Chapters Transcript Video Comprehensive Care for Hypertonicity in Children Thank you all for being here today. We will be talking about the hypertonicity program at Benioff Children's Hospital, particularly the Oakland side. Um, the title Moving Forward, Comprehensive Care for Hypertonicity in Children. I'm a pediatric rehabilitation doctor. Uh, Dr. Sun is our neurosurgeon, and here actually we're going to start off with an, with a picture of the team from, actually I'm not sure how many years ago that is now, Peter, 20. 2005. I grew out my hair since then. Mhm. Uh, and a very recent picture with the current with the team members it currently stands. One of the main conditions that we treat in our hypertonicity clinic is cerebral palsy, which is defined as a group of disorders of the development of movement and posture causing activity limitation that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. It's a lot of words, but what it is, is the second most common childhood disability with a prevalence of 3.5 out of 1000 at age 8. Uh, 950,000 Americans with cerebral palsy. The etiology is a loss of inhibition and modulation from the brain of the Um, of the muscle control, particularly of the reflexes that lead to hypertonicity, excess tone, excess movement, and this particular MRI shows periveventricular leukomalacia from Prematurity and you can see the extra whiteness there. And they are that are the periveventricular leukomalacia. We can classify cerebral palsy in several different ways by area affected, by type of problematic tone, or by functional status. First, by area affected, um, we can talk about monoplegia. Very rare to actually see that. Hemiplegia, which is one side of the body, diplegia, which is both arms rarely, more commonly both legs, and quadriplegia are much more commonly these days, um. Bilateral cerebral palsy, where it affects all four limbs, the trunk and the face, we can also classify it by type of tone. Spasticity, which is the most common one that we talk about, a velocity dependent resistance to muscle stretch. So the faster you try to stretch out a muscle, the harder it is to stretch. If you stretch out that tight muscle slowly, it comes along with you, and if you try to go quickly, it is much more. Uh, it's much more resistance. I'm going to switch slides here and spend just an extra minute and just an extra little bit of time on dystonia because that's one of the harder ones to get a grasp on sometimes and is going to turn out to be important for some of the things we talk about later in this talk, um. Dystonia is involuntary sustained or intermittent muscle contractures. That cause twisting and repetitive movements, abnormal postures, or both. So this is a kind of tone that is often absent when folks are at rest. They can feel very loose, but when they try to do something, or even by when they have a strong emotion, can become tight either overall or with a certain characteristic movement pattern. This, as I say, it's often triggered by attempting voluntary movement or by emotion. This can be really frustrating because you're trying to do one thing and control just your, just a few muscles in one direction, but then the dystonia kicks in. You end up posturing in a direction that's actually counterproductive for the movement that you're trying to do. You get frustrated, that's a strong emotion, and it just piles on top of each other and spirals down and down and down, um. Dystonia, as I said at the beginning, varies in presence and severity over time to a much greater extent than spasticity, so it can be there sometimes and not there other times. One of the things that I often hear from families, and one of my first clues for this is when they tell me about a kid who is relaxed some of the time or especially in sleep, but then when they get excited they're just or mad, they're just so tight and they can't do anything with them. Um And then another way to classify cerebral palsy is by gross motor functional classification system levels or level of function. The majority of kids are ambulatory level 1, so you can walk, run, climb stairs without a rail, level 2, you can walk, climb stairs with a rail, or level 3. You use some sort of a gate aid but are relatively ambulatory and get around in the community with a gate aid occasionally for the longest distances with a wheelchair. Another small, a smaller number are the folks who A largely mobilized using a wheelchair level 4 where you may get around with a very supportive gait trainer. Or use a wheelchair in level 5 where even a good, where you need a good wheelchair with postural control to even sit up straight. Um. In terms of managing hypertonicity, what baseline level of function is, informs goals, informs the appropriate way to manage spasticity. So What are the problems that are caused by spasticity? It can inhibit