Complex Regional Pain Syndrome (CRPS): Difference between revisions

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<div class="noeditbox">Welcome to [[PPA Pain Project]]. This page is being reviewed and updated by participants of a project to populate the Pain section of Physiopedia. &nbsp;The project is supervised and co-ordinated by the [[The Physiotherapy Pain Association]].
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*Please do not edit unless you are involved in this project, but please come back in the near future to check out new information!! &nbsp;
'''Original Editors '''- Katelyn&nbsp;Koeninger &amp; Kristen Storrie&nbsp;&nbsp;[[Pathophysiology of Complex Patient Problems|from Bellarmine University's&nbsp;Pathophysiology of Complex Patient Problems project.]]  
*If you would like to get involved in this project and earn accreditation for your contributions, [mailto:[email protected] please get in touch]!
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'''Original Editors ''' - [[User:Yves Hubar|Yves Hubar]]  


'''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}} &nbsp;  
'''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}} &nbsp;  
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== Search Strategy  ==


Literature was found on pubmed and the vub v-spaces system.<br>  
== Definition/Description  ==
 
Complex regional pain syndrome (CRPS) is a term for a variety of clinical conditions characterized by chronic persistent pain. It is a disease that may develop after a limb trauma.&nbsp;<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref> This appears mostly in 1 or more limbs, usually in the arms or legs. We can say a CRPS is a regional posttraumatic neuropathic pain problem.<ref>RHO, R. e.a., Concise Review for Clinicians: Complex Regional Pain Syndrome. Mayo Foundation for Medical Education and Research, 2002. (level of evidence 3A)</ref> Neuropathic pain disorders are a disproportionate consequence of painful trauma or nerve lesion. <ref>WASNER, G., e.a., Vascular abnormalities in reflex sympathetic dystrophy (CRPS I): mechanisms and diagnostic value. Oxford University Press, 2001. (level of evidence 3A)</ref>
 
CRPS is subdivided into type I and type II CRPS.


== Definition/Description ==
In the literature, there are a lot of names used to describe this syndrome such as ‘‘Reflex Sympathetic Dystrophy’’, ‘‘causalgia’’, ‘‘algodystrophy’’, ‘‘Sudeck’s atrophy’’, ‘‘neurodystrophy’’, and ‘‘post-traumatic dystrophy’’. To standardize the nomenclature, the name ‘complex regional pain syndrome’ was adopted in 1995 by the ‘International Association for the Study of Pain’ (IASP). <ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref><br><br>
 
== Prevalence ==


The international association for the study of pain defines CRPS as a collection of locally occurring painful conditions, usually following traumatic injury, which tends to express itself distally and exceeds the expected pain of the original trauma and usually results in significant motor deficit. <ref name="reu1">L.Verbruggen. Reumatologie. Dienst Uitgaven VUB 2011</ref><br>  
CPRS affects approximately 26 out of every 100,000 people. It is more common in females than males, with a ratio of 3.5:1.<ref name="goebel" /> CRPS can affect people of all ages, including children as young as&nbsp;three years old and adults as old as 75 years old, but typically is most prevalent beginning in the mid-thirties. CRPS type I occurs after five percent of all traumatic injuries.<ref name="Patho Book" /> Ninety-one percent of all CRPS cases occur after surgery.<ref name="turner">Turner-Stokes L, Goebel A. Complex regional pain syndrome in adults: concise guidance. Clinical Med 2011; 11(6):596-600.</ref>  


CRPS is subdivided into type I and type II CRPS. <br>Type I CRPS signifies that no peripheral nerve injury can be linked to the condition, while type II signifies that the condition results from a peripheral nerve injury. <ref name="art1">Groeneweg G, Huygen FJ, Coderre TJ, Zijlstra FJ. Regulation of peripheral blood flow in Complex Regional Pain Syndrome: clinical implication for symptomatic relief and pain management. BMC Musculoskelet Disord. 2009 Sep 23;10:116. (Level A1)</ref><br>
== Characteristics/Clinical Presentation  ==


== Clinically Relevant Anatomy  ==
<u>Signs and Symptoms of CRPS:<br></u>


CRPS can take place in any body part, but the wrist is most frequently affected after fractures.
*pain (pain may not be present in 7%of CRPS sufferers)<ref name="Patho Book" /> <ref name="goebel" /><ref name="Hooshmand" />
*swelling<ref name="Patho Book" /><ref name="goebel" /><ref name="Hooshmand" />
*tremor<ref name="Patho Book" />
*trouble initiating movements<ref name="Patho Book" />
*muscle spasms (may be present in the cervical and lumbar spine regions in advanced cases)<ref name="Patho Book" /><ref name="Hooshmand" />
*muscle atrophy<ref name="Patho Book" />
*temperature changes<ref name="Patho Book" /><ref name="goebel" />
*color changes (red, blue)<ref name="Patho Book" />
*thick, brittle, or rigid nails<ref name="Patho Book" />
*weakness<ref name="Patho Book" /><ref name="goebel" /><ref name="Hooshmand" />
*thin, shiny, clammy skin<ref name="goebel" />
*stiffness or decreased joint motion<ref name="goebel" />
*painful or decreased sensation on skin (some patients report intolerance to air moving over skin)<ref name="goebel" />
*strange, disfigured, or dislocated feelings in limbs<ref name="goebel" />


<br>  
<br>  


An important aspect of the disease is the occurance of vascular disturbances. Mostly affected are primary small vessels, causing an impact on microcirculation, skin temperature and clinical appearance of the limb.<br>A paper described the changes in microcirculation as an increase in the number of capillaries, endothelial swelling and changes in the vessel luminal wall.<ref name="art1" /><br>According to a review, the acute stage features inhibited sympathetic vasoconstriction and exaggerated neurogenic inflammation, whereas the cold stage features vasoconstriction and endothelial disfunction or vascular hyperreactivity while neurogenic inflammation is less severe.<ref name="art2">Wasner G. Vasomotor Disturbances in Complex Regional Pain Syndrome—A Review. Pain Med. 2010 Aug;11(8):1267-73. (Level C)</ref><br>  
<u></u><u>Characteristics:<br></u>  


