bone. So this is during the operation.
Osteotomy and shortening of the tibia and osteotomy of the additional primary fixation with a wire external fixator is attached with lengthening and we can see the pictures of the lengthening and here the clinical picture. OK say amputation. Do we do amputation for fibula million? So when we do the amputation, the only indications for amputation, if the foot is severely deficient and the patient is unable to achieve blunt, degrade foot with reconstruction or could be apparent to us if they don't want to go through multiple extensive surgeries in that case, when you go for signs amputation.
So the reason we go for some amputation because it's more energy efficient than midfoot osteotomy in order to do Simon's amputation, the patient should have a patent posterior tibial artery because this gives the blood supply to the heel pad and the heel pad was act as a cushion to the stomach. Any questions? Thanks, Dylan.
Very comprehensive and I think reasonable, you know, you can, especially in this time of photographs for survivors and the clinicals, you could easily get one of those cases coming up. So I think what we're going to do next is we're going to launch the poll. We'll get you to answer as quickly as we can. And then I think we've only got one question in the chat at the moment, but we'll do the poll first, then we'll do questions and then we'll stop recording and is so the best answer is kill ectoderm rich and fibroblastic growth factor.
So we have 65% of the candidates answer correctly. The second question? It's almost half and half, actually. So the most common foot and ankle deformity and fibula Herminia is required to focus. So 4% to 7% answered this quickly, so correctly, the last question in the X-ray finding to diagnose cruciate ligament dysplasia, actually, all the answers are correct, so the correct answer will be all of the above.
We have 47% chance of this correctly. OK, thank you. Hi, brilliant. Come on, everyone. So in terms of questions, Natalie wants to ask pre axilo post axilo in relation to the access. It's quite a confusing description. Can you explain it a bit more, please?
So say in the upper limb, so the post axilo at the radio site, axilo at the ulnar nerve site. So when you have put it directly on the underside, you know, so this is a pretty active politically. On the other side, on the radio side, it's close to axilo and the same in the lower limb. OK, so the fact is, we can consider that the picture or the thumb with the access, so on the other side, on the lateral side of is post axilo or the other side.
I don't think there is like a role for that, David. OK I just memorize it like that, you know, literally. Does that help? I hope. Dan Schorr, Thanks. OK and then the last one to ask if festive returns are present is the non-functional foot. Sorry, say it again.
It's less than three times at present, so I presume only two toes present is a nonprofit. So this is actually a good question. So there is debate regarding and what's the minimum number of phrase you have in the foot to have functioning foot? OK, so it's because it's controversial. So some people believe that if you have less than three raise, so this cannot be planted.
Right foot or cannot like withstand the whole stresses of the body weight. But actually, this is not right, because sometimes the patient may have to raise about one of these is actually is like a fusion of two race. So imagine if you have a patient with the right to metatarsal, but they may have. One of them is very big and actually it's like a syntactically between the first and second metatarsal.
So if clinically you can, the patient can have planted. Right foot or you feel that the size of the foot is enough to withstand the whole weight of the body while walking. So this means that this limb can be reconstructed if you have a very small remnants of the foot that cannot be reconstructed. It's an indication for amputation, but not the absolute number of metatarsal or toes.
OK so it's based on the ability to be a weight bearing, weight bearing ability rather than the amount of race, so if they are so, you're unlikely to go down that route. But should they ask, say, the candidate should say it's a controversial topic, but the key aim is whether they're able to weight bear on that foot, correct? Yeah, yeah, I agree with you. There were no other questions in the chat.
Is there anything else that burning questions that people want to ask at all? Just just one more question. I mean, what? What is the appropriate age? Can you repeated thing again? What is the appropriate age for the operations. So for lengthening? You start at the age of four.
It's very difficult, you know, to lengthen a child like at the age of two or three due to lots of reasons. Actually, it's because the size of the limit is very small. The ability of the child to cope with an external fixator, it's very difficult. It's very difficult to convince a child that is 4-year-old to do new range of movement to avoid knee stiffness, you know? And so basically, if you are in other causes of limb blends discrepancy, we usually postpone the the, you know, the lengthening and the age of six or seven when the child is cooperative and can cope, can understand the instructions and do the physical exercise as well.
Because in congenital limb deficiency, you have to be multiple episodes of lengthening. So it's better to start early at the age of 4 and then you do one episode of lengthening every four year. So at four, eight 12. And then it's better not to do. It's difficult to do more than three episodes of lengthening. And if you end up having like $0.02 level discrepancy or centimeters discrepancy, you can do episodes of the longer side before skeletal maturity by one or two years, according to you calculated on the multiplier method and see when you can do that.
Other operations apart from the lengthening. So you do at the age of two to three years. So if you are going to do the Super ankle procedure, you can do it at the age of two to three years. You can do the Super hip procedure at the age of two to three years to prepare to you're lengthening at the age of four. OK, thank you. OK, that's great.
Thanks, John, so I'm going to stop the recording now, and I think.
Segment:0 .
Good evening, everyone.
My name is KneeKG. And welcome to our webinar this evening, which is on congenital limb deficiencies by John mean John works at the Evelina Children's Hospital. Ernie Foster's exam in November 2020. So I'm sure he's going to make this complex topic very easy for us all to understand coming up for the exams. I'd like to welcome David, who's my colleague, teaching program convenor, for tonight's session.
So just some general housekeeping. We're not too big this evening, but I'll ask you all to stay muted unless you're talking or contributing just to reduce background noise. I'll just share what the program for this evening is. OK, so we're going to have our lecture on congenital limb deficiencies, and if you have any questions, we'd ask you to write them in the chat and we'll ask them after the mock polls because some of those questions might be answered for the interviews.
After we've done the questions of the mcu's, then we will do some hot seat Bible practice with a maximum of three candidates. So if you're interested in the vyver, if you could raise your hand or send a message to myself or David so we can allocate those for you, I do realize that the exams are only two weeks away, so it's very stressful time. But this is the best practice that you can have is to be better to make mistakes in this part than in the actual exam itself.
