Why is hip dysplasia worse in patients with Charcot-Marie-Tooth?

Not only the feet and legs are affected in people who suffer from this disease. Also the hips are affected, producing pain that appears in the 2nd -3rd decade of life. But why is hip dysplasia worse in patients with Charcot-Marie-Tooth? Because the acetabulum is more deformed, shallow and has more anteversion than in a developmental dysplasia. But also hip subluxation is more severe.

 

 

 

 

 

 

 

 

 

Charcot-Marie-Tooth hip displasia (CMTHD) is more prevalent presented with moderate to severe arthritis. Hip pain on the groin, irradiated to the thigh is the most common location. The pain interferes with walking and with many of your activities. The muscles called the gluteus medius and minimus. Being referred to as the rotator cuff of the hip, may be affected by the disease. Producing a kind of limp that is called Trendelenburg gait.

WHAT CRITERIA DO WE USE IN THE TREATMENT OF HIP DYSPLASIA DUE TO CMT?

Among our patients we have almost 7% who have hip dysplasia. This together with the deformity of the feet and legs, makes walking very difficult. The treatment that we apply depends on several parameters. The age of the patient, the degree of subluxation, or the percentage that the head of the femur is covered by the acetabulum. Also if there are already signs of arthritis, which is a worse prognosis to preserve the joint.

HOW DO WE TREAT HIP DYSPLASIA?

In young patients between 10 to 20 years, without signs of osteoarthritis and with a subluxation of the head of the femur. We perform operations that provide coverage, such as the Chiari osteotomy or a triple pelvis osteotomy of the Tönnis. Apparently they seem very complicated surgeries, but they are simpler and give good coverage to the head of the femur. Sometimes they are combined with another cut that we make in the neck of the femur, to correct the rotation and shorten the bone. In this way we get to introduce the head inside the pelvis. In these patients we manage to repair the dysplasia and they can walk without problem.

 

 

 

 

 

 

 

 

 

 

When we do a triple pelvic osteotomy, the pelvis is cut at three levels and we rotate the acetabulum to cover the femur.

HOW DO WE TREAT SUBLUXATED HIPS THAT HAVE ARTHRITIS?

In these cases, we have already arrived late to preserve the hip, since the patient has lost the articular cartilage. The joint has arthritis, which produces severe pain when walking. That’s why the option is to implant a total hip prosthesis. This is the case of this patient with cavus varus feet, knock- kneed (valgus) and arthritis of the right hip. Now her quality of life has improved remarkably, returning to work in the family restaurant.

 

 

 

 

 

 

 

 

Therefore, a surgeon specialized in treating patients with Charcot-Marie-Tooth, should perform the integral treatment of the lower limbs. Not only are the feet, but also the legs and hips.

We continue integrating the treatments of patients with this disease, restoring their ability to walk steadily and painlessly.

Why didn’t my deformed feet by Charcot-Marie-Tooth heal with splints?

You had splints to treat your deformed feet by Charcot-Marie-Tooth. But when you take them off you cannot walk and  fall to the ground. Your neurologist order several electromyogram, blood and genetic test and medication. But none of these studies helps your feet become stable and well aligned again?

Because all cavus varus or drop feet by Charcot-Marie-Tooth will require surgery. PT and exercise can help maintain a certain muscle tone of muscles that are not affected. If there is a poor response your quality of life is poor. This is why you need foot reconstruction surgery, following the principles of modern Neuro-Orthopedics, developed in Germany and Spain.

As I have spoken many times on my blog, the neurologic feet can be treated surgically, by following three principles: give stability, alignment and balance the muscles that act on the foot. In this way we correct this important sequel to the disease and return the ability to walk normally and with normal shoes, to people who suffer from it.

You can read what one of our operated patients wrote to me, after having reconstructed her cavus varus  feet, which is very exciting:

Good morning, I can say that before the operation of my feet I fell twice a day, before I had a lady who did housework once a week, before I had the walk with my splints, always looking down for fear of stumbling my own feet or with a simple stone that I had, before I cycled and fell on the asphalt without receiving any prohibited signal on my legs, before I was afraid to take my nephew in my arms in case he fell with me, before if I wanted to take a coffee needed the help of someone to walk in front of people for fear of making a fool of themselves before they called me the lame and they looked at me with pity before I went out for a walk I was always at home before I could not smile before I had a depression from which I am going out little by little … I tell other people with Charcot-Marie-Tooth what they are going through and I understand it a lot, but the fear is only in the thoughts of the people and it is a barrier that can be overcome. Not only is the operation important, so is the subsequent recovery, doing a lot of sport and everything sent by the doctor and the physiotherapist. With that you will get cured and return to a normal life.