== Epidemiology /Etiology  ==
*<u></u>sensory impairments<ref name="Patho Book" />
*movement disorders, typically in contralateral extremity<ref name="Patho Book" /><ref name="Hooshmand" />
*ANS dysfunction<ref name="Patho Book" />
*dystrophy<ref name="Patho Book" />
*atrophy<ref name="Patho Book" />
*spreads proximally, to other extremities, and possibly the entire body<ref name="Patho Book" />
*similar presentation to osteoporosis on radiographic images<ref name="Patho Book" />


CRPS is found to result:<ref name="reu1" /><br>- After traumatic injury (65%)<br>  
Patients typically progress through three stages as CRPS develops. CRPS in children does not always follow the same stage patterns and may at times become stagnate or even slowly improve.<ref name="Hooshmand" /><br>  


*1-2% of all fractures result in CRPS
{| width="591" border="1" cellspacing="1" cellpadding="1"
*Largest risk of CRPS for fractures of the wrist
|-
! scope="col" | Stage
! scope="col" | Time Period
! scope="col" | Classic Signs and Symptoms<ref name="Patho Book" />
|-
| '''Stage I''': acute inflammation: denervation and sympathetic hypoactivity
| Begins 10 days post injury;<br>Lasts 3-6 months<br>
| '''Pain''': more severe than expected; burning or aching; increased with dependent position, physical condition, or emotional disturbances<br>Hyperalgesia, allodynia, hyperpathia: lower pain threshold, increased sensitivity, all stimuli are perceived as painful, increased pain threshold then increased sensation intensity (faster and greater pain)<br>'''Edema''': soft and localized<br>'''Vasomotor/Thermal Changes''': warmer<br>'''Skin''': hyperthermia, dryness<br>'''Other''': increased hair and nail growth<br>
|-
| '''Stage II''': dystrophic: paradoxic sympathetic hyperactivity
| Begins 3-6 months after onset of pain;<br>Lasts about 6 months<br>
| '''Pain''': worsens, constant, burning, aching<br>Hyperalgesia, allodynia, hyperpathia: present<br>'''Edema''': hard, causes joint stiffness<br>'''Vasomotor/Thermal Changes''': none<br>'''Skin''': thin, glossy, cool due to vasoconstriction, sweaty<br>'''Other''': thin &amp; rigid nails, osteoporosis and subchondral bone erosion noted on x-rays<br>
|-
| '''Stage III''': atrophic
| Begins 6-12 months after onset of pain;<br>Lasts for years, or may resolve and reappear<br>
| '''Pain''': spreads proximally and occasionally to entire body, may plateau<br>'''Edema''': hardening<br>'''Vasomotor/Thermal Changes''': decreased SNS regulation, cooler<br>'''Skin''': thin, shiny, cyanotic, dry<br>'''Other''': fingertips and toes are atrophic, thick fascia, possible contractures, demineralization and ankylosis seen on x-rays<br>
|}


- After surgical intervention (19%)<br>- Infection (4%)<br>- Prior inflammation (2%)<br>- No clear cause (10%)
<br>  


A review stated that women are predominantly affected, by a factor of 3,5 and a genetic predisposition has also been theorized.<br>The disease affects all ages, though most cases are between 50 and 70 years old, and it is generally believed to occur mainly in caucasian and Japanese people.<ref name="art4">de Mos M, Sturkenboom MC, Huygen FJ. Current Understandings on Complex Regional Pain Syndrome. Pain Pract. 2009 Mar-Apr;9(2):86-99. Epub 2008 Feb 9. (Level A1)</ref><br>  
== <ref>Cristiana Kahl Collins. Physical Therapy Management of Complex Regional Pain Syndrome I in a 14-Year-Old Patient Using Strain Counterstrain: A Case Report. J Man Manip Ther. 2007; 15(1): 25–41.</ref>[[Image:Criteria.JPG|left]]<br> ==


== Characteristics/Clinical Presentation ==
== Associated Co-morbidities ==
 
CRPS may also be associated with:


The following symptoms have been found in literature:<ref name="art3">Maihöfner C, Seifert F, Markovic K. Complex regional pain syndromes: new pathophysiological concepts and therapies. Eur J Neurol. 2010 May;17(5):649-60. Epub 2010 Feb 18. (Level A1)</ref><br>- Autonomic and trophic disorders:
*Arterial Insufficiency<ref name="MD Guide">MD Guidelines, Medical DIsability Advisor. Complex Regional Pain Syndrome: Comorbid Conditions. http://www.mdguidelines.com/complex-regional-pain-syndrome/comorbid-conditions (accessed March 28, 2012).</ref>
*Asthma<ref name="goebel" />
*Bone Fractures<ref name="Hooshmand" />
*Cellulitis<ref name="MD Guide" />
*Central Pain Syndromes<ref name="MD Guide" />
*Conversion Disorder<ref name="MD Guide" />
*Depression/Anxiety
*Factitious Disorder&nbsp;<ref name="MD Guide" />
*Lymphedema<ref name="MD Guide" />
*Malignancy<ref name="MD Guide" />
*Migraines<ref name="goebel" />
*Nerve Entrapment Syndromes<ref name="MD Guide" />
*Osteomyelitis<ref name="MD Guide" />
*Osteoporosis<ref name="goebel" /><ref name="Hooshmand" />
*Pain Disorder<ref name="MD Guide" />
*Peripheral Neuropathies<ref name="MD Guide" />
*Rheumatoid Arthritis<ref name="MD Guide" />
*Scleroderma<ref name="MD Guide" />
*Septic arthritis<ref name="MD Guide" />
*Systemic Lupus Erythematosus<ref name="MD Guide" />
*Tenosynovitis<ref name="MD Guide" />
*Thrombophlebitis<ref name="MD Guide" /><br>


