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Complex Regionaal PijnSyndroom type 1
Abstract
CRPS bij kinderen in Kindertraumachirurgie. Tweede, herziene druk.
Publicatiedatum: 19 mrt. 2019
Bohn, Stafleu en van Loghum
Omschrijving publicatie:
In zestig hoofdstukken wordt door meer dan honderd specialisten een gedetailleerd en actueel overzicht gegeven over de preventie, diagnostiek en behandeling van ongevalsletsels bij kinderen.
Ik mocht meewerken aan hoofdstuk 52 over #CRPS bij kinderen.
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Complex regional pain syndrome (CRPS) is a chronic, intensified localized pain condition that can affect children and adolescents as well as adults, but is more common among adolescent girls. Symptoms include limb pain; allodynia; hyperalgesia; swelling and/or changes in skin color of the affected limb; dry, mottled skin; hyperhidrosis and trophic changes of the nails and hair. The exact mechanism of CRPS is unknown, although several different mechanisms have been suggested. The diagnosis is clinical, with the aid of the adult criteria for CRPS. Standard care consists of a multidisciplinary approach with the implementation of intensive physical therapy in conjunction with psychological counseling. Pharmacological treatments may aid in reducing pain in order to allow the patient to participate fully in intensive physiotherapy. The prognosis in pediatric CRPS is favorable.
Objective:
To compare the effectiveness of pain exposure physical therapy (PEPT) with conventional treatment in patients with complex regional pain syndrome type 1 (CRPS-1) in a randomised controlled trial with a blinded assessor.
Setting:
The study was conducted at a level 1 trauma centre in the Netherlands.
Participants:
56 adult patients with CRPS-1 participated. Three patients were lost to follow-up.
Interventions:
Patients received either PEPT in a maximum of five treatment sessions, or conventional treatment following the Dutch multidisciplinary guideline.
Measurements:
Outcomes were assessed at baseline and at 3, 6 and 9 months after randomisation. The primary outcome measure was the Impairment level Sum Score-Restricted Version (ISS-RV), consisting of visual analogue scale for pain (VAS-pain), McGill Pain Questionnaire, active range of motion (AROM) and skin temperature. Secondary outcome measures included Pain Disability Index (PDI); muscle strength; Short Form 36 (SF-36); disability of arm, shoulder and hand; Lower Limb Tasks Questionnaire (LLTQ); 10 m walk test; timed up-and-go test (TUG) and EuroQol-5D.
Results:
The intention-to-treat analysis showed a clinically relevant decrease in ISS-RV (6.7 points for PEPT and 6.2 points for conventional treatment), but the between-group difference was not significant (0.96, 95% CI -1.56 to 3.48). Participants allocated to PEPT experienced a greater improvement in AROM (between-group difference 0.51, 95% CI 0.07 to 0.94; p=0.02). The per protocol analysis showed larger and significant between-group effects on ISS-RV, VAS-pain, AROM, PDI, SF-36, LLTQ and TUG.
Conclusions:
We cannot conclude that PEPT is superior to conventional treatment for patients with CRPS-1. Further high-quality research on the effects of PEPT is warranted given the potential effects as indicated by the per protocol analysis.
Trial registration numbers:
NCT00817128 and NTR 2090.
There is little knowledge regarding the association between psychological factors and complex regional pain syndrome (CRPS) in children. Specifically, it is not known which factors precipitate CRPS and which result from the ongoing painful disease.
OBJECTIVES: To examine symptoms of depression and anxiety as well as the experience of stressful life events in children with CRPS compared with children with chronic primary headaches and functional abdominal pain.
METHODS: A retrospective chart study examined children with CRPS (n=37) who received intensive inpatient pain treatment between 2004 and 2010. They were compared with two control groups (chronic primary headaches and functional abdominal pain; each n=37), who also received intensive inpatient pain treatment. Control groups were matched with the CRPS group with regard to admission date, age and sex. Groups were compared on symptoms of depression and anxiety as well as stressful life events.
RESULTS: Children with CRPS reported lower anxiety and depression scores compared with children with abdominal pain. A higher number of stressful life events before and after the onset of the pain condition was observed for children with CRPS.
CONCLUSIONS: Children with CRPS are not particularly prone to symptoms of anxiety or depression. Importantly, children with CRPS experienced more stressful life events than children with chronic headaches or abdominal pain. Prospective long-term studies are needed to further explore the potential role of stressful life events in the etiology of CRPS.