                Why a single surgery is required to correct the cavus varus feet?

It is possible that somebody tell you that some surgeries fail or that it is necessary to operate three or four times the feet to correct them. But this happens when you apply techniques not based on the principles that I wrote before. Our patients need only one surgery to repair their feet

 

 

 

 

 

 

 

 

 

 

We need in the future, to figure out how to the layer of myelin, that is lost, can be repaired in the peripheral nerves. Such as stimulating the Schwann cells to produce it and avoid the muscle paralysis and the feet become deformed. The main function of the myelin, is to increase the speed of transmission of the nerve impulse.

That is why we can not ignore biology because when it is discovered, many of the hand and foot deformities produced by Charcot-Marie-Tooth disease will be prevented.

 

5 years later her cavus feet by Charcot-Marie-Tooth are still corrected

This is what my patients usually ask me if 5 years later their cavus feet by Charcot-Marie-Tooth are still corrected. And the answer is always YES, it is not necessary to perform several operations over time, to obtain stable feet, well aligned and with balanced muscles. These are the advantages of modern Neuro-Orthopedics techniques, which we practice and innovate with new procedures.

Still like this young patient who came with both feet badly operated, with multiple scars. They were never functional after several operations done in a hospital in their city. As I have written before, the problem of success or failure is not to apply a surgical technique or another. If not follow a principles of reconstruction, since applying techniques without proper principles usually leads to failure.

As you can see in the video this young patient came with her older brother who was also affected and to walk they had to support each other. The equine cavuss varuss feet or as they are denominated in German Hohlfuß.

 

 

 

 

 

 

 

 

 

 

 

 

 

In the preoperative x-rays, the previous surgeries had not corrected the bad disposition of the hindfoot bones that remained poorly placed, with the talus superimposed on the calcaneus. This made the feet supinated and even in the left one had a stress fracture of the 2nd metatarsal.

                     Children’s with Charcot-Marie-Tooth should be operated on, to prevent the deformity from progressing

The cavus varus feet are the most common deformity that occurs in children with Charcot-Marie-Tooth, type 1 autosomal recessive and that appears before ten years old. The consequence of not being treated adequately or with techniques that do not follow the principles of reconstruction, is the reappearance of the deformity. It gets worse when you reach puberty, as it increases the weight of the body with growth, resulting in falls and twists of the ankles.

                     How to correct the cavus varus feet by Charcot-Marie-Tooth

It has been more than 5 and a half years since we operated her, removing the arthrodesis that had been done and placing the bones of the hindfoot in the correct position. In addition, the supination of the foot was corrected so that it stepped on the big toe and balanced the muscles of the foot, transferring three muscles to the back, to lift it when walking. Not only, it has not lost anything of the correction that we obtained with the operation, if not that the musculature has increased remarkably. Now she doesn’t have instability, she doesn’t stumble when walking, doesn’t have to lift his legs in a marked way to avoid falling. So, she is able to walk longer distances without getting tired as before.

 

 

 

 

 

 

 

What do these things sound to all of you who suffer from Charcot-Marie-Tooth? because all that is maintained once the feet are corrected, with the principles and techniques that we apply. That’s why it’s not strange to us that our patients operated 5 years later, their cavus varus feet by Charcot-Marie-Tooth are still corrected. This is the success of the modern Neuro-Orthopedics in neurological patients, which does not reproduce the deformity, it is more like the good wines, with the passage of the years they improve the results even more.

“My psychology has changed after having operated my cavus varus feet by Charcot-Marie-Tooth”

This is what many of our patients operated on cavus varus feet by Charcot-Marie-Tooth. When they walk without falling, with their feet stable and stop using the antiquated anti equine splints. And this is very important for patients suffering from this disease, to find the safety of a treatment to return to a normal life. These are one of the most important effects in the psychology of people suffering from Charcot-Marie-Tooth.

