I am so thankful to have all of you here this morning to come to our talk on Advanced Hypertonia Management in Children, Who, What, When, and Why Not. We're going to go through this in sort of a, I guess kind of a round-robin sort of way. So it's gonna be really quick-hitting, highlights new things that we're gonna talk about with some different hypertonia management options. Just a little bit for housekeeping, cell phones and audio or video recording. Please silence your cell phones. Audio recording is taking place within the room and this is being live-streamed. Because of that, if you can get to a microphone to ask a question or make a comment, we really appreciate that you do that so the folks at home, whoever is out there listening, can also hear what you have to say. If you're asking a question and can't get to the microphone, we'll try and remember to repeat that for the folks at home. As far as evaluation forms, there are session evaluations are located in the mobile app and the online agenda. Search for the desired session in the mobile app or online agenda and click CME and Evaluations to open the evaluation. We greatly appreciate you guys completing that. That will help in determining if something like this is desired next year or how to improve it. And then visit the PM&R Pavilion as it offers interactive resources and educational opportunities, including the AA PM&R Learning Center with complimentary hands-on education. Throughout the assembly on multiple topics, sponsored educational theaters and Cadaver Lab, as well as an expanded Career Center sessions. Be sure to check out these new opportunities that are free to all attendees. And if you haven't done so already, please pick up your Physiatry Day T-shirt at the Member Resource Center. And with that, we will move on to our session. And I briefly just wanted to introduce everybody who's up here. Dr. Marcy Ward is sitting on the left-hand side. She will be talking about SDR today. She's from Gillette Children's in Minneapolis. And then Elizabeth Martin is next to her. She'll be talking about palliative rhizotomy or selective dorsal and ventral rhizotomy or I think it's got another name in there too, combined rhizotomy. So whatever term you use, she will be talking about that today. And she's from Vanderbilt. Dr. Rita Iyengar is from the University of Michigan. She'll be talking about intrathecal baclofen pumps. Dr. Angel Bowman is also from the University of Michigan. She'll be talking about deep brain stimulation. And I am from Mayo Clinic. And I will be talking about hyperselective peripheral nerectomy. I gave myself the easy one because there's very little literature on that. And I could do that. So today the learning objectives. We're going to describe which advanced hypertonia management options may be applicable to differing pediatric populations. Let me move that. Discuss advanced hypertonia management options with patients including candidacy, risks and benefits and then recognize new and emerging advanced hypertonia management options. And with that, we are going to change slides over and get started with the first talk if it will let me do that. There we go. Okay. Yes, I am. Are you ready? I am. And again, there or there if you want to advance. I love it. Thanks. Good to see so many friends that I haven't seen in a long time for sure. So Jolene invited me to talk about updates on selective dorsal rhizotomy. In about 20 years of working and learning about selective dorsal rhizotomy, I would say that unequivocally, the pendulum has swung from who we recommend it for and the discussions that move around it. So I'm gonna try to cover some of that as we're moving forward. And when we think about the what and the where, I'll go through this pretty quickly. I'm presuming a little bit that a lot of folks understand this procedure already. But basically, regarding selective dorsal rhizotomy, there are two typical approaches that the neurosurgeons use. One of them is the cauda equina approach, which is distal. And for a physiatrist who really wants to know where we are and exactly what we are doing, this is the approach that I love to do because we're totally certain of what nerve roots we are evaluating. The CONUS approach is higher up at the CONUS with less exposure for the neurosurgeon. I'm sorry, Marcy. Oh, got it. Thank you, dear. There you go. Thank you. So the CONUS approach is up higher. The neurosurgeons usually remove fewer elements and some say that it's overall a less painful procedure. We don't really see that in our practice. Generally, the pain for this procedure is very low for the children. When do our neurosurgeons who previously used to do this procedure, not at all, was probably about 15, 10 or 15 years ago where they started incorporating it more routinely. It was more helpful for our adults who were a little bit more mature in their spinal elements. It tended to be the procedure of choice, doing it at the CONUS level in order to reduce the pain and improve the recovery for those older patients. Now, our neurosurgeons, I would say, do about 50-50. About 50% of the procedures wind up being cauda equina, 50% wind up being CONUS level. It really depends on the patient and their circumstances, sometimes related to whether or not the neurosurgeon really wants to be able to know exactly where they are, or, like I said, if they are adults. Well, the classic candidate, I presume everybody knows this, I'm not gonna belabor it for a long period of time, but the child who is born prematurely has periventricular leukomalacia that is contrary to an intraventricular hemorrhage, or had an in utero event, or a challenge in the womb around the late second or early third trimester, resulting in PVL. The child who has diplegic spasticity, the child who is ambulatory, the child who has very little dystonia that can be identified, and then the child who's actually quite strong with better than anti-gravity, or at least anti-gravity strength at the hip flexors and the knee extensors, and good selectivity, good motor control, the ability to isolate movements at the hip and the knee in particular. Bonus points if they could isolate movements below the knee. So it was probably about 10 years ago where we really started to push outside of this box, this typical candidate box, and started to think about, well, what about if we do this work for our children with hemiplegia? So we started to do that at our institution, and interestingly enough, more and more folks over the last 10 years have been publishing their data in this regard, and I have the studies listed here for you, but regarding hemiplegia, Oki in 2010 reviewed 13 patients. The mean age was six and a half. They had spastic hemiplegia, and all of them had a nice reduction in tone, and the majority improved their gait. Park in 2020, I'm grateful that he published his data, a lot of it coming out in the last number of years, but Park published that he performed SDR for 30 children who had spastic, excuse me, 29 children and one adult who had spastic hemiplegia, and they essentially put out a qualitative survey. That qualitative survey data showed when it came back that everyone had improved gait and function, activities of daily living were improved, and there was a real nice reduction in their tone. So when we review the literature that's available, those were a couple studies, regarding hemiplegia, overwhelmingly it's encouraging that this is a good procedure for permanently reducing tone for that group. So when you step outside the box and start to pull in the hemiplegic patients, it seems that this is a safe thing to do when you choose the right candidate. So hereditary spastic paraplegia is a very interesting one. For hereditary spastic paraplegia, Park had put essentially safety data out related to 21 patients that he, excuse me, beg your pardon, 37 patients that he had done rhizotomy on who had HSP. And of those, he reported that 90% of the patients with HSP did have a very nice reduction in tone, but that 10% of them had return of tone down the road. And that echoes what you see in a lot of the other literature that's been published on patients with HSP, that in particular, if it's a form of HSP that is not static, or that in fact, we know is progressive, then you wanna be cautious in recommending this. They may have a nice reduction in tone initially, but may see returning tone that comes in and renders, presumably, the work not very helpful in time. Many other studies about HSP that have been published, including a case series by Kai that suggested that follow-up two years post had shown decreased tone, but that was just a case series of four patients. Sharma in 2016, again, it was a case series looking at four, all had reduction in tone. But interestingly, in Sharma's study, 50% of the patients had functional decline and return of tone. And so again, begging the question, were some of these progressive, and some of them a more static form of HSP? And many of us understand that HSP, we don't always know which form of HSP we are seeing, and we don't always know if it's going to be progressive or not. So making a choice regarding those patients is particularly challenging. Other genetic syndromes, interestingly, when we look at the literature on that, there's not a lot. But Lowcamp in 2020 did show in a review of about 16 patients, four of them, by the way, were HSP, but the other 12 were other genetic syndromes. They followed these patients from anywhere, the follow-up was anywhere from between one to 21 years. It was a retrospective look and discussion with them about their tone. And interestingly, the mean age was 15. So these were older kids, young adults with genetic syndromes, but all of them had tone reduction initially, but they had mixed reported outcomes at the follow-up, regardless of what the time period was, one to 20 years. Again, begging the question, if there's something progressive going on, do we want to take pause? So I'd say for HSP, we're kind of equivocal and be really careful and be prepared to let them know that the outcomes may not be lasting. Transverse myelitis is another