Chapters Transcript Video UCSF500 Testing Helps Personalize Pediatric Cancer Care Precision Medicine in Pediatric Oncology at UCSF Good morning everybody. Welcome to neurosurgery community grand rounds. Um, our topic today is spasticity management and cerebral palsy. It's a topic that I'm really excited about. It's one that Um, really brings joy to me when I see some of these patients and follow-ups, so, um, I'm glad that, uh, you joined us, um, this morning. So I'm Peter Sun. I'm a pediatric neurosurgeon at Bea Children's Hospital, mostly based in Oakland. Uh, joining me as panelists are Yumi. Uh, Matsuya, or rehab physician, Courtney Sager, our rehab physician, Lucy Tomlinson, our, uh, physical therapist, um, So, uh, we want this also to be interactive, so please um ask questions in the chat box and we'll uh try to answer them as we, as we go along. This is our group. This work is a collaborative effort from our spasticity clinic that we started back in 2005. Uh, this is Doctor uh Peacock here. I actually didn't train, uh, knowing how to do SDR, so he, uh, joined our clinic. Um, I learned from him and I'm really grateful for Doctor Peacock's, um, Contribution to start our clinic and we've been going strong since and this is our last uh picture. um Bob and I are the only, the ones that are still there. um Bob Haney of course is our uh other uh rehab physician. So we all know what cerebral palsy is. It's a, um, group of disorders that comes from disruption of the fetal or infant brain development and results in motor and other disturbances. It's the most common childhood physical disability worldwide, and in the United States there's close to a million people with CP, and the incidence is close to 3.5 out of 1000 at age 8. So, uh, we're also familiar with the paradigm. We have some sort of brain injury that results in loss of inhibition and modulation of the intrinsically hyperactive and dynamic spinal reflexes, and the result is we have hypertonia or increased muscle tone. Um So there's various types of hypertonia in cerebral palsy. Uh, spasticity is defined as velocity and resistance to muscle stretch, and it is the most common form of, uh, hypertonia in, um, cerebral palsy, as we can see here, although, um, these types are frequently, uh, mixed together and it's important during physical exam to try to tease out, um, what patients have. So there's a variety of ill effects from spasticity and cerebral palsy. It inhibits motor development, causes muscle contractures, reduces function, daily activities and exercise, interferes with care, positioning, and tolerance of braces, causes people to fall, have chronic pain. Obviously it increases the burden of care for the caregivers and parents, and it certainly creates social stigmatization. So functional performance in CP is classified by the gross motor function classification system from 1 to 4. Level one is defined as basically normal. You can run, uh, may have difficulty on uneven surfaces or with endurance, some balance issues, but very functional. Level 2 cannot run, um, but can get by walking. Level 3 walks with assistive device. Level 4 an assistant transfer. Level 5, nowhere severely affected and cannot stand or sit. So, the goal of uh spasticity management is to improve gait. You know, gait obviously is a coordinated concentric and eccentric muscle contraction and spasticity management can greatly facilitate this and CP patients. We also want to improve motor activities in daily living, other types of motor activities. Uh, we want to decrease orthopedic deformity, improve comfort, minimize pain. Decreased burden of care and also, you know, improving cosmesis is not an insignificant factor. So of course, spasticity management comes in many forms uh and uh we have medication, PTOT, bracing casting, Botox, and other injections. Today as neurosurgery grand rounds or community rounds, we're going to focus on selective dorsal rhizotomy and intrathecal baclofen pump therapy. So, just as an intro to the audience before we get into uh our content, um, I'm gonna ask everybody to just uh see which neurosurgical treatment improves upper extremity spasticity. Intrathecal pump. Uh, SDR selected ulcer rhizotomy, both A and B or neither A or B. All right, we'll move on and uh take a look, yep, OK. All right, so, starting with selective dorsal rhizotomy, um, selective dorsal rhizotomy is cutting the dorsal nerve roots in the lumbar region to reduce reflex and tone. Since we have loss of inhibition, we have the hyperexcitable spinal reflex and selective dorsal rhizotomy is to cut some of these to reduce the reflex and tone. Um, the, uh, reflex physiology was actually worked out by Sherrington back in 1898 and in the 1970s, Fasano introduced the concept of selecting uh abnormal responses from individual nerve roots, hence the selective and selective dorsal