Many times we talk about this disease, but it would be good if you knew that there are 7 types of hereditary motor-sensory neuropathy and I am going to put them here:

Type 1: hypertrophy form of Charcot-Marie-Tooth
Type 2: axonal form of Charcot-Marie-Tooth
Type 3: Dejerine-Sottas disease, which is autosomal recessive (this means that two copies of an abnormal gene must be present for the disease to develop)
Type 4: Refsum disease, with accumulation of phytanic acid in body tissues
Type 5: Spastic hereditary paraparesis also called spastic spinal paralysis
Type 6: The combination of optic atrophy and atrophy of the peroneal muscles
Type 7: Pigmentary retinosis associated with distal muscular atrophy

This patient that we have treated would be within the group of Charcot-Marie-Tooth disease but having started their symptoms before the age of 10, it would be the recessive autosomic form of the disease. She lives in the Canary Islands, since that age she started with foot deformity, frequent sprains and falls. The conservative treatments that had given her, had not worked, so her ability to walk each time was less.

                         Ankle fixation is not the best solution for patients with Charcot-Marie-Tooth

The last time she went to a public hospital, she had been proposed to fix both ankles, which she did not like at all. And I am with her and with many other patients who have been recommended an ankle arthrodesis. There are not any of our patients in the IICOP, with Charcot-Marie-Tooth that we have treated her doing an ankle arthrodesis, due to the poor results it gives.

 

 

 

 

 

 

So since she was a little girl, she had a cavus varus feet for Charcot-Marie-Tooth, the left much more deformed than the left. To walk she used a cane or the help of another person, so as not to fall. This had confined her in her house, barely being able to go out on the street, because of the fear that someone would give her a push and fall to the ground.

                              What are the principles for reconstructing feet with Charcot-Marie-Tooth?

For more than a year we have operated on it, reconstructed her feet, especially the left one that, as you can see, was very deformed. Giving them stability, maintaining the mobility of the ankle, aligning them so that they are plantigrade and transferring tendons to the dorsum of the feet, so that they do not deform again. We put three tendons on the back of the foot so that they act to elevate it.

 

 

 

 

 

 

 

 

Now she goes out without fear of falling and her psychology has changed her like all the patients we operate. When they see themselves walking with confidence, the muscles that were not paralyzed, but atrophied by lack of use, begin to work. That is why they experience these changes and return to normal life that many of them had almost forgotten.

Do you want to see how this patient with Charcot-Marie-Tooth going down stairs after operating his feet?

Seven months after his surgery, this patient with Charcot-Marie-Tooth after operating his feet, can going down stairs without problem. Already at 5 months he had regained his ability to walk normally, wearing normal shoes. When I enter Facebook sheets that deal with this disease, it does not cease to amaze me that in the XXI century. There are still patients who boast of the anti-equine splints they wear. There are also many testimonies of patients operated with 3 and 4 surgeries in each foot, que , who frustrated comment on the poor results they have obtained after so many failed surgeries.



Our patient had already had a couple of failed done surgeries, before to come to the IICOP clinic, which in time had not repaired the deformities but that they had worsened. Which made it even more difficult for them to walk in adulthood. He walked with great difficulty, suffered frequent falls from the instability produced by the Charcot-Marie-Tooth and of course he could not go down stairs.

                                                           Children with Charcot-Marie-Tooth also heal their feet with this surgery

These surgeries that had been done to him, such as those mentioned by other people on Facebook, fail because they apply techniques that do not correct the deformities of the feet. Moreover, because they do not follow principles of reconstruction of the neurological feet and there is failure. When they no longer know what to do, they finally fix the ankle, which makes the patient walk even worse.

We at the IICOP have been operating neurological patients for many years and the results support us. Stabilize the feet, align and transfer muscles to balance the functioning of these in the feet or even the legs. In this case that I bring you also had to make a cut in the tibia and fibula to turn these bones, since they were also deformed. The most important thing is that we can correct the deformity with a single surgery and that the foot never deform again.

                                                           Charcot-Marie-Tooth foot & leg surgery causes patients to stop using Dafos

Now you can see the video, going down stairs of this patient with Charcot-Marie-Tooth after operating his feet and correct both tibias, normally without insecurity and with confidence that he is not going to fall. He is happy for his new life, but we are proud to be able to return these patients to have a normal life.