one that we get asked about a lot. And that when we see these cases come through our spasticity evaluation, we're giving consideration to that. The literature on that is very scant, but there was one study that, or one case report that had suggested that it was safe, which is basically what we found in the patients that we have, which amounts to probably about an N of five, it's been safe and helpful. But again, not a lot of strong literature on that, on the ones that we do have reports on, everything is pointing in favor of. There's no long-term follow-up. Generally, I think we're leaning toward it being safe and a strong possibility with caution, but we don't have a lot of data on it. Now, the spinal cord, you know, in our clinic, a lot of times we're talking about transverse myelitis, which is a spinal cord injury. Isn't it essentially the same pathology? But when we review the data on spinal cord injury, it actually shows, and Reynolds, and I believe Reynolds is part of the PARCC group that does rhizotomies, but Reynolds in 2014 published a case series of three spinal cord injury patients that they offered rhizotomy to. They elected and they followed them. Two of them had a really nice outcome with reduction in tone, and one of them had initial reduction in tone and then return of tone with worsening function. So being very cautious about doing spinal cord injury to really not exploring that right now at this time. GMFCS level four and five, doing it for those patients, the evidence has been somewhat equivocal. Nordmark in 2008 found with all GMFCS levels they had a reduction in tone, but that the ones through threes showed some nice improvement in their gait quality and function. And initially, the fours and fives showed some improvement in gross motor function, but that was not maintained. Now, adults, that's a area where in the last, probably about 10 years, we've been doing more of that. And the literature there actually does suggest that these folks benefit from a pain standpoint and comfort, certainly tone reduction. The outcomes that we have seen have been very good. I would caution that when they're recovering initially, my recommendation was, oh, they'll recover quickly if they're ambulating, and we'll be able to move them along quickly. And some of them have taken longer to recover in general, meaning back to their prior level of function, many of them requiring a mobility device, and then in time being able to get away from that again. So the why. So for me, why do I recommend Rhizotomy? The literature is here. I'm not going to go through it in detail in the interest of time, but still we see in the literature, time and time again, the study that is done echoes. Permanent tone reduction, decreased orthotic use, decreased mobility aid use, and increased mobility aid use. Decreased mobility aid use, that was the Trost study in 2008, showed that GMSCS level threes, about a third of them were able to get away from using a mobility device. Improved independence and ADLs. This one I'm going to touch on a little bit more. The decreased mobility device, beg your pardon. The, ADLs, beg your pardon. The ADLs, there was a study by Forst et al, in the occupational therapy journals, that showed that post Rhizotomy, those patients demonstrated better upper extremity dressing and better lower extremity dressing. That's probably not surprising to a lot of us who work with these patients routinely. We see that, but it was never published. And the reason I want to highlight that in particular is for this purpose. So Munger et al in 2017 published a study that essentially said that when you take patients who have had Rhizotomy and patients who have undergone orthopedic surgery alone, they looked at 24 Rhizotomy patients and 11 orthopedic patients. And they were able to follow them up about 17 years, up to 17 years post. 13 of those they were able to get gait data on. When they looked at those 13 patients and their gait data post Rhizotomy, of those patients, the orthopedic only group and the SDR plus orthopedic group, they performed the same from a gait standpoint as far as their improvements. And so this, they were concluding then, differing courses yield the similar results. And so do we need, therefore, do we need to do Rhizotomy? And from my standpoint, I would say, do we need to do all that orthopedic surgery? There was extensively more orthopedic surgery performed in the ortho group. And in our institution, when Selective Dorsal Rhizotomy is completed, it's a very easy recovery for those patients post-op and good results related to pain reduction, tone reduction, long-term, including decreased injections that are needed afterwards. So when I, and unfortunately, they didn't put my updated slides in this presentation, but the force study that did show that these patients improved their ADLs post-Rhizotomy to a large degree echoes what we hear from our patients. From when I evaluate this as a physiatrist, I say to myself, I often tease the orthopedic surgeons at my institution when they make a comment about an upper extremity. I say, oh, good for you. You remembered there are arms because we're very gait-centric at Gillette. And so the outcomes that we report on are often about gait. But one of the things that I find most important in my patients is their overall function related to what they can do for their independence. I half don't care if they're walking. I'm saying that a little bit tongue-in-cheek, but I've got ways that I can improve that. I want them to be as independent as possible. So it's perhaps that we need to be looking not only at the gait and how good that's looking, but how this impacts the life of these patients. I apologize that I didn't have the other slides and videos that I worked on this morning. Sorry. Okay, we are gonna try and hold questions till we finish at the end. So if you've got something specific, please write it down. I think they are. Okay. Go here. And we'll go here. And here, and I will. There you go, Dr. Martin. Thank you. Well, thank you very much for having me here. So as I get started, I think it's important to know that I am part of a multidisciplinary clinic that evaluates and does perform combined rhizotomies. And one of the articles that I'm gonna talk about today, the 2020 technical note, was from our group. But otherwise, I have no financial relationships to disclose, and I won't discuss any off-label use or investigational products or devices. So what is it that we're talking about today? As Jolene mentioned earlier, there are a lot of different names for this procedure. So in the literature, you can find it referred to combined anterior and posterior lumbar rhizotomy, a combined rhizotomy, palliative rhizotomy, and some other terms. But as you've just had an outstanding talk about selected dorsal rhizotomy, this particular procedure, in addition to cutting the dorsal nerve roots, we're also talking about cutting the ventral or anterior nerve roots, which are containing the motor axons traveling from the central nervous system to the muscle targets. So thank you again to Jolene for this lovely diagram, which shows you that. So there's not a lot of literature out there. On my review, I was able to find four pertinent articles that included a prospective cohort study from 2016 on 50 patients from Bygani, a case series from 2007 on six patients, the technical note that we put out on seven patients in 2020, and then a review article talking about hypertonia management that just mentions the procedure and describes it a bit. There was also an interesting article on magnetic resonance-guided laser interstitial thermal ablation for palliative rhizotomy, which was interesting, but a different procedure, so I'm not gonna cover that. This also doesn't refer to some procedures that I think our institution and others are doing now, which is more of a selected dorsal rhizotomy, but also including some of the ventral nerve roots to the adductors for severe scissoring. So there's a lot of variation in the approaches, both by institution and over time. So starting early on with the 2007 article, they were doing both cervical and lumbar levels. So they described laminotomies that are opening those entire areas, and then within those areas, they're doing 50 to 90% of the dorsal roots, depending again on which parts they're selecting, and anywhere from 50 to 100% of the ventral roots, and there was a lot of variability by patient. In the 2016 prospective cohort study, all 50 patients had an L1 to S1 laminotomy, and they cut 50 to 80% of the L1 to S2 dorsal roots. They determined how much to cut in the ventral roots based on their Barrett-Albright-Dystonia scale. So for those that had the most severe, they cut 80%, and those that were lesser, they cut 50%, with the argument they were trying to prevent muscle atrophy. In our 2020 article, it was specifically describing an approach using the 1 1⁄2 to 2-level laminectomy to expose the conus level in a way that they could better delineate where the ventral roots are in a safer manner. Our neurosurgeon typically cut 60 to 70% of the L1 to S2 dorsal roots, similar to the SDR, and then the ventral roots, they do L2 to S1 and about 2⁄3 of all the ventral roots. As far as rehabilitation after the procedure, again, there's a lot of variability. So in the cohort study, they all had what was described as inpatient intensive for three to four weeks post-op, including four sessions per week. In our study, all patients had outpatient rehab afterwards, and then the case series did mention rehab after. So who might get this? Again, we're talking about this particular procedure because of dystonia, that's why we're including the ventral roots. So we're looking at patients with mixed hypertonia. We think it's important to think about a non-progressive etiology as well. That was also delineated in the cohort study, although interestingly, there were some cases in the case report that did have diagnoses that would be expected to progress. We also tend to think about this in our non-ambulatory patients. So I also found it interesting that in the cohort study, they