rhizotomy. Uh, Dr. Peacock popularized this treatment in North America in the 1980s and 19990s, and Dr. Park, um Uh, uh, refined it to a one-level laminectomy as opposed to a five-level or four-level laminectomy, and I find it interesting that exactly 120 years after Sherrington worked out the reflexes in 19 or 2018, the National Health Services in England concluded that SCR improves its gross motor function and quality of life and would be funded. So today we are moving into an era where STR is a more minimally invasive operation and um we can see benefits for more affected children and its effect on upper extremities. All right, so this is the paradigm. The paradigm is we have patient prone in the operating room. We have electrodes in the various muscles in the anal sphincter. Our setup is that we can also test clonus intraoperatively with a clear dressing. Uh, the clonus is exposed. They're individually stimulated for the most abnormal responses that we can detect in the electrodes. We have worked out our own classification. The normal nerves are just a square little response block. The abnormal ones can be sustained or they can cross over to the other side and affect multiple, multiple other muscle groups. So those are the ones that we classify as the worst nerves which are selected for rhizotomy. So the selection criteria, uh, we like to get to children early if we can. Uh, there's two types of SCR. The first type is functional, where we're trying to improve functions. So, um, age, we want to be young but greater than 3 to 4, uh, so they can cooperate with our 4 to 8 weeks of inpatient rehab program which we think is very important. Uh, patients with the most predictable and consistent favorable outcomes are those, uh, with CP from, uh, prematurity, although other ideologies are considered. We want their hips to be stable. We don't want them to be over lengthened with orthopedic surgery. First, that makes a rhizotomy very difficult to predict in terms of outcome if they have had that. The response to Botox certainly is a good indicator. I tell families that's a good preview of what rhizotomy can do on a permanent basis, and they need to be able to follow directions and participate with therapy for our program. So, also we do STR for therapeutic or palliative effect, and these are patients that are more affected, GMFCS 4 and 5 for improved ease of care and comfort. Um, for these patients, upper extremity can improve as well and we'll, we'll show that, um, and um we want the spasticity to be predominant and little to uh no dystonia um because that can become a massed. Moving on to intrathecal baclofen, uh, baclofen is an inhibitory GABA receptor agonist, reduces spinal reflex. Uh, the, uh, drug can be delivered by pump through an intrathecal catheter directly into the intrathecal space, so we're not taking it orally to affect, um. Um, um, overall systemic issues. It is reversible. It is adjustable, um, the location of the catheter is variable and can be targeted, uh, to the proximities, and it does have an effect on dystonia. No, but it has costs associated with it. Of course, it needs refills and replacements, and there's a variety of hardware complications uh that can result. Um, it's reserved for, um, more severely affected patients, uh, for us and those with dystonia and for spastic quadriplegia patients, it really overlaps with our therapeutic STR and we're leaning more towards STR in these patients due to some of these factors now. All right, so spasticity management, of course, is just part of our multidisciplinary hypertonicity management which includes PTOT, exercise and diet, um, bone and joint alignment, orthotics, and those patients with movement disorders in our clinic, um, you know, some of them may be candidates for deep brain stimulation. So when we refer a patient to our hyper uh to our uh hypertonicity clinic, uh, you know, we're really talking about um a multidisciplinary evaluation by our physiatrists, orthopedic surgeons. We also have a neuromuscular neurologist, neurosurgeon myself, movement disorder neurologist, PTOT rehab. Uh, nurse clinicians, social worker, and dietitians. So collectively we go through these, uh, assessment questions. I think in particular, we want to know their family situation to see if they can stay with us for a prolonged inpatient stay. That's important for the STR program, um, and then we have to set goals and expectations in terms of what we are doing. With spasticity reduction. And these include to simply be more comfortable, um, to have better ease of positioning and daily cares, um, perhaps be able to transfer or use a walker, walk without a walker, even walk better or run and perhaps improve cosmesis. So I think these are all reasonable goals, all goals that we can achieve, but we must be able to delineate. What is possible and I think that's