*Distal Edema in 80% of the patients
== Medications  ==
*Skin temperature changes at the affected body part in 80% of the patients, initially warmer and in 40% of patients gradually cools down until colder in comparison to the rest of the body as the disease progresses. Another review mentioned that 30% of the patients start off from the primarily cold stage.3
*In 40% of the patients skin at the affected body part starts showing redness, but becomes pale or livid in later stages
*In 55% altered sweating takes place, with hyperhydrosis being more common than hypohydrosis.
*Hair and nail growth possibly increase in early stages
*Atrophy of skin and muscles in later stages, as well as contractures may severely restrict movement


- Sensory disturbances (90%) typically in a glove or stocking-like distribution
Possible treatments for CRPS include:


*Spontaneous pain occurs in 75%, usually burning dragging or stinging
*Oral pain-relieving medications including corticosteroids and NSAIDs, as well as acupuncture provide effective pain relief in approximately 20% of those with CRPS, but this is supported by weak evidence.<ref name="Patho Book" />  
<blockquote>
*Treatments may be geared to helping patients manage symptoms. Amitriptyline relieves depression and acts as a sleeping aid. Calcium channel blockers can help to improve circulation through SNS effect. Intrathecal baclofen, among other measures, improves motor dystonia.<ref name="Patho Book" />
*68% felt in deep structures
*Pain intensity and perception of pain is sometimes relieved through use of an implanted transcutaneous electrical nerve stimulation (TENS) unit.<ref name="Patho Book" />
*32% felt in skin
*A randomized double dummy controlled, double blind trial compared the effectiveness of Dimethylsulfoxide 50% (DMSO) and N-acetylcysteine (NAC) in treating CRPS type I. There were no significant differences between the two treatments, but are both successful treating CRPS type I. This study showed that DMSO-treatment is more favorable for warm CRPS whereas NAC is more favorable for cold CRPS<ref name="Perez">Perez R, Zuurmond W, Bezemer P, Kuik D, vanLoenen A, deLange J, et al. The treatment of complex regional pain syndrome type I with free radical scavengers: a randomized controlled study. Pain 2003;102(3):297-307.</ref>  
*In 77% pain shows fluctuating intensity, lesser proportion shows shooting pain  
*Low doses of ketamine infusion has been shown to decrease pain in patients with CRPS type I who had been unsuccessful with other conservative methods of management. Ketamine blocks central sensitization by effecting the N-methyl-D-aspartate receptor which has been shown to be effected in CRPS.<ref name="Goldberg">Goldberg M, Domsky R, Scaringe D, Hirsh R, Dotson J, Sharaf I, et al. Multi-Day Low Dose Ketamine Infusion for the Treatment of Complex Regional Pain Syndrome. Pain Physician 2005;8:175-179.</ref>
*Pain can be increased by orthostasis, anxiety, exercise or temperature changes.  
*Antidepressants may be utilized to treat associated depression.<ref name="Hooshmand" />
*In many cases, pain is more pronounced at night
</blockquote>  
*Sensory gain (Mechanical hyperalgesia, allodynia, ...) or sensory loss (hypaesthesia, hypalgesia, …) may be present.


- Motor dysfunction
== Diagnostic Tests/Lab Tests/Lab Values  ==


*Motor weakness
Diagnosis of CRPS is based solely on examination and patient history.<ref name="Patho Book" /> A triple-phase bone scan is the best method to rule out type I CPRS.<ref name="Cappello">Cappello Z, Kasdan M, Louis D. Meta-analysis of imaging techniques for the diagnosis of complex regional pain syndrome type I. JHS 2012;37A:288-296.</ref> According to Cappello, the triple-phase bone scan has the best sensitivity, NPV, and PPV compared to MRI and plain film radiographs.<ref name="Cappello" /> Radiographic examinations, laser Doppler flowmetry, and thermographic studies may be utilized to assess the secondary issues and symptoms of CRPS.<ref name="Patho Book" /><br>  
*Severe impairment of complex movements
*Impairment of range of motion, initially by concomitant edema, later by contractures and fibroses
*Neglect like symptoms have been found in some patiënts, described as the body part in question feeling foreign.  
*Enhanced physiological tremor in around 50%
*Myoclonus or dystonia, especially in type II CRPS<br>


== Differential Diagnosis  ==
<br>


The differential diagnostic consists of:<ref name="art2" /><br>  
<u>'''The Budapest Criteria'''</u>  


*[[Rheumatology|Rheumatic conditions]]
The Budapest Criteria have been used to diagnose CRPS as well.&nbsp; This method has high specificity and sensitivity according to Goebel.<ref name="goebel" />  
*Inflammatory conditions (infections following bone surgery, neuritides)
*Thromboembolic conditions
*[[Compartment Syndrome|Compartment syndrome]]<br>  
*Peripheral neuropathy (mainly for type II CRPS)


== Diagnostic Procedures  ==
To make the clinical diagnosis, the following criteria must be met:<br>1. Continuing pain, which is disproportionate to any inciting event<br>2. Must report at least one symptom in three of the four following categories:


add text here related to medical diagnostic procedures
*Sensory: Reports of hyperesthesia and/or allodynia
*Vasomotor: Reports of temperature asymmetry and/or skin color changes and/or skin color asymmetry
*Sudomotor/Edema: Reports of edema and/or sweating changes and/or sweating asymmetry
*Motor/Trophic: Reports of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)


== Outcome Measures  ==
3. Must display at least one sign at time of evaluation in two or more of the following categories:


add links to outcome measures here (also see [[Outcome Measures|Outcome Measures Database]])  
*Sensory: Evidence of hyperalgesia (to pinprick) and/or allodynia (to light touch and/or temperature sensation and/or deep somatic pressure and/or joint movement)
*Vasomotor: Evidence of temperature asymmetry (&gt;1 °C) and/or skin color changes and/or asymmetry
*Sudomotor/Edema: Evidence of edema and/or sweating changes and/or sweating asymmetry
*Motor/Trophic: Evidence of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)


== Examination  ==
4. There is no other diagnosis that better explains the signs and symptoms<br>


