Section: New Board Review Category: Neurology

Complex Regional Pain Syndrome

Larry Fallat, DPM

Larry Fallat, DPM discusses the clinical presentation, how to diagnose, and how to treat patients with Complex Regional Pain Syndrome.

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Goals and Objectives
  1. Define Complex Regional Pain Syndrome (CRPS)
  2. Review the clinical presentation of CRPS
  3. Examine how to diagnose patients with CRPS
  4. Review treatment options
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  • Author
  • Larry Fallat, DPM

    Director of the Podiatric Surgical Residency
    Beaumont - Wayne Hospital
    Wayne, MI

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  • Lecture Transcript
  • TAPE STARTS – [00:00]


    Speaker: Alright, let me get through this now. I like nuts and bolts topics but if you are going to do surgery, this is such a big part of it and there is so much importance with this condition. What I hope to accomplish is to give you a working definition of CRPS, review the clinical presentation, you need to be aware of this and how tricky that clinical presentation can be, how you would arrive at least if not a diagnosis, a suspicion of CRPS, review some of the treatment options. I am not going to go into the pharmacology, we don't have time for that, but in general to increase your knowledge of CRPS and how we approach it and how we manage that. So as I talk to residents, a lot of residents that I have worked with, we have a good feel. We can see a patient with RSD, we listen to what they are saying, we can tell by looking at the foot. So here is a quiz. Three weeks after CN Bar resection, patient presents with burning pain, 5/10 pain on top of the foot. This thing is pretty uncomfortable. Just based on what little bit you know is this normal, infection, CRPS, venous congestion or tight dressing. Well, this was just a tight dressing and you can see the crease is on the top of the foot and the dressing was putting pressure on the medial dorsal cutaneous nerve. Take the dressing off. She is -- on my god that feels a lot better. But if you look at this, the residents suspected CRPS and that's good that he is suspecting that, you see the mild swelling. You have a burning pain, tingling sensation, but just a dressing, actually it wasn't tight when we put it on but she became swollen and then it started hurting. Here is another one. 12 weeks after ORIF ankle fracture, 8/10 burning pain, narcotics. First thing, I need pain pills.

    [02:00]

    So the red flags go up when you hear that. Normal venous congestion, faker, CRPS, DVT. This actually was CRPS and I think this case too we are going to talk a little bit more about it in a minute. 10 weeks post bunionectomy; pain 6/10, look at the swelling and look at the erythema. So is this normal, infection, venous congestion, CRPS, postop adhesions, stiffness. Yeah, this was pretty typical. She was afraid to use it. The joint was stiffening up. She wasn't walking that much on it. So she needed a very aggressive course of physical therapy to get her through that, but no CRPS with something like this. Nine weeks after flat foot reconstruction; 4/10 pain, weakness and atrophy of the leg, swelling and stiffness of the foot, some tingling sensation, CRPS, venous congestion, DVT, compartmental pressure syndrome. Just venous congestion. You can see some varicosities on his anterior shin, left lower extremity, good course of physical therapy is reversing muscle atrophy. Two weeks following grade 2 sprain right ankle; 4/10 pain, you can see the swelling onto the right ankle. Is this normal, cellulitis, infection or symmetrical CRPS? Now, we know with CRPS you can get a spread of this condition. It can go to the other extremity. So take a look at that. Well, she had a sunburn. Scared a crap out of me when I went in the room, but she is fine. Some lidocaine anesthetic and sunburn cleared up. So my whole point in showing you this is with this little bit of information, you can't really tell based on the clinical appearance of this.