best done by going through uh some of our patients. So, um, here's patient number one. All right, so patient number one, he's a 6-year-old with a history of premature birth at 35 weeks with spastic dysplasia, was functioning at a GMFCS2 level. He walked independently at age 2, but in what we call a true Aquinas gait with the ankle implantar flexion throughout the stands phase and the hips and knees extended. He also has some duction and internal rotation at the hips. He had fairly good balance, but due to the spasticity, he required handrails to navigate stairs, and he was unable to run without tripping frequently. And here again, you see that his static balance is pretty good, but due to being up on his toes, squatting is difficult without handhold assist. However, he's able to come back up to standing, showing that he has fair hip strength, which is a good prognostic factor after SDR. He had an um instrumented gain analysis at an outside center and was recommended that he not proceed with an SDR. His spasticity was worse at his ankle plantar flexors, and he did have plantar flexion contractures that he had developed over time. The discussion within our team then was whether he would benefit from a a heel cord lengthening procedure versus a brithotomy. With shared decision making with the family though, he ultimately underwent an STR followed by an inpatient intensive rehab course. And this is him on the left 4 months post-op at an outpatient follow-up visit. He's requiring a walker to take steps with a heavy reliance on his upper body to maintain an upright position and with a lot of effort to do hip flexion to advance his steps, but he's starting to get some good heel contact on the floor. We do see that a lot of times after removal of spasticity post STR with aggressive stretching and things like serial casting and splinting during the acute rehab course, we can get significant improvement in range of motion at the knees and ankles. And on the right, 2 years post-op, he essentially has a normal physiologic gait. And on the next slide, here he is as a young adult living his best surfer life with his feet beautifully flat on the surfboard, and your balance really can't get any better than that. So with the SDR, his GMSCS level improved from 2 to 1. He now has a normal gait that has been sustained over time, and if we had gone with the heel cord lengthening instead, we may have wrist over lengthening at the Achilles, leading to development of a crouch gait. It wouldn't have addressed the hamstring or adduct or tone, and with spasticity still an underlying problem, the plantar flexion contracture may have recurred over time. He did have an L5 pars defect that required repair as a teenager that Doctor Sun will touch on in a later slide. Yes, so we started off doing the multi-level laminoplasty. I just wonder if that had any um uh impact on on the development of the pars defect, but I think this patient nicely illustrates what we see in the literature and that our STR results are sustained over time and that independent walkers and diabetic patients will have the smallest amount of subsequent orthopedic surgery post STR. Um, from a multi-level laminoplasty, we've really turned this into a minimally invasive surgery where we do a single level laminoplasty. Laminoplasty means that we don't even take off the bone. We lift it up as like we hinge a door and we put it down, and the exact level of the laminoplasty is determined by a quick scan and MRI, which just takes 5 minutes and these patients don't even need sedation for us to know exactly where the connus is. Once we visualize where the connus is, we design our cut to expose the connus and you can see the incision now is only 3 centimeters, you know. So after we expose the connus, uh, this is the dura. We open the dura, we simulate. nerves like we were talking about to find the worst nerves and 50 to 70% are cut per the neurophysiological findings and as it turns out, sensation is very overlap and we don't have any permanent sensory impact and there's some evidence even now besides the clinical improvement we see that on muscle biopsy or muscle measurements, it facilitates muscle regrowth. So moving on to our next patient. Yep, our next patient is 6 years old. He was a GMFCS 3 community ambulator with a reverse walker and could take steps also with handheld assist, but in Aquinas, a crouch at the hips and addduction and internal rotation left worse than right, with his knees, especially on the left, not getting much extension during swing phase due to hamstring spasticity, and on stand phase, he shows hyperextension at the knee from a combination of the plantar flexor spasticity and quadriceps sweepness. His balance is poor as you can see, and you'll also note that in taking steps, his arms are in a flex position due to dynamic tone kicking in at the