Taylor Brandy

November 2018
Advanced Orthopedics
Literature Review Paper

Complex Regional Pain Syndrome & Reflex Sympathetic Dystrophy

According to the Mayo Clinic, complex regional pain syndrome (CRPS) is a

chronic pain condition in which the pain perceived by the patient is out of proportion to

the injury. CRPS can present after a heart attack, stroke, an injury or surgery to an

extremity. Although there is not a clear understanding on the cause, early intervention

has shown to be effective. There are two types of CRPS. Type one is more prevalent; it

accounts for 90% of cases. CRPS type 1 is also known as reflexive sympathetic

dystrophy (RSD). Type 1 is presents after an injury to an extremity, heart attack, or

stroke with no direct nerve damage to the affected limb. Type 2, once called causalgia,

is similar to type 1 but the main difference being in type 2 patients, there is nerve

damage. Peripheral injuries causing CRPS can be forceful such as a crush fracture,

amputation, or fracture, but they can also be as minor as a sprained ankle. There is no

one single test that diagnoses CRPS. A diagnosis is made by a physician based on

your medical history and physical exam. In some cases bone scans can detect

changes. Sympathetic nervous system tests can detect changes in blood flow in the

affected limb when compared to the unaffected limb. And at times MRIs can detect

tissue changes consistent with CRPS.

Symptoms can include: swelling in the affected limb, sensitivity to touch or cold,

changes in skin temperature, changes in skin color, changes in skin texture, changes in

hair and nail growth, joint stiffness, muscle spasms, tremors, muscle weakness, and
muscle atrophy. Sensitivity to touch or cold often present first, along with pain and

swelling in the affected limb. Changes in skin color can vary greatly from white to

mottled, to red or blue. Patients also frequently first present with hypersensitivity.

Treatment options are widely varied due to limited research on effective

treatments. However, there has been some evidence for utilizing heat therapy, TENS,

biofeedback interventions, topical analgesics, mirror therapy, spinal cord stimulation,

and intrathecal drug pumps. Some evidence based prophylactic interventions showing

reduced risk for CRPS involve taking vitamin C following a fracture and early

mobilization following a stroke.

The following interventions have been studied to address symptoms of CRPS:

multimodal physiotherapy, graded motor imagery, mirror therapy, virtual body swapping,

tactile discrimination training, ultrasound of stellate ganglion, and TENS. The Cochrane

Library’s systematic review compiled 21 trial reports to summarize quality of evidence

on a variety of interventions to address CRPS type I and II (Smart, Wand, & O’Connell,

2016). All included articles were randomized controlled trials, which were assessed for

bias based on the following criteria: random sequence generation (selection bias),

allocation concealment (selection bias), blinding of participants and personnel

(performance bias), blinding of outcome assessment (detection bias), blinding of

outcome assessment (detection bias), incomplete outcome data (attrition bias), and

selective reporting (reporting bias) (Smart, Wand, & O’Connell, 2016). The studies were

grouped by intervention to measure quality of evidence for each intervention. The

quality of evidence was defined into four categories: high quality, moderate quality, low

quality, and very low quality. Smart, Wand, & O’Connell provided clear delineations
between the levels of quality. High quality defined as we are very confident that the true

effect lies close to that of the estimate effect. Moderate quality was defined as we are

moderately confident in the effect estimate; the true effect is likely to be close to the

estimate of the effect. Low quality defined as our confidence in the effect estimate is

limited; the true effect may be substantially different from the estimate of the effect. Very

low quality was defined as we have very little confidence in the effect estimate.

Multimodal physiotherapy included interventions performed by physical therapists

as well as occupational therapists. The therapies included under physical therapy were:

pain management education, relaxation exercises, connective tissue massage, TENS

and exercise. The interventions included under occupational therapy were: splinting,

desensitisation, and functional rehabilitation. Overall the quality of the evidence for

multimodal approaches was very low. Although the articles were randomized controlled

trials, they had high potential for bias. The evidence demonstrated a small, not

statistically significant improvement in overall function.

Based on the included studies in Cochrane Library systematic review, graded

motor imagery interventions included one protocol consisting of limb laterality

recognition for two weeks, imagined movements for two weeks, and lastly, two weeks of

mirror-box therapy. Another protocol consisted of limb laterality recognition for one

week, imagined movements for one week, and four weeks of mirror-box therapy in

addition to conventional care through occupational and physical therapy. Both protocols

demonstrated a statistically significant reduction (between 25% and 34%) in pain scores

through reliable assessments administered during six week follow-up (Smart, Wand, &

O’Connell, 2016). Two studies also demonstrated an improvement in function as well.

However, due to the high risk of bias, the quality of the evidence is still considered very

low (Smart, Wand, & O’Connell, 2016).

In Cacchio 2009 trial, mirror therapy interventions included a protocol utilizing

four weeks of mirror therapy along with conventional stroke rehabilitation. Participants

for the included trials not only had CRPS type 1 but also had recently had a stroke. In

another 2009 study by Cacchio, found long term improvements in pain in movement

and pain at rest. During a six month follow up pain scores had improved by 38% and

45% respectively (Smart, Wand, & O’Connell, 2016). Although the authors did provide

data on improved pain scores for patients, there was little to no data collected on other

outcome measures such as: function or quality of life. There is very low quality evidence

that mirror therapy improved upper limb function and perceived pain in CRPS type I

patients, post stroke (Smart, Wand, & O’Connell, 2016).

Another included study, in 2014, Jeon examined complete virtual body swapping

with mental rehearsing interventions in patients diagnosed with CRPS type I.

Participants underwent a single session with an immediate follow up demonstrating no

changes in perceived pain (Smart, Wand, & O’Connell, 2016). No data was provided

from this article despite the authors of the systemtatic review requesting further

information. Once again the authors failed to report any data on quality of life changes

or changes in function. Therefore, the quality of the evidence for virtual body swapping

was very low due to high risk of bias.