    [04:01]

    So getting back to CRPS, this used to have so many different names and there just was mass confusion. I might talk to somebody on the west coast and we are talking about the same condition, but we are calling it different things and so there was confusion with that. So in 1994, the association of anesthesiologist met in Orlando, Florida and they say we got to do something. Let's standardize the definition. This is what they came up with CRPS. I don't want to run through all of this, but that's a heck of a definition and CRPS 2 is the old causalgia. This is Silas Mitchell. He was in Civil war neurologist and surgeon and he used to find these soldiers after they had amputations sitting by a creek just pouring cool water over their amputation site to reduce that burning pain. Anyway, you have those long definitions and they are in every publication you've, but really what we are talking about here, and CRPS is a complex form of neuropathic pain. It is severe chronic pain characterized by sensory autonomic, motor and dystrophic signs and symptoms. There is a sympathetic alternation. Something is wrong with the sympathetic nervous system, usually due to an injury or surgery, but there are cases where there is no etiology, no precipitating event has even been discovered and there has got to be some degree of limbic system dysfunction. So that's the part of the brain that will scramble or unscramble emotion and so on, but nobody is able to do anything with that. Treatment has to be geared at other levels. But if you take a look what I am after here? The important thing is there is a neurovascular instability and neuro-inflammation.

    [06:02]

    And because of this, I can see a patient today and limb is hot, red, swollen and send them to the pain clinic and three days later they show up and it's fine and they are doing great. That's why tomography never was a good diagnostic tool because it didn't take into consideration the fluctuation of the neurovascular instability that occurred. There is a pathogenesis. I don't mean to blow this off but nobody knows. I make it aware of these different things, but I don't think you are going to see this on any of the boards. Well, this is from Schwartzman. He has written quite a few articles on CRPS. This is an older study, but in this study, motor vehicle accidents 23% of the time resulted in CRPS, falls 14%, contusion and so on. Surgical is on there, of course. When I talk to residents, many times they will yeah, man, it can be RSD because we just took an ingrown toe nail out. It doesn't always have to be a major traumatic injury that results in CRPS. It doesn't have to be a fracture or blunt trauma like this. Degloving injury and laceration or gunshot wound. It can be something as benign as this where we do meticulous dissection to take an aroma out or to correct a bunion. The exception of course is in wars. Soldiers who get shot, especially if there is a nerve damage, have high incidences of CRPS. Yes, that's a little different case than what you and I are going to see on a typical day in our clinics. Previous classifications at least have stages. Doctors like stages.

    [08:00]

    And we used to say, okay, stage 1, I have stage 1 RSD, oh my god, and well, these stages because they fluctuate, this criteria, this classification is fallen by the side now. And so if you take a look, this was the stage 1. The limb is dry. It's red. It looks like it got a little bit of sunburn there. Stage 2, the dystrophic stage. Had hyperhidrosis and you can see the moisture and sweat would just drop right off of the foot. This is stage 3 and this is bad news. This is a cold, withered limb. It's [indecipherable] [08:31] dead and this is the one you don't want to operate on except who did operate on that. I did. I took the painful screw out of her medial malleolus, but sometimes they are just and so much, it's --the limb is so far gone. You try and do something to help remove that one area of focal pain that's just killing him. So the problem with the stage is it implied sequential progression and we used to say stage 1, I hope it doesn't go on to stage 2 and stage 2 is here. So this system was not real good. Now, we look at the symptoms and this is what the pain doctors want to hear. Let's take a look at them. The vasomotor dysfunction changes that we see, the skin temperature, the skin color and whether it's symmetrical or asymmetrical and to do this, you have got to have both socks and shoes off. And take a look at this. This is a great little picture because it shows you the shiny appearance of the limb that's affected with CRPS. Everybody should have one of these. You just document the temperature very easy way rather than just palpating or feeling the skin on top of the foot. Then we look at swelling and sweating. I have shown you this picture already. Here is another one. This was on an older female who had hammertoe and bunion surgery and take a look at the hyperhidrosis and the shiny appearance to the CRPS limb on the right.