OK, so. We do have some upcoming courses coming up, so we've got the mock exam course. And we've got dates throughout the year. We've got the case discussion's course at various points as well, and we're also running our first basic sciences for the folks this weekend. You can keep an eye on any relevant courses that are coming up on our website, which is orthopedic Academy.
Co.uk and we are also available on social media. And so at the moment, I'm recording this session, and it will be available on our YouTube channel in a couple of days. So don't panic if you missed any part of it or stop the recording when we start the rivers to protect your identities. And without any further ado, I'm going to hand you over to John for our lecture.
Oh, Thanks a lot, KneeKG. Good evening, everyone. Actually, before I start, I just would like to thank everyone in this fantastic group. I passed my exam in November 2020, and I have to say without the help and the support I got from this group, I wouldn't have passed my exam. And so Thanks.
So everyone helped me to pass my exam for those who don't know me. I'm John and I'm a senior fellow at the London general hospital, and I'm interested in pediatric orthopedics. So today we're going to speak about congenital limb deficiency. So, so basically, this is a tough topic. Actually, it's a very complex cases. The reason we are approaching that topic to do teaching for today is because sometimes it come up in the exam.
So I thought it would be better if we have a resume of the topic and how to answer the questions in the exam. I will try and keep it simple. We will not go into depth in these complex situations. You are not, you know, you don't need to be expert in managing these cases and the exam. OK, so let's just start.
So today. OK, so today, what are we going to do? So first, we will discuss like a general approach to any AIDS case that you met in the exam, either in Dubai or in the clinical. And then we will start our topic by speaking about the embryology. I will speak about the Lempert development.
And then we will discuss congenital femoral deficiency. And then we will discuss what happens in the knee in any case of congenital limb deficiency and what are the things that you should look for? And we will speak about fibular at the last thing in our teaching today. So let's go. Uh, but first, I would like to start with this clinical approach to pediatric cases.
Actually, I use this approach in while I was preparing for the exam and I thought it's useful. So for any case of pediatrics, you start by taking history. So in history, you usually ask about the complaint, the complaint. In other cases, it's usually pain in children. It may not be pain. And if it is pain, the key to you is this pain is significant or knot is to ask if the pain is affecting the patient function or not.
Like, how do you do in and body and their sport participation and all these kind of things? And then you focus on the present history and the present history. You should ask the patient if he had any previous treatment, his condition. And then in the past history, we all know about the prenatal, perinatal postnatal history and the postnatal.
We ask about the developmental milestones generally. And it is very important to ask about the family history in any case, in any pediatric case, especially for those conditions who have a genetic background or usually have a positive family history. And then you should ask if there is other system of like if you have a patient who is a congenital limb deficiency, you need to know if he has congenital heart disease.
Arena, Genesis or anything of this associations, so you should ask the patient if you ask the parents if they are seeing other specialties, if they are seeing the ophthalmology, for example, for any ophthalmological problems, if they are seeing any other speciality endocrinology, pediatric neurology and all these kind of things. And then when it comes to examination for any pediatric case, you should examine the patient from the tip to the toe.
So in other cases, if your, for example, you have a hip case, so you usually discuss examination of the hip. And then after discussing examination of the hip, you mentioned roughly, you mentioned briefly the rest of the general examination, but indeed it's better to have a system in your mind, whatever the case, to try and check on these kind of things. So we're starting from tip to toe. So it mentioned about examination of the head in the head, you look for any abnormal phases that the patient have any syndromic faces.
Does you have or have any abnormal shape of the head like plager? Carefully microcephaly? You look at the eye. Is there any evidence that blows clearer that can point to a surgeon slap perfecta? You look at the development of the teeth and all these things. And then in every pediatric case, you should check the spine and need quick neurological examination.
And don't forget to examine the upper limb and end the lower limb. You should look for any evidence of coronal or sagittal plane deformity. You look for any rotational abnormality, you look for any length discrepancy. So to resume that, it's in every pediatric case, you should say I will look for the face. I look for any abnormal flashes you would look for.
I need to examine the spine. I would look for any neurological deficit. I would. I have to examine the upper limb. I examined the lower limb by you look for any such terraplane deformity and irritation of abnormality. Any length discrepancy. Regarding investigations, it's according to each condition, sometimes in the X-ray city, the MRI is, but my father is in pediatric cases, always ask for more views because as we mentioned earlier, if you are seeing a patient to have a lower limb deficiency, so he may have a associated condition in the upper limit, but you may have a condition at this point and whenever you are also expecting something a little that, please ask for more investigations.
And if you are, if you have a case like, for example, this abnormality the ankle, then you have to check the whole leg. OK hey, next, you're going to speak about embryology. I have proclaimed please. OK, so let's start.
So we all know that embryology is just the heartbeat. You know, it's like a dry topic, and I usually find it difficult. So what are you trying to do today? I try and make it as simple as I can. And so usually the Lempert and very Genesis stars between the ages of 4 to eight weeks. And it starts at an outgrowth of mesoderm into the overlying ectoderm.
So this is the embryo. So this is the metal dog and the Black line that is covering it at the ectoderm. Ok? the mesoderm, the outgrowth and form. The Lempert is formed of two types two layers of mesoderm. The first is somatic mesoderm that will give rise to the muscle tissue later on, and the lateral Mason mesoderm that will give rise to the bone and cartilage.
So when we describe the development of the Lempert, we usually. Describe it in 3 axes, and each axis has a different molecular pathway, and to be honest, this I know that it's a bit difficult and it's a bit difficult to memorize the name of the genes and the axes and everything, but it does come up in the exam and does come up as an empty key in the first part.