specifically selected those that were GMFCS levels three and four. All of ours were GMFCS level five, and then GMFCS level wasn't mentioned in the case series, but based on the descriptions, they were likely about five. So as far as the age range, it's pretty wide. So they were described as young as four and up to age 18. Again, we're talking about both spasticity and dystonia management, so all described their modified Ashworth scores, as well as their Barry Albright dystonia scores. There was a wide range, as you can see in the case series. Both the cohort study and the technical note had a modified Ashworth mean of about three. It was also interesting to me that in the 2007 article and in ours, they described the Barry Albright based on the mean or what's happening in one extremity, whereas in the Ghani article, they actually do the mean summation of all extremities, which I think is the accurate way to report the Barry Albright, but it made it a little bit hard to compare between them. So when would we do this? So again, we're really thinking about this in patients who have dystonia. We're also thinking about this as more of a last resort, so patients who have failed other methods, things like oral medications, intrathecal baclofen, or have a contraindication for that. We tend to think about this for patients who have primarily lower extremity hypertonicity, similar to what we're thinking about for our SDR patients, but it was interesting that we do have cases out there of doing cervical levels as well. Our goals tend to be comfort and ease of caregiving, but I also found it interesting in this cohort study, they actually had functional goals as well, the ability to roll in bed and sitting with support. So why would we not do this? As far as surgical complications, most of the ones that are described seem similar to what we might see with L-selective dorsal rhizotomy. There are some patients out there described as having urinary retention afterwards, but it all seemed to resolve in a couple weeks. I think what is most important is really the weakness, which not surprisingly, we're cutting eventual roots. So in our technical note, we noted that none of the patients had more than three to five strength in any of the lower extremity muscle groups after the procedure. So this is not a reversible procedure, so if we don't like the outcomes, you can't really go back. So it's important to be thinking about who you're selecting for this, and it does cause weakness, which can have a potential negative impact on any functional goals. So if you have a patient that you think might have a reasonable goal of working on helping with transfers with their lower extremities, or parents think it's really important to continue ambulating in a gait trainer, this might not be the right procedure. We also wonder if that weakness might have long-term effects on things like osteoporosis risk, or impacting their venous return in the lower extremities. Personally, I'd like to see a lot more data on the other benefits or the sequela of the procedure. So in our experience, we've done 18 patients between 2019 and 2022. We are gathering data both prior to surgery and then a one-year follow-up. Unfortunately, our coordinator joined us in the end of 2021, so when we were looking back, we realized a lot of the patients hadn't gotten their follow-up, and we're having to go back and recollect that data and their follow-up, and it's not quite a year. But this is a nice table that I borrowed from our neurosurgeon that summarizes the measures that we're gathering both for our different populations for our SDR, and then for our palliative and combined rhizotomies here. Most often, parents are reporting that they're seeing improved care, especially with diapering, and improved comfort afterwards. And the other outcomes that we have published are kind of coming from Ghani's article. It was interesting to me that more than 60% still needed orthopedic procedures afterwards. They did see a significant reduction in their modified ASHRAE score, but again, I found it interesting that at the 12-month, it seems to start creeping up a little bit compared to their immediate post-op score, and they see a significant reduction in their barrier outbreak. So I'm gonna go ahead and stop there. Thank you. So we are going to move on from rhizotomies to intrathecal baclofit pumps. Here we go. Do you want the laser pointer on right now? Sure. Okay. And I'll just set it down there for you, and then that if you want it, and that too. Okay. All right. Good morning, everyone. I'm here to talk about ITB, or intrathecal baclofen therapy. It's been around for quite some time, and so there's a lot that's been published about it, but not too much that's really changed. So in terms of disclosures, there's no financial interest or affiliation regarding material discussed here. However, I will be discussing ITB use beyond the FDA-approved condition of spasticity in children. We'll be talking about dystonia, paroxysmal sympathetic hyperactivity as well, so I just wanted to mention that. In terms of reviewing, just go over the basics a little bit about ITB and how it works. I always find that going back to the basics is helpful as we think about our patient clinically. Go over who makes a good candidate. I will have a case presentation to review the Michigan Medicine experience, why ITB, why not ITB, and my wish list for the future. So what is ITB? It's delivered into the CSF that baits the spinal cord. Those are just about one millimeter or so under the outer surface of the spinal cord, so the various regions of the cortex. And that, there, of action at the spinal level versus the brain, and thus further decreasing cerebral side effects, meaning that when you have slow intrathecal infusion, most of the catheter position, in relation to the targeted spinal cord segment, then may point towards that. Phylic bulk of it stays in the CSF. So ITB doses can be 100 to 1,000. Effects of ITB can help with paroxysmal sympathetic hyperactivity. And so in terms of the motion, and then there's sex. So when ITB was first approved for children with spasticity of spinal and cerebral origin in 1996, it was recommended that patients be over four years of age and weigh greater than 35 pounds with an obligatory trial of oral baclofen. That's since evolved to where pumps are recommended for children weighing as little as 28 pounds, and successful pump placement has even been reported in infants as young as 11 months and weighing around 13 pounds. You know, that really, that's not something that we've experienced as much at Michigan, but it's certainly out there, that people are implanting pumps in younger children and finding that they're having safe effects. An obligatory trial of oral baclofen is not necessary, and pumps are now considered for children with secondary dystonia, and also for early rehabilitation conditions, like refractory paroxysmal sympathetic hyperactivity. Of course, other conditions like medical compliance, strong psychosocial support, good nutrition, and having specific goals of function, reducing caregiving burden, and improving comfort remain critical to good candidacy. So, you know, raises the question of, should all patients have had titration of oral baclofen before ITB? In general, looking at the literature, yes, for spinal origin hypertonia, but what about cerebral origin? And that seems to vary. We'll come back to that, but give it some thought. So, of course, you know, for ITB, there's the first step is selecting your candidate. The next is doing an intrathecal injection screening trial. That's typically done as a lumbar puncture, starting at 50 mics, and then depending on response or not, you take it up a notch to 75 mics, and then go up to 100. 25 mic boluses have been described for, or recommended for smaller kids, and also something to consider when you're dealing with primary ambulators. So, typically, but what if your patient has mixed hypertonia? You know, we see our patients, and they're not all purely spastic. A majority of them have mixed hypertonia, and they may have dystonia. So do we say, then, that ITB's not effective for them if they fail that 100 mic test dose? So, in these cases, one may pursue a continuous infusion trial, starting at 50 to 100 mics per day, and then take that dose up steadily by 10 to 20%. We've typically taken the dose up every 12 to eight hours, closely monitoring for adverse effects, and adjusting frequency of changes accordingly. So, this continuous infusion trial, of course, as many of you know, involves implanting an intrathecal catheter that's connected to an external pump, and these trials may typically last three to five days, sometimes longer, and high doses can be reached. You can get into doses as high as 400, 500, or more micrograms per day till you get to see what your response is. Higher rates of infection and adverse effects certainly can be expected. And then there are situations where you kind of go, do we have to do this trial, or it's very hard to do this trial. So, one may go straight to a continuous infusion trial in situations where an LP has proven difficult, or is expected to be very difficult, such as when you have patients with severe scoliosis. And that also brings the question is, are these trials really essential or not? And, oops, sorry, go back. Because 98%, it looks like these are not my updated slides. It looks like 98% of patients seem to respond to the intrathecal test dose, and especially if they have predominant spasticity. So that begs the question, is this trial really essential or not? And what I found in my experience is that trial time is really critical to success, long-term success with the pump, because patients, you have them there, they're a captivated audience, and so you can really go talk about problems that might be encountered, what they should expect long-term, et cetera, and that's really helpful. So, what about the catheter and where the tips place? Surgeons vary in their preference of the older silicone two-piece catheter than your Ascenda catheter that was introduced in 2011. So this