Dr. Ravinder Brar presents "Orthopaedic Management of Cerebral Palsy" at the UCSF Pediatric Musculoskeletal and Sports Medicine Conference 2023 in Berkeley, CA.
Next up, we have Doctor Ravi Brah. Um Let's see, she has an MD N MP H. So she did got her master's of Public Health at um Dartmouth and then went to med school at Virginia Commonwealth University stuck around in Virginia for her residency or the residency at the Medical College of Wisconsin. Then she did her P Ortho fellowship uh in Atlanta at Atlanta Scottish, right Children's Hospital and she's been with us at U CS F since 2016. Let's see, three years ago. Was it 20? She's also got this title of being the medical director of um U CS F S C P and Spasticity Center, which is really cool. And she's my go to person for neuro neuromuscular Ortho things. Um Thank you. I gotta say mine is like the best height person ever too. Um So I'm really happy to work with her and our entire rehab department. Um So in general, I put too many slides and too small a font. Uh So I apologize for that. I did cut out like 15, there's still too many. Um And thank you all for still being here. It's a beautiful day out and we're talking about complex things in the afternoon after lunch. So I know it's hard. Um But yes, I'm Ravi Bra, I'm an orthopedic surgeon. I have no financial disclosures, but U CS F is a member of the Cerebral Palsy Research Network. Um And most of us are members of the American Academy for C P and Developmental Medicine. Um And since there's gonna be a lot of people talking about C P um and neuromuscular conditions, I'm gonna quickly go through the definition um and highlight what's important uh like throughout this talk for orthopedics. Um So, cerebral palsy is a static encephalopathy. That's what everyone's taught. But what we care about in Ortho is that it has progressive effects on the musculoskeletal system um that affects like movement, tone and posture. And so it's heterogenous effects and the clinical manifestations are all dependent on the location and severity um of the part of like the parts of the brain that are injured. So I'm gonna adjust some things over here. So I can see better um C P effects muscle size, composition and force. And so it's all a vicious cycle on your skeleton. Um There's abnormal neurological signals and spasticity that results in the muscles being um fewer, shorter and stiffer the fibers of them and then a longer tendon. So then you have a weaker muscle with diminished excursion um and a decreased cross sectional area which leads to decreased joint range of motion and decreased power. Um The smaller stiffer weaker muscles also don't have good selective control. It's worse in the muscles that cross two joints, your biarticular muscles. Um and there's a discrepancy between muscle growth and bone growth, which can lead to more weakness, deformities of your bone and your joints, loss of function and pain muscles. This is a um this is a cycle I'm talking about. They need a stretch stimulus to grow. And so if you have a motor delay or joint contractures or tone abnormality, it limits your physical abilities that provide that stretch stimulus. So then you have inhibition of your normal muscle growth and development. And there's a mismatch between the muscle strength and the person's body size. And as they grow, um they can have a heavier body and worsening joint contractures and worsening weakness. And so that's why we see during the adolescent growth hurt um that patients who are ambulatory or have a certain level of function can sometimes decline in their function and lose the ambulate ambulation potential. Um And so that's the natural history. Unfortunately, in some kids for that deterioration of gait during adolescence. So what can orthopedics do? Well, we can help with an atomic issues when it comes to soft tissue, we can address tight tendons. Um There's often a muscular imbalance where something like the abductors are stronger than the abductors and we can address that imbalance by weakening the stronger one. Um we can address contractures, Toral abnormalities, limb malalignment and some dynamic issues. When it comes to gate, what can we not do? We cannot improve strength. You just heard me say when there's a muscular imbalance, I don't do something surgically to make it stronger. I just weaken the stronger muscle. So physical therapy is what can help with insufficient strength. Um I can't correct issues with balance such as ataxia or the brain's inability with proprioception to control that balance. Um I cannot improve selective motor control and unfortunately, we can't improve the communication between the brain and the muscle. So anything I do is not gonna increase or decrease tone, the way we talk about um cerebral palsy and communicate to each other. Uh is about the gross uh has to do with the gross motor function classification. Um And so to distill it down, there are five levels and since C P affects kids to different extents is how we communicate it. And this is also what affects prognosis. So those who are level one are um are high functioning, they can walk, run and jump um without issues, but sometimes there's a little bit of um like decrease in speed or balance or coordination. Level two. That's when I ask kids, like when you walk upstairs, you have to hold on to a railing. Um Can you take a hike on uneven ground and not have issues? Three? Is that turning