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Surgery/Fractures/ Falls Questions

  1. Why is it that non-union fractures so common in CMT patients? Do you feel that the peripheral nerves are necessary for healing?
  2. A 29 year old CMT patient had surgery on the ball of his foot. The incision failed to heal and he is receiving intravenous antibiotics several times a day. Do CMT patients have healing problems?
  3. I am 43 years old and having pain in the knee. The doctor said my CMT is pulling my kneecap to the side, causing the pain. What can be done for this?
  4. I've seen reference to the potential need for foot/ankle surgery in some cases of CMT. Is the surgical procedure specialized? Are there specific indicators or contraindicators for this surgery?
  5. Recently, I was standing in my den when my right knee suddenly collapsed. It happened so quickly that I'm not sure if I pitched forward over my foot and then fell backwards, or if I immediately fell backwards. Whichever way it occurred, I severely sprained the top of my right foot. I was wearing my AFO's at the time. On the assumption that the buckling of my knee was associated with my CMT, I wonder if any of the doctors on your panel have seen this before and have any advice about strengthening certain muscles or any other advice about avoiding that kind of fall.
  6. I am an orthopaedic surgeon in Peterborough, Ontario, Canada. Recently a 13 year old girl presented to me with previously diagnosed CMT (I don't know which type) and a second episode of patellar dislocation. While I'm aware of lower extremity weakness in CMT patients, I can't find any info on treatment of recurrent patellar dislocation in CMT patients. Are you aware of any publications on this problem?
  7. I have CMT and am 53. I have had 11 operations on my feet, including the last which fused my ankles. In the last 8 months, I've started having problems around my knee. When I walk about 10-12 minutes, or stand still for that long, my left leg becomes basically paralyzed. Around the knee region, I lose the use of the leg. Could it be the CMT spreading above the knee? If it is, can you tell me what I can do?
  8. Two orthopaedic surgeons both suggest surgery for my daughter who was diagnosed with CMT 1B. She has had this problem most of her life and has worn AFO's for the past three years. According to the doctors, the type of surgery should depend on the type of CMT and now, one says it is crucial to have a plantar fascia release done before she stops growing and the cartilage hardens and her bones become deformed. She has a severe arch in both feet, claw toes and without her AFO's, she walks with great difficulty and turns her ankles. Our concern is the "hurry" to do this operation, the pain, a lack of bettering the situation and the need for later surgeries for her toes. In short, we are looking for the best solution to the problem.
  9. Hip replacement surgery is often a boon to those who suffer from arthritic joints and there is no other way to get relief. The operation, in itself, in the hands of a skilled surgeon, usually goes smoothly. The complication arises when the surgery is performed on people with CMT who have a tendency to fall when walking unaided or when using a cane. Orthotics help but are no protection against falling. I had my second hip replacement and am experiencing many more falls than I did with my first one. The obvious answer is to use a walker, which is much safer and more secure, but my objective is to get back to normal with no cane or occasional use of the cane. If I take the suggestion of my surgeon, it is to get back on the cane as quickly as possible to preserve my prior-to-surgery level of mobility. My therapist, concerned about my falling, wants me to spend more time on the walker. How would you solve this problem?
  10. My 11-year-old daughter had a visit with her orthopaedic surgeon a few weeks ago and he mentioned surgery to fuse the bones on the top of her foot. Can you tell me what you might know about this surgery and how successful it is with CMT patients. I have a very active 11-year-old who, in the last year, has fallen many times and has had sprains and fractures. This happens often and I would like to know if this surgery would help her.
  11. The physicians in my area don’t seem to be very familiar with CMT. I am concerned that the deformities of my feet are just getting worse and that eventually I won’t be able to walk. What surgeries are typically done to fix the foot deformities common in CMT?
  12. I am a 63-year-old patient with CMT. I underwent foot surgeries at ages 9 and 10. I’ve had several foot surgeries in adulthood, as well. Last week, I had surgery for lengthening of my right Achilles tendon. At present, I have dressings and splints. I am scheduled to have a cast put on next week. Is there anything that can be done to lessen the atrophying which occurs with casts? Or, are there other devices to use while healing? Also, whenever my legs have been in casts, I’ve experienced severe pain in the affected leg. I’ve heard that other people also have such pain. (It might be called ventriculation.) Can anything be done to reduce or prevent this? I am able to tolerate only low dose and infrequent pain medications.
  13. Are people with CMT prone to reactions from anesthesia when having surgery?
  14. I’m having surgery and will be given Versed. I read somewhere about being careful with Versed. I have CMT 1A and am wondering if that medication will be okay.