motor development, reduce function, daily activities and exercise, cause muscle contractures and musculoskeletal deformities, interferes with care, position, and tolerance of braces, increase the risk of falls, cause chronic pain, and increase the burden of care. When do we consider tone management when hyper this is my, these are This is, this is my set of criteria. Everybody uses a slightly different one, but when hypertonia is interfering with function, positioning, cares, or comfort. Goals of spasticity management are to improve motor activities in daily living, decrease orthopedic deformities, improve comfort, minimize pain, decrease burden of care, and decrease social stigmatization. A little detour here for the next slide into gate. We talk a lot in this later in this slide about. Um We talk a lot later in this talk about gait and improving gait, so just. A quick Comment that gait is obviously our concentric and eccentric muscle contraction contractions. To cause us to be able to progress our bodies forward, spasticity in CP makes some muscles turn on at the wrong time, makes some muscles turn on for too long, and and other times not turn on at the right time. This is compounded by the poor motor control, weakness, and poor balance that are also part of the impairments of motor control and cerebral palsy. We can also see that Function in cerebral palsy deteriorates over time, and that's in part from spasticity and especially in the lower GMFCS levels. On the other hand, after an SDR, function improves over time. The Multidisciplinary CP hypertonicity management is one of the cornerstones of our program here. We always, everybody needs PT, OT, diet, exercise, and physical activity. Then we have spasticity management, bone and joint alignment, orthotics. Um, and ultimately for folks who are, who are more difficult to manage, referral to our multidisciplinary hypertonicity clinic. Spasticity management can consist of bracing of medication. Uh, of botulinum toxin and and phenol or ethanol injections, and then of surgical tone management, including selective dorsal rhizotomy or intrathecal baclofen. But In terms, but to come back to the question of do we improve function, does all spasticity need to be treated? Debatable because there are some folks who we talk about, well, is this person using their spasticity to help them move around, to help them get up, to help them walk, and we'll see some examples later on in this talk of patients in whom we debated this before we decided on what treatment to provide. Um, I'm gonna hand over to Peter for the next little bit to actually talk about a bit about selective dorsal rhizotomy from a surgical perspective. Thank you, Kate. Uh, hello, everybody. I'm Peterson. I'm the pediatric neurosurgeon that's At the spasticity clinic, we started it years ago and we're very proud to continue it. It presently under the expert guidance of Doctor Sigford. Um, so, one of the main modalities, uh, that we treat spasticity and CP is with, uh, selective dorsal rhizotomy. You know, this is not a new operation. Many of you have heard it before. The physiology has actually worked out in, um, You know, the 19th century, and I find it um Um, um, kind of ironic that it's exactly 130 years later, just, uh, um, um, in 2018, uh, the, um, National Health Service in England finally approved it, uh, to treat kids because it, um, uh, improved function and, and, and reduced spasticity. But basically, uh, we have, uh, uh, Doctor Sickford mentioned, there's a loss of inhibition and modulation of intrinsically hyperreflexive. Uh, spinal reflexes and the rhizotomy is to cut those nerves, uh, that mediates the response. Um, uh, they are the sensory nerve roots because the sensation is so overlapped that we can actually reduce, uh, the spasticity by cutting some of these nerves without losing any proprioception or, um, Um, tactile sensation, yeah. Um, We'll go on to the next slide. So, yeah, this is kind of what the setup looks like. Um, we, um, do, uh, just a very small opening in the back, uh, at the level of the conus. Uh, we tease out the ventral and dorsal nerve roots. Of course, we don't want to touch the ventral nerve roots, nor the sacral nerve roots that mediate motor function and bladder function. And then we identified the uh dorsal nerve roots, uh, and then we test them individually to see which one are most responsible for spasticity, you know, as it turns out, some of these, when we stimulate them, don't, um, respond in a hyperreactive way. Some, when we stimulate them, all the muscles go off and they're sustained or they're tonic, or they go to the contralateral side. And we use this physiology as a basis of the rhizotomy, hence the S part, which is the selective. Yeah, so, we do, uh, rhizotomies for two class of patients. One is functional, which we want to improve their