upper extremities as well. Yeah, he's pretty weak, isn't he? And what are our goals going in? So it was, um, for him, you know, goal setting was uh a challenge kind of setting realistic expectations. We weren't sure um with the spasticity removed, how much balance he actually had, but after his STR it became apparent he actually had fair balance when asasticity was out of the equation, and this is him one year post-op and he's now walking independently. And he still has impaired ankle dorsiflexion and has a mostly flat foot contact and with some quad weakness with that hyperextension that you still see on stand. However, with AFOs on, his knee hyperextension resolves, and he was even able to run, which he couldn't do before. And you can also see that his arms are now more nicely extended at his sides instead of in a flex position. To get to this point, post-surgical inpatient rehab is really crucial for the initial neuromuscular retraining to have the therapist really guide his movements so he could relearn the more physiological muscle activation pattern for moving and walking and not revert back to his previous walking pattern. Yeah, I just think this kind of result is really made possible by our extensive inpatient rehab part of the, the rhizotomy program. So we know that um perspective, there are perspective studies for SCR that shows that it gives better um motor uh measure scores uh than PT alone, but I think, uh, you know, uh, we'll have Lucy talk a bit about our inpatient rehab program or the PT component which is integral to uh our SCR program and enhances the outcome. Hi, yes, so, um, in the, after a rhizotomy, it's different than other, um, injuries that you see in, uh, rehab, whereas from a brain injury or something, you're trying to revert back to where you once were. But after a rhizotomy, it's different than, um, trying to revert back to where you were or developing in general, because what you're trying to do is, uh, get to have a new motor pattern. And that's why it's very, very important to go back to a basic strengthening level, because these kids were using tone instead of true string. And yeah, it was in a non-deal position, usually a crouch gate or something, where some of the muscles like your quads are in a lengthened position when they contract, and other um muscles like hamstrings and uh gastros are in a more contracted position when they're trying to contract or shortened position when they're trying to contract. So we need to get those muscles to contract in the more lengthened or shortened positions, and that means that It is, it kind of in the basics in the, in the beginning with a lot of more um strengthening prone exercises, things of that nature, and not walking right in the beginning. You can't just get up and walk, and I think that's something that needs to be expected. Um, I think you saw in the patient, and the, the first patient we saw, the 4-month post-op, you know, before you're like, oh, they were walking. Without a walker up on their toes, and 4 months post-op, they're walking with a walker, but it's a very much of a long game, and you can't just jump right back into it, and you have to give them the supports that they need in the beginning, so that we can actually have these very long-term results. So it is a long-term game with therapy going on for for a lengthened period of time, but most importantly, getting, uh, getting kind of a A better contraction in the new positions they are before getting them up on their feet and just walking. Um, I think the fact that it's a long game is so important to emphasize, uh, for example, um, Um, this patient right at, um, age 4, you can see how weak we are, um. Um, spastic dye, um, I think this is another patient who you would not imagine this result without an extensive inpatient rehab that we see five-year post-op, um. I would say this patient we saw initially at 4 waited till 6. Today, um, I might um push for this patient to be done a little earlier because we still have, uh we have some muscle contracture issues, but he's, you know, I, going from here. To where he's walking independently, I would have to say I couldn't really say that we knew that was going to happen. So I think PT or inpatient rehab program, is critical. All right, um, next patient. Um, so our next patient, um, came to us at 8 years of age. He was a GMFCS2 and a community ambulator with AFOs, but we can see that he has very poor postural control and balance, some scissoring up on his toes, but really almost kind of an atoxic gait with variable foot placement with each step and arms that come up to high guard to maintain his balance. He was very conscious of his gait and how it made him stand out so much from his peers, but in our pre-op discussions, again similar to our previous patient in setting realistic expectations and goals, we weren't