Surgery/Fractures/Falls Answers

1. Why is it that non-union fractures so common in CMT patients? Do you feel that the peripheral nerves are necessary for healing?

Certain kinds of fractures are more common in people with neuropathy, including inherited neuropathy. Possible reasons include decreased bone density, greater risk of falling, and decreased sensation. Nerves are probably not necessary for healing in a strict sense, but activity (which requires movement and therefore, nerves) is required.

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2. A 29 year old CMT patient had surgery on the ball of his foot. The incision failed to heal and he is receiving intravenous antibiotics several times a day. Do CMT patients have healing problems?

The circulation problems in CMT and decreased sensory loss could slightly affect healing

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3. I am 43 years old and having pain in the knee. The doctor said my CMT is pulling my kneecap to the side, causing the pain. What can be done for this?

Generally, unless the CMT starts in childhood, the quadriceps, the muscle that pulls the kneecap to the side is usually not affected. However, there may be deformity in the knee caused by arthritis or other disease of the knee joint, even unrelated to CMT disease. That would cause pain. Then, there is reason for the quadriceps to pull the kneecap to the side and the treatment for that is to take care of the underlying problem. It would be important to know if this person was male/female (females may have a tendency to have this problem more frequently), height/weight and whether it is one side or both. Also, knowledge of the muscle grading (how strong the muscles are) in both lower extremity, including knee, ankle and foot would be useful.

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4. I've seen reference to the potential need for foot/ankle surgery in some cases of CMT. Is the surgical procedure specialized? Are there specific indicators or contraindicators for this surgery?

Surgery should only be done if there is a need and a benefit. The outcome should be a frank discussion between the patient and the surgeon. Before surgery is done, there is an "informed consent" that a surgeon gives to the patient and all questions need to be answered so that the patient would know what exactly is going to be done, the expected results, the problems, complications, difficulties and benefits. Alternative measures need to be discussed. This is universal practice in the USA.

Medical doctors do not consider operating on CMT patients to be routine surgery for any orthopaedic surgeon. There are some orthopaedic surgeons who have had special training in foot and ankle surgery via fellowships and who specialize and possibly even limit practice to foot and ankle surgery in most major U.S. cities. Because of the variability and the ongoing weakness, each case has to be assessed individually and surgery planned carefully.

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5. Recently, I was standing in my den when my right knee suddenly collapsed. It happened so quickly that I'm not sure if I pitched forward over my foot and then fell backwards, or if I immediately fell backwards. Whichever way it occurred, I severely sprained the top of my right foot. I was wearing my AFO's at the time. On the assumption that the buckling of my knee was associated with my CMT, I wonder if any of the doctors on your panel have seen this before and have any advice about strengthening certain muscles or any other advice about avoiding that kind of fall.

It is difficult to give an exact answer without examining the individual and accessing the entire limb. However, I will try to answer the question with the information given. Experiencing the knee giving out in a backward direction and hyper-extending the foot and ankle is not an uncommon occurrence in people with Charcot-Marie-Tooth Disease. Due to poor proprioception (the ability to feel position) and atrophy of leg muscles, it is difficult for all CMT individuals to maintain good balance. The calf muscle (behind the lower leg) crosses and helps stabilize both the knee and ankle. Because of weakness in this muscle due to CMT, the knee can hyperextend (recuvatum) and cause instability and balance loss. This can cause the individual to fall backwards, thus hyperextending the foot and ankle.

Strengthening exercises usually offer little value due to the muscles' inability to function from CMT. A knee brace can help, but they are bulky and cumbersome to wear especially if one is already wearing AFO bracing. There is no specific treatment for this problem other than being aware that it is present and taking precautions to avoid being injured in the fall. Continued use of a crutch for extra stability would also be recommended.

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6. I am an orthopaedic surgeon in Peterborough, Ontario, Canada. Recently a 13 year old girl presented to me with previously diagnosed CMT (I don't know which type) and a second episode of patellar dislocation. While I'm aware of lower extremity weakness in CMT patients, I can't find any info on treatment of recurrent patellar dislocation in CMT patients. Are you aware of any publications on this problem?