function. Uh, and, uh, ideally, these are the younger patients, GMFCS 1 to 3. Um, and then, a very important part of our program is actually we keep them in the hospital for, um, 4 to 8 weeks, um, for intensive inpatient rehab that you can never get when you go home. So, the, Therapists work with them 2 to 3 times a day, 6 days a week. I think that's part of the reasons that you'll see some, uh, from our patients that we get such good functional outcomes. I think this inpatient rehab part is vital. Uh, and one point I want to, I'll have everybody in mind is when we want to have someone, Um, uh, functionally improved with spasticity treatment. It's best to reduce their spasticity with STR neurosurgical management or other spasticity management before we do orthopedic surgery cause sometimes when the tendons are cut, it's hard to predict how well they'll respond. And one clue is that they often respond well to Botox as a selection criteria. Yeah, so we want to do this early on, um, Uh, before, uh, they have bony contractures because, um, then the morbidity of spasticity kicks in and then we're dealing with, uh, you know, bony surgery or salvage surgery for musculoskeletal, uh, decompensation. This is actually an old, this is actually a relatively old graphic from arguing against doing starting with the early surgery, which turned into a problem called birthday surgery. The kids got a surgery just about every year as the orthopedic surgeries tried to chase the consequences of the spasticity. We're trying to get ahead of things by doing the surgery, by taking care of the spasticity first. Yeah, exactly. All right, go ahead, Peter. So, uh, there has been randomized trials, uh, looking at, um, uh, the effect of STR and the, uh, three centers were pulled, and then there is a statistically, uh, significant, uh, improvement in the, um, GMF uh, gross motor function measure. Um, so, And we can um. So, um, yeah, next slide. So we also do this for therapeutic patients, for comfort and care, and these are the GMFCS 4 and 5 patients. Um, and these are actually some of the most grateful families where we can reduce, uh, spasticity and reduce the burden of care in this, uh, patient population. And um for that patient population, used to, uh, we used to put in a lot of intrathecal baclofen pumps, um, uh, you know, oral baclofen has a weak capacity to penetrate the pumping barrier, can make patients sleepy. So intrathecal baclofen was developed and placed in a, a subfascial or subcutaneous pump, and the medicine gets delivered directly by catheter to, um, Um, the, um, spinal cord and it's a very effective way to reduce spasticity. However, um, you know, it does have a significant cost component. It escalates the medical care by requiring medications and replacements over 4 to 6 years depending on how fast the dosing is run. Now, we wanna show Our audience that we've many times have replaced this modality with our selective orthorrhizotomy for therapeutic patients, which is of course just a one-time operation. Mhm. Um, the previous, uh, argument for doing the pump is that it has, um, good upper extremity effect as well, and we'll show some data that, uh, you know, our STRs, while we're cutting lumbar nerve roots, axle somehow rewires the circuitry so that there's a good effect in the upper extremity as well. So, prior to, so prior to having any of these surgeries and for anybody who were wondering about the best way to manage, we bring them into hypertonicity clinic, sometimes gets called CP clinic, but really all of us manage, all of us who are involved in hypertonicity multidisciplinary clinic get Uh take care of a lot of folks with CP in individual clinics. The folks who come to the hypertonicity clinic are often those who were considering surgical tone management or have been proven to be difficult or puzzling challenges. Um, so we consider the etiology of the hypertonicity. What are the neuroimaging findings? Has there been or does there need to be genetic testing? What kind of hypertonia is there? What interventions have been tried and what have been the responses? We use a couple of different scales. The modified Ashworth scale measures spasticity. The hypertonia assessment tool helps distinguish spasticity from dystonia from rigidity to give us an idea of what we're talking about. And as Dr. Sun was speaking a minute ago, you heard him talk about dystonia being a contraindication for a selective dorsal rhizotomy, which is why we're trying to figure that out. We also do an orthopedic evaluation to look for hip dysplasia, for scoliosis, for contractures that might affect our outcomes. Um, we also get a functional gait analysis in clinic and often refer people for a three-dimensional computerized gait analysis. We also make sure to clarify goals and expectations. Uh, what is the family coming in, what are they hoping