catheter, the Ascenda catheters, significantly reduced catheter-related complications. But a big problem with it, if you can look here, you can see this is the old catheter, and you can actually track it going up, and you can see it going up in here as well. But when you have the Ascenda catheter, it's really not visible and you can't track it. And what you can see is this tiny dot over here, and that's the tip of the catheter. So it really is something that's difficult for us to radiographically image compared to the older catheters. On the plus side of it, we've had far fewer problems with the new cath, once, since we've started using the new catheter. So this was a study that we published with our information back in 2011, and this was all with the old catheter, and we were seeing so many more problems. And then reflecting back between 2011 and now, we've had very, very few dye studies that we've had to do or other imaging studies for problems. And yet, we know that because we are dealing with a mechanical device and a system where you have to keep replacing it for because the battery runs out, it's bound to run into problems over the course of the lifetime. So what are some imaging techniques that are helpful? One is where you do the plain x-ray and you're looking to see how's that pump working. You can do the fluoroscopic dye study or C2 Milo, where you're looking to see how it goes. And then you have a follow-up CT. So that's a change that we have done over the, looking at over the past few years is, or soon after our study there, is we added CT very early and follow it right after our fluoroscopic dye study because we found that it really has increased our ability to detect problems. And more recently, something that we have done is iodine subtraction in those situations where you kind of see this spot and you're not quite sure whether it's dye or whether it's surrounding tissue. To help differentiate, our radiologists have started to, we've done it at least in a couple cases where we've used iodine subtraction and been able to say that that spot is a leak, a very small leak, and been able to identify that. So differences in practice remain with different aspects of ITB therapy. The German study by Stephan Berwick and his group back in 2013 looked at data of more than 400 pooled patients from 14 different German centers. They assigned ACBDM levels of evidence to the available literature with emphasis on children and adolescents and also described practical approaches from the 14 centers. So that was more practice-based evidence. We also then in the U.S. had an ITB therapy best practices expert consensus panel and I see some people here who may have been on that panel, Cindy, there. And this comprised of 21 multidisciplinary clinicians in private practice and academic medical centers in the United States who were managing PEDS and adult patients. And what these consensus studies in general pointed was that consideration of ITB in patients with hypertonia functioning at GMFCS three to five levels where high tone is interfering with function, caregiving, comfort, and not responding to other forms of pharmacologic and non-pharmacologic treatment would be appropriate. The European study strongly recommended doing a trial of oral meds first, even for hypertonia of cerebral origin while the Americans felt one could proceed quickly to ITB in carefully selected patients. One advantage that's mentioned for trying the oral baclofen is that it allows for evaluation of allergy to baclofen prior to pump implantation, but I've not yet seen a problem with that allergy and looking through the reports wasn't able to, through the literature wasn't able to detect that either. And the other question that comes up is, oh, you do an oral baclofen trial, but what you're looking at if they have an allergic response is that really to baclofen or is it to a filler that's in that tablet and how, if you really wanna test for ITB, do we need to be giving them that intrathecal baclofen to test for allergy or not? And this was something that was discussed in the American Association of Allergy Immunology, which was the only report that I could find of some discussion about this. It's important, both of these studies noted that it's important to look at the effects of hypertonia on the growing skeleton and its implications for hip and scoliosis management must also be considered. Yeah, I need that to move down. How do I get this cursor to move to the next? Do you want the cursor to move? That's okay, that's fine. All right, so here's a case that I'd like to share because, and this really started my turn toward to making some changes. So this is Alan. He had MIECHCP at GMFCS level three. When he came to see me, he was seven years old and he had had a dorsal, selective dorsal rhizotomy at two and a half years of age. He'd failed two trials of oral baclofen, managed with botulinum toxin A injections in general. So we evaluated him for a pump. He had an initial test dose at 50 and wasn't so convincing. So he then, they were really nervous about having to go through multiple. And so we actually skipped because what I detected at the time was that he clearly had dystonia. And so we skipped from the 50 and went straight to 100 micrograms in him. And he had a very good response to that. After about, he then had the pump implanted. After about two years of improved tone and function, improved function, he then ran into a series of problems. He was a twiddler. He had a flipped pump a few times. He then, you know, had tear at the connector and eventually had problems with infection. And so he had his pump implanted, re-implanted, you know, revised multiple times in a span of about a year there. So then after that, they of course got gun-shy, did not want to, you know, do this for some time. And we were too, wondering what, you know, how best to manage him. So he was managed on oral baclofen. We started really slow and built it up slowly and also adding other meds for dystonia. Then got lost to follow up with us. He was following elsewhere locally. He returned at about 13 years of age, again interested and wanting to reconsider the baclofen pump. We did a trial again and this showed improvement and this improvement was greater than what he was achieving with oral meds alone. However, don't know what happened. They again, you know, didn't pursue the pump. He returned again at 20 years of age for ITD consideration. Recommended again, but did not follow through. And he's now returned at 23 years of age to establish care in our adult CP clinic. Was started back on oral baclofen and now is up to 90 milligrams per day, but he's still not pursued the pump. And so this kind of, you know, really highlighted to me the importance of spending that time talking over things and really looking at, you know, because when they run into problems, clearly for him, when we do the trial, it turns out to be a good treatment. Functionally, it was a great treatment, but I think that negative experience of the multiple problems he encountered, you know, has made him unshy about that. And so how have things changed for us? We in Michigan have been, we initiated this program in 1998. We have over 250, had over 250 patients and in all this time had only had two patients that had their pump implanted directly because of spinal fusion and severe hypertonia. And they've actually done, responded well and done, you know, well with that. I've always included both PT and OT in my evaluations during the trial because it was very evident to me early that it would often what seemed to convince the patient and the family to move towards a pump was not so much that spasticity reduction in the lowers, but really the subtle changes that they were seeing in their upper extremities that would translate potentially to function. And so we'd looked at that and I'd kind of presented this at a couple of our APMNR meetings and posters about the changes we were seeing in the upper extremities and how that was helping with, could potentially help with function. So I found that to be more convincing. Depending on dystonia degree and oral dose, I will not hesitate to start the trial, to start at 75 or 100 mic dose instead of doing that 50 mic dose. I've seldom needed to go up to, go to continuous infusion trials. We've only done five so far. Most of our patients seem to respond adequately to the 100 mic bolus dose, enough to be convincing to proceed with pump implantation. We may not necessarily have that full, you know, one-point drop, but then the changes seen in the upper in terms of some functional changes has been helping. The other thing that we've done is we've, you know, adopted CT early and also do 3D CT to assess the catheter during dye studies. So why or why not? I mean, there have been studies conveying, in general, when you look at the literature and look at, there's plenty of systematic reviews coming out now, which is great. These studies all convey low-level evidence for effectiveness of ITV therapy in children. While many studies show improvements in spasticity, those improvements don't necessarily seem to translate to improvements in motor function. Most studies showing improvements in spasticity and dystonia are non-controlled. How do I get my cursor to move? I want to get to this side. You can't get it off the screen. If that's what you want, I can shut it off. Oh. Here, let me shut it off. Hang on just a second. Oh, there we go. Okay, perfect. And it's there. All right. So when we look at a systematic, you know, these reviews, the systematic review by VISER and other studies highlight the need for larger cohort studies with systematic assessment of individualized goals. That's something that's coming out in the literature is that individualized goals are where the changes seem to be more noticeable rather than looking at, you know, broader things. So looking at individualized goals, long-term effects, that's what's needed. A recent systematic review update and meta-analysis of pharmacologic and neurosurgical interventions by Darcy Feelings on her group, looking at CP and dystonia shows only