And it can be asked, you know, in the second part and the 5 and the basic science table. OK, so the first asks, is it the proximal to distal axis, so the proximal to distal axis growth of the limb, but is organized by the epicondyle ectoderm ridge? So this ethical ectoderm ridge is a thickening of the ectoderm overlying the mesoderm of the limb, but it secretes fibroblastic growth factor.
And this fibroblast, the fibroblastic growth factor, stimulate the underlying mesodermal cells to differentiate into specific types of cells that give the Lempert its shape. Eventually, the effect of removal of the epicondyle dermal rate is growth the rest of the Lempert, which will present later as a congenital amputation of the limb or transverse deficiency.
Uh, so we can see on this photograph this is an example of a congenital amputation or transverse deficiency of the limb part, and it resulted from removal of the epicondyle ectoderm or rage or damage to the epicondyle ectoderm ridge. The other reason? Uh, if you have a typekit plantation of the epicondyle ectoderm road rage, it'll cause extra them.
It's not a common kind of thing to see. Uh, here you can see an example of that, where you can see the two feet are growing in one of them is because the power of the cells of the epicondyle to the marriage are implanted far away from where it should be. So the typical ectoderm marriage after a while will have entered Interdigital necrosis, and this will result in the separation of the fingers or the toes.
If this fails to happen, this will result in syntactically. Here's an example. It's a photo of the Fort where you can see there is syntactically in the second and third toes. So basically in the exam and the basic science table, we can't get any of these pictures. So if you see any of these pictures, don't panic because you will not be asked about this specific condition.
But all the discussion will go into where the limbo develops. And I have to say that this picture is a transverse deficiency of the limb actually is a picture that I had on my exam. So Uh, now we speak about the second access in the Andrew posterior axis. So if you look at the picture on the right hand side of the screen, so this is the embryo.
And this is the limb. But and this square is just a magnification of the limb pop. So let me. So from what we can see here, the limb pod has a both and posterior border. So it's a quite confusing because this empty border will give rise to the radial side of the forearm lateral and this posterior border will give rise to the ulnar nerve border of the forearm.
So these this entry procedure access development is regulated by the zone of fertilizing activity. So cells cells on the posterior aspect of the Lempert get thickened and active and regulate the differentiation and to enter and posterior axis. So this is regulated by the sonic hedgehog gene and the Indian hedgehog gene to make things more complex.
So the sonic hedgehog gene also contribute to proximal to distal limb, but development through a feedback loop to the epicondyle ectoderm ridge, so you can see that on the right hand side of the screen. So this is the typical ectoderm ridge. They said the zone of polarizing activity. On the posterior boarder of the. So the ethical ectoderm rich regulate proximal to distal growth through secretion of the fibroblastic growth factor.
You can see that the zone of paralyzing activity secrete or activate the sonic hedgehog gene, which regulate the development of the limb body and the Anthropocene axis, and this regulate again the proximal to distal development through a negative feedback loop. Transplantation of the zone of paralyzing activity from posterior to anterior aspect will cause myriad duplication of the ulnar aspect of the hand.
I know that it's a bit tough, but we have to go through it. Let's continue. So this is the last access its dorsal to ventral axis. So growth through the dorsal and ventral aspect or differentiation into dorsal and ventral aspect is regulated by the wingless type signaling pathway, and it comes from the dorsal ectoderm.
Uh, so they signaling pathway induce the underlying mesoderm to develop characteristics of the docile structures and because the activity of this pathway is blocked on the ventral aspect. So this will allow the ventral mesoderm to differentiate into ventral structures, say the w.a. pathway also contribute to regulation of the S gene.
And this reflects the complex interaction and coordination among the 3D pathways responsible for limb development. So and the pictures on the bottom of the screen, we can see that this is the dorsal aspect where we have the w.a. gene active and this is typical ectoderm ridge where we have the fibroblastic growth factor. And they said the about where we have the SH gene.
And if you and you can draw the example drawing, look at this one, it can help you memorize these kind of things. So I hope that no one of you guys now is covering his AIIS regarding the rest of the teaching today. We're going to think that are more clinical and you'll find it more interesting. So for congenital limb deficiency, we will start by congenital femoral deficiency, so congenital femoral deficiency is the new term, and it now replaced proximal femoral focal deficiency because it includes a wider spectrum of pathology.
So demographics of congenital femoral deficiency, so the incidence it's a bit higher, so it's one case in every 50,000 people, the male to female ratio is one two. It's usually unilateral in 85% to 90% of the cases, so it's rarely bilateral when unilateral, the right femur is usually most commonly affected.
It can be diagnosed by in utero ultrasounds, where they can measure the length of the femur to reflect on the gestational age. So in case of discrepancy between two sides or in case the discrepancy of both femoral regarding the rest of the milestones are measured in utero ultrasound, we can make an earlier diagnosis. So as we have mentioned earlier, the congenital femoral deficiency is a spectrum of disease.
So it starts from having a normal but slightly short femur to having FEMA with just abnormal Nick shaft and go to FEMA with severe available. And sometimes the second step in the spectrum is having through the proximal femur or at the end of the spectrum, you have a complete absence of the proximal femur and this is called proximal femoral focal deficiency.
So the term congenital femoral deficiency reflects better the whole spectrum of disease. So let's start. So are you going to mention about the non-federal structures? Sorry, just listening. The non-federal structures that are also involved in congenital femoral deficiency.
So whenever you have a case of congenital femoral deficiency, you have to look at the limb from starting from the acetabulum down to the toes, because as we are mentioned earlier, lots of congenital deformities can be associated together. So your first look at the acetabulum. So the acetabulum is usually dysplastic. And if it is dysplastic, so it's usually the established dysplasia is usually progressive, which means that with time it gets worse.
And this is contrary to what we see in the media age where with growth, the acetabulum grow and become deeper and remodel into a better acetabular index. And then if you look at the thigh, usually you have muscle hyperplasia. Some of the muscles are absent, some of the muscles are hypoplastic, some of the muscles are contracted, and you have to assert that on a case by case basis. The other thing is the blood vessels are neurovascular.