entirely sure what his ultimate potential would be since he had such poor balance as well as apparent weakness that would be further unmasked following removal of his leg spasticity. So, um, you can see also that he had a lot of clonus there, um, and then here, um, this is him 8 years later. And what a difference, right? His feet now come nicely down to the floor. His knees still stand some flexion throughout the gait cycle, and he still has some um truncal sway, but he was happy with the results of his STR, stating he could now walk without his AFOs because, side note, as we see in most of our kids, um. They stopped wearing their AFOs once they get to preteen age, since they don't want to wear anything that makes them stand out from their peers, but he was able to walk long distances, not fall, and he said at that time he now feels normal. And following the appointment at which the previous video was taken, he underwent ileosois and hamstring lengthening at both legs, leading to an even smoother gait, and at 22 years old now maintained his hobby of playing basketball with his buddies. And we can still see a bit of that tranquil sway there, but he has a gait pattern that a non-medical person would likely not notice as being different and may simply characterize as walking with some swagger. Yeah, what a difference, right, comparing him to 8 years ago, 14 years ago. Um, Yeah, so we know that adult CP patients have a higher rate of anxiety and depression, and I think this patient's a nice illustration of, um, you know, you could get to a point that you essentially look normal, and SCR patients being followed for long term actually have similar anxiety and depression scores compared to typical developing adults, and I think in large part due to the effects that you were able to see there. Um, all right, moving on to our next patient. Our next patient is um a 7-year-old who was 7 years old when he presented to our multidisciplinary spasticity clinic with a past medical history of prematurity, born at 29 weeks, and perventricular leukomalacia seen on MRI suggesting diplegic cerebral palsy. Uh, he didn't take his first independent steps until, um, around age 4. But, um, his parents stated their functional goal was, uh, with surgery was to make him look normal. And he's already walking at a GMFCS1 level, so the question is whether he was too good for an STR. Um, you can see him on the left walking with decreased dorsi flexion. He makes initial contact to the forefoot, but he's still able to get his foot flat and stance. He has mild increased abduction from normal with a shorter stride. And uh his gait would not be described as um typical, but it is kind of more normal than what we'd see for others in this presentation. Um, but then we see him one year post SDR and you see him, uh, taking longer steps with less scissoring, faster cadence. Uh, he still has some decreased dorsi flexion with whole foot contact while walking, as well as a little bit of a steppage gait to compensate. But overall, the gait does appear more normal, so parents' goal was achieved. He does go from being a GMFCS1 to a better one. he'll walk further distances and he'll hopefully have less breakdown at the mid-foot in the future, uh, with better placement of the foot and ankle and stance. Yeah, terrific. Right, onto our next patient. This is a more difficult patient. Yeah, definitely. Um, he's 8 years old, born at term with increased perioventricular signal on MRI, history of Sjogren-Larsson syndrome, and his primary method of mobility at home was crawling. Um, his initial video shows significant scissoring and severe flexion at the hips and the knees, and he's high up on those toes, and he requires handhold assist for the limited mobility that he has, putting him at like a GMFCS 3 to 4 level. Um, it's not really like a true functional walking, as he would not be able to sustain this for a notable period of time. But if left untreated, he would eventually lose the limited walking that he has and require a wheelchair for all mobility, making him a GMFCS4. And I think for me, if, uh, for any therapists that are walking, watching this, you would look at this and say, uh, I can't really do much with this in the community right now as far as physical therapy to get walking to be better when you're in this state. There's not much to, um, to go off of from here without some further intervention from the medical team. Definitely. And so here on the next slide, we see him post STR and he's still requiring handled assist or a walker to ambulate, but you can see how noticeably more comfortable he is with more dynamic range of motion. His hip and knee contractures are obviously still persisting, but he removing that spasticity will allow for improved um orthopedic outcomes when he eventually gets those released. Um, it will make his future surgery