No golden standard has been developed yet, but included here are the Budapest criteria.<ref name="art2" />
== Etiology/Causes  ==


The following must be met<br>- Continuing pain, which is disproportionate to any inciting event<br>- Must report at least one symptom in three of the four following categories:<br>  
The cause of CRPS remains unknown, however there are explanations for the presentation of the condition.&nbsp; After trauma or external stresses placed on the body, the nervous system has a heightened response to stimuli.<ref name="Medline Plus">Medline Plus, the U.S. National Library of Medicine and the National Institutes of Health. Medical Encyclopedia: Complex Regional Pain Syndrome. http://www.nlm.nih.gov/medlineplus/ency/article/007184.htm (accessed 29 March 2012).</ref>&nbsp; The inflammatory process of the body overreact leading to red hot extremities due to exaggerated blood supply to the area,&nbsp;or blue, cold extremities&nbsp;due to blood vessel constriction.<ref name="Hooshmand" />  


*Sensory: reports of hypaesthesia and/or allodynia
[[Image:Cycle of CRPS.jpg|291x217px|The cycle of CRPS]]&nbsp;<ref name="Aurora">Aurora Health Care. Health Information: Complex Regional Pain Syndrome. http://www.aurorahealthcare.org/yourhealth/healthgate/getcontent.asp?URLhealthgate=%2296853.html%22 (accessed 28 March 2012).</ref>
*Vasomotor: reports of temperature asymmetry and/or skin color changes and/or skin color asymmetry
*Sudomotor/edema: reports of edema and/or sweating changes and/or sweating asymmetry <br>
*Motor/trophic: reports of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)


- Must display at least one sign at time of evaluation in two or<br> more of the following categories:<br>
== Systemic Involvement  ==


*Sensory: evidence of hyperalgesia (to pinprick) and/or allodynia (to light touch and/or temperature sensation and/or deep somatic pressure and/or joint movement)
CRPS primarily affects the vascular system, but over time it can spread to other systems including the nervous, endocrine,&nbsp;cardiovascular systems.&nbsp;&nbsp;Problems develop such as headache, dizziness, tinnitus, urgency/frequency of urination, erection disturbances, renal&nbsp;bleeding,&nbsp;hypertension, nose bleeds,&nbsp;syncope, abdominal pain, peptic ulcers, nausea, vomiting, weight loss, diarrhea, chest pain, abnormal heart beat, tachycardia, heart attack, and&nbsp;falls.<ref name="Hooshmand" />
*Vasomotor: evidence of temperature asymmetry (&gt;1°C) and/or skin color changes and/or asymmetry
*Sudomotor/edema: evidence of edema and/or sweating changes and/or sweating asymmetry
*Motor/trophic: evidence of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)


- There is no other diagnosis that better explains the signs and symptoms<br>  
<br>  


== Medical Management <br> ==
== Medical Management (current best evidence) ==


Concerning pharmacogenic treatment:  
*Spinal cord stimulation was more effective than conventional medical management in reducing pain in patients with CRPS type I<ref name="Simpson">Simpson E, Duenas A, Holmes M, Papaloannou D, Chilcott J. Spinal cord stimulation for chronic pain of neuropathic or ischaemic origin: systematic review and economic evaluation. Health Technology Assessment 2009;13(17):1-179.</ref>
*Spinal cord stimulation was shown to be effective in completely eliminating pain in adolescent females 2-6 weeks after stimulation<ref name="Olson">Olson GL, Meyerson BA, Linderoth B. Spinal cord stimulation in adolescents with complex regional pain syndrome type I. EUR J PAIN 2008;12(1):53-59.</ref>
*Use of surgical and chemical sympathectomy show moderate improvement in pain scores in patients with CRPS. There were no significant differences found between the surgical and chemical groups when comparing pain scores from day one to four months. More high quality research needs to be done before recommending this as a first line of defense.<ref name="Straube">Straube S, Derry S, Moore RA, McQuay HJ. Cervico-thoracic or lumbar sympathectomy for neuropathic pain and complex regional pain syndrome. Cochrane Database of Systematic Reviews 2010;7:1-14.</ref>
*High frequency repetitive transcranial magnetic stimulation on the motor cortex in addition to pharmacological management was effective in reducing pain. This was demonstrated by the scores on the McGill Pain Questionnaire and Short Form-36 which include different aspects of pain such as sensory-discriminative and emotional-affective.<ref name="Picarelli">Picarelli H, Teixeira M, deAndrade D, Myzkowski M, Luvisotto T, Yeng L, et al. Repetitive Transcranial Magnetic Stimulation Is Efficacious as an Add-On to Pharmacological Therapy in Complex Regional Pain Syndrome Type I. J Pain 2010;11(11):1203-10.</ref>
*Surgery, casts, and ice should be avoided when treating CRPS because they further aggravate the nervous system. Surgery leads to further stress, inflammation, and immune system disturbances.<ref name="Hooshmand">Hooshmand H, Phillips E. Spread of complex regional pain syndrome. Vero Beach, Florida. Neurological Associates Pain Management Center.</ref>
*A stellate ganglion block, or sympathectomy, blocks the nerve pathways causing pain. This may be most beneficial in the early stages of CRPS.<ref name="Patho Book" /><ref name="goebel" />


- Pathophysiologically oriented pharmacogenic treatment include application of glucocorticoids, tnf-alpha antibodies, free radical scavengers and sympathic blockade.<br>- Symptomatically oriented pharmacogens include opioids, gabapentin, NSAIDs and baclofen.<br>- To inhibit osteoclastic activity calcitonin, bisphosphonates and mannitol and vasodilating drugs may be given.
{{#ev:youtube|izOYrLUuNd8}}<ref>Arizona Pain. Stellate Ganglion Block. Available from: http://www.youtube.com/watch?v=izOYrLUuNd8 [last accessed 3/29/12]</ref>  


<br>  
<br>  


Definite reports on the efficacy of sympathectomy are currently lacking and there is a risk of developing post sympathectomy pain syndrome.<ref name="art2" />
== Physical Therapy Management (current best evidence)  ==