low-level evidence that ITB may improve dystonia, pain, and quality of life in CP, as well as attainment of individualized goals. But it did not show improvements in motor function again. The same review, however, noted improvements in motor function with DBS along with dystonia, pain, and quality of life. Again, all with low level of certainty highlighting the need for more rigorous and larger cohort studies. Shruti Thomas and her group here did a cross-sectional study looking at 137 patients with severe spasticity with and without dystonia. And their results match our clinical experience at Michigan in that the bulk of patients with severe spasticity also have dystonia. Of their 137, only 23 had pure spasticity and the rest were mixed tone. And that's what we typically find as well. And typically, the patients who have mixed hypertonia or dystonia have more rostral, higher placement of the catheter. They require flex dosing and a higher dose than those just with spasticity. A recent prognostic systematic review of pain intensity in CP noted beneficial effects with ITB therapy six months post-pump implantation. And this is in keeping with other studies in the literature that consistently note improvements in pain with ITB. Studies of ITB and SDR both show a comparable reduction in spasticity and an improvement in motor function in non-ambulant GMFCS4 and 5 children. But complications such as device-related problems were more notable, of course, in the ITB group. Looking at patients with pumps at end of battery life or needing replacement after removal of infection was another study. And what they found was that patients in this situation were offered SDRs at the end of where they needed pumps to be replaced at the end of battery life. They offered them SDRs instead of a pump replacement and noted that all patients showed improvements in upper and lower limb spasticity after the SDR and a little more than a quarter showed improvement in neurologic function. So over 90% of parents and carers then felt that functional outcome with the SDR post that baclofen pump was improved compared to where they were with ITB alone. There are continuing debates in the ITB world. Is there a need for screening trial? What about ITB in ambulant children? You know, the literature really shows that this is a group where we need to be more cautious and where adverse effects are noted to be great. They haven't really panned out with any certainty to show that there's been benefits. And yet on an individual basis we definitely see that there is improvement there. What about scoliosis surgery? Should that happen first or should it be ITB? Our practice generally has been to address the scoliosis, I mean to put in the pump first because controlling the spasticity actually helps with management post-surgery. And that seems to be what the literature also is suggesting. In terms of GMFCS-5 patients, should it be ITB or a rhizotomy? And that's something that we can certainly talk about here after hearing Elizabeth's talk there. And then there is dystonia, and should it be ITB or DBS? As I said, the studies are suggesting that motor function improves more with DBS than with ITB. So why use ITB? Intrathecal, more effective delivery of baclofen, reversible treatment, flexibility of dosing, ability to tailor. Certainly with dystonia it's effective so we can be helped with higher doses, facilitates caregiving and quality of life. And importantly, it can also be used very early for early rehabilitation, especially with anoxic brain injury, paroxysmal sympathetic hyperactivity control. Why not ITB? Recurrent surgeries, you have a mechanical device bound to break down over time, wear and tear over a lifetime, labor-intensive, certainly requires a lot of compliance on the patient's part and a lot of vigilance on the provider's part, follow-ups very critical. And high risks that persist with infections, withdrawal, overdose, and managing those. The systematic reviews are raising the question that do risks outweigh the benefits if there are no motor functional gains in patients with dystonic CP? Now is this really the case or is it more a limitation of how we are able to assess that? What we need then is to have more studies look with greater numbers, with pooled data from multiple centers, data from multiple registries. It's really good now that we have the European registry, you have the U.S. CPRN network starting with it, and then the Australians are in the midst of a 10-year prospective study looking at their pooled data as well. There is clearly more need for longitudinal studies and, of course, improved troubleshooting. So boldly looking to the future, I'm excited about the CPRN registry and our ability to pool our data in a standard format. The question is, will real-time data capture regarding activity become commonplace? And would that be a good way to assess function and changes? Will AI algorithms help us determine best treatment selection? And will there be advances in the pump catheter system to allow for multilevel drug delivery? Because if it's delivered right near the tip and doesn't spread out too much at a simple continuous rate, then could there be a multilumen catheter that delivers it at multiple levels to be able to improve our effects? What about our ability to measure flow changes in the system and improving our radiographic assessments? So these are all to consider. Sorry. Thanks, Rita. I gave Rita, I think, the biggest lift for this. So I appreciate doing that. And then we will move on to our deep brain stimulation. There's angels. Here we go. And, Angel, do you want the laser? And you can always turn it off too by just clicking on that. Thank you. As I make it go away. Okay. That's fine. All right. So I'll be talking about deep brain stimulation today. I am not a neurosurgeon. So any detailed questions, please hold for the neurosurgeons. So back in 1996, the first child to receive DBS was an 8-year-old girl who was severely dystonic. She had severe generalized dystonia. They targeted her bilateral globus pallidus internus. And 20 years later, 20 years post-op, she was still having very good efficacy in the control of her dystonia. Then fast forward to 2003 when the FDA granted a device exemption to start using this in pharmacologic-resistant dystonic children aged 7 years and, oh, shoot, these aren't my updated slides either. It left off up. That definitely wasn't me. That definitely wasn't me just not noticing that on my slides over the 20 times I went over them. So currently, they use DBS in carefully selected younger children, and that's also showing some benefit, but it's a case-by-case basis. It's not standard of care. That was definitely just me not seeing my slides and missing them and left that word off. You're okay. So what is it? It's a neurosurgical procedure. It happens in two parts, though, which most of you might not be aware of. So in part one, the leads are implanted. There has recently become a new stage or a new practice in doing the lead implantation, which I'll get to in a minute. And stage two is where kind of the pacemaker part is implanted, the brain pacemaker, and that happens one to four weeks after the first stage. They started to do the two-stage approach due to high rates of infection when they were doing it as a single procedure. One month after the brain pacemaker is implanted, they start to do the programming, and that can take anywhere from a few months, usually up to around a year. And so it's not a quick and easy, done process. It's quite a prolonged process to get to the right spot. And so it targets circuit-based neuromodulation, normally the GPI or the subthalamic nucleus. However, there is a new procedure that Singer's group came up with that introduced this very specific targeting protocol in which they inserted some temporary electrodes, and they placed them at multiple targets throughout the brain, including the basal ganglia, the hypothalamus, and then during a one-week inpatient admission, the patient was kind of probed to see what responded best to the stimulation. And so that was actually found to significantly influence surgical placement, permanent placement of the electrodes, and they were placed oftentimes at locations which otherwise would not have been selected or targeted. So that made a big difference in target selection for these kids. And then in adults, typically they are awake during some of the parts of the procedure. However, in kids, waking them up partway through was not going well. And so we have this interventional magnetic or this interventional MRI-guided placement for children, which has begun to work very, very well, in which they do real-time confirmation of the placement of the leads with the MRI, and the clinical outcomes are actually comparable to traditional placement techniques that take place in adults. So this is kind of the new thing that they're doing for the implantation in children. Just for those of you who were not aware, this is what I was speaking of earlier. So the electrodes are implanted. The lead comes out through a small hole in the skull and then goes just underneath the skin down along to the part that we've been referring to as a brain pacemaker. It's the pulse generator just under the skin. So ideal candidate inclusion is usually medical treatment failure. A lot of us are doing this for a very long time. We know these severely dystonic patients are just resistant to a lot of treatments. It's not controlling it adequately. So these are some great kids to try this on. Any person or child who's having a major impact on functioning or significant disability, dystonia is so bad that they're locked inside their own body. They can't participate in activities. And very importantly, an unequivocal diagnosis of dystonia, so where we're not thinking that this is anything else. We just had a patient referred to my surgical spasticity clinic who was referred for possible DBS placement, but they were largely with functional neurologic symptom disorder, causing these dystonic-like movements. And not a candidate, guys. Don't do that. Exclusion criteria would