What my charge was today was to basically tell you everything I know about cerebral palsy, which is not very much, I don't think. I think the more you do this kind of surgery and evaluation, the more humbled you are, as many people have said earlier. This is where I work, so many people here are too young to realize it, but I kind of put this up there because if you remember the show Dallas, the opening scene, they were showing this with the big hole in the petroleum club. This is the hospital where I work, but in the distance here is sort of the sign of Dallas, so if anybody's old enough to recognize it, that's what it is. I have no real disclosure about this. Cerebral palsy has actually been described way in the times of B.C., even in the Roman Empire, but really it wasn't until about 1862 when William Little, and he's a very big name in orthopedics because he started orthopedics in England, and he became interested in this topic. His quote back then in 1862 was that the upper extremities are sometimes held down by prepondering action of pectorals, teres major and teres minor, and latissimus dorsi. The elbows are semi-flexed, the wrists partially flexed, pronated, and the fingers incapable of perfect voluntary direction. So what about cerebral palsy? When you look at it, it's really a static encephalopathy. There's about 1.5 to 2 per thousand live births, plus or minus a few in there, and it really hasn't changed over time. The number's pretty stable. It's an upper motor neuron dysfunction. It's a non-progressive neurologic disorder, and the musculoskeletal system, even though it's non-progressive, it responds to persistent deforming forces, and that's what causes you to have sort of the classic position that's seen in cerebral palsy patients. So the manifestations of it are due to disorders of the motor control, abnormalities in sensibility, and many of these patients, which we need to take account to, and I think you've sort of heard earlier today by Scott and other people, is that it also has to do with the patient and their sort of intellectual abilities, and some of these children have impairments that affect some of their outcomes. So you would think that this is easy to recognize, but it's really not, because if you think about it, when it's early on as an infant, it's very difficult to see these patients and to diagnose them, and frequently we actually see them because they come into our brachial plexus clinic. I don't know if Scott may be the same, but sort of early clues to these is that primitive reflexes, like for instance a Moro reflex is normal until you get to three months of age when you sort of startle the kid and they go like this, and then if that persists after three months, there's something going on, and so that should tip you off, but really it's very difficult, and so we talk about increased tone, as described a little in the fracture pronator grasp, you know, sort of this thumb and palm sort of positioning, delayed motor control, gross and fine, and other things you tend to see are abnormalities in speech, vision, hearings, some of them have pretty significant seizures, behavioral problems, mental retardation, you know, so tone is this thing that we talk about. So what is this tone? What is this hypertonia? So this was out of a paper that was actually done by one of our neurologists, Dr. Delgado, so hypertonia is defined as abnormally increased resistance to externally imposed movement about a joint. It may be caused by spasticity, dystonia, rigidity, or a combination of features, and that becomes important because, you know, as surgeons, we like to put everything into the box. We like to have it clear cut. This is this and this is that. Well, I think this is one thing that's not, so we try to put it into where the location of these lesions are, so the spastic or pyramidal lesions, which apparently have been quoted to be about 50%, sort of dystonic, athetoid, extra pyramidal locations, which are about 40%, and the mixed, which are really 10%, and I think that anybody that does cerebral palsy realizes that that's not really the case. I mean, there's some pure spastics, and a lot of the, and pure dystonics, but some of them, or a lot of them, probably a lot more than 10%, I think, really fall into sort of that mixed bag and sort of mixed tones, and I don't think it's a pure sort of event, and so it's really important, and I'll discuss it over and over, that you see these patients several times, and I think that, you know, Scott would probably agree that we see these patients several times in the office as well as other tricks to make sure we check on their tone. So the characteristics of this sort of pyramidal or spastic type is really spasticity, synchronous activity, hyperactive reflexes, clonus, myostatic contracture, weak, slow, voluntary actions, and really the importance for this is that, as opposed to the dystonic type I'm going to show you in a little bit, these ones are patients that are probably good or reasonable surgical candidates to think about. I had a video, actually, in my talk that's not as good as Scott's, so I went ahead and stole his, that he sent