A review has been found, describing the positive effects of Spinal Cord Stimulation and several theories regarding its effectiveness.<ref name="art5">Prager JP. What Does the Mechanism of Spinal Cord Stimulation Tell Us about Complex Regional Pain Syndrome? Pain Med. 2010 Aug;11(8):1278-83. (Level C)</ref><br>  
It has been found that physical therapy and occupational therapy are effective in reducing pain and increasing function in patients who have had CRPS for less than 1 year<ref name="goebel" />. Physical therapy should focus on patient education of CRPS and functional activities.&nbsp; The typical preferred practice patterns for CRPS are as follows: 4A, 4D, 5G, and 7B.<ref name="Patho Book" />&nbsp;


== Physical Therapy Management <br>  ==
Physical therapy intervention could include any of the following:


The following interventions were found in literature: <ref name="art2" />  
*TENS: Somers, et al found that high frequency TENS contralateral to the nerve injury reduces mechanical allodynia, while low frequency reduces thermal allodynia in rats.<ref name="Patho Book" /><ref name="Somers">Somers D, Clemente F. Transcutaneous Electrical Nerve Stimulation for the Management of Neuropathic Pain: The Effects of Frequency and Electrode Position on Prevention of Allodynia in a Rat Model of Complex Regional Pain Syndrome Type II. Phys Ther 2006;86:698-709.</ref>
*aquatics:&nbsp;Aquatic&nbsp;therapy allows activities to be performed with decreased weight bearing on the lower extremities.<ref name="Patho Book" />
*mirror therapy
*desensitization<ref name="goebel" />
*gradual weight bearing<ref name="goebel" />
*stretching<ref name="goebel" />
*fine motor control<ref name="goebel" />


*Lymphatic drainage to facilitate regression of edema
It is important for physical therapists to recognize that CRPS typically follows blood vessel pathways, and therefore symptoms may not always follow neural patterns.&nbsp; Also, due to the spread pattern, CRPS treatment should be provided bilaterally, due to the contralateral connections present between the extremities.<ref name="Hooshmand" /><br>
*[[Mirror Therapy]]
*Graded motor learning
*TENS (Unless patient cannot tolerate the therapy due to allodynia or hyperalgesia)


A paper on movement disorders in CRPS stated that splints or plaster casts are often ineffective and might even worsen dystonic postures related to CRPS. <ref name="art6">de Mos M, Sturkenboom MC, Huygen FJ. Movement Disorders in Complex Regional Pain Syndrome. Pain Pract. 2009 Mar-Apr;9(2):86-99. Epub 2008 Feb 9. (Level D)</ref>
== Differential Diagnosis  ==


== Key Research  ==
CRPS needs to be differentiated from the following diagnosis<ref name="turner" />:


add links and reviews of high quality evidence here (case studies should be added on new pages using the [[Template:Case Study|case study template]])<br>  
*Bony or soft tissue injury
*Neuropathic pain
*Arthritis
*Infection
*Compartment syndrome
*Arterial insufficiency
*Raynaud’s Disease
*Lymphatic or venous obstruction
*Thoracic outlet syndrome
*Gardner-Diamond Syndrome
*Erythromelalgia
*Self-harm or malingering<br>


== Resources <br> ==
== Clinical Guidelines  ==
 
[http://www.rcplondon.ac.uk/sites/default/files/complex-regional-pain-full-guideline.pdf Complex regional pain syndrome in adults&nbsp;UK guidelines for diagnosis, referral and management in primary and secondary care.] Royal College of Physicians, May 2012.
 
Complex regional pain syndrom: practical diagnostic and treatment guidelines, 4th edition. Pain Medicine. 2013, 14: 180-229
 
== &nbsp;&nbsp;Case Reports/ Case Studies  ==
 
*[http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?vid=6&hid=107&sid=b31938a9-e11b-4a82-b517-b07f03b65ccb%40sessionmgr104 More Than Meets the Eye: Clinical Reflection and Evidence-Based Practice in an Unusual Case of Adolescent Chronic Ankle Sprain]
*[http://www.jospt.org/issues/articleID.447,type.2/article_detail.asp Thoracic Spine Dysfunction in Upper Extremity Complex Regional Pain Syndrome Type I]
 
<br>  


add appropriate resources here <br>  
{{#ev:youtube|jaTlI6bfF64}}<ref>CNN. CNN Report on Reflex Sympathetic Dystrophy. Available from: http://www.youtube.com/watch?v=jaTlI6bfF64 [last accessed 3/28/12]</ref>  


== Clinical Bottom Line  ==
<br>


add text here <br>  
== Resources <br> ==


== Recent Related Research (from [http://www.ncbi.nlm.nih.gov/pubmed/ Pubmed])  ==
*[http://www.rsds.org Reflex Sympathetic Dystrophy Syndrome Association]&nbsp;
*[http://www.theacpa.org American Chronic Pain Association]
*[http://www.mayoclinic.com Mayo Clinic]
*[http://www.ninds.nih.gov National Institute of Neurological Disorders and Stroke]


see tutorial on [[Adding PubMed Feed|Adding PubMed Feed]]  
== &nbsp;Recent Related Research (from [http://www.ncbi.nlm.nih.gov/pubmed/ Pubmed])  ==
<div class="researchbox">
<div class="researchbox"><rss>http://eutils.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=1fAD01Rzfn7B0ZRzIfL12TBhNQObAZ_2nJk-8802GMR8R-nYIR|charset=UTF-8|short|max=10</rss></div>  
<rss>http://eutils.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=1P7PR1QO6IS9xQ724YOoSHF8On2gkKwiE3UGU6x5C6gE847gBI|charset=UTF-8|short|max=10</rss>  
</div>  
== References  ==
== References  ==


see [[Adding References|adding references tutorial]].  
see [[Adding References|adding references tutorial]].  
 
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Revision as of 11:03, 4 October 2015

Original Editors - Katelyn Koeninger & Kristen Storrie  from Bellarmine University's Pathophysiology of Complex Patient Problems project.