Bundle is not in its usual anatomical landmarks, so it can be displaced immediately, laterally. So whenever you do surgery, you should be aware of that. If we come down to the knee so the knee can have a spectrum of disorders in cases of congenital femoral deficiencies, the patient may have hypoplastic lateral femoral condyle, and this will present to his general Falcon. The patient may have patella hyperplasia or may have cruciate ligament dysplasia or absence of the cruciate.
If we look at the tibia and fibula, we look for any tibia and fibula. And on the ankle, we should look for any evidence of partial coalition in case of any of coalition. The patient may have a Bowl. And so the joint where the top of the tails is acting like the Bowl and the rest of the ankle or the Mortis is acting like a socket. And the reason of having ball and socket joint because the subtalar joint is fused, so the ankle joint will take over and do the function of all the joints of the foot, sir, to take the shape of the ball and socket joint to give a wide range of movement and illustrate the kind of thing.
Then we have to look at the foot and the foot. You look for any absent trace, you look for any evidence of syntactically. And then as we have mentioned earlier, don't forget to look at the other side. Don't forget to look at the upper limb, look for any ulnar nerve deficiency, which is the most commonly associated deformity in the upper limb.
And you look for any other deformities in the upper limb like syndesmosis between the radius and the ulnar, radial Amelia. You look for any finger abnormalities, you look to examine the shoulder. You should examine all to the spine and look for any evidence of spine disease like congenital scoliosis.
So you have to look to the limb as a whole, and by looking to the limb as a whole, then you will complete the puzzle and reach the diagnosis at the end. OK, so. Uh, we were speaking now about how to approach a case, so whenever you have a case of congenital femoral deficiency, then you start with history taking, as we have mentioned earlier, you start with examination.
So if you keep the other deformities that we have, we have mentioned here. So this will be the phases of the clinical examination will be the pages of the investigation that we will go for later on. So an examination? Don't forget to examine for limitless discrepancy. So these patients have discrepancy. So you should assess how much discrepancy they have and roughly can.
You can say I can see that the severely short and the ankle joint is below or above the level of the knee joint on the other limb. But then you have to examine the hip and see if there is any contractions in the hip. If there is any flexion flexion deformity examined for the range of movement and then examined the knee, you look for any contractions, you look for any patellofemoral disorder, you look for any limb deficiency, you look for any deformity and then you examine the ankle and again, you look for any contracture deformity.
Seven the range of movement and it's very important to examine stability of the ankle as well. So we'll come back to each joint. So for the hip, look for any contraction like external rotation contractor. Fix it flexion deformity do the Thomas test as they do in adults. Look for any iliotibial band contracture do the egawa test.
If there is decreased abduction, this could be due to savara for the need, look for any fix flexion deformity, look for any program that will be associated with lateral femoral hyperplasia. Look for any ligament plaques to examine the patellofemoral joint. To look for any hypoplastic patella, look for any patellofemoral tracking.
These patients may have small patella and may have congenital patella dislocation. Examine the knee. Look for any meniscal clunk. They may have described meniscus and the ankle. Look for any evidence of iguanas. You have to commend if the ankle isn't vigorous or focused if the ankle looks normal. You should comment on any stability is unstable in focus or favors.
The ankle itself may look normal, but may have decreased or flexion due to abnormality on the distal tibial articular surface. Look at the level of the lateral medial. It may be more proximal than the mesial medial or at the same level, which is again, is no is abnormal. And again, you look at the foot. Look for any absent race. You look for any syntactically.
And don't forget the other things that we mentioned earlier, so look for the other limb. Look for the upper limb. Look for the spine, though neurological examination.
And then for imaging. We should have a full and robust your standing leg with the particular pointing forward. And if there is a limitless discrepancy, you can use a block to better balance the pelvis and will help with this full length X-ray to be most accurate. We can get a full limb lateral X-ray and maximum extension to look for any flexion deformities.
You can get a pelvis entrepris, 2x ray and supine position and check the acetabular index. You look for ossification of the femoral head. So the acetabular index, as we can see on the right hand screen, we can see it's measured between the helgeland line and a line along the roof of the acetabulum. It's normally around 20 degrees.
So you can ask for an MRI scan, and the role of MRI scan is to look for any proximal femoral femur that is not it's not ossified. So if the proximal femur does not show up in the x-ray, this means that it could be a cartilaginous, are not classified as yet or could be deficient. So the MRI will help with that. And look for any. You can ask for.
MRI is the need to look for any congenital ligament deficiency. Cities can has a lower role, actually, but you can. You can ask for MRI scan in older kids to look for it to assess the acetabulum and the pelvis. OK, so classification. Classification is a bit difficult.
Don't worry if you can't memorize it fully, we will go through it. But yeah, again, I know it's a bit complex, but it's got the treatment, so it would be better if you have just an idea about it. So these are historical classifications. They are not used anymore. So first, the etkin classification and then in 1959.
So it's basically classify the content of deficiency into those with good acetabulum. And these are a or B and those with poor Statlander or C and d, and this is so classified into a or b, according the appearance of the proximal FEMA. Uh, perhaps in 1983 made it a bit more complex, but again, it does not dictate which treatment you're going to give the patient.
So again, it's not in use right now. Yeah, so all these classifications are not satisfactory. The mostly used classification right now is the paleo classification, and it's done in the 1998. So for type one, so type 1 as intact femur and the hip and the knee are normal and by. OK and for type one, it is so classified into one a where the patient will have normal certification of the proximal femur.
But the abnormality in one egg could be in the neck shaft angle could be the whole femur slightly short, the neck shaft angle could be inverse, could be in retro virgin and in time be. The femur is well formed, but there is delayed or suffocation of the proximal femur, and the late ossification could be either only in the neck or could extend to the softer kanturk area or could be combined Nick and slap to contact types.