for, and is it something that we think can be achieved? And then we formulate our Pre and post-op rehabilitation plan. Goals can be fairly wide ranging. They can be family just wants to be comfortable or or make it easier to position and take care of the child. Maybe they want to use a walker or a gait trainer or walk without a walker, walk better or longer, run or look normal, um. So a wide range of goals. Some folks' goals are modest, some folks' goals are ambitious, and some folks' goals are achievable, and sometimes we have to counsel through, um. Gonna let you Peter, I think you actually may have, may know this kid from the beginning. Yeah, this is one of our earliest rhizotomy patients. As you can see, um, At 6, he has spastic dysplasia, and when he walks, he's in quinas. There's some scissoring. Um, he has really good balance but can't really run this way. And of course, uh, he won't be able to walk like this over time. It's not a sustainable gait pattern into, um, middle age and adulthood. Yeah, so, a PT assessment, he obviously has, besides his spasticity and his equinus contracture there, he has good balance and strength, but as it turns out, actually, we'll see his equineness is pure spasticity. So we felt this child was a good candidate for STR. In fact, one of the outside labs, he said he was not suited. Our discussion centered on whether he uh was suited for STR versus just heel cord lengthening. Um, this is back then, we did, um, a five-level laminoplasty, uh, to get to each nerve root. Right now, we've, uh, uh, uh, refined that to just a one-level, uh, um, exposure, but, um, you know, um, yeah, let's take a look at how he looks afterwards. Uh, this is, um, 4 months later after he went to our intensive inpatient rehab. You can see he's still using a, a, a walker. But he's down on his toes. His equinness has reversed, uh, and, uh, you know, um, we also incorporate some casting sometimes when we're in the, uh, immediate, uh, inpatient post-op period. Um, and then, uh, two years later, you can see his gait is essentially normal. And This is without, uh, any orthopedic surgery, right? I mean, if one just cut his heel cords, um, it would not have addressed his scissoring and then he could have relapsed or if one, overdid it at his heel cords, he could even be crouching. But taking away this patient's spasticity and this classic spastic thigh patients, you can see he has a essentially normal gait now. And then we got a hold of him years later, recently, um. Yeah, so they sent us, uh, he sent us this video at 19, uh, wave surfing, and then, uh, he, uh, recently graduated from a master's program and getting his degree. So this is, this is obviously very rewarding because, um, they not only get better, they get better with time, you know, as if, uh, in the And as opposed to, um, just, uh, getting worse and worse over time with, uh, increasing, um, musculoskeletal morbidity from his spasticity. So this is a really good outcome to share and really heartwarming to see. So, um, he did develop a little pars defect, and I will mention that, um, because of the multi-level laminectomy and then we, uh, uh, did modify our technique and refined it to, uh, just one level. Yeah, so, um, the literature also shows us that, you know, STRD results are sustained over time. Uh, this is from Doctor Peacock's original cohort that he, uh, popularized the operation in South uh, and, um, Uh, originally in South Africa and brought it to North America. Who I learned the operation from. Yeah. And then, uh, review of orthopedic surgeries after STR also shows that we have reduced number of orthopedic surgery that's required, uh, particularly, uh, in patients with, um, Um, higher, um, um, uh, uh, level of function. There have been some interesting, most of the, most of the studies do show that there are fewer orthopedic surgeries after a selective dorsal rhizotomy than without, though there have been, there's been at least one interesting multi-center study that seemed to depend, the number of orthopedic surgeries seemed to depend more on the center than on the. Spasticity management. Um, but. Um, so, Here we have another, here we have another patient who we had, who we did. This is somebody who was GMFCS3 to begin with. They weren't able to walk independently. See, they're, they're needing handhold assistant falling over. Even so, their gait is scissoring, their legs are crossing, they're high on their toes. Um And they're weak. So what do we hope for from the surgery for this? Well, to get them flat, hopefully to get their legs to do less crossing. Maybe be able to get to some walking. So this is one year. This is one year post op. You can see that they are not scissoring anymore. They're walking with essentially a heel toe or at least a mid-foot to toe progression. They're walking independently, most importantly. Um, and