    [10:01]

    We have motor dysfunction. We get tremors and this is a good example of tremors that affect the hand in 1987. You see this woman's handwriting and then after getting CRPS, look at the handwriting. We see the same tremors in the foot as well and we see the weakness and decreased range of motion that can have a flexor contracture in the foot. This is more typical. This is a spastic contracture. It's there 100% of the time. The tibialis anterior muscle and you can see it adheres and permanent spasm. This is dystonia and that is a tough condition to treat. I actually operated on that guy and eventually I was giving him Botox injections to release the spasm and that would work for a while and then always come back. Look at these trophic changes though. Look at the hair growth pattern. I hope you guys can see it from where you are at. The CRPS limb is on the right and see how there is decreased hair growth on the foot and take a look at this. The CRPS limb is on the right and look at the coarse hair that has grown. So many times these are late findings, but I point this out to you. So you might look at these things. You might see them and it may help you with a diagnosis. This is the dermatitis that's very typical with CRPS patients. When you see this, don't think of contact dermatitis, don't think of tinea pedis. This is CRPS. And look at this excessive dryness. When sympathectomies were invoked, the patients would get dryness like this, but sometimes trophic changes can result in dryness like that. Look at the nails on the right. Look at the ridging on the left. Great toe has onychomycosis on the right. That ridging is characteristic of the trophic changes we see with CRPS. And then of course, the allodynia and to me, this is a huge one and that's where the patients report pain with stimulus that should not hurt them.

    [12:05]

    So if you touch them lightly that will start to pull their foot away. Many times they can't put a sock on because it hurts and lot of times the nails are extremely thick and long because it hurts too bad to have them trimmed. This is the type of pain that people have virtually the full spectrum of symptoms that you can ever remember. These fluctuate. On Monday, they have can burning sharp pain and they are staying away from you and then in coming two weeks later, yeah, it feels okay, little sore. That's the fluctuation. The characteristics, these are like little tips and pearls with CRPS. The entire limb tends to be painful, but it may be over showered by a focal area of pain and that focal area of pain may not be where the original surgery or injury occurred. We call this a spike type of pain and it might follow a peripheral nerve but it may not. It could be in a different location. So you might have a patient who has a fifth metatarsal base fracture but developed spike pain in the area of the medial dorsal cutaneous nerve or in his heel. The pain is out of proportion and that's what throws us all off. Because we are convinced when they tell us, oh, it's killing me that they want drugs. They want narcotics. They don't want to return to work. So that's always a problem for us. Think in terms of the patient having pain when they shouldn't have and discomfort. So if they sprain an ankle and five, six, seven, eight weeks out, the pain is 9/10 and there is something else going on there. So initially, symptoms can be vague and that is why early diagnosis is so tricky because many patients will say, yes, it hurts but it will be okay and they blow you off and that will throw you off too. The symptoms may not be obvious. They can change with each visit, may not involve the entire limb and that's something else that can throw you off.

    [14:00]

    Spike pain and peripheral nerve pain may delay the suspicion of CRPS and do they want time -- well, they can't work because it's too bad, but is this legitimate or they faking it? How do we arrive not a diagnosis so much because in court of law, they are going to say we can't make a diagnosis but the anesthesiologist or neurologist can, but how do we arrive at an index of suspicion of CRPS? Well, an accurate history of course and the time from the injury or surgery to presentation in your office and by the way as a rule of thumb, ballpark, it takes about six weeks for CRPS symptoms to start developing. So sometimes, I will get a call from a resident. The patient has come in to the ER four days out from surgery or injury and the resident suspects CRPS and probably not. May take longer than that and I have seen take as long as six, seven, eight months to show up. Pain is out of proportion. Look at the subjective and objective symptoms. You know what to look for now. And supplemental diagnostic studies, so taking a look at something like this. Does this look normal for the time period that you are examining the patient? Okay, so let's say you got a little suspicion something is going on. So we can run some lab test, find out what's going on. No way, Jose, there isn't a lab test that you can use that's going to help with the diagnosis of CRPS. And over the years periodically, new study will come out and say, well, IgE was up a little bit. Maybe that's it or this and that but no, there is really nothing that you can do. Initially, it's a clinical diagnosis, but there are few things we can do to try and narrow it down. There are lot of questionnaires studies too that the pain clinics will do that sometimes can be helpful.