point where um they use an assistive mobility device, uh those who are level four, so are more impaired, can sometimes walk short distances with a walker but are still reliant heavily on wheeled mobility um for community ambulation. And then our G M F CS five kids um are the most affected and they often lack head and trunk control and there's no independent mobility, uh neuromuscular acquired hip dysplasia. This is something orthopedics cares a lot about um and the path of physiology of it uh is that the spasticity or dystonia and the lack of the muscle control causes abnormal um uh imbalance of the muscular forces across the joint. And the illustration that you see on the right there um is showing how you have a normal uh hip on the left and an abnormal one on the right and how the imbalance of forces are causing um the the joint like acquired dysplasia. So the actors um are pulling the leg in um and causing it with the tight flexors to sub lux out. And as that femoral head um is being pulled up, it's causing lateral pressure on that acetabular roof. And so instead of that nice round socket, it keeps getting hit by the femoral head which then causes it to be more vertical. So then you don't have a nice deep socket. Um you have increased an aversion, you have increased neck shaft angle. Um And then with that femoral head constantly abutting um unevenly the socket, then it gets um deformed as well. And so you don't, you no longer have that nice round head. Um You can have a wind swept pelvis, which the dislocation, which then causes pelvic obliquity, which as we know, um can then contribute to neuromuscular scoliosis. The risk of hip subluxation and eventual dislocation is directly correlated with the G M F CS level that we just discussed. It's a pretty significant relationship, it's linear and it's directly proportional. And so to me, that means that any child with C P gets a pelvis x-ray, even if they're high functioning just as a baseline. Um And so throughout childhood, the total risk for displacement for um G M F CS five is about 90%. G M F CS four is about 80%. So that's pretty significant. Um And that's why um hip surveillance is kind of like my soapbox. So why is this all important? Especially if a kid um doesn't like even if a kid doesn't walk because a located hip, a hip that is meant that it's in the right place where it's meant to be moves better and is less painful than a hip. That is not where it's supposed to be. And even if you don't walk, it's a stable platform for sitting. Um And if it's in the right location, then you're able to maintain that motion and what activity you do have with aging when it does subluxed or it does dislocate some kids don't have pain, but many do, especially if it was all, if it was in the right place to begin with because then your femoral head is a butting a part of a p the pelvis that it's not supposed to hit when you open, um, the hips to say, put on a diaper. So I think it's a huge problem even in our kids who don't walk and dislocation and subluxation, it causes that misshaped femoral head or that miss shaped socket. And so when you have two shapes that don't match, that's arthritis and stiffness and pain and it makes it really hard to take care of the kids on a daily basis who can't advocate for themselves. Why do all the Ortho people have issues with working on a computer? I don't understand. I'm sorry. So this brings us to hip surveillance. Um And so hip surveillance is just monitoring the hips regularly to identify early indicators of hip displacement and that's with regularly scheduled physical exams and x-rays. Um And this matters even in kids whose hips don't hurt because even though they have good range emotion now and no pain now, it doesn't mean that they won't have pain in the future. So you wanna be able to catch it early. Um And the goal is to treat or prevent pain with this early intervention and we can improve outcomes and reduce the number of reconstructive surgeries and also reduce the need for future salvage surgery because salvage as it implies with that name is not a great surgery, but sometimes it's our only option. So there are three main components to hip surveillance. It's ideally a population based targeted evaluation. And in other countries, everyone participates this from the physical therapist, the rehab physicians, the general practitioners, um everyone's involved. Uh but we at the, in the US don't have as coordinated of an effort. Unfortunately, because we have such a heterogenous population um with our different states and everything. But even within California, we don't have a nice surveillance program, but it's regularly scheduled physical exams and radiographs. And the frequency of that is based on the G M F CS level on physical exam. As Doctor Dia was saying, like we just like to eyeball certain things. And so for me to start delineating um G M F CS level and looking at head control their ability to sit on their own. Um And then I'm asking about difficulty with diapering. And then I actually asked parents how they change the diaper because they'll say they don't have an issue because they've been changing this child's diaper for like 15 years and then you'll see them like wedge their body in between the knees and like somehow change a diaper. And that is not sustainable as the child continues to get stronger and older. And so I think it's an important thing to ask because just