this is somebody who went through the 4 to 8 weeks of inpatient rehabilitation, uh, which is, which is critical, as Dr. Sun said, because folks who have Because folks who have learned to walk, learned to move with the spasticity in place, learned a whole set of motor patterns to compensate for that and to work through it. If you just let them go back to walking, they will. resume a lot of their old habits and patterns. Instead, what we have is doing physical therapy to work on retraining the into a more typical gait pattern. This is another person we were able to get a hold of much, much later who's just demonstrating their essentially normal appearing walk and jump in their kitchen. Yeah, this is really a remarkable result, uh, Kate. A lot of people will look at that first video and say this child is too weak. You know, um, STR will make them weaker. But in fact, with our protocol with the inpatient rehab, once spasticity is gone, we can take advantage of that immediate period of neuroplasticity and improve their strength too. This is, uh, the patient went from a 3 to 1, basically. So this is really gratifying for our team, um, and a great result. Yeah. A little bit more here on the role of PT and OT. In addition to the post op role, they have a significant role preoperatively. They, they do an evaluation as part of the multidisciplinary clinic to find current functional status, current range of motion, and current tone. They also help with determining goals and get a sense of what the families are thinking. They also evaluate also in their evaluation, they're able to get some. Sense of whether the children are able to listen and to follow directions and will they be able to Actually manage 23 hours of therapy a day, 5 or 6 days a week, or will this just be too much to ask. Um, and then of course, there's a post-op therapy as mentioned before. There have been studies to show that selective dorsal rhizotomy combined with physical therapy is better than physical therapy alone in terms of the gross motor functional measure change. We think the PT is important, but that it can, but we now, we know that it can only go so far without changing the underlying spasticity, which is what's impairing a lot of, uh, which is what's impairing a lot of the function. Um, Yeah, this is uh one of our earlier patients as well. You can see this child, uh, uh, has a spastic dysplasia, but also the balance is not great. Can't walk, uh, without, uh, you know, These are Um, very fancy crutches, um. And um His initial evaluation was at 4. We subsequently felt that uh he was a good candidate and, and he went through the um rhizotomy program with us and we have a video of him 5 years post-op. And his gait pattern is not, uh, normal, of course, but he is walking independently, um, from using, you know, uh, crutches to walking independently. And then like Kate was saying, these kids, you know, they get, they do get also better over time and we have another, um, uh, follow-up video, I think, of this patient, uh, in his, uh, in college now or grad school again, um, you know. His gait, you can still see a, a somewhat spastic diplegic pattern, but he's much, much better and able to ambulate independently and run, uh, as opposed to, um, using crutches. I, I think if nothing was done for this child, uh, he would be. Probably in a wheelchair in the community. Um, I I think we're gonna, I'm gonna move us with our, um, I know that we're supposed to have a good period left for questions and I want to get to, uh, no, actually this is, this is a decent one to do before we jump ahead a little bit. You mentioned about the um Um, yeah, the psychological, um, yeah, because this is a good one to do the, because the psychological slide follows this one. So we got another, another person who's, I mean, walking independently, but that's not a gait pattern that's going to get you, that's either going to get you around very well in the community and it's going to draw a lot of it and it's going to draw a lot of attention, um. He's doing a lot to compensate for his, for his poor balance with his arms, and so they're waving all over the place. It's just a gait pattern that's probably not efficient in the community and is going to draw a lot of attention. Yeah, big social psychological impact. So here he is, uh, 7 years after his rhizotomy near. You know, I can, I can really put on my physiatrist hat and see a couple and see a couple things that are different with that gait, but It's pretty typical, but it's pretty close to typical. You're not going to really notice them walking down the walking through the airport. OK, and here at the end. At 22, Jumping Walking again, just maybe a little bit of extra trunk sway, but really close to typical. Compared to Where we started at the top. Which brings us into the psychological aspect. Peter was alluded to. I'll