Without any further details and assuming that this child does not have congenital anomalies or growth development problems, the dislocation of the patella (which is generally laterally) to the outside is usually not related to CMT disease, but does happen quite frequently in girls, because of the positioning of the patella (kneecap) in its "groove" or "track" as it extends the knee.

Girls, especially those who develop early, may be obese have kneecaps that "track" towards the outside and with a bit of trauma or with a "trick" movement, can dislocate it. Once that happens, the dislocation can occur more frequently. A pediatric orthopaedic surgeon should easily recognize this problem and if a "brace" does not work, it may need surgical repair.

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7. I have CMT and am 53. I have had 11 operations on my feet, including the last which fused my ankles. In the last 8 months, I've started having problems around my knee. When I walk about 10-12 minutes, or stand still for that long, my left leg becomes basically paralyzed. Around the knee region, I lose the use of the leg. Could it be the CMT spreading above the knee? If it is, can you tell me what I can do?

I would strongly suggest that he see his neurologist, preferably the person who diagnosed him with CMT. He should get an updated neurological exam and a manual muscle test of his leg strengths to see whether there is muscle weakness or not.

If this does not produce a satisfactory explanation, then it would be important to re-assess the operations that he has had. It seems that there are a lot and we, as orthopaedists, seldom see or would recommend fusion of both ankles, unless they were involved in severe accidents, had severe degenerative changes or had congenital malformations. The position of the ankle (which by definition, after fusion, is stiff) would put additional stress on the knee joint and depending on the attitude of the heel and foot (something that runners frequently refer to) could cause knee problems. Also, his height, weight, amount of activity such as work and recreation are also factors to consider.

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8. Two orthopaedic surgeons both suggest surgery for my daughter who was diagnosed with CMT 1B. She has had this problem most of her life and has worn AFO's for the past three years. According to the doctors, the type of surgery should depend on the type of CMT and now, one says it is crucial to have a plantar fascia release done before she stops growing and the cartilage hardens and her bones become deformed. She has a severe arch in both feet, claw toes and without her AFO's, she walks with great difficulty and turns her ankles. Our concern is the "hurry" to do this operation, the pain, a lack of bettering the situation and the need for later surgeries for her toes. In short, we are looking for the best solution to the problem.

There is definitely a "time-frame" in which foot surgery may be more effective in the growing child. The best time is before the foot bones stop growing and then, the deformity becomes "fixed" and the shape of the bones will change, governed by the deforming forces on the foot.

The surgery that may be most effective for a child who has ankles that turn in because of the imbalance is not necessarily a plantar fasciotomy. The strength of the muscles that turn the ankles in and out needs to be known, and, if that is causing the imbalance, then, the tendons may need to be moved to a different location so that it will pull in a more natural direction. Obviously, if the foot has a very high arch, then plantar fasciotomy may by helpful, but as a secondary procedure.

Just flattening an arch (which is actually not an easy procedure, no matter which way it is done and which should affect other tissues as little as possible) without knowing what causes it to be "drawn up" or high, does not solve the problem.

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9. Hip replacement surgery is often a boon to those who suffer from arthritic joints and there is no other way to get relief. The operation, in itself, in the hands of a skilled surgeon, usually goes smoothly. The complication arises when the surgery is performed on people with CMT who have a tendency to fall when walking unaided or when using a cane. Orthotics help but are no protection against falling. I had my second hip replacement and am experiencing many more falls than I did with my first one. The obvious answer is to use a walker, which is much safer and more secure, but my objective is to get back to normal with no cane or occasional use of the cane. If I take the suggestion of my surgeon, it is to get back on the cane as quickly as possible to preserve my prior-to-surgery level of mobility. My therapist, concerned about my falling, wants me to spend more time on the walker. How would you solve this problem?

It would be my understanding that the surgeon has probably done his/her job and the joint replacement is stable and pain-free. It is now up to the individual to improve his function. Falling is not good and when uncontrolled, it could lead to the dislocation of the hip and a major complication.

Age, balance, shakiness, memory, patience, shortness of breath, shoes, the ground they walk on and the obstructions are all important factors. The therapist should do a manual muscle test and determine just where the weakness is. Also, a functional evaluation of the person's surroundings--home, yard, furniture placements, exercise, and so on--is important. A therapist who has an interest in rehabilitation would be able to use this information and design a program to accomplish a reasonable, doable, and mutually agreed upon goal. In this case, I believe it is to prevent falling and to go back to walking some distance without aids.