because they can get by doesn't mean, it's the right way to be doing things and it's gonna be harder in the future. I look for asymmetries, pelvic obliquity, any limited range of motion and skin breakdown, especially from any braces and rotational abnormalities. Um When it comes to referring to orthopedics on your physical exam, if you find that hip abduction is less than 30 degrees with the hips and extension, so not flexed up. Refer to us if you see hip abduction is asymmetric, please refer to us. If you notice that the hip abduction is decreasing, please refer to us. And if hip extension is decreasing, please refer to us oftentimes um it's hard to get the right x-ray for an A P pelvis because of um the lower doses and the lumbar spine. So sometimes we have to ask our radiology techs to put um like bumps underneath the knees to deal with contractures to get that right uh x-ray and it does matter. I even have this printed out um at our radio at our like uh x-ray room so that people know what we like. Um But all you really need for routine surveillance is the A P view because that's how we do all our measurements. We want the legs parallel, we want the the like the knee caps patella facing up and we need to um reduce the lumbar lower doses. The migration percentage is what tells you how uncovered that hip is how much it's migrating out of the socket. Um And what you can see in this, um this example, it's the same child and in infancy, the hip was normal. Um at about like 18 months with only a 10% migration percentage at one year. Um or one year later, at 2.5 years, it increased to 25% by age five, it was 40%. Um And then age eight. Uh and on, you can just see how it got progressively worse and deformed in that last photo. Um There's severe acetabular dysplasia where it doesn't even exist the socket. It's just like this wall without any concavity to it. Um And you can see that there's just a chunk missing out of the femoral head laterally there because there wasn't the forces from the aceta for it to grow and to form that nice round head. When it comes to x-rays, please refer to orthopedics if the um x-ray shows a migration percentage greater than 30%. And so you might be thinking, well, I don't wanna do calculations um on my x-rays and like draw lines and then do a division with the calculator that you bring up on your computer, which is the same thing. I don't wanna do that either. And so doctor Carney, an orthopedic surgeon at the shrine in Sacramento developed this free app on your phone. Um And this is what I use every day. So the other day I forgot my phone, which besides that being my pager and being a problem, I couldn't do my migration percentages and I was literally at a loss, pulling up the calculator and like drawing lines and dividing. I hated it. So this thing is amazing when you have a chance download it for free. It's cool because it also shows you guideline summaries, like frequently asked questions, it has that protocol for how to get the proper x-rays that you can print out and put somewhere. Um And so what you do is you just pull up the x-ray, you take a picture in the app and then you have these horizontal lines and that's for rotation. So many of the kids are not gonna lay like perfectly straight and so you just rotate it to get it as level as possible. I use um the tri radiate cartilage or like the bottom of the is to, to get it level. And so you rotate, using that and then you in on either side. And so that red box exists because once it hits the red box and what you're looking for is the lateral edge of the bony aci taul if it's within that um red box or touching the red box. So inside it refer to orthopedics. Um and because that red box is 30% so each line here is 10%. So that's why it's the red one is the third one. And so if you look at this bottom one here. What you do is you line up the black line on the medial aspect of the thermal head and the white line on the lateral aspect, it's hard to see it sometimes because this kids like the lateral aspect is not easily seen because it's not getting the right contact pressure. So it's not developing as well, but you line it up and then you can count here and that's a 70% migration percentage. So it makes it really easy. Um And quite fast. So I highly highly recommend this app and it's like it's an award winning app and everything. Um I alluded to this earlier, we need organized hip surveillance. Um There's nothing nationally, there's nothing within California but Australia, some European countries, British Columbia. Um They all have surveillance programs led by primary care, physicians and therapists, rehab physicians, orthopedic surgeons as part of their evidence based care. And that has resulted in, in like not having salvage surgery. So kids aren't getting their hips cut out because they're so deformed. And so that's a huge deal. Um And I think since other people can do it, it's something that we should be lobbying for to get more um more attention to it through our CCS uh clinics and things like that. But it's really hard to organize. But I just, I'm just constantly on a soapbox about getting x-rays about the pelvis because as an orthopedic surgeon, I am single minded when it comes to the hips and I'm quite aggressive about it. But I think it does make a huge difference. Your surgical options when it comes to hips are soft tissue and then bony procedures. So you can have your soft