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- [00:01]
A
Welcome to AOFAS Ortho Podcast, where leaders in foot and ankle orthopedic surgery discuss the issues that affect you and your practice. Please note that the views expressed on this podcast do not necessarily represent the views of the AOFAS or its members.
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B
Welcome to the AOFAS Ortho Podcast. I'm Pam Luke and I'm here again with my co host Joe Park. We have the great pleasure of having Dr. Glenn Pfeffer today with us as part of our series Expert Spotlight and Masterclass today talking about Cabovaris foot deformities, more specifically Charcot Marie Tooth and its related foot deformities. Dr. Pfeffer is currently a professor of Orthopedics at Cedars Sinai Medical center in Los Angeles, Director of the Foot and Ankle Center, a co director of the Cedars Sinai USC Dance Medicine center, past president and co director of Hereditary Neuropathy Program at Cedars Sinai, and also a member of the research council for the Hereditary Neuropathy Foundation. It's many accolades with Dr. Pfeffer, but these last two specifically made our committee really want to invite him to shed some more light for us today about cmt, what we can do as orthopedic surgeons to manage these patients better, both clinically as well as surgically. Welcome Dr. Pfeffer and Joe.
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A
Thanks for having me.
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C
Thanks, Pam.
- [01:33]
B
So Dr. Pfeffer, I think both things are like on our minds is how did you get into becoming the expert in orthopedics for this very special niche? Mainly because if I Google CMT and orthopedics, I think you're the first maybe five, ten hits on Google. So how did you kind of find this?
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A
You're right. I'd never used ChatGPT until about a week ago. I didn't know if I liked it or not. It's kind of silly, isn't it? And I said to ChatGPT, who's the best CMT surgeon in the world? And I liked its answer. So now I paid 79 for some kind of advanced form anyway that everyone's in the foot society has known me a long time. I don't really have a big ego. So take that with a grain of salt. But it's true story. You know, I've been doing this a long time and I went into orthopedics to do hand surgery and I did a hand fellowship. I ended up in a job in San Francisco where I was running the hand service at the Army Hospital and doing hands in private practice. But in my hand fellowship I thought you had to do replants. And I was not really interested in being up every other night as I was in my fellowship doing replants. So I did another fellowship in foot and ankle where I didn't think there'd be too many all night replants. And when I got to San Francisco, I did both. And I was there for about 18 years. I was running the foot and ankle clinic at UCSF and in private practice. It was just wonderful. And in about 1988, just a wonderful young woman came to my office and she said, I'm having some problems with my feet. And I thought, this is cool, this is great. What is a sports injury? Maybe an arthroscopy, Ankle laxity. And she said, do you know what CMT is? And I lied. And I said, sure, yeah, of course. And she said, well, that's good because nobody really knows what it is or heard about it. And then I noticed there were crutches in the corner of the room. And she was probably about 23. And she said, I've never been on a date. No one has ever seen my feet, including my parents, for as long as I could remember. So I'm going to like to show you. And she showed me these gnarled, like live oak, twisted feet. And I realized she was barely walking. That was the beginning, really. She had cmt, I think maybe I didn't know anything about that from residency or never did fellowship. And maybe, maybe there was what, a five second discussion during medical school. There wasn't much out there then, you know, Hansen, Roger Mann, the founders of the foot society, Nathaniel Gould had been interested in this. There's literature, but there was no clear consensus on how to take care of it. And I did. And then I remember years later, this is still AOL day, she said, I want you to know, Dr. Pfeffer, I've gone out on a date for the guy. It's kind of corny, but it's just, you know, it was very potent. And then, then a few years after that, she said, listen, I'm getting married anyways. Very moving to me because I didn't. I don't like orthopedics much. You know, I'm not big in. I don't really love putting nails in. And I never like big fractures. And I left hand surgery. And this was a perfect combination for me. And then I realized that. What do you think? 60%, 65% of these are kind of straightforward, right? The weak brevis tibialis anterior, weak cavo varus foot plantar fascia contracted. And then Greg Guyton, Alistair Younger wrote some nice reviews. But then I was faced with the problem as I got more and more patients by word of mouth that at least 30, 35, 40% of these patients do not fit the mold. And there was nothing really about what to do. And it was kind of exciting for my brain to figure it out. And it. That's the story.