let you address that a little further. Yeah, I mean, you can see that his original gait pattern would have a lot of um impact socially, um, you know, um, self-awareness leads to depression, anxiety, but, you know, uh, as this um patient uh illustrates and as this study shows, you know, STR patients in adulthood have uh similar rates of anxiety and depression compared to, um, They're non-CP peers. So, um, uh, physical function is, uh, obviously, uh, very important, but they're also, uh, aids in their psychological well-being. Mm OK. Then here is somebody who was who's a little bit less typical. This is somebody who had, who was born full term but found to have a genetic condition, Sjogren-Larson syndrome, leading to his spasticity. The classic paradigm is that SDR works best for folks who have spastic diplegia secondary to paraventricular leukomalacia from interventricular hemorrhage due to associated with premature birth, and You know, there's been a question, you know, who does it help beyond that? Does it help folks beyond that? This is one of the folks we saw. Who has a different ideology. He got around at home primarily by crawling. You can see that he is able to walk with some help, but he's way up on his toes. Knees are bent, leaning way forwards. And so here we see him post-op, uh I don't know. Here we see him about a year. I don't know why he's not working very well. Post-op by about a year, we don't see that his GMF-CS level has changed. He's still walking with handhold assist or with a walker, but his comfort is improved. His range of motion is improved. He's not, so he's, and he's not dealing with contractures in the same way that he was before, meaning that his long term prognosis for maintaining a functional, his current functional status is improved. Uh, we're supposed to leave about 20 minutes for questions. I want to pull us through here to another one who was a. Who was a debate, as we, as we talked about at the beginning, there's always some questions about is this somebody who's a good candidate for rhizotomy or are they too weak or are they using their spasticity for something. Here's somebody who was atypical in a couple of ways. This is somebody who was older than typical at 13 and Concern that he was actually needing his spasticity because he was so weak he needed his spasticity to stay upright. Would we actually improve anything with his With a rhizotomy. Right. The fear is always we would make them a bit weaker and instead of being able to use this gait trainer, he would just, uh, kind of collapse down. Um, Um, Couple of things here though, he still has a decent gait pattern, right? So we felt that by taking his spasticity away, um, Uh, he could be better, so we went ahead, yeah. Um, Brilliant. My whole computer may be in the process of crashing. OK, there we go. It seems to be, it seems to have come back. It was just offering to restart itself because nothing worked. Um, so here he is, uh, here he is after surgery. Yeah, something has gone wrong with my, something has gone wrong with my computer. It's giving, it's throwing all sorts of error messages. What we can't see is that he is ambulating with handhold assist and with an improved gait pattern, he's hitting, he's striking with his heels and with his, with his heels first. And has not in fact collapsed. Peter, do you have the link to the slides handy? All right. I can also share them from my end if you want to send them to me, that's helpful. Whatever is easier. Mhm. Yes. Let me bring it up. I'll share it from my computer. Sounds good. Yeah. So that patient was older, had some contractures already. Certainly will need additional orthopedic surgery, but, you know, uh, surgical spasticity management, uh, the STR can help, uh, older, uh, patients as well, certainly. Um, we've done a little bit older, 1617, but we really like to get to them. Um, much earlier. Um, I think, uh, the decision-making for us, uh, in that case is, in the, in younger patients is much more straightforward. Um, after a lifetime of spasticity and compensatory changes, um, um, sometimes it's, um, harder to predict how people will do. I will have to say that I think in our program with SDRs, I don't think we've made uh people worse with the exception of what Kate was mentioning. Children who have, uh, dystonia, perhaps masked by their spasticity. Once their spasticity is gone, uh, for some patients, we've seen their dystonia kick in a lot worse. All right. So, um, I'm gonna share Uh, replace current share. Are we seeing our slides now? Yes, we can see them. All right, let me run this down to where we are. Oh We were at 54, I think. Let me just say that for now. OK. All right. So this um patient, one of my It may take a moment to load the videos. Here we go. Yes. So now he's able to take some steps independently. I mean, this is Really important for