Knowledge of where the weakness is in an elderly, active, ambulatory person with long-standing manifestations of CMT, could allow the therapist to develop a strengthening program for these muscles. For instance, the abdominals, the hip extensors, hip abductors and quads and, together with lightweight AFOs, allow a person to balance better and walk with minimal aids.

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10. My 11-year-old daughter had a visit with her orthopaedic surgeon a few weeks ago and he mentioned surgery to fuse the bones on the top of her foot. Can you tell me what you might know about this surgery and how successful it is with CMT patients. I have a very active 11-year-old who, in the last year, has fallen many times and has had sprains and fractures. This happens often and I would like to know if this surgery would help her.

Eleven-year-olds should be and are expected to be very active. They have their activities, play sports, dance, etc. However, they don't get a lot of sprains and fractures of the foot. Are the sprains in the ankle? If they are sprains, what has been done about it besides having the orthopaedist suggest surgery?

If the problem is due to clumsiness because of a high arch and turning in of the foot and ankle, then something needs to be done. A manual muscle test to determine the strength of the posterior tibial, anterior tibial and peroneal muscles would be useful. The foot may need to be balanced. If the sprain is at the ankle, then high top shoes and their support would be helpful and would not require fusing the bones on the top of the foot. If the problem is otherwise (including being extremely overweight or heavy), I would need to know specifically which bone is fractured. The fracture or underlying problem itself may need to be addressed.

In any case, the foot of an 11-year-old is still under development (growing). Doing surgery can interfere with the growth and cause secondary deformities, shortening, and scarring and not solve the problem. The orthopaedic surgeon should be a pediatric orthopaedic surgeon or someone very experienced in the care of deformities of the child's foot, not only fractures.

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11. The physicians in my area don’t seem to be very familiar with CMT. I am concerned that the deformities of my feet are just getting worse and that eventually I won’t be able to walk. What surgeries are typically done to fix the foot deformities common in CMT?

There are many surgical procedures available for people with CMT, but one that is quite effective is reconstruction of the foot and ankle. Options include the straightening of hammered toes, the repositioning of an in-turned heel and the lowering of the arch. The end result is a foot that is stable and is able to stand flat on the ground. As well, tendon transfers on the lateral side of the foot and ankle can provide increased strength and a more normal gait.

You don’t say whether you wear orthotic devices (ankle-foot orthoses) to help with stability and balance, but that is always the first option that should be considered before surgery is undertaken.

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12. I am a 63-year-old patient with CMT. I underwent foot surgeries at ages 9 and 10. I’ve had several foot surgeries in adulthood, as well. Last week, I had surgery for lengthening of my right Achilles tendon. At present, I have dressings and splints. I am scheduled to have a cast put on next week. Is there anything that can be done to lessen the atrophying which occurs with casts? Or, are there other devices to use while healing? Also, whenever my legs have been in casts, I’ve experienced severe pain in the affected leg. I’ve heard that other people also have such pain. (It might be called ventriculation.) Can anything be done to reduce or prevent this? I am able to tolerate only low dose and infrequent pain medications.

Given your situation and being post-operative, thus, part of a planned event, I don’t think there is anything that can be done to lessen the atrophying which occurs while in casts. I am sure that you are aware that after the lengthening surgery, the tendon needs to heal. Depending on whether the lengthening was done percutaneously or by open technique, there is a certain amount of time that is required for healing. Thus, after the dressings and splints used postoperatively, the cast is needed to keep the ankle immobilized so that the tendon, which may or may not have been repaired with sutures, can heal and become whole again.

I am not familiar with the term “ventriculation”, but in all cases, pain is a very individual and personal matter. The use of medication for pain, its tolerance, especially when prescribed post-operatively, needs to be discussed between the patient and his/her physician. The prescription, its quantity, strength, and period between dosages needs to be carefully adjusted and monitored via patient feedback.

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13. Are people with CMT prone to reactions from anesthesia when having surgery?

Most people with CMT will have no problem with anesthesia. Only those with respiratory involvement (very rare) or vocal cord paralysis (a very rare variant) may have problems with anesthesia above that seen in the general population.

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14. I’m having surgery and will be given Versed. I read somewhere about being careful with Versed. I have CMT 1A and am wondering if that medication will be okay.

I know of no special concerns with Versed and CMT unless it is used for an extended period of time unlike the brief exposure for anesthesia before surgery.

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