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C
And you were just commenting off mic before, but at this stage you were saying, how many CMT corrections do you think you've done at this point?
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A
Yeah, we, I ended up with the fellows last year when we were. Two years ago when we were work, you know, doing some 1500, 1500 CMT cases. And someone the Foot Society came up to me, said, you mean 1500 CPT codes? Yeah, because we do 80 a year. You know, I'm 72, I've been doing this a long time. And that young woman I told you about, she had friends and there were some patient groups. And then I reached out and then what happened is the day I showed up at Cedars 20 years ago, you have nothing to do in a new job. And I contacted the people at Cedars and said, I want to set up a CMT center. People always oblige you when you're in that honeymoon phase in a new job. And they said, okay. And then we started doing more and more. And then I reached out, I think the second week to the medical advisor, the national groups, there are two big groups, Hereditary Neuropathy foundation, cmta, there's other groups, CMT Cure, that have formed. So I got to be on the medical advisory board of those groups. And then I started giving lecture to those groups. And then, oh, maybe eight years ago, a young woman said, Dr. Pfeffer, she says, I'm from Arkansas. Nobody knows about you in Arkansas. I said, what could I do? And she said, set up an Instagram site. And I was very cool about it, you know, nonchalant. I said, okay. And I went to the room with the resident and I said, what's Instagram? I don't know. I don't know if he has completely stored stumbling along, you know, but so we set it up. You know, you can do that in a minute. So we set up Charcot re tooth surgery, and then I got a haircut the next day. And I said, how do you put a video on? And now they're probably 700 posts by me. We have 23, 3000 followers. And it's astounding. Some of these posts have 5, 6 million views. So I Spend several hours.
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B
What do you post on? What do you, what videos?
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A
Charcoal, Marie Tooth. They're before and after videos, patient stories. It's all about patience. Anyway, I don't mean to talk too much, but that's.
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B
No, this is perfect.
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A
That's what did it not. My advice to anybody is know, follow your passion. We spend 35 years being somebody else a little bit, don't we? Writing things. High school and college and how to be a perfect medical student, orthopedic resident. It's hard sometimes for our personality to come out what we really love. And it was hard for me, very hard. Orthopedic surgery was not a natural bent for me, let alone engineering issues. And this is what I love. And when you love something, it'll tend to work out for you. You know, when you're working something on a Saturday afternoon or a Sunday afternoon, not getting paid for it and you love it, you know you're in the right place.
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B
That's awesome advice.
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C
Yeah.
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A
Oh, I left you speechless.
- [08:58]
C
Yeah. Doctor, obviously, you know, I want to try to learn as much as I possibly can for you in this time. So could you just briefly go through, let's say a young person like the young woman you mentioned who's in her 20s. Can you just go through your exam? Like I've read some of your articles, you comment about documenting strength of all the tendons, the different tendons. Can you just go through quickly? I know it's a lot of information, but just like what are the main take home messages you're looking for on that first visit?