him, right? He can now transfer much easier. He doesn't need a caregiver for activities of daily living such as transfers, and that basically makes him from a dependent person to an independent person if he can uh ambulate sufficiently, uh, to take some steps to transfer. So we felt that this would make a really big impact for this patient. Uh, over a lifetime as well. All right. Here he is taking some steps with support, um, but minimal support. All right, So, moving on to the more severely affected patients, uh, this is a child with a severe spastic cardia pregia secondary to a noxic brain injury from cardiac arrest. And You can see, uh, we're having a really hard time, um, bending the leg here. Um, mom has a hard time, um, changing, cleaning, um, Just have a real hard time um achieving activities of daily living. And here we are post-op from the STR a lot looser. I mean, these are not the dramatic pictures that we're showing with our functional patients, but, um, in terms of, um, you know, family satisfaction. Um, it really makes a big difference, uh, to reduce the, um, burden of care, and, uh, this is what that mom said to us in clinic in follow-up. Right. Our family is relaxed more now that he's more comfortable. I have so much energy. I signed up for to run a half marathon. I have more time and energy. So, and I saw, and I, I, I've continued to follow him. He's one of my primary patients and uh have heard reports on how that half marathon went, which was successful, and she's now committed to doing it, to doing many, many more. All right, she finished, huh, for her. So I mean that in some ways that I think is, well, it's not, well, you can't necessarily quantify effort of taking care of somebody with severe spasticity. I think that makes, I think that gives you as a qualitative a comparison as possible. It's about as hard as running a half marathon. Yeah. So, studies have shown this uh impact of selective rhizomeome to cerebral palsy children, uh, reduces caregiver's burden and um this, in this paper. Um, and also improves the quality of life, um, in terms of pain nursing, positioning and dressing. Um, this is our, uh, cohort that we, um, followed, and, um, one of our nurses, uh, Melissa Hirshhorn, uh, did, uh, a number of family, um, questionnaires, uh, and, um, asking, um, to assess the quality of life. Um, after a rhizotomy versus a, a group of patients that we placed baclofen pumps, and you can see that, that, that they're generally much uh or strongly agree that the, the quality of life has improved for, uh, the family, um, and for the patient. I think, I think this illustrates that, you know, I think, I think both groups actually have comparable decrease in the Ashworth scale in terms of You know, the spasticity on bedside measure, but I think the um. Escalation in medical care for, um, patients, uh, with pumps in terms of refills and, uh, going back to the hospital for that. Uh, the prospect of repeated surgery, I think, um, you know, kind of drags that down a little bit. Um, but we do have patients that, um, respond well to the pump, particularly patients with both spasticity and dystonia because rhizotomy does not address the dystonia, as I was mentioning, or Complications actually are increased dystonia when patients have both, when the spasticity is reduced and a masks the dystonia. But when patients have both dystonia and spasticity, as you can see, this child has stiffness and also uh dys dystonic arm movements. Um, these are the patients that's ideal for, uh, uh, intrathecal baclofen pump. And this patient, um, had a spinal fusion. Um, he, um, and let's go back. Yeah. So, you see on the right after the pump, oh, this video was playing both. Yeah, you can see after the pump on the right side, he had, he's much less dystonic, right? Those dystonic movements have reduced, his spasticity has improved, so he had a good result from the intrathecal baclofen pump. But he also illustrates some of the complications we have with pumps, you know. Um, he did go on to have a spinal fusion in age, uh, 13. His, um, catheter, his intraspinal catheter, uh, kinked. Uh, he went through withdrawals. He had emergent surgery for pump catheter revision, um, because of the kinking and, of course, uh, the refills are every 5 to 6 weeks. And kind of like our other patients with shunts, whenever something's wrong with the patient with the baclofen pump, uh, we always think, uh, that it's because of the pump. You know, people kind of focus on the pump, whether it's, um, functioning or, or not. And, um, but, you know, patients do get other things and, um, every time he does go to the emergency room for any medical problems, viral illness or UTIs, he gets an extensive workup with imaging. Uh, hospitalization, etc. to work up the pump. All right. So that's a fairly large number of patients. This is the patient that