So this is type 1 a, as we can see in this picture, so the femur is normally formed, but it's slightly shorter than it should be. And type 1 be the proximal femur is still cartilaginous with the late ossification. Say and then type two, typekit has mobile, so the arthritis. And say the greater Toronto hypothesis is usually present and then he is usually more white.
So just to differentiate between one B and type 2. So in one B. The proximal femur is formed by still cartilaginous and there is delayed acidification of the proximal femur. But in type 2 a, there is missing part in the proximal femur, and this missing part is called pseudo arthritis, where these two parts of the femur are connected with fibrous tissue with no cartilage, no bone connection between these two.
And again, to type 2 is classified into type two, where the femoral head is mobile and the acetabulum type 2b, where the femoral head partially fused to the acetabulum and type c, where the femoral head and the sternum are completely fused or could be absent. Don't worry about the sub classification, but now we know that type 2 is a type where the proximal femur has to do astrologist and part of the femur.
These two parts of the female are not connected. Type 3 is a severe type where the patient will have their physical deficiency of the femur, but the greater the greater Toronto process is absent. So this how to differentiate type III from type 2. So again, it's classified into three areas where the best advice is there and the emotion is more than 45 degrees or typekit, where the distal Fischer said there.
But the emotion is less than 45 degree, and typekit is complete deficiency of distant femur or fusion of the distal female remnant to the tibia. Again, I would say, don't, don't, don't make things complex, guys. You don't have to memorize the subtypes of type III and type 4. Is this deficiency of the femur? But the proximal end is normal, as we can see on this photograph here.
So we speak generally about the principles of surgical treatment. They so. The step one and treatment, so if we say we have a short limp, so in order to lengthen the limb, you should have a stable hip and stable knee because if you don't have a stable joint above unstable joint below, you can't lengthen the femur. If you lengthen the femur while there is like so arthritis in the proximal femur.
So the lymph will not, will not grow, will not increase in length. But what will happen is the proximal femur will continue to migrate approximately at the site of the pseudo. So step one in management will be to stabilize the hip and stabilize the knee in case they are unstable. If they are stable, then you will skip step one and go to step three.
So how? How do you stabilize the hip? So at the age two to three years, you should go for operations to stabilize the hip, so the principles of management will be to release any contraction if there is any contraction of the flexes. The doctors, the elite band, you have to lengthen those. If there is any proximal femoral deformity, you have to do proximal femoral osteotomy to correct the errors, the retro virgin, the external rotation.
If there is acetabular dysplasia, you have to do pelvic osteotomy to correct the acetabular dysplasia. And if you do all these steps, this is called super hip procedure. Uh, so this is an example of a super hip procedure where we proximal femoral osteotomy is done together with a pelvic austerity to correct the acetabular dysplasia, and the osteotomy is fixed with a screw along the neck.
So the aim of the screw is to encourage the ossification of the proximal femur. There is whole cartilaginous at that age, and they put a rush spin in the Russian pan inside, interim medullary and augment this with a figure of eight tension band wire. However this kind of operation ended up with complications. So the most common complications was recurrent various or the proximal femur and delayed ossification of the proximal femur.
And since these operations, these kind of complications are reported frequently. The surgery developed into this new techniques of surgery. So to avoid the recurrent virus, we shifted to using fixed angle plate either pediatric DHS or plate plate or proximal femoral looking plate, and to enhance calcification of the proximal femur. You can add bone morphogenesis routine and to the proximal femur through a separate drill hole on top of the plate, as we can see here.
When you mentioned, so this is an example, so you can see on the left hand side, the proximal femur is not ossified, but with MRI scan was confirmed that the proximal femur is still there and it's cartilage. And so and the neck shaft angle isn't severe virus. So and then we can see here.
That the various is corrected and it's fixed with a fixed angle blade plate and one more photogenic protein was added through a separate drill hole on top of the blade plate. And we can see on the right here how the proximal femur. Now was defined and maintained the neck shaft angle. So when you be careful when you mentioned about the more photogenic routine in the exam because it's not FDA approved for use in children, so it's used in children and off-label fashion, and it's used in other indications like you are through the stevia and memorabilia.
The reason why is not. It's not FDA approved for use in children because it has a theoretical risk of oncogenes, particularly in pediatric cases. They believe that it induces the mesenchymal cells to transform into cells with malignant potential. However, this is a theoretical risk is not approved by a clinical study. And as we all know, it's used in adults for spinal fusion to decrease the risk of pseudo arthritis.
And there is an evidence I quoted the reference here, where they proved that it doesn't cause any increased risk of cancer. So we summarized how you stabilize the hip and we will figure out how you stabilize the knee. So basically the most common associated ligament deficiency is cruciate ligament deficiency. But even in those who have cruciate ligament deficiency, they tend not to have clinical intricacy and stability.
So even if there is isolated entrepreneurial instability in the knee, it's not necessarily an indication to the knee ligament reconstructive surgery in children with congenital femoral deficiency. The surgery can be done at the adolescents if it is symptomatic. And so if you have severe instability where there is no endpoint, then you can argue that you can do extra articular like Macintosh procedure to reconstruct the anterior cruciate ligament and during lengthening, you should be careful because when you lengthen the femur, if there is an associated cruciate ligament deficiency, this can cause progressive subluxation of the knee.
So you have to spend the knee and add the ring of the external fixator and at the proximal tibia and hinges at the knee level. So he's an example, so we can see here on the X-ray that the female on the right side is shorter than the female on the left side. OK, so this was lengthened by an external fixator over nail, and we can see that there is a ring spanning the knee to avoid progressive subluxation of the knee, and we can see the clinical photograph here where this ring at the knee below the knee level is attached to the main external fixator with hinges, which will allow the patient to do a range of movement and avoid knee stiffness.
OK, now we move to step two in the management, so we mentioned that step one will be stabilizing, help stabilize the knee and then step two will be serious lengthening of the femur and the tibia if needed. So the first step to plan you're lengthening is to predict the total ambulance discrepancy at skeletal maturity.