be very young age, as we talked about, normally seven and up. There are some cases of doing younger ages. Any of those kiddos with a high infection risk, a very low weight, or people with unrealistic expectations. So you want to have those talks with the families and the patients beforehand. Also for exclusion, which we know is also the case for pumps and SDRs for therapies after, would be the inability for adequate follow-up. It does take a year. It does take frequent revisits to program this appropriately. So we need good follow-up. And then something where I could not find the reason, but I'm going to put it out there for you guys also. An exclusion criteria was active suicidal ideation. I think there's obvious reasons for that, but I couldn't find any other than the obvious. One, the earlier the better. Multiple, multiple reasons, which I'll explain more in the next slide. But we also want it to happen before joint contractors occur. If we're taking away this dystonia, but then the child is already contracted and has 45 degree knee flexion contractures or elbow contractures, can't use a stand or still, even though we've taken this toe in a way, and then we still have to do all of these procedures, that's not the optimal time to do it. So the earlier the better before contractures, and not as a last ditch effort. Like I touched on before, a lot of us are struggling with these dystonia kids, years of trying all of the medications that we can think of, everything that's written up in the papers. And when we can only see these kids every month, two months, three months, six months, this process takes a lot of time. And before we know it, it's been five years that we're treating this child unsuccessfully, and then we're going to the DBS. And so that's not what we want to do anymore. We want to have that in the back of our minds as we're thinking about all of these treatments along the way, and try and have this as an earlier intervention. So long-term outcomes. So there's lots of different dystonias. There's lots of different primary dystonias. I'm going to go over this a little quickly so that we have time for questions at the end. But in primary dystonia, there was a huge benefit. Improvements from anywhere from 40% to 100% in their dystonias. 61% of patients had discontinued all dystonia-related medications at follow-up in some studies. The subthalamic nucleus stimulation location had reported long-term improvements ranging anywhere from 70% to 90% for certain types of dystonia. And then, oops, sorry, going back up here. 91% of patients had discontinued at least one medication at follow-up in this study. So the primary dystonias tend to demonstrate greater and more consistent improvements than those with acquired dystonias. And then in primary dystonias, the GPI stimulation had been more beneficial in certain types of dystonia. And then a majority, the follow-up for all of these had been the three months and then a 15-year. So complications, I mean, expected with a neurosurgical procedure, but about 8% per electrode a year, a 12% rate of wound infections, and then a 30% rate of hardware-related issues. And this is from a recently published German study in their registry. And so the main takeaways from this, primary dystonias are highly responsive. Secondary dystonias are highly variable in their responses. But DBS has been shown to resolve over 90% of status dystonicus, regardless of the etiology of that. So that's also a big one because a lot of us struggle with treating this. Younger patients with shorter disease duration tend to have greater and more long-lasting benefit with DBS compared to older patients with the more prolonged dystonias. Most of these are all MRI compatible. However, with the T2-weighted sequences, those should be avoided because the device can actually heat up. Don't do that, please. Check with the manufacturer. The palatal DBS is the most effective in patients with that isolated dystonia, irrespective of the underlying etiology. And then genetic testing can also help inform patients of the expected benefits. As I was talking about, multiple different types of dystonia, so genetic testing can also be helpful. So you can have those informed conversations with the patient and their family. Awesome. Thank you, Angel. Okay. Last but not least, here we go. So I have the luxury of talking about the procedure which has the least amount of data to support it, which gets to be a little bit fun because it's a little more anecdote, I think. Let me move on here. There we go. So I'm going to talk about hyperselective peripheral nerectomy. How many of you have heard about this for tone management? There's a few in here. Yay. Okay. Fantastic. So no disclosures. When we talk about hyperselective peripheral nerectomy, we're really looking at the area — let me turn my cursor on here — in this bright green area. So right where that nerve comes in contact with the muscle. So it's sort of like a rhizotomy at the muscle level. It doesn't involve that spinal cord. It's really selective for that particular muscle that we're talking about and distinctly different from some of our other invasive procedures that we've talked about already today. So what and who when we're thinking about hyperselective nerectomy? So I have three pictures up here. Y'all are pretty far away from that. If you're like me and can't see distances, it's okay to come up a little closer. But the first picture, A, is a traditional selective peripheral nerectomy. So this was actually the very first procedure that was introduced back in 1913 and was done at the median nerve in the upper limb to treat spasticity. So they basically went in and sectioned the nerve itself, took out a hunk of it to prevent that from reinnervating. But as you can see, that also involves part of the sensory and motor nerves. So it took out both parts of that. Actually, this technique was then abandoned partly due to discomfort from it, from that sensory part. But also there was high rates of recurrence and limitations of anesthesia and antimicrobial medications at that time. But in the 80s, this procedure was revised, first starting with a proximal selective motor neurotomy where they went in and identified then that motor section, so going a bit more distal in that nerve, and cutting out a section of that again, trying to make it a larger section to reduce reinnervation that can occur with that. And they were using about 50% of that nerve that they were sectioning, but actually had a greater rate of recurrence with that than they wanted at the time. And so they started looking at doing this procedure even distally, so isolating those nerves right at the area, that's in Part C, right where they're going into the muscle, so really getting those rami at the level of the muscle. So when we're looking at that, when they're thinking about sectioning these, if they leave about 30% of that innervation, individuals will still have anti-gravity strength. So if they cut 70% of those motor rami, they can still have anti-gravity strength preserved. So when we think about things like doing this procedure, when they're looking at doing a neurotomy or a neurectomy, they want those for folks who don't have significant volitional control. When they're going into that hyper-selective neurectomy, they're thinking about that a little bit more for individuals who still have some volitional control so that they can actually use it to enhance function and not just for tone reduction. So for this procedure, at this point when we're thinking about who, why, and when of this, the who would be permanent spasticity reduction. And I think you've seen that with most of the procedures that we talked about today is that permanent spasticity reduction piece. So proximal selective neurotomy, that one where you're doing it in the knife, is still in that motor nerve that's away from the muscle. Let me turn that. There we go. So up in this area here. That would be for somebody who has less muscle function. they don't have as much motor control, we just want tone reduction of that particular muscle. But in those who still have some volitional control and we want to enhance function, then we're thinking about the hyperselective nerectomy where we're doing that more in that distal location close to the muscle. In our institution, we're thinking about this when we want to, when we're having a difficult time either getting good control in a focal way with our botulinum toxins or our phenol, when tone is responding, but we wish to preserve muscle and we don't want to submit the child to repeat procedures. So if we're starting this when a child is three or four and we're having to do this repeatedly and there's really a focal area that we want to take down the tone, we find that this procedure has been helpful for us if they're losing range of motion in that particular muscle or again for hygiene or positioning purposes. And let me back, there we go. So when we're doing a hyperselective peripheral nerectomy eval for an individual for a lower extremity muscle, and I thought this was gonna run, hang on. Let me try it one more time. There we go, fantastic. So this is actually a little girl who is a five-year-old who was born at just over 24 weeks gestation. She's got a little bit of a mixed tone picture. She's a bilateral asymmetric CP and the left side is the side that was really giving us a run for our money. We were doing a bunch of botulinum toxin to that leg and I'll back out of it. Let's see if I can get to run again. There we go. A bunch of botulinum toxin and particularly to that gastroc muscle and despite that, she was developing a progressive contracture with that and we did casting and it was really that particular area, that gastroc soleus complex that was giving us the most trouble with her walking. So you can see her walking is not entirely normal even thinking about that. Her family was very interested in something more permanent but really wanted to really focus on that area. So our lovely orthopedic surgeon went in, took a look at it. We said, this is what we're really looking for and that hyperselective