Top Contributors - Katelyn Koeninger, Kristen Storrie, Laura Ritchie, Laure-Anne Callewaert, Yves Hubar, Angeliki Chorti, Admin, Melissa Coetsee, Evan Thomas, Scott Cornish, Elaine Lonnemann, Kim Jackson, Rachael Lowe, Gayatri Jadav Upadhyay, Gwen Wyns, Matthias Steenwerckx, Vidya Acharya, Lucinda hampton, Habibu Salisu Badamasi, Claire Knott, Lauren Lopez, Jo Etherton, Maria Galve Villa, Michelle Lee, Richmond Stace, WikiSysop, Wendy Walker and Naomi O'Reilly  

Definition/Description[edit | edit source]

Complex regional pain syndrome (CRPS) is a term for a variety of clinical conditions characterized by chronic persistent pain. It is a disease that may develop after a limb trauma. [1] This appears mostly in 1 or more limbs, usually in the arms or legs. We can say a CRPS is a regional posttraumatic neuropathic pain problem.[2] Neuropathic pain disorders are a disproportionate consequence of painful trauma or nerve lesion. [3]

CRPS is subdivided into type I and type II CRPS.

In the literature, there are a lot of names used to describe this syndrome such as ‘‘Reflex Sympathetic Dystrophy’’, ‘‘causalgia’’, ‘‘algodystrophy’’, ‘‘Sudeck’s atrophy’’, ‘‘neurodystrophy’’, and ‘‘post-traumatic dystrophy’’. To standardize the nomenclature, the name ‘complex regional pain syndrome’ was adopted in 1995 by the ‘International Association for the Study of Pain’ (IASP). [4]

Prevalence[edit | edit source]

CPRS affects approximately 26 out of every 100,000 people. It is more common in females than males, with a ratio of 3.5:1.[5] CRPS can affect people of all ages, including children as young as three years old and adults as old as 75 years old, but typically is most prevalent beginning in the mid-thirties. CRPS type I occurs after five percent of all traumatic injuries.[6] Ninety-one percent of all CRPS cases occur after surgery.[7]

Characteristics/Clinical Presentation[edit | edit source]

Signs and Symptoms of CRPS:

  • pain (pain may not be present in 7%of CRPS sufferers)[6] [5][8]
  • swelling[6][5][8]
  • tremor[6]
  • trouble initiating movements[6]
  • muscle spasms (may be present in the cervical and lumbar spine regions in advanced cases)[6][8]
  • muscle atrophy[6]
  • temperature changes[6][5]
  • color changes (red, blue)[6]
  • thick, brittle, or rigid nails[6]
  • weakness[6][5][8]
  • thin, shiny, clammy skin[5]
  • stiffness or decreased joint motion[5]
  • painful or decreased sensation on skin (some patients report intolerance to air moving over skin)[5]
  • strange, disfigured, or dislocated feelings in limbs[5]


Characteristics:

  • sensory impairments[6]
  • movement disorders, typically in contralateral extremity[6][8]
  • ANS dysfunction[6]
  • dystrophy[6]
  • atrophy[6]
  • spreads proximally, to other extremities, and possibly the entire body[6]
  • similar presentation to osteoporosis on radiographic images[6]

Patients typically progress through three stages as CRPS develops. CRPS in children does not always follow the same stage patterns and may at times become stagnate or even slowly improve.[8]

Stage Time Period Classic Signs and Symptoms[6]
Stage I: acute inflammation: denervation and sympathetic hypoactivity Begins 10 days post injury;
Lasts 3-6 months
 Pain: more severe than expected; burning or aching; increased with dependent position, physical condition, or emotional disturbances
Hyperalgesia, allodynia, hyperpathia: lower pain threshold, increased sensitivity, all stimuli are perceived as painful, increased pain threshold then increased sensation intensity (faster and greater pain)
Edema: soft and localized
Vasomotor/Thermal Changes: warmer
Skin: hyperthermia, dryness
Other: increased hair and nail growth
Stage II: dystrophic: paradoxic sympathetic hyperactivity Begins 3-6 months after onset of pain;
Lasts about 6 months
 Pain: worsens, constant, burning, aching
Hyperalgesia, allodynia, hyperpathia: present
Edema: hard, causes joint stiffness
Vasomotor/Thermal Changes: none
Skin: thin, glossy, cool due to vasoconstriction, sweaty
Other: thin & rigid nails, osteoporosis and subchondral bone erosion noted on x-rays
Stage III: atrophic Begins 6-12 months after onset of pain;
Lasts for years, or may resolve and reappear
 Pain: spreads proximally and occasionally to entire body, may plateau
Edema: hardening
Vasomotor/Thermal Changes: decreased SNS regulation, cooler
Skin: thin, shiny, cyanotic, dry
Other: fingertips and toes are atrophic, thick fascia, possible contractures, demineralization and ankylosis seen on x-rays


[9]
Criteria.JPG

[edit | edit source]

Associated Co-morbidities[edit | edit source]

CRPS may also be associated with:

  • Arterial Insufficiency[10]
  • Asthma[5]
  • Bone Fractures[8]
  • Cellulitis[10]
  • Central Pain Syndromes[10]
  • Conversion Disorder[10]
  • Depression/Anxiety
  • Factitious Disorder [10]
  • Lymphedema[10]
  • Malignancy[10]
  • Migraines[5]
  • Nerve Entrapment Syndromes[10]
  • Osteomyelitis[10]
  • Osteoporosis[5][8]
  • Pain Disorder[10]
  • Peripheral Neuropathies[10]
  • Rheumatoid Arthritis[10]
  • Scleroderma[10]
  • Septic arthritis[10]
  • Systemic Lupus Erythematosus[10]
  • Tenosynovitis[10]
  • Thrombophlebitis[10]

Medications[edit | edit source]

Possible treatments for CRPS include:

  • Oral pain-relieving medications including corticosteroids and NSAIDs, as well as acupuncture provide effective pain relief in approximately 20% of those with CRPS, but this is supported by weak evidence.[6]
  • Treatments may be geared to helping patients manage symptoms. Amitriptyline relieves depression and acts as a sleeping aid. Calcium channel blockers can help to improve circulation through SNS effect. Intrathecal baclofen, among other measures, improves motor dystonia.[6]
  • Pain intensity and perception of pain is sometimes relieved through use of an implanted transcutaneous electrical nerve stimulation (TENS) unit.[6]
  • A randomized double dummy controlled, double blind trial compared the effectiveness of Dimethylsulfoxide 50% (DMSO) and N-acetylcysteine (NAC) in treating CRPS type I. There were no significant differences between the two treatments, but are both successful treating CRPS type I. This study showed that DMSO-treatment is more favorable for warm CRPS whereas NAC is more favorable for cold CRPS[11]
  • Low doses of ketamine infusion has been shown to decrease pain in patients with CRPS type I who had been unsuccessful with other conservative methods of management. Ketamine blocks central sensitization by effecting the N-methyl-D-aspartate receptor which has been shown to be effected in CRPS.[12]
  • Antidepressants may be utilized to treat associated depression.[8]

Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]

Diagnosis of CRPS is based solely on examination and patient history.[6] A triple-phase bone scan is the best method to rule out type I CPRS.[13] According to Cappello, the triple-phase bone scan has the best sensitivity, NPV, and PPV compared to MRI and plain film radiographs.[13] Radiographic examinations, laser Doppler flowmetry, and thermographic studies may be utilized to assess the secondary issues and symptoms of CRPS.[6]


The Budapest Criteria

The Budapest Criteria have been used to diagnose CRPS as well.  This method has high specificity and sensitivity according to Goebel.[5]

To make the clinical diagnosis, the following criteria must be met:
1. Continuing pain, which is disproportionate to any inciting event
2. Must report at least one symptom in three of the four following categories:

  • Sensory: Reports of hyperesthesia and/or allodynia
  • Vasomotor: Reports of temperature asymmetry and/or skin color changes and/or skin color asymmetry
  • Sudomotor/Edema: Reports of edema and/or sweating changes and/or sweating asymmetry
  • Motor/Trophic: Reports of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)

3. Must display at least one sign at time of evaluation in two or more of the following categories:

  • Sensory: Evidence of hyperalgesia (to pinprick) and/or allodynia (to light touch and/or temperature sensation and/or deep somatic pressure and/or joint movement)
  • Vasomotor: Evidence of temperature asymmetry (>1 °C) and/or skin color changes and/or asymmetry
  • Sudomotor/Edema: Evidence of edema and/or sweating changes and/or sweating asymmetry
  • Motor/Trophic: Evidence of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)

4. There is no other diagnosis that better explains the signs and symptoms

Etiology/Causes[edit | edit source]

The cause of CRPS remains unknown, however there are explanations for the presentation of the condition.  After trauma or external stresses placed on the body, the nervous system has a heightened response to stimuli.[14]  The inflammatory process of the body overreact leading to red hot extremities due to exaggerated blood supply to the area, or blue, cold extremities due to blood vessel constriction.[8]

The cycle of CRPS [15]

Systemic Involvement[edit | edit source]

CRPS primarily affects the vascular system, but over time it can spread to other systems including the nervous, endocrine, cardiovascular systems.  Problems develop such as headache, dizziness, tinnitus, urgency/frequency of urination, erection disturbances, renal bleeding, hypertension, nose bleeds, syncope, abdominal pain, peptic ulcers, nausea, vomiting, weight loss, diarrhea, chest pain, abnormal heart beat, tachycardia, heart attack, and falls.[8]


Medical Management (current best evidence)[edit | edit source]

  • Spinal cord stimulation was more effective than conventional medical management in reducing pain in patients with CRPS type I[16]
  • Spinal cord stimulation was shown to be effective in completely eliminating pain in adolescent females 2-6 weeks after stimulation[17]
  • Use of surgical and chemical sympathectomy show moderate improvement in pain scores in patients with CRPS. There were no significant differences found between the surgical and chemical groups when comparing pain scores from day one to four months. More high quality research needs to be done before recommending this as a first line of defense.[18]
  • High frequency repetitive transcranial magnetic stimulation on the motor cortex in addition to pharmacological management was effective in reducing pain. This was demonstrated by the scores on the McGill Pain Questionnaire and Short Form-36 which include different aspects of pain such as sensory-discriminative and emotional-affective.[19]
  • Surgery, casts, and ice should be avoided when treating CRPS because they further aggravate the nervous system. Surgery leads to further stress, inflammation, and immune system disturbances.[8]
  • A stellate ganglion block, or sympathectomy, blocks the nerve pathways causing pain. This may be most beneficial in the early stages of CRPS.[6][5]

[20]


Physical Therapy Management (current best evidence)[edit | edit source]

It has been found that physical therapy and occupational therapy are effective in reducing pain and increasing function in patients who have had CRPS for less than 1 year[5]. Physical therapy should focus on patient education of CRPS and functional activities.  The typical preferred practice patterns for CRPS are as follows: 4A, 4D, 5G, and 7B.[6] 

Physical therapy intervention could include any of the following:

  • TENS: Somers, et al found that high frequency TENS contralateral to the nerve injury reduces mechanical allodynia, while low frequency reduces thermal allodynia in rats.[6][21]
  • aquatics: Aquatic therapy allows activities to be performed with decreased weight bearing on the lower extremities.[6]
  • mirror therapy
  • desensitization[5]
  • gradual weight bearing[5]
  • stretching[5]
  • fine motor control[5]

It is important for physical therapists to recognize that CRPS typically follows blood vessel pathways, and therefore symptoms may not always follow neural patterns.  Also, due to the spread pattern, CRPS treatment should be provided bilaterally, due to the contralateral connections present between the extremities.[8]

Differential Diagnosis[edit | edit source]

CRPS needs to be differentiated from the following diagnosis[7]:

  • Bony or soft tissue injury
  • Neuropathic pain
  • Arthritis
  • Infection
  • Compartment syndrome
  • Arterial insufficiency
  • Raynaud’s Disease
  • Lymphatic or venous obstruction
  • Thoracic outlet syndrome
  • Gardner-Diamond Syndrome
  • Erythromelalgia
  • Self-harm or malingering

Clinical Guidelines[edit | edit source]

Complex regional pain syndrome in adults UK guidelines for diagnosis, referral and management in primary and secondary care. Royal College of Physicians, May 2012.