to me, so it's with permission, right, Scott? Yeah. So I'm using it as a little bit different, so I'm trying to show you what a spastic patient would be. So the reason this is probably a good candidate is that she's got volitional control, she has these deforming forces, but again, if you do tendon transfers, you can probably make her a lot better, because of what you're seeing there, that she has this volitional control, this spasticity. And that is opposed to sort of this extra pyramidal type, or these lesions that are sort of outside of the normal areas, and those patients have athetosis, no fixed contracture, tremors, ataxia, rigidity, and dystonia, and if you see these patients, it's pretty scary, actually, because it looks like they're possessed. So you may be talking to them, and their limb is doing this, and sort of doing that, and you tell them to do their wrists, and they're rigid. And so an example of that would be this, and this is sort of someone that I think you could tell, if you did try to do something to this patient, tendon transfers or things, that they just wouldn't work. There's so much rigidity, dystonia, and the limb actually appears at least to have a mind of its own. So what are the deformities? So deformities have been talked about quite frequently. In the shoulder, you get internal rotation, adduction contractures, elbows flexed position, forearms in a pronated position, the wrist is usually in a flexed and only deviated position, fingers are flexed, thumb and palm, and many of these patients also have swan neck deformities. An important assessment that I think everyone needs to do when you think about a potential candidate patient is that you want to look at what their functional classification is, and that's what this so-called house classification is. It's a zero-edit classification, and as far as what you remember of it, is basically the seven or eights are pretty almost normal. They have very good spontaneous use and almost complete control of it, whereas the ones that are zero to ones really are basically doing nothing. I mean, they're just sort of in a fixed posture, and they don't have very good volition control or any volition control at all. And so the ones that you can really affect difference are obviously in six, seven, and eights because you're going to make a big difference in them. But when you look at this group here, the two, three, fours, and fives, if you can take someone that actually is about a three that has a good passive assist and actually put them into a four, which is a poor active assist, or a full active assist, you usually really change their life. And I actually have a video of that later, so we're going to go over that a little bit more. But this kind of gives you, when we look at patients, that's kind of what we look at is sort of what kind of volition control they have, what kind of use they have of it. So any time we're talking about surgery, we also have options, whether it's therapy and sort of Botox that come into play, and surgery, and also combination and all of the above. So if you look at these sort of non-surgical components, the therapy, does it really work? Well, we have excellent therapists like Scott does, and they're just devoted to our hand service. So as far as the patients, I think that they get the best care because they're going to need therapy not only before you entertain surgery on them, but also throughout life or until they get out of our system, which is at 18 years of age. So the non-surgical therapy, if you look at what the reports are, this is just one report in 42 studies. When they looked at this in a review article, 13 different upper extremity therapy approaches included 1,454 patients. Their inclusion was 0 to 18 years old, unilateral cerebral palsy, and they actually evaluated the efficacy of non-surgical upper extremity therapy or adjunctive therapy in combination. Outcome measured upper extremity unimanual or bimanual capacity and performance, achievement or individualized goals or self-care skills. And when they looked at it and summarized all these data, they basically found that modest evidence, that intensive activity-based goal-directed interventions, which are really this sort of constraint therapy and bimanual training, were probably more effective or are more effective than standardized care improving upper extremity and individualized outcomes. So in patients that haven't been operated or are not operated, that you can probably affect a difference or make a difference in them if you do some of this bimanual training as opposed to sort of the unimanual training that many of the therapists have been taught during their training. And strong evidence that goal-directed OT home programs were effective and could supplement hands-on direct therapy. And if you go on, and that reference is here, it's a big article in Pediatrics, 2014, so it's fairly recent. And what about Botox injections? So one of our colleagues, a well-known hand surgeon in North Carolina, Dr. Coleman, published his experience of 73 patients injected with Botox divided by the house classifications. Ten patients were 0 to 2. Fifty-four