- [09:25]
A
Oh, okay, first visit. Let's see if I can be really pithy on this without too much editorial comment. For anyone with a chronic disease, impairment becomes the new normal. So they're okay. These people are not depressed CMT patients. They're kind of upbeat, walking along with their crooked feet, maybe in braces, maybe in pain. They're used to it, they're functioning people. But they come to your office for a reason and part of it is explaining to them to get some perspective on their life, helping them. I tell them, talk to your brother, talk to your best friend. Especially if they're adolescents, you know, because again, their impairment has become the new normal. They haven't let it get them down and they live in pain. Two neurologists and others have not supported them. CMT patients are lost in our system. They just not enough of them. I spoke to somebody in our society who does 1200 cases a year of surgeries. It's a lot of cases and I said, how many CMTs do you do? And the person said, oh, I do a lot. I do about six, seven a year, you know, so it's really hard, you know, would anyone in our society recommend that you do complex total ankle cases if you do six a year? Probably not. And I think CMT is much harder than total ankles, especially these days, you know. So the next is the issue of the that their neurologists have told them there's nothing to do. Neurologists are often, forgive me, but the biggest impediment to someone with crooked feet getting good care. One of the great honors in my career was in Richard Lewis, a very well known, you know, muscular neurologist with a great interest in cmt. And he said, glenn, it's been a real honor working with you in the CMT center here. I've really learned a lot. He said, I learned that people shouldn't have crooked feet and that you could help them. He said, we are not taught that really as neurologists. So that's a big issue. And if somebody is interested, any of the listeners in doing cmt, go to your neurologist, reach out to them, be pushy and say, I can help, I can help these patients. Next, the muscle strength issue you asked me about, Joe, is for me, is the muscle strong enough to transfer or not? That's sort of it. You know, a lot of these patients have a flail lower extremity. Well, not a lot, you know, I don't know the percentage. Maybe it's 20% have a flail lower extremity and they're fine. They do well in braces, you know, ground reaction braces, because their foot's flat, it's supple, it's the crooked feet that are the problem. And I do a lot of video chats or consult, they're not really consults, but discussions with patients, you know, around the world actually. And sometimes it's hard to tell on a video call what they're really doing. Just look at their bottom of their foot and when they've got that big callus at the base of the fifth metatarsal, you know, they're not doing well. And remember, these people have some sensory neuropathy, so you know, they're able to pull, push through it. So then the question is this term rigid foot. These are not rigid feet. These are all correctable feet, except in the most severe cases that have end stage ankle arthritis. And that's usually some 40, 50, 60 year old. So these feet we could say are rigid, but it's because of the soft tissues. If you really sit there with them and try to bring it around, you can get a sense of what's causing the contractures. And then an exam. The most important and the hardest parts are one, the Achilles contracture. Well, first of all, why wouldn't somebody with a drop foot or a weak tibialis anterior have an Achilles contracture? I mean, who's the perfect person? It's these patients. And yet when we did our consensus statement, most people didn't feel the Achilles was contracted. I just heard a webinar that the Foot Society put on it was terrific. And Anthony Riccio, I think that's his name, at the University of Texas, he said he disagreed with the consensus statement because he thought most people had an Achilles contractor. And I completely agree with him. But the key to remember is the Achilles inserts slightly medially. So if it's contracted, it brings the heel into varus. And you have to lengthen that almost in every case to get that heel out of varus to stop that varus pull. The peroneus longus is very hard to evaluate. People tell me, oh, it's easy to evaluate that strength. I think that's really tough, you know, to sit there to see if the first metatarsal is pushing down. And the other thing is, in young people, they've learned to compensate. The extensor digitorum longus, right? The long toe extensors, it's masquerading as a tibialis anterior or perineal. You know, I didn't know this. No one ever taught me this. Maybe you guys have thought about it. But the extensor edl, the extensory longest is lateral to the midline. So