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Complex Regional Pain Syndrome (CRPS) Type 1: Reflex Sympathetic Dystrophy

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This is an 11-year-old with a painful

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foot after a fall six months earlier,

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and the patient has diffuse pain,

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medial, lateral, extending into the heel.

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And I am showing the case like

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I would review it by myself.

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Sagittal T1, fat-weighted, in the middle,

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all-purpose, proton density, fat suppression.

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Spare, spur, special, fat-sat.

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The quality of the fat saturation is

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very good, but not great, because the

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bones aren't black, but they're pretty

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dark, and the water is pretty bright.

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On the far right is an additive gradient

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echo, an excellent sequence to look at

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cartilage, even fissial cartilage, very good

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for articular cartilage and intracapsular

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tissues, but not very good for medullary bone.

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So, it's up here as a reference, because we

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don't know the answer yet, But it's always

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nice to scroll and look at the sagittals

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together unless you need a reference in

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another plane, which sometimes you do.

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But this is how I would do it alone.

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And scrolling, it's obvious that there is

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some swelling within the medullary space.

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So that's really what the case is about.

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We know that there's been a trauma.

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The growth plates are open.

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The child is 11 years old.

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And I'm looking for the most heinous things first.

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For instance, I'm looking at the growth plates.

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I want to know if there was a missed Salter

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Harris fracture and the child's been walking

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around on a Salter Harris 1 through whatever.

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He's not.

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I want to see if there has been

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a missed or a colt fracture.

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And what am I looking for?

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I'm looking for a line.

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You might say, well, it's been quite a while.

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Will there still be a line?

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You bet there will.

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Lines or scars from fractures may persist for

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a year, and sometimes they may never go away.

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You may be able to see

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the remnants of a fracture decades later.

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So, six months is not enough time for

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the line of a true macro fracture

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that has been missed to resolve.

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If it was a low-grade bone injury, well,

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okay, maybe it resolved, but why does the

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child still have pain six months later?

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So that would not make sense.

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You gotta synthesize the case.

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So most of you are honing in on these

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little spots and blots and dots.

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Which are signs of a high bone

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turnover scenario in the child as

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they run around and do what they do.

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It is not uncommon for them to

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complain of some low-grade discomfort.

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Occasionally, they will coalesce into an

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actual stress injury in one specific locus.

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But most of the time, in children, you'll

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see a little bit of an effusion, a little

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bit of an overuse syndrome-type pattern.

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Of spotty osteoedema with no coalescence.

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It'll be scattered and random.

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It'll be mostly towards the center of

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the bone, and you will not see any lines.

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If you see some lines, then you may have to

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get a little more aggressive in terms of your

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descriptors for overuse fatigue syndromes.

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But that's not the case here.

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Yes, we do have some of these spotty, dotted

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areas of osteoedema, but we also have a child.

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That's six months out, still has pain, and

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we don't have an explanation, other than

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we have edema in bones that is not spotty.

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It's not dotty.

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It's not punctate.

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It is rather peripheral in the navicular.

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It is rather peripheral in the cuneiform.

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It is even peripheral or

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subcortical in the cuboid.

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And if you look very carefully,

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it's even peripheral in the talus.

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There are not too many disorders that will have

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this peripheral pattern of subcortical edema.

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One would be a reaction to a joint inflammation.

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In other words, rheumatoid can produce

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subcortical, subchondral swelling, which shows

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up on an x-ray as juxta-articular osteopenia.

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But, we have a child without a major effusion.

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It's in virtually every single bone.

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There is no pannus.

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There are no erosions.

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There was a history of trauma.

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The whole compilation of imaging

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findings and clinical history do not fit.

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There's not a large effusion.

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Rheumatoid or seronegative inflammatory

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arthrosis are not a good choice.

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Nor are crystalline diseases in 11-year-olds.

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So, you might say, well,

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what about septic arthritis?

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In every joint, with every bone swollen?

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I don't think so.

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Now, there is a condition of

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hematogenous osteomyelitis.

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But that's gonna look a lot

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more aggressive than this.

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And the child will be sick.

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So, that doesn't fit.

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Although, it should have crossed your mind.

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So, what are we left with?

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We are left with a diagnosis you absolutely,

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positively have to make in an adult, but

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especially in a child, because they can lose

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their extremity, and that is Complex Regional

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Pain Syndrome Type 1, also previously known as

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the ARDIS, RSD, Reflex Sympathetic Dystrophy.

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Now, what's Type 2?

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Type 2 is when you have a discrete

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nerve injury with burning and

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symptoms in one nerve distribution.

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But when it's generalized. It is a result

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of usually a minor trauma in individuals

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with a heightened autonomic nervous system.

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So the nervous system vasculature reacts and

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makes the nerves hypersensitive, hyper-twitchy.

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And I don't mean on the motor

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side, I mean on the autonomic side.

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So the patients initially may feel a

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little bit of warmth, but eventually the

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extremity, especially the foot, turns cold.

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They get pillow motor changes.

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The skin gets coarse.

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The hair gets more conspicuous, and when you go to

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examine their foot, they say no, don't touch it.

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Because that's how sensitive the

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skin really is in this disorder.

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And the longer it goes, the

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harder it is to reverse.

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Now, many of you are thinking, well,

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there's not a lot of soft tissue swelling.

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Well, guess what?

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There isn't RSD until you get into the endgame.

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And we don't want to be in the endgame.

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Because then our child, or our adult, has

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a massive problem with massive osteopenia.

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We're trying to prevent that.

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So in the early part of the game, we

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want to make this diagnosis and get the

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child immediately into movement therapy.

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The extremity has to be moved,

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a lot of times passively.

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Sometimes it takes anesthesia or a lumbar

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block to actually get the extremity moving.

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So, repeated lumbar blocks are one

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of the most important treatments.

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for Complex Regional Pain Syndrome Type 1.

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There are other, newer therapies, all of which

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are meant to quiet the nervous system down.

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Gabapentin can be a very effective drug for

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reflex sympathetic dystrophy, as can ketamine

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in very severe cases, where the patient

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undergoes twilight anesthesia for two or

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three days to settle the nervous system down.

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If you don't settle it down, and this persists,

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you can have an ischemic loss of the extremity,

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and the extremity will be rendered non-functional.

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Now, this is, unfortunately, a systemic disease.

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You'll see people that have heightened allergies

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when they develop their RSD, because the entire

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body is functioning in a fight or flight response.

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So, they'll start to sneeze more.

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It can get into their throat.

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They may complain of a sore throat.

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It can get into the viscera, into the bladder.

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It can get into almost any organ in the body

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that has a strong autonomic nervous system.

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It is not a common condition to see in the hip.

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The further away from the center of

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the body, the higher the incidence

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of reflex sympathetic dystrophy.

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Now, in the Jurassic period, when

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I trained, we thought, "Okay, minor

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trauma, RSD, three months, six months."

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A delayed time frame for RSD to appear, but

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we now know that especially in the foot,

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RSD, now known as Complex Regional Pain

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Syndrome Type 1, can occur the next day.

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There does not have to be a delay, so do not

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let that dissuade you from the diagnosis.

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This is Complex Regional Pain Syndrome Type 1.

Report

Description

Faculty

Stephen J Pomeranz, MD

Chief Medical Officer, ProScan Imaging. Founder, MRI Online

ProScan Imaging

Tags

Trauma

Musculoskeletal (MSK)

MSK

MRI

Foot & Ankle

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