And the easiest way to do that nowadays is via the multiplier method. It's a smartphone mobile app that every person can download and use. So you should aim for around two to three lengthening and and each lengthening the lens in the FEMA about 5 to 8 centimeters. Don't worry about these numbers if you can't memorize. So the first lengthening is usually around the age three to four years.
And if you apply the rule of four, then you can do one episode of lengthening every four years. And so in those who are young, you can use external fixator, and in those who are approaching skeletal maturity, you can use the magnet nail. Uh, at the age of 12 to 15 years, consider a facilities of the longer limb if there is only a small amount of residual resemblance discrepancy.
And when you lengthen the femur, the complication that it could happen is an ifixit flexion deformity. This is because stretching of the bone and stretching of the soft tissue can cause flexion. Deformity of the knee should be careful about that or when after you remove the external fixator, the patient may have bending of the regenerate at the side of the lengthening because the original is usually is not as strong as the rest of the FEMA.
Or sometimes they may have free fracture at the side of the regenerate to avoid that. The patient should have excessive physiotherapy and be encouraged to move the knee while doing the lengthening and keep an eye on the new range of movement to avoid flexion deformity and to avoid bending of the femur or fracture. It's better either to lengthen over Mayo or to put an interim dollar in the femur after he finished lengthening.
So from what we can see here, so this is a congenitally short femur that was lengthened with an external fixator and then the patient presented with a fracture. The fracture could happen either at the bedside or at the side of the regenerate. So this is an example, so you can see here there's a congenital femoral deficiency that is told through this in the proximal femur, and this is a long legacy.
And we can see here after doing the Super hip procedure. So this would work through this healed and the deformity in the femur corrected with a good neck shaft angle and good acetabular index. And then the patient had his first episode of lengthening. We can see that the ambulance discovered the femur is longer than this picture. And this is the last episode of lengthening, and we can see that the external fixator across the knee with the ring below the knee level to avoid knee suffocation.
So step three, as we mentioned earlier, it's either him thesis or complete with his thesis. So you can't do contralateral thesis around the knee as an adjunct to equalize the balance discrepancy or if laterally, you can do him if it's your thesis, if the patient has got a bogus deformity of the knee. So this is another example, this patient had a super hip procedure and then had lengthening those external fixator.
And we can see here a ring across the knee to avoid subluxation at the knee. And after removal of the external fixator and entering the rod was inserted to avoid fracture at the regenerate site. And this is the last episode of lengthening, and it's done by a magnet needle and became this patient developed or had a fungus needed for empezo and the same X-ray.
You can see there is 8 plate on the medial side of the femur to correct any bogus deformity. So what happened is the hip is an unreconstructed, all like complete deficiency of the proximal femur. What are the options of management? This is a very complex situation like we see in Paley type three, when a person that can be done for these cases is rotational velocity, so rotational capacity is you rotate the whole limb 180 degrees, so the knee joint will act as a hip.
The ankle joint will act as a knee and then you can attach a prosthesis started from the ankle Downward. Let's see an example for that, so here we see. So you can see that now the whole limpets rotated 180 degree and the fibula is on the medial side now and the femur is fused to the alien. So this is an X-ray of a Paisley type III congenital femoral deficiency, and coincidentally, this patient has got multiple hereditary X-ray sources.
So they said just to zuma, to the pelvis. And this work has been done to him, so this is a rotation policy, we can see that the whole TVA is rotated 180 degrees. The female is fused to the Alien and a processes that attach it to the land. So here's the post picture, you can see the earlier femoral fusion.
So this is a clinical photograph after this operation, you can see that the patient, the knee joint access, the hip and flexion of the knee will be like flexion of the hip, but he will not have any range of reduction or reduction. And the ankle joint will act as a knee and then will attach oversees a prosthesis. The other option is to do pelvic support osteotomy, so pelvic support osteotomy is when you have deficiency of the femur, so you do an osteotomy at the level of the skin and move the proximal femur to be touching the skin, say, and then it's a complex operation.
I don't want to go into much detail, but it will allow the patient to do is kill weight bearing. Briefly, you mentioned about the knee in congenital limb deficiency, what you look for in the knee. So you look for any kind of deformity like Volker's deformity is the most common and the reason or the cause of the fungus deformity is when you have a hypoplastic lateral femoral epicondyle on the distal femur.
Or it could have a proximal tibial origin as well when the latter part of the proximal of the proximal tibia is delayed notification. We can see on this X-ray on the right how to measure the height of the lateral femoral epicondyle, and it's usually less if it is compared to the medial epicondyle, and it's less if it is compared to the lateral femoral condyle of the other intact limb.
Look for any knee instability the patient may have hyperplasia or at plaisir of the cruciate ligament, either the anterior or the anterior and posterior. Some of these patients may have fixed it. Flexion deformity look for any patellofemoral joint deformity like patella hyperplasia, lateral dislocation or trochlear dysplasia. I mentioned briefly about the cruciate ligaments dysplasia. Say this is a radiographic classification of cruciate ligament dysplasia.
You don't have to memorize the classification, but if we mentioned it briefly, then you can. You can know how to look on the X-ray. So in type one, the patient will have hypoplastic lateral tibial spine and normal medial tibial spine. And if this is the case, so this patient will have hyperplasia of the anterior cruciate ligament and normal posterior cruciate ligament. So in type t, the patient will have complete absence of the lateral tibial point and hypoplastic medial tibial spine.
So this patient in type 2 will have complete absence of the ACL and hypoplastic ACL in typekit three, where both the tibial spine are not there. So they are completely deficient and we can see that the femoral the femoral node is not developed as well. So this patient will have complete deficiency of the ACL and the PCL.