and recovery easier with less chance of contracture recurrence. Um, and you'll also notice that, um, he Uh, he has his feet flat in this video where he didn't in the post-op video. So this kind of demonstrates the importance of having the SDR before the um orthopedic intervention because he would have been at risk for over lengthening the tendons. Um, on that thing, and so this is kind of we're seeing that on this slide as well. That our ideal timing is um age 4 to 6, um, and then prior to orthopedic surgery. And then they may need some intervention for um postoperatively with orthopedic uh orthopedic surgery to release contractures. Yeah, we really want to try to get to them before they get to stage 3 and 4, Um, as optimal candidates for STR. And this is what Uh, Dr. Segar was saying the natural history of certainly 34, and 5s are that it can get worse over time, and we can see from our last patient there was no way he would have maintained even being able to do what he was doing, but, um, patient status post SCR do improve function over time, um. All right, our next little patient. She presented around 8 years old and with spastic diplegia and bilateral equinous contractures that were definitely not braceable. And the stated goal of her and her parents was to tolerate brace use and to emulate better using a walker. You can see here that she is a 3 at presentation. She's requiring a walker to move. And prior to her STR, she's up on her toes bilaterally, secondary to tone and those ankle contractures. Her hips are not consistently abducted, but she's still crossing midline with each step and circumducting and hiking her hips to clear her toes. And then you see her at the MTU post STR ambulating for shorter distances with contact guard assist on the left. And she won't be walking long distances like that, but it really would improve um helping with transfers and shorter distances at home. And you'll notice that her, her ankles are in a better position because she's tolerating bracing now. Um, in the second video, you see her being much more confident with her reverse walker. She's still up on her toes more, a little bit more on the left than the right, and her overall gait pattern is much improved. Um, she'd be able to sustain ambulation all day during school with the walker, which is a big improvement from where she was. Yeah, definitely. All right. Our next patient is a different sort of category. We're moving on to our therapeutic uh patients. Mm. Yeah, he is 7 years old at the time of his SDR. Um, he was a very premature kid at 24 weeks, a spastic quadriplegic CP GMFCS4. Um, he was able to take, uh, to stand and take a few steps around age 1.5, but then had some regression, was no longer able to walk after age 2.5. We tried some botulinum toxin injections, but they, if any effects that we're seen were very short-lived. And you can see here in the video that he has severe adductor tone limiting his ability to bear weight or through his feet at all, um, even with considerable, uh, considerable support by our therapist, the trunk, um, and his goal was to use a gait trainer. The video on the left is still pre-SDR and he required abductor straps, which are essentially exering his bands on either side of the walker to keep his legs apart. Um, even with that intervention, you can see that he's not really able to advance his steps to take uh, to move his feet forward. Rather, he's kind of pushing himself forward with both feet to propel the walker forward just a short distance. And um post STR you'll see that he is using a gait trainer without the straps. He's still kind of doing that push off method and almost hopping forward to propel the walker, which isn't true walking, but it's certainly more enjoyable than what he was doing before. And he's able to bear the weight through his feet, which is better for bone health. And I think it's also important to point out that it's really psychologically rewarding for both the parents and family to see any kind of um uh form of walking with their child, even if it's not necessarily permanent. Um, and the other thing I think to point out with him is that you can't see it, um, in this in this video, but you can kind of imagine how much more comfortable it is throughout the day for him to just exist, um, doing cares for him, including bathing and dressing and diaper changes now that his legs can open up is substantially easier for the family. Um, and he's also able to prevent future orthopedic complications due to contractures with the less or the decreased tone in his lower extremities. Yeah. Moving on to an older patient, um, Yeah, this is a 13 year old, he's a little bit older than the rest of our kids at the time of presentation. Um, he has a history of prematurity, shunted hydrocephalus, and a mitochondrial, uh, disorder, leading to a spastic quadriplegic CP. We