tissue lengthening and your releases. And so this is an actor toomy. I do open, I know there's percutaneous but I do open because I feel like you can really um really, really lengthen it well and you do graduated releases. If it's not enough to just get the abductor, then you just keep doing more, um including the grill and abductor, uh Brevis. And so this is a kid who had really, um like had a lot of spasticity at two years old and you can see how far that hip was going out there, especially on the right and um at 50%. And then so I just did soft tissue lengthening because, you know, one of the things is this kid was still showing us that he was progressing. He was learning skills. And so if I were to do bony surgery, I would set him back because he would then be weaker. He wouldn't be allowed to bear weight or anything. So we did soft tissue lengthening and it bought us time until he was older. Um, and he still hasn't had surgery yet. I think he's six now and I am planning a surgery like a bony surgery on him. But, you know, I did not interrupt his progress with physical therapy, um and skill learning um at this time. So I'm really happy we did this surgery because it bought us about four years before. I'm now planning his surgery when we talk about bony surgeries, bilateral V D R OS. So various de rotational um and shortening osteotomies and pelvic osteotomies. Here is an example of it. Um The idea is to put the hips in the socket, make them more varu because um normally you start with vagus hips when you're born and as you grow and develop, they get a little more vas and that doesn't happen in our kids with C P. And so we um we do these bony surgeries. This is just walking through that, doing the V D R O first, then doing a pelvis omy. Um And you just put something in there to widen it and bring that roof flat to do a bony containment. Um And then that's what it looks like afterwards when I say salvage surgery, that just means hip resection, meaning we just take out the hip because it's not helping, it's just causing pain. Um And so you can see here that, you know, it's not a great option. It does create a floppy leg which makes it easy to uh diaper, but it um it can still, and it removes the immediate pain. But if your muscles are still tight and you still have that spasticity that um residual femur can still start hitting your pelvis and cause pain. So it doesn't eliminate pain in every child. So the key points are that hip surveillance is important, important, important. Um You should get a pelvis x-ray at the initial visit and continue to get x-rays. Um Please refer to us if there's any concerns and the risk of hip dislocation is directly correlated to G M F CS level. Just remember 30 degrees um or 30% in terms of migration percentage or uh lack of range of emotion on abduction and hip reconstruction is effective in improv improving positioning, mobility and transferring comfort and diapering. Oh my gosh, I need to speed it up. Ok. Lower extremity issues in surgery. Um You can have deformities at each joint, the knees, the ankles and the, the feet. We like to do seals, which is single event, multi-level surgery. That's when we just correct as many things as we can in one sitting as uh as uh if we can be safe doing it. Um And that eliminates the need for birthday surgery, which is like a surgery every year. We involve other specialists such as our rehab colleagues to do Botox on um like like the upper extremities while we're working on the lower extremities or we have our plastic surgeons do transfers on the upper extremities. It reduces hospitalizations, it reduces periods of rehab. It's more cost effective. It's less disruptive to, to, to school and to uh parental um leave from work, knee flexion, contractures, tight hamstrings, weaker quads, weak soleus, it has um increased energy consumption and uh we evaluate it using just like checking the po angle and checking the contracture. There are different ways to address it. Um You can get 15 to 20 degrees of lengthening by just doing hamstring, lengthening, plus or minus casting or bracing. And so this is just us lengthening the hamstrings. Um There's been a move towards uh growth modulation now. So what we do is we place screws in the anterior part of the um distal femur to stop the growth anteriorly which allows growth growth posteriorly. So you can imagine that can over time like over two years. So not immediate success, you can get about 20 degrees of correction, the last option and this is actually a great option. But you are, you know, the first two, you can bear weight immediately, this last option. Um the distal thermal extension osteotomy, you just create a deformity to counteract the knee um flexion contracture by hyperextending above it. Um that immobilizes the kid and doesn't allow them to bear weight for six weeks. But it is a great option um in our kids who need to be able to stand um for transfers um and have the ability to do so but have like significant knee flexion contractures when it comes to the foot and ankle. Our goals are to have braceable, painless and plan to grade feet. Please note that that's done. That does not mean that we're gonna do a surgery that prevents you from needing to wear a brace for the rest of your life. You will still need to wear a brace and I often won't do surgery if a child will not agree or the patient will, uh, sorry, the patient's family will not agree