Yeah, so this is the classic matter, so it's difficult to apply this classification before the age of six years, and the reason behind that is most of the proximal will be cartilaginous at the age. Uh, yeah, so then we were speaking about media. So fibular Amelia is a condition where part or all of the fibula bone is either hypoplastic or dysplastic or is not there at all, and it could be associated with hyperplasia and dysplasia of the tibia.
And hyperplasia dysplasia or a plaisir of part or all of the foot? OK, so the incidence is quite rare as well, so it happens. The incidence ranges from 1 in 135,000 to 1 in 50,000. Uh, the majority of the cases are unilateral. It's usually negative family history. And in patients who have bilateral fibula Romania, it's usually autosomal dominant.
So let's look at what problem these patients will have, and then we will think about how we sort this out surgically. So the problems in patients with was fibula humiliate that they may have limb discrepancy, they will have foot and ankle deformities. And this is the most important. And this will be the focus on the treatment that we have. Foot and ankle deficiencies as well. They will have tibial deformity.
They will have journey home and knee instability. So regarding the limitless discrepancy it could be femoral, the patient could have associated congenital femoral deficiency, could be TVO could be coming from the foot. We discussed before you look for any knee deformity, any instability, the tibial deformity is usually enter and remedial bombing and the reason behind the intermediate bombing, and that the remnants of the fibula will act as a tether and prevent the tibia from growing normally.
So if the data at the site of the fibula. So it will be on the posterior lateral aspect of the tibia. So this will cut the tibia to grow into intra medial bearing. You may have a skin dimple at the apex of the tibial vowing. Like, in this case, so we can see the trivial spying and immediately there is a temple at the apex of the TV and deformity and the ankle is in vulgar deformity.
The ankle is usually unstable, and this is the most challenging part in the management of mania, so we can see on this photograph how the patient is standing with the ankle is in severe vulgar. And this is another photograph. And the reason behind the new focus is the fibula is not there, so they lose the lateral butters to the ankle, so the tail has nothing to support on the lateral side, so drifts into Volcker's classification again will mention it briefly.
So this is the pain classification that is most commonly used nowadays. So in type one, fibula at the ankle joint is stable and the fibula is just slightly shorter than the other side. It's usually mild time it may not be diagnosed, and the balance, discrepancy and maturity is less than 5 centimeters in type 2. They tend to have ankle Volker's, but it's dynamic so you can correct it passively.
The fibula is usually shorter than the tibia, and they usually have a bone and socket joint ankle joint, and they have limited dorsiflexion. And type three, there is a fixed angle equine fungus, so it is subdivided into three a wear. So though, three a where it's the anchor type. So the origin of the Vargas is coming from the ankle, the distal tibial a purposes is a broken item and Volker's or type 3b, where the origin of the fungus is in the subtalar joint.
So there is some coalition that is more United and various and equines and typekit 3c is confined ankle and subtalar type for the ankle deformity is an equine virus. The coalition was United in various position and there is associated distal tibial deformity and differential diagnosis of this case will be clubfoot.
Surgical treatment. So as you have mentioned before, a major the expected limitless discrepancy and maturity, and we lengthened the tibia. Uh, below centimeters more than 5 centimeters in young children can cause growth inhibition. So in the fema, you can learn some more than you can listen on the TV.
And again, we can apply the four year rule. So you do two to three lens openings and you start at the age of 4 and you lengthen your spirit, you're lengthening episode four year apart. Consider it prejudices of the long limp around adolescence. If there is associated congenital femoral deficiency, you can plan femoral lengthening either simultaneously or in a separate sessions. Uh, you have to do a surgical plan and then you decide according to the classification.
So in type one, the only problem is slight shortening of the lower limb, so you can plan for it, just tibia or fibula lengthening. You don't have to do any food fixation and the patient doesn't need any kind of food surgery. So in type typekit, that is ankle instability. And because of the ankle stability, as we have mentioned earlier, the fibula is short. This X-ray that is on the screen is a normal ankle X in a child where deficiencies of the distal fibula is at the level of the ankle joint.
OK we can see here this is the X-ray of a case of Romania, where the prices of the distal tibia is at the level of the distal tibial voices. So in typekit. So the fibula is short. So the way to stabilize the ankle is to shorten the tibia. So you relatively lengthen the fibula, so you do an operation culture, the operation where you do shortening realignment osteotomy of the distant tibia to correct the Vogel's and stabilize the ankle after this operation or together with the tibia, can be lengthened.
So this is how we do this operation, so you do immediate incision, t-shaped incision in the Persian. And then you do a small incision at the syndesmosis level, you separate the two from the fibula. And then you do the osteotomy, it's a pipeliner osteotomy to correct the deformity and the distal tibia.
And then after you do that, you shorten the tibia and you preliminary you fix it with wires. And the next is to be stabilized, the syndesmosis was a tightrope. And here an example, so you can see this is the three city before the operation and these are the X-rays. We can see that the five lights, it's short and the ankle is drifting into August, and this is the operatively image with the osteotomy and the shortening of the TV.
And this is after fixation. We can see after fixation that now the forces of the distal fibula is at the level of the ankle joint. In typekit, 3 and four, we do the Super ankle procedure, and super is not it's an acronym for systemic utilitarian procedure for extremity reconstruction. So in part 3 and four, lengthening is often combined with the soccer ankle procedure, so you do both at the same session.
So the principle of it is it's quite similar to the management of type t. So here we can see the deformity of the distant tibia. So the lateral distal tibial angle is less than 90 degree, and there is deformity of the anterior of the end of the anterolateral distal tibial angle. It's more than 90 degree, and this act as a mechanical block for the ankle, those reflection.
So to do this, to do this operation, we do two long incisions on the lateral aspect of the leg, one proximal and 1 distal. And the first step in the operation is to remove the fibula and leg. Any remnants of the fibula should be removed because these will act as a tether for the growth of the tibia and cause tibial deformity.