<⁄span> Historically, in both adult and pediatric populations, a lack of knowledge regarding complex regional pain syndrome (CRPS) and absence of clear diagnostic criteria have contributed to the view that this is a primarily psychiatric condition.
<⁄span> To test the hypothesis that children with CRPS are more functionally disabled, have more pain and are more psychologically distressed than children with other pain conditions.
<⁄span> A total of 101 children evaluated in a tertiary care pediatric pain clinic who met the International Association for the Study of Pain consensus diagnostic criteria for CRPS participated in the present retrospective study. Comparison groups included 103 children with abdominal pain, 291 with headache and 119 with back pain. Children and parents completed self-report questionnaires assessing disability, somatization, pain coping, depression, anxiety and school attendance.
<⁄span> Children with CRPS reported higher pain intensity and more recent onset of pain at the initial tertiary pain clinic evaluation compared with children with other chronic pain conditions. They reported greater functional disability and more somatic symptoms than children with headaches or back pain. Scores on measures of depression and anxiety were within normal limits and similar to those of children in other pain diagnostic groups.
<⁄span> As a group, clinic-referred children with CRPS may be more functionally impaired and experience more somatic symptoms compared with children with other pain conditions. However, overall psychological functioning as assessed by self-report appears to be similar to that of children with other chronic pain diagnoses. Comprehensive assessment using a biopsychosocial framework is essential to understanding and appropriately treating children with symptoms of CRPS.
A complex regional pain syndrome (CRPS)--multiple system dysfunction, severe and often chronic pain, and disability--can be triggered by a minor injury, a fact that has fascinated scientists and perplexed clinicians for decades. However, substantial advances across several medical disciplines have recently improved our understanding of CRPS. Compelling evidence implicates biological pathways that underlie aberrant inflammation, vasomotor dysfunction, and maladaptive neuroplasticity in the clinical features of CRPS. Collectively, the evidence points to CRPS being a multifactorial disorder that is associated with an aberrant host response to tissue injury. Variation in susceptibility to perturbed regulation of any of the underlying biological pathways probably accounts for the clinical heterogeneity of CRPS.
The objective of this study was to investigate the association between psychological factors and complex regional pain syndrome type 1 (CRPS1). A prospective multicenter cohort study was performed involving the emergency room of three hospitals, and patients age 18years or older, with a single fracture, were included in the study. At baseline (T0), participants completed a questionnaire covering demographic, psychological (Symptom Checklist-90), and medical variables. At plaster removal (T1) and at T2, the participants completed a questionnaire addressing symptoms of CRPS1. Psychological factors that were analysed were agoraphobia, depression, somatization, insufficiency, (interpersonal) sensitivity, insomnia, and life events. In total, 596 consecutive patients were included in the study, and 7.0% were diagnosed with CRPS1. None of the psychological factors predicted the development of CRPS1. The scores on the Symptom Checklist-90 subscales fell into the range of the general population and were, in most cases, average or below average when compared with those of pain patients or psychiatric patients. No empirical evidence supports a diagnosis of CRPS1 patients as psychologically different, and the current results indicate that there is no association between psychological factors and CRPS1.
Current IASP diagnostic criteria for CRPS have low specificity, potentially leading to overdiagnosis. This validation study compared current IASP diagnostic criteria for CRPS to proposed new diagnostic criteria (the "Budapest Criteria") regarding diagnostic accuracy. Structured evaluations of CRPS-related signs and symptoms were conducted in 113 CRPS-I and 47 non-CRPS neuropathic pain patients. Discriminating between diagnostic groups based on presence of signs or symptoms meeting IASP criteria showed high diagnostic sensitivity (1.00), but poor specificity (0.41), replicating prior work. In comparison, the Budapest clinical criteria retained the exceptional sensitivity of the IASP criteria (0.99), but greatly improved upon the specificity (0.68). As designed, the Budapest research criteria resulted in the highest specificity (0.79), again replicating prior work. Analyses indicated that inclusion of four distinct CRPS components in the Budapest Criteria contributed to enhanced specificity. Overall, results corroborate the validity of the Budapest Criteria and suggest they improve upon existing IASP diagnostic criteria for CRPS.
Reflex sympathetic dystrophy is well recognised in adults. It occurs in children but is rarely mentioned in paediatric textbooks. Children presenting with painful limbs often have many investigations and the diagnosis may be considerably delayed. This results in prolonged distressing pain for the child, anxiety for parents, and difficulty with treatment. We describe four cases to show the characteristics of the disease in children.