to be compliant with bracing afterwards because then you'll put a kid through surgery, they won't wear a brace and you will have done all this work for no reason. Um So I don't like, you know, I, I like really screen kids out for that. Um This is a 20 year old kid with a terrible spastic flat foot and this is like one of the big reconstructions that we can do. Um, this kid is non ambulatory uh but was having a lot of issues fitting into shoes. Um And this is a good six hour surgery. You would think it's a foot. So it shouldn't take that long, but there were two of us there and we just took out the navicular and um you can see it already looks much better there. Um It's still swollen immediately because now you just have this space where there used to be bone and now it's just soft tissue and um you get a little bit of blood that collects there in the beginning. And then this was a couple of months later, this kid very happy, the family is very happy that he can fit into shoes. Um And so we can do something as impressive as that. It just takes a lot of time. And so we have to scream for the right patients who benefit from it. Um There is a high fracture risk in kids with C P, especially in those who don't walk because they have low bone mineral density. Um There's sort of increased risk for fracture, especially if they have anti epileptics, like they have seizures, they're unable to communicate sometimes when they have pain. And so um sometimes we get a diagnostic delay and then when you immobilize them, they can't use their standard, they have decreased weight bearing and worsening osteopenia. So we have to be mindful of that just because they have hardware in. It doesn't mean they're gonna, you know, that's not gonna cause a fracture. The problem is, is one of my kids. Um he had that played in for about three years, but if they're gonna get a fracture and there's hardware in, it's gonna be a per prosthetic fracture. And that's because of the transition from the stiffness of having metal in there to no metal. So if they're gonna break, they're gonna break where the metal is. So I think goal setting is the biggest thing when it comes to surgery. Um especially in our more affected kids, we wanna help by providing a painless existence with reasonable positioning and no significant contractors that prevents like putting on clothing and taking care of them feet and different things to protect the caregiver because that child is gonna grow and become heavy. And so it's important even though we might see a problem to actually ask the family what the problem is and how you can best help them. Sometimes it's small things like you can see a bigger problem that you can address, but all the family really needs is like a small minor change to make their day and they taking care of the patient easier. Um and better. And so it should all be shared realistic goals. Um The plan should be discussed and I think orthopedic surgery is heavily reliant upon. It is interdisciplinary team which at its minimum includes um rehab our therapist, orthotist nutrition. Um and, and seriously much, much more. And again, your goals depend on your G M F CS level. We can improve git um we can reduce some reliance on walking aids. Um Often you'll still be using braces, we can provide a little more symmetry for aesthetics. And then quality of life is the biggest thing when it comes to our G M F CS fours and fives, preoperative optimization is a big thing. That's when you may hear from me. Um And we take a lot uh we, we send out so many emails um uh from our nurses here will contact you and ask you if you think if you're worried about anything, um, before doing surgery on our patients, um, and we rely on our pediatricians to lead a comprehensive review, um identifying any issues of concern communication. Um And if you think like there's gonna be an issue with follow up or anything and there's so many per operative considerations. Um And sometimes we'll pre admit before surgery uh to improve nutrition um to improve respiratory status because nutrition, you know, compromises wound healing and immune function. And so what we found and this is a retrospective chart review um for preanesthetic clearance is that if a pediatrician saw the patient prior to anesthesia's evaluation, then there was less likely to be last minute changes in our plan that would then cancel the surgery. Um So for us, it's really important to get everyone involved. Lastly, there's a lot of access challenges for care with C P. We work at a children's hospital. Um So we have a lot of resources for our kids um who qualify, but even though C P is defined as a childhood disease, um the life expectancy um is quite long and there are so many adults in the US living with C P. Now that many centers across the country including U CS F are building programs um to help provide comprehensive care to adults with C P. But with that, we need more providers who are willing to provide that care. Um and they are looking to all of us who are in pediatrics to help with that because a lot of adult providers feel like they haven't been trained since their residency years um in cerebral palsy and other neuromuscular conditions. And so this is something that even the N I H um has a task force dedicated to it. And so we um would like to work on this more and if anyone has interest come up and talk to me and so thank you for your attention and I'll be.