In order to do that, you have to dissect and protect the common ulnar nerve and superficial peroneal nerve. And then after you do that, he released a capsule of the ankle joint. And then you do osteotomy of the turtle coalition, so you correct the alignment of the calkin to the details, as we can see here.
To do that, you have to rotate the continuum and translate on the same time and then you fix the tail details in the ankle joint. And then after that, you do shortening realignment osteotomy to the distant TV. And this will be the last. They define a picture of what we have done, so it's a subtler osteotomy, distal tibial osteotomy and preliminary fixation.
And then you add a frame on the top and you put this ring here to allow the patient to do weight bearing. And if you plan to do lengthening on the same session, you can do a proximal osteotomy and you can lengthen on the same operation. So here's an example. So we can see here that the ankle isn't severe, but there is a skin temple at the apex of the tibial deformity.
It's untrue medial bearing of the tibia we can see on this X-ray ray. The femur looks pretty normal as compared to the other side. You can see the ventromedial bearing of the tibia the severe focus of the ankle. We can see on the lateral view here that the whole bones of the ankle are fused into one single bone. So this is during the operation.
Osteotomy and shortening of the tibia and osteotomy of the additional primary fixation with a wire external fixator is attached with lengthening and we can see the pictures of the lengthening and here the clinical picture. OK say amputation. Do we do amputation for fibula million? So when we do the amputation, the only indications for amputation, if the foot is severely deficient and the patient is unable to achieve blunt, degrade foot with reconstruction or could be apparent to us if they don't want to go through multiple extensive surgeries in that case, when you go for signs amputation.
So the reason we go for some amputation because it's more energy efficient than midfoot osteotomy in order to do Simon's amputation, the patient should have a patent posterior tibial artery because this gives the blood supply to the heel pad and the heel pad was act as a cushion to the stomach. Any questions? Thanks, Dylan.
Very comprehensive and I think reasonable, you know, you can, especially in this time of photographs for survivors and the clinicals, you could easily get one of those cases coming up. So I think what we're going to do next is we're going to launch the poll. We'll get you to answer as quickly as we can. And then I think we've only got one question in the chat at the moment, but we'll do the poll first, then we'll do questions and then we'll stop recording and is so the best answer is kill ectoderm rich and fibroblastic growth factor.
So we have 65% of the candidates answer correctly. The second question? It's almost half and half, actually. So the most common foot and ankle deformity and fibula Herminia is required to focus. So 4% to 7% answered this quickly, so correctly, the last question in the X-ray finding to diagnose cruciate ligament dysplasia, actually, all the answers are correct, so the correct answer will be all of the above.
We have 47% chance of this correctly. OK, thank you. Hi, brilliant. Come on, everyone. So in terms of questions, Natalie wants to ask pre axilo post axilo in relation to the access. It's quite a confusing description. Can you explain it a bit more, please?
So say in the upper limb, so the post axilo at the radio site, axilo at the ulnar nerve site. So when you have put it directly on the underside, you know, so this is a pretty active politically. On the other side, on the radio side, it's close to axilo and the same in the lower limb. OK, so the fact is, we can consider that the picture or the thumb with the access, so on the other side, on the lateral side of is post axilo or the other side.
I don't think there is like a role for that, David. OK I just memorize it like that, you know, literally. Does that help? I hope. Dan Schorr, Thanks. OK and then the last one to ask if festive returns are present is the non-functional foot. Sorry, say it again.
It's less than three times at present, so I presume only two toes present is a nonprofit. So this is actually a good question. So there is debate regarding and what's the minimum number of phrase you have in the foot to have functioning foot? OK, so it's because it's controversial. So some people believe that if you have less than three raise, so this cannot be planted.
Right foot or cannot like withstand the whole stresses of the body weight. But actually, this is not right, because sometimes the patient may have to raise about one of these is actually is like a fusion of two race. So imagine if you have a patient with the right to metatarsal, but they may have. One of them is very big and actually it's like a syntactically between the first and second metatarsal.
So if clinically you can, the patient can have planted. Right foot or you feel that the size of the foot is enough to withstand the whole weight of the body while walking. So this means that this limb can be reconstructed if you have a very small remnants of the foot that cannot be reconstructed. It's an indication for amputation, but not the absolute number of metatarsal or toes.
OK so it's based on the ability to be a weight bearing, weight bearing ability rather than the amount of race, so if they are so, you're unlikely to go down that route. But should they ask, say, the candidate should say it's a controversial topic, but the key aim is whether they're able to weight bear on that foot, correct? Yeah, yeah, I agree with you. There were no other questions in the chat.
Is there anything else that burning questions that people want to ask at all? Just just one more question. I mean, what? What is the appropriate age? Can you repeated thing again? What is the appropriate age for the operations. So for lengthening? You start at the age of four.
It's very difficult, you know, to lengthen a child like at the age of two or three due to lots of reasons. Actually, it's because the size of the limit is very small. The ability of the child to cope with an external fixator, it's very difficult. It's very difficult to convince a child that is 4-year-old to do new range of movement to avoid knee stiffness, you know? And so basically, if you are in other causes of limb blends discrepancy, we usually postpone the the, you know, the lengthening and the age of six or seven when the child is cooperative and can cope, can understand the instructions and do the physical exercise as well.
Because in congenital limb deficiency, you have to be multiple episodes of lengthening. So it's better to start early at the age of 4 and then you do one episode of lengthening every four year. So at four, eight 12. And then it's better not to do. It's difficult to do more than three episodes of lengthening. And if you end up having like $0.02 level discrepancy or centimeters discrepancy, you can do episodes of the longer side before skeletal maturity by one or two years, according to you calculated on the multiplier method and see when you can do that.
Other operations apart from the lengthening. So you do at the age of two to three years. So if you are going to do the Super ankle procedure, you can do it at the age of two to three years. You can do the Super hip procedure at the age of two to three years to prepare to you're lengthening at the age of four. OK, thank you. OK, that's great.
Thanks, John, so I'm going to stop the recording now, and I think.
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