patients were in that 3 to 5 area, so either there were passive assist or active assist. And six to eight and nine patients were in this category of, you know, basically very good for listening control, very good active use of it. And they either used Botox or placebo injections. And they looked at them at baseline 8 and 20 weeks is when they injected them. OT evaluated them as well as the surgeons, and they had an evaluation of screening baseline 8, 20, and 26 weeks. And then they put it all together and used what's called the Melbourne Assessment Unilateral Upper Limb Function instruments for pre- and post-injections to kind of compare what kind of results they had of it. And there's a lot of sort of systems and a lot of evaluation systems for CP, and I think people could argue which one is the best, but it's one that's certainly accepted as an outcome measure. And so children receiving Botox experienced a short-term improvement when compared to surgery. Repeat injections were well-tolerated and safe. Use of Botox for managing upper extremity spasticity is available for children for whom surgery is not recommended. So, in other words, some of these dystonic patients that you want to sort of get them out of their fixed posture, Botox may be an alternative for them when you know that you're doing tendon transfer surgery is not going to be successful and will lead to failure. So if we go on to surgery, so how do we really decide what to do? And I think this is one of the hardest decisions in surgery to make. Patient evaluation is crucial. So you have to know what the sort of generalized intelligence of the patient is, level of motivation, type of neuromuscular disorder they have, type of spasticity. And then in terms of the upper extremity range of motion, sort of the grasping release pattern, deformity sensibility, functional pattern. And we're going to talk about the SHUI because that's the Shiner's Upper Extremity Evaluation, it's a videotaped evaluation, and I think it's crucial to have some sort of evaluation on these kids before, but that's what we use is the SHUI, and I'm sure Scott does because that's a Shiner's instrument. But this is also something that I don't think people have talked about enough, but this is really the evaluation of digital tightness. So when you think about these kids that have this spasticity when their wrists are flexed, how do you know if the fingers are tight, if the finger flexors are tight? Well, this is a good way to do it. So with the fingers in extension, you bring the wrist up into extension from its flexed position, and then this so-called Volkman's or Eaton's angle is the angle that's recorded when the fingers start to bend down as you bring the wrist up. And that's important because that tells you or gives you a sense of how tight the finger flexors actually are and whether or not you need to do something during your surgical intervention. So if you did this and you're able to get the wrist straight with the fingers straight, that you know that that wrist sort of problem is really from the wrist flexors and doesn't have very much of a component, if at all, from the finger flexors. And it's important treating it. So when you look at the treatment options for these things, you have to really, and again, I think it's been emphasized all day today, is that you have this sort of Venn diagrams, you know, from grade school math, but you have these wishful thinking and these realistic ideas. So wishful thinking by the parents is there's a lot of guilt sometimes in the mom, and they want you to make that extremity normal, quote, unquote, or be like the other side. And, you know, realistically, we know what we can provide, and so we have to get those ideas to mesh somewhere in the middle. So we know that it's going to be an improvement, and there's things that we can do to make the child better, but it's not going to be normal. And that's really important, I think. The problem is really a CNS problem, and so it's a problem with the connection between the brain and the arm because the muscles are there. It's just the connection is not getting there. So you can't fix that. The surgical treatments available deal with musculoskeletal structures in the periphery. The treatment is really dependent on the reasonable preoperative goals, and parents must be aware of what is and what is possible. And I'm sorry for repeating it, but I think that that is really, really important. And the best predictor, I think, of improvement of function is really a child's attempt to use the hand volitionally. The goals really have always been and always will be improvement of function, decrease in deformity, a change in position. Many of these kids, when they get to be teenagers, don't like the wrist down. They would rather have it straight, whether or not they have a lot of function in it or not. Facilitation of hygiene and custodial care, improvement in appearance. And again, to go from a house zero to eight is not going to happen. Some of those patients you can make from a three to a five or a six to an eight, but you're not going to make them from a zero to an eight. So it's