Complex regional pain syndrom: practical diagnostic and treatment guidelines, 4th edition. Pain Medicine. 2013, 14: 180-229

  Case Reports/ Case Studies[edit | edit source]


[22]


Resources
[edit | edit source]

 Recent Related Research (from Pubmed)[edit | edit source]

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References[edit | edit source]

see adding references tutorial.

  1. ↑ O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)
  2. ↑ RHO, R. e.a., Concise Review for Clinicians: Complex Regional Pain Syndrome. Mayo Foundation for Medical Education and Research, 2002. (level of evidence 3A)
  3. ↑ WASNER, G., e.a., Vascular abnormalities in reflex sympathetic dystrophy (CRPS I): mechanisms and diagnostic value. Oxford University Press, 2001. (level of evidence 3A)
  4. ↑ TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)
  5. ↑ 5.00 5.01 5.02 5.03 5.04 5.05 5.06 5.07 5.08 5.09 5.10 5.11 5.12 5.13 5.14 5.15 5.16 5.17 5.18 Cite error: Invalid <ref> tag; no text was provided for refs named goebel
  6. ↑ 6.00 6.01 6.02 6.03 6.04 6.05 6.06 6.07 6.08 6.09 6.10 6.11 6.12 6.13 6.14 6.15 6.16 6.17 6.18 6.19 6.20 6.21 6.22 6.23 6.24 6.25 6.26 6.27 Cite error: Invalid <ref> tag; no text was provided for refs named Patho Book
  7. ↑ 7.0 7.1 Turner-Stokes L, Goebel A. Complex regional pain syndrome in adults: concise guidance. Clinical Med 2011; 11(6):596-600.
  8. ↑ 8.00 8.01 8.02 8.03 8.04 8.05 8.06 8.07 8.08 8.09 8.10 8.11 8.12 Hooshmand H, Phillips E. Spread of complex regional pain syndrome. Vero Beach, Florida. Neurological Associates Pain Management Center.
  9. ↑ Cristiana Kahl Collins. Physical Therapy Management of Complex Regional Pain Syndrome I in a 14-Year-Old Patient Using Strain Counterstrain: A Case Report. J Man Manip Ther. 2007; 15(1): 25–41.
  10. ↑ 10.00 10.01 10.02 10.03 10.04 10.05 10.06 10.07 10.08 10.09 10.10 10.11 10.12 10.13 10.14 10.15 10.16 MD Guidelines, Medical DIsability Advisor. Complex Regional Pain Syndrome: Comorbid Conditions. http://www.mdguidelines.com/complex-regional-pain-syndrome/comorbid-conditions (accessed March 28, 2012).
  11. ↑ Perez R, Zuurmond W, Bezemer P, Kuik D, vanLoenen A, deLange J, et al. The treatment of complex regional pain syndrome type I with free radical scavengers: a randomized controlled study. Pain 2003;102(3):297-307.
  12. ↑ Goldberg M, Domsky R, Scaringe D, Hirsh R, Dotson J, Sharaf I, et al. Multi-Day Low Dose Ketamine Infusion for the Treatment of Complex Regional Pain Syndrome. Pain Physician 2005;8:175-179.
  13. ↑ 13.0 13.1 Cappello Z, Kasdan M, Louis D. Meta-analysis of imaging techniques for the diagnosis of complex regional pain syndrome type I. JHS 2012;37A:288-296.
  14. ↑ Medline Plus, the U.S. National Library of Medicine and the National Institutes of Health. Medical Encyclopedia: Complex Regional Pain Syndrome. http://www.nlm.nih.gov/medlineplus/ency/article/007184.htm (accessed 29 March 2012).
  15. ↑ Aurora Health Care. Health Information: Complex Regional Pain Syndrome. http://www.aurorahealthcare.org/yourhealth/healthgate/getcontent.asp?URLhealthgate=%2296853.html%22 (accessed 28 March 2012).
  16. ↑ Simpson E, Duenas A, Holmes M, Papaloannou D, Chilcott J. Spinal cord stimulation for chronic pain of neuropathic or ischaemic origin: systematic review and economic evaluation. Health Technology Assessment 2009;13(17):1-179.
  17. ↑ Olson GL, Meyerson BA, Linderoth B. Spinal cord stimulation in adolescents with complex regional pain syndrome type I. EUR J PAIN 2008;12(1):53-59.
  18. ↑ Straube S, Derry S, Moore RA, McQuay HJ. Cervico-thoracic or lumbar sympathectomy for neuropathic pain and complex regional pain syndrome. Cochrane Database of Systematic Reviews 2010;7:1-14.
  19. ↑ Picarelli H, Teixeira M, deAndrade D, Myzkowski M, Luvisotto T, Yeng L, et al. Repetitive Transcranial Magnetic Stimulation Is Efficacious as an Add-On to Pharmacological Therapy in Complex Regional Pain Syndrome Type I. J Pain 2010;11(11):1203-10.
  20. ↑ Arizona Pain. Stellate Ganglion Block. Available from: http://www.youtube.com/watch?v=izOYrLUuNd8 [last accessed 3/29/12]
  21. ↑ Somers D, Clemente F. Transcutaneous Electrical Nerve Stimulation for the Management of Neuropathic Pain: The Effects of Frequency and Electrode Position on Prevention of Allodynia in a Rat Model of Complex Regional Pain Syndrome Type II. Phys Ther 2006;86:698-709.
  22. ↑ CNN. CNN Report on Reflex Sympathetic Dystrophy. Available from: http://www.youtube.com/watch?v=jaTlI6bfF64 [last accessed 3/28/12]
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