classify him GMFCS 3 to 4. Um, you'll notice he has a very high steppage skate in the first video. Ankle contractures are more impaired in the second as he can't really get his feet flat, but you'll see that he's really bearing a lot of his weight through the platform walker to stay upright, um, and just kind of moving his feet forward distally. Um, and the question here is whether he's too old to benefit from an SDR, as it may be harder for him to build his strength back up and relearn to walk given his size. There's also the question of whether he has adapted to using his spasticity to maintain that upright position for transfers and limited walking, which we'd hate to take away. Yeah, I mean, also a bedside exam shows that he has clonus and hyperreflexia, so we felt that even though he may be using some of his spasticity, um, right here it's highly dysfunctional. He's scissoring, he's kinda, his crouch, he's got that mid-foot break, right? So he's got a lot of tone there. Um, so we went ahead, um, with him. Yeah, so you're gonna see him post SDR and he didn't bring his walker to clinic that day, so he's walking with handhold assist. He's still in a bit of a crouch position, but he's using his support person more for balance than to really support his weight, which he was before with that platform walker. Um, and this change of function, um, would decrease his caregiver burden substantially as he gets older, as he's more easily able to transfer and walk short distances within the home. It'll maintain his ability to be upright for longer. Yeah. Um, Lucy, did you, did you know him? Yes, yeah, he was actually my rehab kid, but, but, uh, yeah, when I was in rehab, I worked with him for quite some time. Yeah, good job. Thanks. All right, we have a few more patients left. OK, so, uh, this is our 9 year old with spastic quadriplegia secondary to anoxic brain injury following a cardiac arrest that happened post liver transplant. He's dependent for all mobility, ADLs, and communication, though he's working on using an IEAS communication device. He has significant spasticity at all of his extremities despite being on multiple spasticity medications and having had multiple Botox injections. And the lower extremity spasticity in particular was really making his daily cares extremely hard. So in the last video, you see the quadriceps spasticity making bending the knee difficult, and when he would get upset or sick with a cold or something, his adductor spasticity would also kick in, making diaper changes and lower body dressing really difficult for mom. He also has significant equinovarris contractures at the ankles, and on the right, um, you can see Lucy here arranging his legs much easier. Um, post rhizotomy and even his ankles now get passively almost close to neutral, um, much easier to tolerate bracing and with bracing hopefully can prolong or avoid future orthopedic surgeries for his ankles. And after, uh, sorry, yeah, uh, but like, um, we'd be like, well, what is the point of this, right, well, the point is Yeah, so after his rhizotomy, his mom said something that really wowed us that she now had so much more time and extra energy that she signed up to run a half marathon. And um parents of some other patients have also given us feedback of maybe unexpected, but kind of cherry on the top type effects they were experiencing after their child's rhizotomy. Um, one parent told me they were no longer having to do digital stimulation to induce their child's bowel movements. Another parent was crying when I visited them at bedside on post-op day 2, saying they had never seen their child smile so much in their life, and she was already like 8 years old. Um, many have told us that their child is now sleeping through the night, allowing the whole family to get better sleep. And we get to know most of these patients really well leading up to the surgery, since a lot of the time we're seeing them repeatedly in our rehab clinic for medication adjustments and injections, and like Peter mentioned earlier, it gives us so much satisfaction and joy to not only see these patients being more comfortable and happy, but also to see their families really thriving. Yeah, and we see this er in the literature as well. Uh, this is a publication this year er that shows that the impact of selective ulcer rhizotomy to these children really improves the caregiver's burden, the surgical group, um, with, um, um, you know, statistically significant change there. Uh, we also have, uh, literature that's coming out to suggest this is also published this year that, uh, selective dorsal rhizotomy includes the lives of children with stage 4 and 5, or, um. Level 4 and 5 cerebral palsy, um, with pain positioning, dressing, uh, these are all significantly improved. Um, yeah, it's one thing looking at these charts, you know, but what, um, Yumi