    [16:01]

    Look at the imaging though and this is one thing you can do. And this is a polar type of distribution of spotty osteoporosis, very characteristic of sympathetic involvement and this is [indecipherable] [16:14]. This is an old one from '75, but he did such a great job of it that I like it and I keep using this picture. Take a look at these. Look at that washed out appearance on the lateral view. You look at that and you say holy cow, that's full blown RSD. That patient had sprained an ankle or something. By the way, that's not RSD. That may represent an exaggerated sympathetic response but she had no pain. Once the fracture was healed, some physical therapy and off she went. Look at the AP view. This was an OCD repair with metaphyseal bone grafting. That is RSD. So radiographically, you can't tell. Nobody in the world can tell. Severe disuse osteoporosis, radiographically is identical to the changes we see on x-rays of patients with CRPS and certainly, there is got to be a degree of sympathetic involvement with both conditions. Bone scans can be a little bit more specific and this is just a beautiful triphasic bone scan. Take a look. This was the fifth metatarsal base fracture. Look at the generalized uptake in the mid foot and the ankle joint. And look at the periarticular uptake at the metatarsophalangeal joint. This is indicative of CRPS. This is what you look for. Not always this easy because most of the time it's just non-conclusive. These people also get painful range of motion. They will come in and tell you, oh man, I can't walk. I don't want to bend my foot or ankle and here is the reason. Because of alteration of sympathetic nervous system, there is increased activity of the fiberblast. They start multiplying. The joint capsules in the fascia becomes very thick, stiff and painful and if this happens, it can become permanent and there is nothing that can reverse that.

    [18:09]

    So the minute you see this, they go to physical therapy. No matter what else may be going on, I need to get a therapist started to rehabilitate them and minimize that stiff pain that they are going to get. Pain mapping is another thing you can do. Allodynia is an issue. So these people have RSD, but I have outlined the area of greatest pain. You see the axis. These are like trigger points or spike points of pain. This can throw you off sometimes though it can make you think that this is a peripheral neuritis, but it's not. This is RSD. Take a look at the patient where I have the diagonal lines. That's not RSD. That neuritis because with RSD I can't make that many lines on it without getting beat up by her. Diagnostic nerve blocks can be very helpful. They can be diagnostic but they can be therapeutic as well. I have not had flareup of CRPS giving diagnostic block. Doesn't always work but it is one option that you have to try and reduce the pain and help you decide if this is CRPS or peripheral neuritis. Early diagnosis and early referral to a pain clinic is mandatory. You have to do this. Years ago if our patients got CRPS, we [indecipherable] [19:29] for it and as podiatrist and doctors and surgeons became more aware of this condition and we are starting to make referrals to the pain clinic, then attorneys jumped on and said, well, you didn't refer him early enough. Just be aware that's why I have given this talk. This is so important because if they get full blown CRPS, they aren't going to work again and somebody is going to have to pay for their disability. So legally it's not just anymore referral to the pain specialist, it's early diagnosis.

    [20:01]

    Here is some tips that I have come across. Lot of times when they are starting to get CRPS, the first symptoms will be stiffness of the ankle, metatarsophalangeal joint or the heel and when it's the heel, it mimics plantar fasciitis. And they will say to me, you know, my broken ankle is feeling better now, but boy, just the bottom of the heel would get better and so we get them to physical therapy as quickly as possible. Location of pain may not correlate with the injury site. Musculoskeletal pain, sprain ankle, feels better when you get off of it. With RSD, they tend to have pain when they are off of it and that is a big diagnostic tool. Have to examine both feet at the same time. If you suspect CRPS, this is our battle plan. In my area, this is what we do, immediate referral to physical therapy. And it has got to be good therapist. If the therapist is a great big muscle guy, I am glad I’d done it. I am going to get you rehabilitated, patient can't go there. It's got to be a therapist who understands CRPS and it's more of a gentle range of motion, stretching and massage. Remember trauma causes CRPS and so if you've therapist, it's going to crank and dislocate and get them in the headlock that could probably make it worse. Triphasic bone scan, a lot of the pain doctors in our area want this scan. So we order that so that when they see the pain specialist they have it all there and then refer them to the pain clinic. I am going to just pass by these to save us a little bit of time here and this is a sympathetic block. This is the most important thing they can have done. This is not only diagnostic but therapeutic. They may require a series of these and initially this may only last for a few hours but that is the one treatment that can actually cure this condition.