We also operate children with Charcot-Marie-Tooth and poorly aligned legs and flat feet

The German physician Friedrich Schulz, already described in 1884 the cases of children with Charcot-Marie-Tooth and misalignment of the legs and flat feet. They are patients with type 1 of the disease, which manifests itself mainly before and at the onset of puberty. They begin with shortening of the calf muscles and then the body weight, helps the foot to become flat valgus and equine. If we add to this the weakness of the leg muscles and misalignment. The result is an important inability to walk and move.

Thus conservative treatments can be applied initially with orthoses or modified shoes, but the deformity is reproduced so in the case of and misalignment of the legs and flat feet, it is necessary to operate them and to correct their deformities. In this way we manage to improve gait stability and prevent the deformity from progressing with growth and increase in body weight.

How have we operated?

What we have done with this boy, has been to correct in a single surgery the two tibias in varus and external rotation and the two flat flat feet. For the tibias we had to cut to two levels, one close to the knee to correct the varus and another above the ankle to correct the excess external rotation. We then lengthen the calf muscles, correct valgus flat foot by artroerresis with a screw, and transfer tendons to the dorsum of the foot and other minor procedures.

The most important thing is that now, 5 months after the operation, our patient has substantially improved the way he walks. His legs are aligned and the feet already have a normal plantar arch. He has started his training with a personal trainer, to maximize his muscles and improve his physical condition. In addition to performing swimming and stationary cycling on a daily basis.

Thus, the modern Neuro-Orthopedics, applies what we call retro innovation, which is to use good procedures of the past, to combine them with the current ones. In this way we stumble on the invention of new techniques based on the experience of operating many patients with Charcot-Marie-Tooth, in addition to being helped by the current technology.

 

 

 

 

 

 

 

Once patients with Charcot-Marie-Tooth have been operated on, they stop using Dafos¡Tweet it!

There are many teenagers and children with Charcot-Marie-Tooth and poor alignment of the legs and flat feet, who can benefit from these techniques. To prevent their deformities from progressing and further deteriorating their quality of life. For this the doctors who work in the IICOP of Madrid work intensely with our patients.

We have already removed it from the wheelchair to this patient with crouched gait by spina bifida

This young patient with crouched gait by spina bifida or myelomeningocele is the second person who traveled with us to Germany last June. This trip she did once we had surgery on both legs. In them we correct the position of the knees, the rotation of the tibias and the two flat feet values. Her deformities was from birth to having affected the spine at L4 level.


Several surgeries performed in a hospital in southern Spain, not only did not solve the problem but rather aggravated, as the parents said. This is the eternal question, since many institutions can be very good, but it does not mean that the professionals who work in them have a solid training and up-to-date knowledge, to successfully deal with this type of patients.

So when I saw her, she came in a wheelchair, because no one gave her hope that she would walk again. She also dragged a very heavy orthopedic device with a design from the beginning of the last century. Her appearance as you see in the first part of the video was unfortunate. So we had to operate on both legs, with a space of 48 hours between one and the other. We spent a whole morning of these days dedicated to her.

We started at the hip by tenotomy of one of the adductors, we lowered the kneecap to a normal position. To correct the external rotation of the distal tibia, we proceeded with a desrotatory osteotomy of distal tibia and finally we made several cuts of tendons of the foot and transferred the gastrocnemius tendons to the anterior part of the tibia and then continued with the correction of the foot. By means of these procedures we corrected her crouched gait by spina bifida at level L4.

Her evolution was very good, the bones healing properly. But the end of the treatment was to place some orthoses that were up to the height of the surgery that we had done.  That’s why I proposed to the parents to come with us to Germany, to have the orthosis done there. This seems complicated, then turned out to be easier and the benefits soaring. We are all happy about what we have achieved. Where there was no hope and there was only mediocrity, we all put our grain of sand to heal this Ceuta girl from her crouchet gait by spina bifida.

 

 

 

 

 

We can treat the patient’s feet with Charcot-Marie-Tooth and sequels of previous surgery

Of course we can reconstruct the feet of patients with Charcot-Marie-Tooth and sequels of previous surgery. And this I write I do it with absolute humility. Three days ago I saw a web side, a testimony of an operated patient from  America. He wrote with emotion, that he considered the type of surgery that had been done for other patients suffering from the same disease and showed his x-rays with their feet still misaligned . Of course these types of techniques, together with ankle arthrodesis are not the best solution.