imperative that you examine the patient several times, and I think it's really important to note that when a child comes, sees you in the clinic for the first time, there's a lot of anxiety. Even if you don't wear a white coat, they're very anxious. And so their tone could be much different than when you see them three or four months down the road. So typically we'll get a good exam on them, send them to our therapist, and the therapist will work with the parents with bimanual exams. We don't do too much constraint therapy, but bimanual sort of activities, and then we'll reevaluate them in about three or four months. And I think that's really important to see what their tone is, and again, write down what you feel like is on their exam. And then one of my mentors, Pete Carter, I think he's full of, a lot of people know Pete here, but he's sort of full of great one-liners. And one of them is the two-four rule. So when you're thinking about doing a surgery on a child especially, or anybody, you wanna remember that ultimately you wanna do something for them, and not just to them, because there's a lot of operations we can do to them. But if you're not gonna do something for them, don't do it. And it's important that the family and patient, again, have realistic goals. So what about this SHUI examination that I talked about? Well, it's a videotaped examination where the child is asked to go through a bunch of tasks. And it's videotaped so you can review it again. And it's a validated study, and a validated tool. Includes spontaneous functional analysis and dynamic positional analysis, as well as some grasp and release tasks. And it's able to evaluate all of those. So it evaluates, basically, the spontaneous functional analysis from zero to nine, and going from zero to complete neglect, to five indicating a spontaneous independent function. And then dynamic positional analysis, how do they perform those tasks? What is their wrist position is? What their finger position is? And again, it's graded. This is what it sort of looks like, the worksheet that our therapists go through when they actually score it. So what happens is there's a sheet of, and there's tasks that they do. It's videotaped, and then the therapists go back, and they look at exactly what happened during that videotape, and they put scores on it. They come up with actually a percent that's recorded. So, just to show you. So this is a 14-year-old that has right spastic hemiparesis. And this is sort of part of the task. And you would say that he's probably in the level, sort of probably a house threes, as you'll start to see as he sort of uses as a passive assist. But you see his wrist is down, so that character's position, his forearm is pronated. He does want to use it, though. I mean, he does use it as a helper. It's not totally neglected, like some of the kids that are really rigid and dystonic. And they also carefully, as I said, examine sort of what they're grasping, and how they're using it, and sort of the grasp and reach pattern that they use. And so then you say to yourself, what do I do now? And I think this is, in the past, we hadn't used this as much, but I think it's a great tool, not only for us as, where I am, but it's also a great teaching tool, especially for the residents and fellows that come with us. I don't know if you use it very much, Scott, but it's good, because we can sit in a pre-op conference, and everybody can sort of talk about what would you do, what do you think the best treatment for these children are? So the issues really are in that chapter, and I think it's a great tool, and I think it's a great tool for us, so the issues really are in that child, if you looked at him, is his forearm, so his forearm's pronated, his wrist is in that sort of flexed, on or deviated position, fingers and thumbs are flexed, and so you really want to do this decision-making where you're gonna make the right decision, and the right decision and the wrong decision, and sometimes there's a pathway that you've got on both ways before you come up with, actually, what's gonna be good. So in the forearm, what are your options for a pronation deformity? Well, it has to do with, really, the pronator teres, so you can do a straight pronator release, you can do a pronator rerouting, a flexor pronator slide, if they're really, really tight and you don't think you can get it with just a pronator release. These two, between a release and rerouting, really has to do with if you can evaluate the supinator and see if they have some active supination, and you know that if you just release the pronator, they would have supination, or if you release, if the pronators are really working, it has felicitative control, but their supinator is not, if you reroute it, you actually give them some supination. In the wrist, the EC to ECRB transfer, like Scott had talked about earlier, is what I tend to use. Sometimes you have to be very careful to determine that the ECU is actually working, because sometimes it's pulled so strongly and the FCU can mask it and lead you to believe that the ECU's really there and working when it's not. But for reasons that