peripheral anorectomy would be that ideal procedure. Prior to having this option, we probably would have went in and did a selective dorsal rhizotomy for this kid. So he specifically went in and you can see this picture on the bottom. The picture on the top is the view of the leg when he's starting the surgery. You can see it's a pretty long incision that they're doing here. So about halfway down that calf muscle, that incision goes and they're really opening this up entirely so they can see those nerves as they're going into the gastroc and soleus muscle and then sectioning pieces of these. So he ended up sectioning 70% of the rami so leaving 30% of that innervation for that motor innervation to that gastroc soleus complex. And then after that procedure, let's see if this video will run and I may have to back back into it. Oh, I know, here we go. It'll start running now, there we go. So after the procedure, you can see her walking slows down a little bit. It actually looks a little bit more controlled when looking at that left side, but that heel is not yet down. So we've taken away the tone, but the kiddo still has a contracture that we need to address. And so then our other orthopedic surgeon went in and addressed that contracture with doing a heel cord release. And we were able to get some lovely heel strike even with having that midfoot collapse that's present on that little one. So when we think about this type of procedure, one of the things that we need to ask in particular with something new like this is why not? Why should we be avoiding this or why should we be thinking twice about doing this? And one of the first things is that this is a newer procedure for children. There's a couple of articles out there in the literature that are starting to show more of that technique piece. So we're very early in the stages of this. So what is the technique for doing a hyperselective peripheral nerectomy? Why would we consider doing this? We don't really know who the ideal candidates are. At our institution, I found the kids where it's really a focal, a tone area that's giving us a lot of trouble in particular that gastroc soleus complex. And we've been particularly successful with our hemiplegic CP kids or our kids with really asymmetric diplegic CP where those gastrocs are just giving us a run for our money. That's where I've been really happy with the hyperselective nerectomy. I've also done it in another kiddo who had more mixed tone CP, affected everything but really was affecting that gastroc soleus complex the most. And I wasn't as happy with the outcomes of that as far as the tone reduction. So I've really leaned more towards using it in my hemi CP kids or asymmetric dyes. We do this in a staged manner at our institution. So they do the tone reduction part first and let the kids recover. And it takes the kids somewhere between one to three months to really feel better after the surgery. I think they really have to open them up. There's a lot of swelling after that. They're really quite sore for some time. So I've learned to counsel the families that even though the surgeon will tell you you'll be better in four to six weeks, it's gonna really be closer to three months where your child's feeling better. And then we're going to need to take a look and see what's left of that contracture. Is there something there that we need to address from an orthopedic standpoint? And then we don't know the long-term outcomes. Is there re-innervation that's going to occur after we do this type of procedure? We're not sure. Is there more detriment to denervating a muscle in a child than there is in an adult? Are we gonna get some sort of atypical osteoporosis that's in that area because that muscle's not as active or set them up for risk of fracture? We just don't know. And then could it impact further surgical interventions in that area? So for the gastrocnemius muscle when we're doing that hyperselective neurectomy, it does impact what lengthening approach they can do. Our surgeons can't do the Strayer approach because that's the more proximal approach in the muscle and that's exactly where they did the neurectomy and there's scar tissue there. So then they have to do that more distal gastroc lengthening approach. And that's all I have for that. So we are a little bit over but we have a little extra time in this room. And if anybody has any questions, wants to ask about a couple things, really appreciate that, come up to the mic. We're happy to address those. Hello. Hi, is this working? You're able to hear me okay? I can hear you. Awesome. Thank you so much for the talk. I really appreciate your time and information. This was briefly mentioned in the DBS talk but I really was hoping to get the panel's input. The title of your talk is Advanced Hypertonia, Who, What, When and most importantly, Why Not? In a 2021 paper by Dr. Chris Steven in Annals of Clinical and Translational Neurology, it was stated that of the 1,400 new patient referrals for dystonia treatment, 99 patients met criteria for clinically established functional dystonia, including 14 pediatric patients. With the understanding that functional dystonia can mimic organic dystonia but has a very different pathophysiology and treatment algorithm, I'm curious what your institution's approach is in identifying these pediatric patients early in effort to avoid unnecessary investigations and procedures. Thank you. Angel, you wanna start with that? So asking about functional. Yeah, as far as just dystonia. Yeah. So as you said, it's very difficult to determine if this is frank dystonia early. A lot of the treatments kind of help us weed that out. Our institution does not have a individual or a, I guess, written protocol that we use. We have recently started a surgical tone intervention clinic that we're having multidisciplinary approach with that PT, OT, neurosurgery, and PM&R. And so that's kind of helping us to have all four, you know, hands on deck looking at these patients at the same time, doing different assessments, HAP, BAD, all of that kind of stuff, and seeing if we can weed out and what our best guess is. Also with getting the information from the parent, getting videos, all of that information is helpful in trying to make an educated decision on this. At Mayo, we also have a movement disorder lab. So they do different types of EMG assessments on individuals with dystonia. And so can use certain parameters to weed out to most extent those that have more of a functional appearance versus those that have a primary or secondary dystonia too, which is quite helpful for that. Thank you all for wonderful presentations. I was interested in the hyperselective neurectomy. And what I was interested in is if you all had any experience with the cryoneural lysis and if that could mimic that and give you some sort of insight into whether that would be a good intervention to pursue or if you've had any sort of experience with cryoneural lysis for spasticity management in general. Great question. I do not have any experience with that, but I know Josh Vova who's sitting back there does and could talk a little bit more about that. I don't know that that would necessarily give us some insight. I feel like when doing phenol, Botox, that can give us an idea of if a child may respond to it. For some of the kids that I've done it, I either can't get enough botulinum toxin into that muscle, but I can respond a little bit, but it seems like it's still really tight, really stiff. I've also found the neurectomy to be helpful for kids that really seem to have that focal tone of the gastroc and they're also having a lot of trouble with pain, which I know we're not supposed to do these type of procedures for pain, but it's actually been helpful for pain reduction for that. And Josh, you wanna talk about the cryoneural lysis? Sure, and actually, the answer would be, I believe so. And so the way that the cryoneural lysis works, it's exactly what you do for a selective. And the whole reason that they started cryoneural lysis was because of the selective neurectomies and some of the surgical complications that were happening with the selective neurectomies. That's why they started doing cryoneural lysis. It came from, because they do a lot of selective neurectomies in Europe. And so I'm looking at those problems. And so the biggest problem, especially when you're doing a selective neurectomy or a cryoneurotomy or whatever you wanna call it, right, is trying to decide how much function to take away. And so your pluses and your minuses. So what we do before we do one is we actually go into a selective neuroblock and decide, especially like in the case that Jolene presented, do you wanna do the gastroc? Do you wanna do a soleus? What's it gonna look like? How much you're gonna take away? And then you go back and after going and do a cryoneural lysis and take away some of your function, so it does actually mimic that without the surgical complications. However, it's only gonna last for a certain period of time. The nice thing about cryoneural lysis is it does not cause any scarring, it does not cause any damage to muscle tissue, it will not interfere with any surgical procedures that you need to have in the future. And so that's why we're doing it. Thank you so much. I love that we're growing tools in our toolbox. So Stacey Stipp from Milwaukee. So more of a comment for you for Jolene, because we're doing these neuroctomies as well, but doing it same patient population a little bit different, only sectioning to the soleus and a lot of times doing a strayer at the same time. So it'd be interesting to kind of compare our outcomes and see which works better in terms of gait and things like that, because we're not getting as much pain and swelling obviously post-op because it's not as large of an incision and things. That's great to hear. So our surgeon who does the neuroctomy is an adult surgeon. So that's part of why we do it in two stage, because that's what they do on the adult side of things. So trying to bring those practices together. So we actually get two orthopedic doctors to operate and talk