Recommending this type of operation, but seeing the Rx that went up, really the foot was not corrected, it continued in varus with the talus and  calcaneus misaligned. Fusion of the 1st toe (Jones’ op.). These patients seem to be better off, but these techniques have proven for years the poor results in the short and long term follow-up.

And I say it because years ago, I did the same, influenced by what I learned in my stay in Seattle and especially by reading books written by American authors. But after seeing the results we had, they really were not good at all. Especially if we compare it with the ones we get in Europe now.

¿What is the difference?

For it is in the principles of treatment and in the techniques that are used in many hospitals under the influence of the English publications.  They really apply techniques, but I believe, without follow a principles and this is what makes them fail. They have nothing to do with the ones we use here in Spain and also in specialized hospitals in Germany.

This occurs in our patients with Charcot-Marie-Tooth and previous sequelae of surgery, such as the case that I bring to you, since they had performed, osteotomies of the calcaneus and 1st, 2nd and 3rd metatarsals. In a vain attempt to correct the alignment of the foot, but useless to give stability when walking, so that the foot became deformed again. They did not reduce the position of the talus on the calcaneus. The same as the patient operated in America. When your doctor warned you that it would have to be re-operated several times. Big mistake!

How have we treated these feet?

By following the basic principles that we have already said other times: to give stability, to correct the alignment and to balance the muscular functioning. This translated in techniques is: a Lambrinudi’s procedure, correct the supination and the varus, to finish transferring tendons to the dorsum of the foot so that it does not fall when walking. The result is that our patient has walked again well, does not stumble and has gained confidence to lead a normal life.

As you see, there is a situation that must change in many orthopedic surgeons, so that they think if the operations that are done in patients with Charcot-Marie-Toot, really are effective. We will continue to reconstruct the feet of patients with Charcot-Marie-Tooth and previous surgery sequels and thus give them hope that this disease that affects the feet, can correct their consequences, without having  more failed surgeries or fix the ankle.

Do you want to see how a patient walks, 5 months after operating his equinus varus feet by Charcot-Marie-Tooth?

The answer is within this post and people who want to read these lines and who suffer from Charcot-Marie-Tooth, can benefit from the innovative treatments that we apply in the IICOP. Someone may doubt these surgeries and their results. But many people can find here the solution to repair equinus varus feet solution to repair equinus varus feet (clubfeet)  with and claw toes. This way the patients can walk without having to use the old-fashioned Dafos. In addition to being able to wear normal shoes and most importantly, do not stumble when walking.

The most important thing is that we can definitely solve the deformities of the feet. You can watch the video before and after surgery with only 5 months of difference. Therefore, we can talk about a well planned and performed surgery, the benefits are very large. By having your feet steady, well aligned and balanced your muscles. It makes patients regain their ability to walk independently and most important, return to a normal life.

Also the consequence of these procedures for repairing equinus varus feet by Charcot-Marie’Tooth is that patients return to physical training. Many of the leg muscles are unaffected, only atrophied, for lack of physical activity. When they get their feet and toes corrected, they are able to go to the gym and with a personal trainer, to recover the physical function. We do it for many patients, start for six weeks with physiotherapists and then with a personal trainer. Getting excellent results!

What is the modern Neuro-Orthopedics?

All these reconstruction procedures, we group them in what is called the modern Neuro-Orthopedics. And it makes orthopedic surgeons specialized in treating Charcot-Marie-Tooth disease, let us be able to repair not only the problems of the feet.

Also, let us have experience, aligning the legs by osteotomy of tibia or femur. It is even cases of hip dysplasia, which occurs in 10-15% of these patients, we can repair the lesion. Preserving the hip using techniques performed by us as the Chiari or triple pelvic osteotomy. In this way we cover the head of the femur, without needing to replace the hip with a prosthesis.

As you can see, at the IICOP in Madrid, Spain, we continue to innovate and carry out a comprehensive treatment of the injuries caused by Charcot-Marie-Tooth disease. I trust that in specialized centers that are dedicated to study these diseases. There are surgeons who begin to know the innovative treatments of orthopedic surgery, to solve the sequelae of this disease.