he said, you can get a supination deformity from the FCU to ECRB, which, if you think about function that you would use, having full supination in your forearm is actually worse than having pronation. So you can actually do a lot of harm to them if you do that. But certainly it's written about the green transfer, FCU to ECRB, and you can actually fuse the wrist to be straight. And as far as the fingers go, what do you want to do? So you have to determine, again, with that sort of Eaton's angle, how tight the fingers are. If the fingers are tight, you can either Z-lengthen them, intramuscular lengthen them. Again, if they're real-world tight, so the Volkman's or Eaton's angle is up at 60 degrees, you may have to do a flexor pronator slide, which is a big operation that you release everything off the medial epicondyle and off the ulna and even off the radius, because if you think where the FPL starts, it starts way in the mid-radius. And so you're really, it's a big, big operation to do. It's not just like going in there and doing carpal tunnel release. And the thumb options, if you look at that child, is you could do MP stabilization, some sort of fusion, have to do something to sort of release the thenars, FPL release and EPL rerouting if you think they need more extension. So in the child after you've, in that particular child that we're gonna talk about, that we talked about, we decided that we would release his protein or teres and reroute it. Wrist flexor lengthening, ECRB tendon transfer, FTS, intramuscular lengthening, and thenar release to get his thumb out of his palm and also give him some stabilization. So if you look at him, this is him for his post-op SHUI score, and he's just kind of basically showing that he can bring his wrist up and his fingers. And when he's doing some of these tasks, again, it's not like the other side, so it's not gonna be perfect. But in him, really what's really important to note is that you really sort of increased his use of it. So if you remember back to where it was more of a passive sort of assist, it's now become more of an active assist than it was. And for these children, it makes a huge difference in their ability to perform ADLs and their ability to use the upper extremity. And now he sort of really used, although it's not quick, he still uses it to tear the paper, to grasp the paper, whereas before, if you remember, he was just putting his elbow down or his forearm down. And so when they graded the SHUI pre and post, it went from, they do it in percentage, 22 to 36%, and dynamic position of 28 to 81, and grasp and release went really from 50 to 100. So his overall house when they scored it went from a three to four, which you think is just one grade, but it actually, like I said, is really important for him because it really went from a passive assist to an active assist. We won't do too much of this child, but this is one that was more rigid, more dystonic, and intellectually was not really there except he wanted his wrist to be straighter, did not use his arm very much. This is kind of a pre-op SHUI that kind of shows you that in the interest of time. We'll just sort of go on to the next. When we do our wrist fusions, we actually do a proximal carpectomy because it shortens everything down. Do a wrist fusion, put a plate on it. As you see here, and many times if you do that because you're shortening down the column, you don't have to do as much of the finger flexor. So in those cases, you want to really fuse the wrist first and then do something to the fingers later. And this is just basically showing his wrist straighter, and he, if anything, may use it a little more, may not, but because of his straighter wrist position, he was very happy with it. And when they looked at his scores, it increased also nine to 27, and sort of spontaneous dynamic was 35 to 61. Grasp and release was zero to 33. And if you try to sum all this up, it's really that I think every patient is different. You can't put them into specific boxes. You have to really know and see those patients quite often before you operate them. It requires a multidisciplinary approach. So we have our own neurologist there, and they're great because a lot of these kids have seizures that need to be controlled before you operate them or someone's spasm. They need to be on Baclofen, a good OT department. Realistic expectations are so important in these kids. Participating guided therapy. And I really think that you should have some sort of video cataloging of these kids before, whether you do a shoe or a Melbourne that you videotape. I think it's really important for that. So that's it. And there's actually gonna be a full day of this in Rotterdam for anybody that's interested in the future. So. Thank you. Thank you. Thanks very much, Scott. Questions for Scott? Jeff? Jeff Friedrich. Thanks. Great talk, Scott. I always think that it's somewhat akin to rheumatoid hand surgery in that there's an aesthetic component to this that people don't want to talk about. At least patients don't want to ask about that as their first ask. But it seems important. How do you factor in the aesthetics? Because