to each other, which I know can be difficult, but we've been able to do that at Mayo. And so it's lovely to have that conversation and we're just early in that process of sorting that out. It's nice to know your experience too. Yeah, so we have the benefit of the PEDS neurosurgeon and the ortho working together. Fantastic. Question about combined rhizotomies. One, are you guys doing anything pre-op to test their bladder function? So we are thinking about, do you do a CMG? Do you do ultrasound? What do you do to kind of test bladder function? We have a lot of international kids who are coming to us and ended up with combined rhizotomies. And I think that's a question. And then also, we send our kids who get SDRs to inpatient rehab, but we have not been sending our kids who are GMFCS fours and fives to inpatient rehab because their level of function isn't changing. It's a quality of life caregiver. And it's not really that big of a change to really qualify. So like, how are you deciding that? So it's a good question. We actually do send all of our kids that were going to do a combined rhizotomy on to urology and they get UDS before the procedure. And we don't have the benefit of an inpatient rehab unit in our state. We are currently building one. So that is a good question for the future. I think it's gonna depend a lot on the goals because we are seeing kids with our SDRs who seem to qualify for different levels of rehab afterwards. There are definitely those that we are trying to send out of state for inpatient rehab because we know that they need it. And there are others that are doing quite well with their intensive outpatient program. So I think it's been case by case for us. Marcy, are you guys doing inpatient rehab for your combined? We are doing a short inpatient rehab stay, but that's mostly to stabilize them on our unit rather than the neurosurgeons continuing to just watch their surgical health afterwards. So really to get the caregivers used to caring for them with their new reduced tone and getting them up and do equipment. And do you do bladder testing ahead of time? Yeah, unequivocally that's been a part of our work now is before the ventral dorsal rhizotomy, they all get a bladder workup. The urinary retention concerns have really prompted us to have frank discussions with families beforehand about how that might change so that that's not a surprise if that does in fact happen. Has it had a role with the UPS or like with the EMG? Or is it? Yeah, it's a VCUG, KUB, and yes. So they're looking at the EMG and the complete aerodynamic studies. Have you had any bladder issues persist? Yeah, we've definitely had bladder issues that have persisted. That's been one of our biggest challenges and concerns. And that's why we're now talking to families about it. Very, very frankly, almost preparing them that it's gonna be a certainty because it's definitely a dissatisfier if it does happen. And we've done just a handful of them. Our population is much smaller. I have not been doing aerodynamics ahead of time, but now I'm really thinking I probably should be. And those kids do not come to our inpatient rehab unit partly because of the functional piece for it. So it's good to hear your perspectives on that. So I have a question about SDR and GMFES-3 for children with intellectual disability, autism, behavior issues, and where you fall with those kids. Part of the larger talk is usually talking about how well kids cooperate in rehabilitation settings. So that's a piece that we consider, but it's not a hard stop for us. I would say that the tone reduction is there. And I think about my three slash four group that have significant cognitive and intellectual challenges. We're not looking to get those kids up. If it's a three and a four, we're not looking to get them up to ambulating with an assistive device. If that happens, boy, that would be great. But I just saw one back, and the child's now 18 years old and had his rhizotomy close to the age eight, and the tone reduction is there. But to be true, he's moving about in his environment at the same level that he did. I haven't done phenol or Botox on him for 10 years, for which I'm very grateful to not have had to put him through that because he does have autism. And the orthopedic surgeons haven't had to lay a hand on him, so that's my thoughts. It's every individual patient and what they're going to need. It's not a hard stop for us, but we certainly don't expect that the patients are gonna be participating at any higher level than they already are. So I actually have a question about the partial nerve ectomies. Because there's all that literature and that struggle with sarcopenia and botulinum toxin, and everyone has their own opinion, when you're doing, and botulinum toxin is a partial denervation, when you're doing a surgical procedure in a young child that's more of a complete denervation, are you concerned about what's gonna happen to that muscle as the child grows? Because that's something I've always been struggling with, especially once I'm doing the cryo and having this period of time is, what am I going to do with a more complete nerve lesion to this developing muscle? I'll start with that, I guess. Great question, and the answer is, like everything else that we talk about, I don't know. I think it's important to have that conversation with families about the long-term effects. We're not sure. Right now, we're having this sort of struggle. Here is our goal, but we don't know exactly what this is gonna look like down the road. One of the ways that I look at it is that this is a really overactive muscle that's causing us a lot of trouble, and if we don't do something about it now, we're going to put the child through multiple orthopedic surgeries, potentially. So weighing that risk-benefit of multiple orthopedic surgeries over that lifespan versus potentially sarcopenia in a muscle that's really not helping them that much at this point, I'm not sure, and is that sarcopenia really gonna be that important for them when they get older? I don't know. There's a lot that we don't know about muscle in our adults with CP, and a lot that we don't know about what we're doing and how that impacts muscle versus doing absolutely nothing. And so there's a fine line that we're trying to ride here, and I certainly appreciate that. And when talking to my parents about what we are proposing to do and putting out the options of, here are the tools in my toolbox, here's what we know, here's what we don't know, what do you think about it? And I have some families, by the time I get done, their eyeballs are really big, and they're like, what? And at that point, we're like, we are gonna hold on doing anything, and we're gonna give you time to think about it, and we're gonna let little Johnny or little Jill grow just a little bit more, and we'll keep doing what we're doing. And then we'll have this discussion again and see where we're at. And also having the child participate in that discussion as they're getting a little bit older too, because we're doing this to them, and we want to make sure as doing this to them, we're doing it for them. So it's probably a little bit of a long-winded discussion of saying, I don't know. Anybody else? I'm just wondering with ultrasound getting better and better in its ability to discern, oops. I'm just wondering about the use of ultrasound as it's improving in its image quality, whether you're able to actually see the nerves as they're getting in there into the muscle, and whether doing phenol blocks at that level, kind of like the cryo part would be something that, is that something that you've had any experience with? I haven't used it for that in that purpose, but phenol itself, no matter how much you finesse it, it's gonna fibrose everything it comes in contact with. So even if you finesse it to that point, we're still gonna have some muscle fibrosis with it. And that's something I always talk to families about too when doing phenol is about that fibrosis piece and that we're watching for and the fact that that muscle will and absolutely does change when we're doing the phenol with that. Question? Yes, for the deep brain stimulation, do we ever use it for like a localized, someone just has dystonia in one arm? Would we ever do deep brain stimulation or is it always for more diffuse dystonia? For all of the things that I've seen, it's for the more diffuse dystonia. So if we're looking at more of the localized one arm, we're looking more at then the toxin injections or things like that that can be more adequately controlled to be more precise. I was thinking maybe it would prevent, you know, it starts in the arm sometimes and then becomes diffuse over time. I was thinking maybe it could prevent it from becoming diffuse. Yeah, that's an interesting idea. I don't know, I'd have to look into it more or give you my institution's neurosurgery people and you can ask them. Thank you. And I think if you're thinking about DBS and you have access to somebody who does that, if in doubt, it doesn't hurt to refer just to have the families have the discussion. I've had a couple of younger kids that I've referred. I know they're a little bit little, but I see it as something that's probably on the horizon. And so I have them meet with our DBS team to discuss what does this look like? What do we think about this? And when we're thinking about timing, when should we think about it? Again, giving my parents the tools to make those decisions and to know what tools to use and to know what tools are in the toolbox that we have at this point to help their kiddos and try and make that best decision they can with the information they have. We have gone terribly over and I am thankful that you all sat here listening to this and we're happy to answer any questions afterwards too. Thank you.

advanced hypertonia management

children

selective dorsal rhizotomy

palliative rhizotomy

intrathecal baclofen pumps

deep brain stimulation

hyperselective peripheral neurectomy

spastic hemiplegia

genetic syndromes

mixed hypertonia

dystonia

cerebral palsy

individualized treatment