Kate was talking about. Uh, uh, uh, he is 14 years old. He has hypoxic brain injury. On exam, he has clonus. He has hyperreflexic, uh, and, um, well, should, should, what are the goals of, of spasticity managed for him, right? Um, um. He is entirely reliant on mom holding him to generate any gait. um, and, um, when asked about the goals of treatment, family would say, well, we want him to walk better. This is the kind of person where, uh, the, his spasticity helps him, uh, in terms of what he is able to do right now, uh, which is stand, pivot, and transfer. If this patient had a rhizotomy, this patient would definitely collapse down and not be able to do the stand, uh, um, pivot and transfer. Um, OK, what, what do you see when you look at this patient? Um, I see that he is, can we restart one of the videos. So mom is providing, mom is providing all balance. I don't think she's providing a ton of strength support, um. But he is largely using a very stereotyped gait pattern as in it's very reflexive. He doesn't have a whole lot of selective control. Which is a concern in terms of thinking about going on to a rhizotomy. So selective motor control is the ability to move one joint in the absence of moving all the rest. I have great selective motor control at hips, knees, ankles, and toes. I can move any one of those without moving any of the other. Many of the folks we look at for rhizotomy will have good selective motor control at hips, probably knees, and then trouble at the ankles and the toes. Why this is important is because to have a good, to have a typical gait, you need to be able to control those joints individually. In the absence of good selective motor control, the reflexive gait pattern actually depends on some of those synergies that you get from the spasticity and the and Having the lack of reflex inhibition. Yeah, I think is, we don't, we don't see selectivity testing on him, but his whole gait looks like it's pretty much entirely um reflexive. Yeah. So, um, we did not end up, um, uh, doing a, uh, rhizotomy on him because we would think that, uh, it would make him, uh, uh, uh, worse, um, more certainly probably not better, uh, because of the reasons, uh, Doctor Sickford had just mentioned. Yeah. So, this is the, uh, um, Um, caregiver reported outcome measures that we showed the bar graph on. Um, patients are quite satisfied with their results. Um, Yeah. Great. So, um, Kate, any other comments? um. Or we have uh a few minutes. I have a couple of questions in the Q&A, yeah. Um, both of them are related to care for adult patients. The first is, uh, understanding what is available for adult patients with spasticity and Parkinson's syndrome, and the second, um, Is SDR ever used in adult stroke patients to improve their function? There is, I'm going to start with, I'm going to start partly with the second one first, and it is that there are a small number of surgeons doing selective dorsal rhizotomy for adult patients with cerebral palsy, largely cases, largely people who were a little bit too old to or uh. A little bit too old to have been able to get to a center when rhizotomy was just at, you know, a real specialized surgery at one or two centers, and some of those folks are having nice, are having nice outcomes. I have not heard yet about it getting used for stroke yet. Have you, Peter? No, um, stroke, of course, is mostly, um, uh, hemiplegia. The ideology is, uh, a little bit different. They do get spasticity after their acute, um, period. Um, But we have not done, there has not been a large body of clinical literature on, on stroke patients. I think one of the things with a rhizotomy is the rehab that we talked about. I mean, we do make patients weaker, um, initially to allow their uh neuroplasticity to help them improve once their spasticity is gone. So, I think, um, It would be hard on the stroke patients given their overall, you know, general age and associated morbidities. Um, for the adult, uh, CP patients, we only have experience doing them on ambulators and, uh, with, uh, extensive rehab afterwards, uh, they, they do improve. Um. But, uh, we only have experience with, uh, uh, patients with who are ambulating. Mhm. Um, as far as adult patients with spasticity, uh, obviously the same non-surgical treatment options are available. The medication, the medication options, um, the chemo denervations, the Botox and botulinum toxin injection options, um. Yeah, with, uh, Parkinson's, we have another type of neurosurgical treatment. It's called deep brain stimulation that works, uh, uh, very well, um, by interrupting the Parkinson's circuitry. Um, so, um, yeah, so that would be more of, uh, um, functional neurosurgery on the adult side with, uh, um, Uh, deep brain stimulators or uh microablations using ultrasound, uh, for, um, Parkinson's and other, uh, adult movement disorders. Created by