when their toes are strong, they evert. Some of these kids have tremendous eversion strength, and yet their peroneals are fantastically weak. So you have to pick that up. And also you think their tibialis is strong tibialis anterior, but it's their toe extensors doing it. And then you look down and they've got clawing of their toes. So why take the posterior tibial tendon in someone like that unless it's a significantly contracted deforming force? So I don't want to take the posterior tibial tendon if I don't have to. It's a really important muscle. It's certainly much more important than the toe extensors. So what I'll do in those people is I'll transfer the toe extensors or the hibs. Everyone can look knows how to, you know, has read about that into the cuneiforms. And the other issue, Joe, I guess I'm not going in order is the algorithm. I really try not to fuse patients really ever so well, let's back up. What do you need? You need a plantar grade foot, you need hind foot. That's stability with something giving eversion. You want a foot that goes up and down, ideally out of a brace or maybe a person doesn't want to wear a brace. So how do you do that? But the in terms of the specifics, first I'll do the peroneus longus to brevis transfer to get the eversion strength. If that doesn't exist, I'll transfer the fhl. The flexor hallis is longest and if that doesn't exist in strength, I'll fuse the subtalar joint. That's about the only fusions that I do routinely is a subtalar fusion. And I only do it for stability. I've heard people in the foot society lecture and they go, well yeah, we do a closing wedge of the, of the subtalar joint and you can't correct the hind foot so we fuse the subtalar joint. Hogwash. You can't get the heel out of varus by fusing the subtalar joint. You have to do a calcaneal osteotomy. So it's not uncommon that I'll end up fusing the subtalar joint because there's no other motor and doing a calcane osteotomy. And then Joe, specifically with adolescents, a big problem in the country I think is adolescents are adults. Once you're 13, 14 years old, think of the CMT patient as an adult. Right. A lot of the pediatric orthopedic surgeons, I don't know how many they don't and they're still doing very simple soft tissue releases on 14 year olds. And of course I'm seeing a select group in the office that have failed that. Right. But I wouldn't think as a 13 or 14 year old, as an adult, the scariest patients, the hardest are the 6 and 7 and 8 and 9 year olds. They're a tough bunch because they're in evolution. During adolescence they can get worse. They're often hyper mobile. Right. And so do what you have to do with cmt. You have to do everything to give them a plantar grade foot that's stable. And the problem when you do that is sometimes you could end up with an overcorrection. That's been my biggest Problem, not recurrence, but overcorrection. I'd say 5% of my patients, especially the younger ones, have ended up with a plano valgus foot. You know, one out of 20, it's about right. It happened a lot. Not a lot, but it happened more to me when I was releasing the subtal ligaments. Right. So you don't want to ever do that. First thing you do is you. First thing you do is examine the foot. If it's a gastroc, it's a gastroc. I find it's an Achilles. I do a hook lengthening. Fine. I'll check the ankle for laxity. It's rarely lax. If the ankle is lax, it's usually the calcaneal fibrillar ligament. That's isolated. People don't believe that. Look at it. They don't get anterior laxity. They're not somehow mobile enough. And then all soft tissues will be released and medially will release the posterior tibial tendon. If it's a deforming force, which it often is, except in younger people there it may not be. And if it's not a big deforming force, I have no problem lengthening the posterior tibial 10th in a centimeter or two. If the longus is strong, you want balance. So if I get a posterior tibial tendon that's no longer deforming at a strong longus, I'll do that, and then I'll release the capsule, divide the spring ligament, always in an adult, sometimes in an adolescent, never hardly in a child, and leave the septib ligaments alone. That's when I got. I used to divide them, but that's when I got an overcorrection. I'll stop there.
- [19:04]
C
Just a quick question. I always struggle with these patients that in my mind, you know, the posterior tib is the deforming force, and then I always am nervous about releasing it without transferring meaning. Like if later on they need a posterior tib transfer, let's say their disease progresses. Do you have any tips as to, like, have you ever tagged the posterior tib? My fear is I've weakened it, and then maybe it'll atrophy, so then it won't be a very good dorsiflexor evert. Or if I transfer it to the lateral canary.