In 2009 Moseley, implemented four protocols using tactile discrimination training

interventions in patients diagnosed with CRPS type I. During follow ups held two days

post, Moseley reported no statistically significant differences in self reported pain

measurements. The author also did not report any data on functional outcome

measures or quality of life outcome measures (Moseley 2009). The quality of the

evidence was considered very low due to high risk of bias (Smart, Wand, & O’Connell,

2016).

Two included studies, Askin 2014 and Aydemir 2006, implemented ultrasound

therapy on stellate ganglion and ultrasound on placebo area. The studies also

implemented conventional therapies in addition to the ultrasound. In 2014, Askin

implemented two different frequencies of ultrasound to two groups of patients. The

experimental group received 3.0 watts to the stellate ganglion and the other group, the

control group, received 0.5 watts to act as a placebo. In 2014 Askin determined the

conventional treatment would include medication as well: gabapentin, vitamin C, and

prednisolone. Conventional therapy was determined to be: contrast baths, TENS, active

and passive ROM, stretching, resistance exercises, as well as mirror box exercises. The

conventional therapy and the ultrasound intervention were implemented daily over the

course of 20 days (Smart, Wand, & O’Connell, 2016). Although there was no data

provided in the article that could be utilized in a meta-analysis they did report

stastitically significant results with ultrasound interventions (Smart, Wand, & O’Connell,

2016). In 2006, Aydemir examined the different effects stellate ganglion block with

ultrasound to blocks with lidocaine and placebo conditions. Similarly, the two patient

groups also received exercises, TENS, contrast baths, compression, and oral

paracetamol (Smart, Wand, & O’Connell, 2016). In 2006, Aydemir showed no

statistically significant evidence that ultrasound reduced pain more than the effects’ of
the placebos (Smart, Wand, & O’Connell, 2016). The quality of the evidence is low due

to imprecision, bias, as well as limitations of the studies.

In 2005, another included study Hazneci examined the impacts of ultrasound on

stellate ganglion to TENS in patients with acute CRPS type I. Both the control and

intervention groups received conventional therapies. Outcome measures on pain were

taken at the end of the three week trial. The ultrasound group, the intervention group,

demonstrated lower pain VAS scores by roughly 27% (Smart, Wand, & O’Connell,

2016). However it was unclear whether Hazneci used random sequence generation and

allocation concealment during the 2014 study, thus the study was considered to have a

high likeliness for bias. Furthermore, the sample was no more than 30 participants

(Hazneci 2005). There is low quality evidence that ultrasound is an inferior intervention

when compared to TENS due to inconsistency, risk of bias, and imprecision (Smart,

Wand, & O’Connell, 2016).

In 2004, Maillard, Davies, Khubchandani, Woo and Murray examined the effects

of multidisciplinary approach to treating reflexive sympathetic dystrophy among the

pediatric population. The authors determined that based on previous research, the

disciplines included should be a rheumatologist, physiotherapist, occupational therapist,

psychologist. A total of 23 children participated in the study, which took place over the

course of two weeks. Of the 23 participants, 19 participants had follow ups for less than

six months and the remaining 4 participants had follow ups for at least six months

(Maillard et al., 2004). The patients were split up into two groups, outpatient treatment

or inpatient treatment, depending on family structure, severity of symptoms, and

availability of local therapy supports. All patients were provided with daily treatments
from all disciplines. Physiotherapy and occupational therapy included aquatic therapy

and conventional treatments. For patients participating in the inpatient group, after the

first week the patients went home to practice their new skills with their families over the

weekend. For more severe cases, inpatient stays could be extended to four week stays.

Of the 23 participants, 18 were females and 5 were males; the median age of onset for

all the participants was roughly 12 years (Maillard et al., 2004). All participants in the

study reported severe pain in the affected limb as the chief complaint (Maillard et al.,

2004). Furthermore 91% of the participants had restricted range of motion, 61%

reported sensory symptoms, 50% reported swelling, 56% reported color changes in the

affected limb, and 26% reported abnormal sweating (Maillard et al., 2004). Based on

Maillard et al. definition of full recovery: no recurrence of symptoms within the initial

three months following resolution. Full recovery was achieved in 18 of the 23

participants. Maillard et al. found no significant difference in the time reported to

complete recovery between the two groups, inpatient and outpatient. Both groups

achieved complete recovery in roughly six months. However, there was a noted

different in observable improvement in symptoms between the inpatient and outpatient

groups. Participants who received inpatient care, showed improvement in an average

time of roughly 3 weeks (Maillard et al., 2004). Of the 19 participants who completed the

study and less than 6 month follow ups, 11 remained complete well (Maillard et al.,

2004).

Although there is not a clear consensus on best practice for treating CRPS type 1

or type 2, there is evidence that early intervention is effective. There is also evidence

that patients will likely take several weeks and even months to show results.
Furthermore, there is often times a psychological component that may need to be

addressed either in occupational therapy treatment sessions and/or by other healthcare

professionals. In conclusion, when treating patients with CRPS type 1 or type 2, it is

important to intervene early. Patients may also benefit from multidisciplinary approaches

through physical therapy, occupational therapy, and counseling. Patients may also

benefit from graded motor imagery as well as conventional treatments such as passive

range of motion, stretching, desensitization, aquatic therapy, and pain management

education.
 References:

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ultrasound therapy for stellate ganglion blockade in complex regional pain syndrome

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AydemirK,TakaynatanMA,Yaziicio luK,ÖzgülA. The effects of stellate ganglion block

with Lidocaine and ultrasound in complex regional pain syndrome: a randomized,

double blind, placebo controlled study

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