    [22:01]

    If it doesn't for whatever the reason, they got into late or the blocks weren't that accurate, then of course peripheral nerve stimulation, dorsal column stimulators can be utilized. There are many modalities out there that can help manage subdue the pain of CRPS. Quickly, I will take a look at two case reports here. A 51-year-old female, bimalleolar ankle fracture, ORIF and so we operate and give her a nice operation. She is immobilized. Pain 2-3/10. 10 weeks postop, her pain has increased. Edema, erythema, limited painful range of motion, ankle and subtalar joint. She requests narcotics and she was the last person that would ever request narcotics. She is in physical therapy. This is 12 weeks out and we have mapped her and that's the area of greatest pain. Although her whole limb was uncomfortable and you can see where the allodynia. So it's much more distinct. A bone scan, okay, it's showing increased uptake, I don't know if that ever help me too much because she broke her ankle and that's going to light up for couple of years. Look at these sequential x-rays. On 05/30, you see the quality of bone. On 07/01, starting to become osteoporotic. On 08/26, more osteoporotic. She was referred to pain clinic. They gave her sympathetic block and diagnosed her as having CRPS. She continued in physical therapy, given oral medications. We gave her peripheral nerve blocks in that area where pain was the greatest. Five months postoperatively and with treatment at the pain clinic, pain was at 2 out of 10 and she had returned to full activity. One year postop, pain 1 out 10 and then look at in August, you see the osteoporosis in her tibia and then by October you see it's starting to reverse as the sympathetic excitability has reduced.

    [24:03]

    This lady, I feel kind of bad for her. She sprained her ankle and she had two months of conservative care by another doctor. She saw us in March 2013. Pain is 8/10 and pain was right at the ankle, no place else. MRI indicated ATFL and CFL. ATFL I think was blown out. CFL attenuated. Referred her to PM&R. We got his opinion on her. We treated with CAM boot and PT. No improvement. Severe pain. No suggestion of CRPS. Surgery on 09/20/13, three weeks postop she came in. We tried to take the stitches out. She is crying and can't even talk. She is crying, sobbing. At five weeks, pain 7/10. Can't wear the Swedo brace, tingling and numbness in the toes in November, but every visit she is crying, she is sobbing. She can't talk and she is refusing our peripheral blocks and she had been sent to a pain specialist but refused the sympathetic blocks. Gave her gabapentin, referred her to psychiatrist. On 02/11, lidoderm patches seemed to help her. On 04/14, did have sympathetic block, eliminated the pain she had at rest. We saw her just a couple of weeks ago. Pain is 6/10 and walks with a cane. I think this is going to be a permanent disability. You see a TENS unit, which she thinks helps her, but I don't think she is ever going to go back to work again. So this is a pretty benign procedure. Repair of ankle ligaments and she gets CRPS. I hope she gets disability because if she doesn't, she tends to want to come after anybody, anyone else. So in summary, pain out of proportion. Look at the timeframe for the pain. Look at vasomotor, pseudomotor, trophic changes.

    [26:02]

    These things should give you an index of suspicion and then you have to refer them out to a pain clinic. We have already talked about disability and legal issues. So anyway that's the information I have you on CRPS. I hope one of you ever see this on your patients, but if you do, maybe you can at least recognize it and jump on it.


    TAPE ENDS - [26:25]