- [19:34]
A
It's an excellent point. Okay, just about the post tib. The most important thing I can say in the podcast, other than the issue of the neurologists and, you know, follow your star in life and follow your passion is the posterior Tibial tendon has to be dissected out to its insertion on the cuneiform. There's absolutely no possibility of doing the surgery correctly. 100% time is impossible unless you do that. And that seems to be percolating through a bit in the foot society. But people don't do it. And I think it's just when you really talk to people close up and they'll say, well, you know, often I can't get the post tib into the cuneiform, so I'll put it into the navicular or the talus, or I'll put it into the cuboid. I think it belongs in a central area, which is. I feel the lateral cuneiform. To me, that's the central axis of the foot. Some people think the medial cuneiform. But even if the medial cuneiform is the central axis, I'd like the lateral cuneiform because it gives a little, then eversion, you know, to the hot, what you're trying to correct. But take an osteotome and take a little tiny sliver of the navicular off, just a little. We just tap, tap, and dissect it all the way out to the tibialis anterior. The first thing we do is pick up the tibialis anterior. If there's a strength in it, I don't want to cut it accidentally. And we'll lift up the post tib and then we do the rest of the transfer. Joe, in terms of your specific question, I don't think you could release it. If it retracts, then it's going to atrophy, you know, then it's like an Achilles rupture that's three months old. I don't think much of that, but there's controversy. The people in our consensus statement felt that you could not lengthen it. I always felt you could. But these are rare, rare cases. But they're definitely. When I speak to the pediatric orthopedic docs, they said, we have no problem lengthening it. So Vince Moscow, before he retired, was the smartest person on the planet. Well, he's still the smartest person on the planet about this, but for kids, you know, he wrote the book literally, and he said he'd have no problem, you know, doing that. So you lengthen a little bit of the Z lengthening, leave the plantar, most fibers intact, because those are the ones that go all the way down to the bottom of the foot. And I've seen people that have had lengthening, and the muscle's still good. Sometimes it's very scarred in down there. But why would a muscle that you, you know, lengthen the musculotend unit centimeter and a half, why would that cause any problem long term? I don't think it does, but it's rare cases because you have to say anybody over the age of 25, really, I don't think this is possible because they've gotten, you know, real contracture in that posterior tibial tendon muscle. You know, it started to contract, you really have to probably take it. So I'm really talking about adolescents and young people under the age of 25 where it will do it and I've had some wonderful results in adolescence. You know, I. They're posted on Instagram if you'd like to see.
- [22:31]
C
That's great, that's great.
- [22:32]
A
Anyway. But those are rare cases. I, I wouldn't leave the take home message that you can lengthen the posterior tibial tendon a lot, but I am counteracting the people that made me feel so stupid in the foot society. I was always sitting there listening to CMT talks, feeling so dumb because people would say you always have to transfer the posterior tibial tendon. And I'd be sitting here thinking, really? And people be sitting there saying you have to fuse the subtilary joint to get correction. I thinking, God, you know, anyway, I'm just trying to push back on that a bit on those.
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C
Yeah, yeah. So were you saying just previously you were saying that if they're older than 25, you struggle to do a posterior tip transfer or you are.
- [23:11]
A
No, you can't just do a lengthening. See, I had the CMT for a while is some people get this, you know, not until later in life, but if you've had the CMT for a while with that supination deformity, what happens? Everything gets more contracted, right? The ligaments are more contracted, but the whole posterior tibial muscular tendinous unit, the muscle gets contracted. So you really can't just lengthen it. If the deformity in an adolescent or very a 20 year old is because the peroneus brevis is weak, really, if that's the primary problem and the posterior tib is just over pulling some, then, then you can lengthen the post tib, do tissue releases at the tail and avicular joint, you know, right through the Z lengthening and then give them something laterally in you or me or a 25 year old that you can't do it. But as a matter of fact the problem is getting enough length in the posterior tibial tendon when it contracts. I mean, that's a real problem. But I always try to, you know, always go out to the, always go out to the tibialis anterior insertion. And while we're on the tibialis anterior, I'm just going to mention I'm doing a study on this now in the lab. But sometimes the tibialis anterior is also an inverting, deforming force. So watch out for that. And all I'll do is something simple. I don't believe in split transfers. I think a tendon should do one thing that's controversial. But while you're there, you can divide the tibialis anterior distally and just move it to the dorsum. Just put it right into the. Where the biotenodes go into the dorsum of the medial cuneiform, you know, and then it no longer is an inverting force. These are rare cases, but still it's important to know for someone who wants to do a lot of CMT surgery, that's great. That concludes part one of our two part series on the CMT CAVO virus foot. We want to thank Dr. Glenn Pfeffer for taking time from his busy schedule to join us. Stay tuned for the conclusion of our discussion and thank you for listening to the AOFAS Ortho Podcast. Thank you for listening to the AOFAS Ortho Podcast, a Convey Med production. To learn more about joining our dynamic community of highly skilled orthopedic specialists, visit aofas.org.