that position of the hand is almost, it's almost like a scarlet letter. It's very easy to spot and easy to make fun of at school. Well, you know, the teenagers that we see that come in that haven't had a, so the teenagers that come in that we see that have not had surgery and their wrists are really in a flexed, ulnar deviated position, they want their wrists fused. And they don't really have any problem with a scar. Because like you said, I mean, being like this walking around is much different than sort of having a straight wrist. So I haven't seen that being a problem with the scar. And Jeff, the giveaway is the adolescent will come in and they'll say to you that my arm's getting worse. That's like the biggest red flag. And you say, well, what do you mean it's getting worse? It's getting worse. And then it takes a few minutes to realize it's really not getting worse. It may be more psychologically painful to them. But as you get older, the things you do in life become more complicated. So that's number one. And number two, as you get older, you don't want to be different. So in those kids, I think it's fine from a realistic standpoint to fuse the wrist. Like Scott showed, you have to be careful about fusing the wrist in extension because there's a loose ability to open at all. And you may even have to fuse them in some flexion, even though it'll be a better appearing arm. And I think it's totally appropriate to fuse them. And I guess that's my feeling about it is as an indication for surgery, it's not a bad indication. The aesthetics of that part. Because if you think about it, the real indication for that besides the look is that many of them have hygiene problems when they're flexed down like that and they're so locked. So when you bring a wrist up, you can actually bring the fingers out by whatever procedure you sort of like to do that with. But I think that's important, the hygiene aspect. David? Scott, great talk. I have a lot of TBI patients from the surrounding bases. And I find myself doing a lot of flexor pronator slides for those people. And I just wonder if you would comment on your experience with that and how you decide when to do a flexor pronator versus something maybe less ablative. Well, so the flexor pronator slide for me really depends on what that evaluation first shows. That you get a good idea when you look at the Volkman's or the Eaton's angle on it, how tight their finger flexors are. I mean, if their finger flexors are 50 or 60 degrees, you know, then you, I mean, I basically tell the parents that we're gonna have to do that because there's no intramuscular lengthening or no z lengthening you're gonna be able to do. So for me, that's what I determine. The difference is when you're doing a wrist fusion again, because even if it's 50, 60 degrees to start with, once you shorten that column down with a proximal row, many times you can get away with doing a z lengthening or intramuscular lengthening. Because I think, I mean, you would agree, I mean, that's a very daunting, I mean, it sounds like, okay, we're just gonna do a flexor pronator slide. Well, I mean, it's a big operation. I mean, it's not only an operation like this, but you really have an incision like way up from above the elbow to down. So it's a difficult operation. But did I agree for the bad deformity? If you do the owner approach like Milan talks about, but just like Scott said, you can't stop until you're on the radius. Yeah, if you, not checking out, but if you start and you go, oh, I'm not gonna get to the radius, you're not gonna get to the FPL, you're not gonna get them out. So for the really bad TBIs that are really, really spastic, I think it's the only operation that you can do to get that relatively non-functional hand and arm in a better position. But you gotta keep going, and you gotta tell them it's gonna be a big, long incision along the owner's side. I agree. Last question, David? Any ideas on the durability of some of these procedures, especially in the younger child and the need for long-term splinting? Because you've always got gravity that's working against you. You've got a skeleton that's continuing to grow. So, I mean, I think that probably what Scott said earlier, I didn't wanna really disagree with it, but I would say that when you do the transfers in a young child for the wrist, the ECU, ECRB, when we looked at our experience, I don't have an exact number for you, but many of those patients come back as a teenager because they have that flexion deformity again, and we end up fusing their wrist. Do you have that, Scott? I mean, we see that the finger ones we don't see and the pronator ones seem to last. We like to do them, like Scott was saying, young. I mean, I like to do them at four or five, whenever they can sort of help you with the rehab, as far as the initial surgery with CP kids. But the wrist flexors, I think it's a tight, even if you release them, and they go back down. So they may need to have fused as a teenager. Okay. Thank you, Scott. I have to pledge. Thank you.

cerebral palsy

neurologic disorder

motor control disorders

hypertonia

deformities

treatment options