just talked about just really brings it home to, uh, see the impact of, uh, uh, positive impact on quality of life and reduction on, um, caregivers' burden, um, uh, for these, for these children. Uh, our own, uh, questionnaire that our, um, Melissa, our, our, uh, uh, nurse clinicians sent out to families also backs this up and that, uh, you know, our patients are happier after rhizotomy, um, as much as baclofen pump or even more so, uh, that, uh, there's a positive impact on the quality of life, um. So, um, this is a patient who had a baclofen pump. I think it's, it's illustrative of something we want to point out that this patient had a baclofen pump, but was kind of tired of the Uh, changes in the pump, the frequency by which she had to come in for refills. Um, this is her with the pump, um, so she still has a lot of tone. Um, the catheter level is at T8, so we had hoped that there will be some positive effect on her arm, but she really wasn't able to. Uh, use her arm that well. I think if we had put the catheter level at a higher position, maybe T1 or C7, that would have done more for the arm. Um, obviously, she has a hemiplegia on the left, but in terms of the right arm, um, but then if we had put it that high, we may have lost, uh, some, um, tone reduction in the legs. So she was converted to an STR. And I, I want you to take a look at her right hand. She has much more um control of her right arm and um also reciprocal abilities in her lower extremities, and in fact, she's able to um ride a tricycle after the rhizotomy and um able to steer it with her right arm. So, more and more we're seeing in the literature that upper extremity spasticity and function does improve after lumbar selective dorsal rhizotomy. Um, we have hypothesis to explain why that might be the case, but we don't know for sure, but clinically, uh, we definitely are, are seeing that as illustrated by the last patient, and this is again, um, Our questionnaire for our patients, and we can see that uh many have responded that the surgery has also improved upper extremity function uh um. Uh, as well as, uh, uh, the other questions in this, um, in this, uh, A patient questionnaire measure. OK. And lastly, we have patient 12. Um, his initial surgical evaluation was at age 4. He had a pump placement around age 7. He has a history of spastic quadriplegic, CP secondary to HIE. His tone was mixed with both spasticity and dystonia, which is why a pump was favored, and you can see him on the left even struggling to, um, stand given the tone and posturing, uh, limiting his intentional movements. Um, post um placement, uh, you can see him taking some very, uh, supported steps with less spasticity and dystonia, um, but his gait is truly not functional. Uh, the video demonstrates improved range of motion and some decreased posturing with his intentional movement. Yeah, less, less dystonic leg movements there, doesn't he? However, however, um, we chose this case to demonstrate some of the downfalls of using an ITB pump. Uh, he underwent a spinal fusion at age 13 and subsequently developed a kink in the catheter that caused significant withdrawal syndromes, and he, uh, symptoms, and he required emergent ITB pump, uh, catheter revision. Additionally, he had, um, he's on a very high dose of baclofen to maintain the improvements that he has, so he has to come in for the, to the hospital for refills every 5 to 6 weeks. Um, another downside is that, um, whenever he presents to the ED with any kind of medical concern, the pump function uh malfunction is always suspected to be the cause, and so it has to be ruled out before really any other workup is started, so this delays his medical care. He's having unnecessary imaging and pump interrogation consistently upon arrival, and he's presented with increased tone secondary to viral illnesses, UTIs, and most recently for um recurrent kidney stones. They've sent him home from the ED twice, um, uh, for with UTIs and only on the 3rd presentation did they check for stones despite a known history. Um, he lives a ways out from Oakland, but he has to consistently come here for care so that providers would be comfortable with managing him at all because he has the pump in place. And all this is to say that when, when we can, SDRs are pretty preferable to pump placement at this point, um, when they're appropriate with fewer longer term complications and risks. Yeah, so in conclusion, um, spasticity is of course just one component of cerebral palsy, but it is amenable to treatment, um, unlike some of the other more difficult aspects of CP, um, and spasticity treatment, including STR can improve function and quality of life, and this has to be done in a multidisciplinary setting. So thank you for your attention. Created by Related Presenters Amanda Marinoff MD Pediatric hematologist-oncologist View full profile