### CASE 1
An 11 year old athletic girl presented to this hospital with a 10 week history of increasing pain in her right leg. She had first seen her general practitioner with pain over the tibial tubercle of her right knee but no history of trauma. Her local orthopaedic service had diagnosed Osgood-Schlatter's disease and she had been advised to rest and use crepe bandage and elasticated tubular bandage.
Five days later her leg became cold and blue below the knee, with burning pain radiating from the knee to the foot. Deep vein thrombosis was diagnosed and she was treated with anticoagulant drugs. After a week anticoagulant treatment was stopped, but the leg continued to be painful, cold, and blue when in a dependent position. A full length plaster of Paris cast was applied in an attempt to relieve the pain of the Osgood-Schlatter's disease, but within days the symptoms were worse, affecting her whole leg up to mid-thigh. The blueness resolved only when her leg was elevated. The plaster of Paris was removed, but by this stage the pain was so severe that she would not put her foot on the ground and she was confined to a wheelchair.
On first examination at this hospital the leg was noted to be cold and blue and the foot oedematous. She had allodynia (pain provoked by a stimulus that does not normally cause pain) and hyperaesthesia up to her …
Few series on reflex sympathetic dystrophy syndrome (RSDS) have included children. The present series reviewed 10 affected children. The group consisted of 9 girls and one boy with an average age at onset of 11 years (5 years to 16 years). The diagnosis was based on the clinical findings of pain, dysesthesia and autonomic system dysfunction. All patients underwent x rays and bone scans. Their results showed great variation. Minor trauma was the most common trigger factor. The lower extremities were more often involved. The treatment consisted of pain relief and progressive mobilization. Less conventional treatments in children, such as calcitonin and bisphosphonate were also used. The severity and duration of the disease varied greatly among these children. Moderate pain and sympathetic dysfunction persisted often up to two years after onset. Reflex sympathetic dystrophy is more common in children than previously thought. There are differences with the adult form in presentation and clinical course: the diagnosis is often delayed, the lower extremities are more often involved, girls are affected more often and idiopathic forms are frequent. Significant emotional dysfunction is found in a majority of patients and they are best treated as inpatients by a multidisciplinary team.
Reflex sympathetic dystrophy (RSD), an unusual diagnosis in general paediatrics, is well recognised by paediatric rheumatologists. This study reports the presentation and the clinical course of 46 patients (35 female, age range 8-15.2) with RSD. The patients saw professionals from an average of 2.3 specialties (range 1-5). Twenty five (54%) had a history of trauma. Median time to diagnosis was 12 weeks (range 1-130). Many children had multiple investigations and treatments. Once diagnosis was made, treatment followed with physiotherapy and analgesics. Median time to recovery was seven weeks (range 1-140), with 27.5% relapsing. Nine children required assessment by the child and adolescent psychiatry team. This disease, though rare, has significant morbidity and it is therefore important to raise clinicians' awareness of RSD in childhood. Children with the condition may then be recognised and referred for appropriate management earlier, and spared unnecessary investigations and treatments which may exacerbate the condition.
An unusual case of a 2 1/2-year-old girl with reflex sympathetic dystrophy (RSD) of the left arm is described. She is the youngest RSD case ever presented in the literature. Upper extremity involvement is also rare in childhood RSD. She had both physical and psychological trauma in an earthquake preceding the disease. The association of RSD with a psychological disorder is stressed and awareness of the condition to the general paediatrician is recommended for early diagnosis and successful treatment.
Limited research suggests that there may be Warm complex regional pain syndrome (CRPS) and Cold CRPS subtypes, with inflammatory mechanisms contributing most strongly to the former. This study for the first time used an unbiased statistical pattern recognition technique to evaluate whether distinct Warm vs Cold CRPS subtypes can be discerned in the clinical population. An international, multisite study was conducted using standardized procedures to evaluate signs and symptoms in 152 patients with clinical CRPS at baseline, with 3-month follow-up evaluations in 112 of these patients. Two-step cluster analysis using automated cluster selection identified a 2-cluster solution as optimal. Results revealed a Warm CRPS patient cluster characterized by a warm, red, edematous, and sweaty extremity and a Cold CRPS patient cluster characterized by a cold, blue, and less edematous extremity. Median pain duration was significantly (P < 0.001) shorter in the Warm CRPS (4.7 months) than in the Cold CRPS subtype (20 months), with pain intensity comparable. A derived total inflammatory score was significantly (P < 0.001) elevated in the Warm CRPS group (compared with Cold CRPS) at baseline but diminished significantly (P < 0.001) over the follow-up period, whereas this score did not diminish in the Cold CRPS group (time × subtype interaction: P < 0.001). Results support the existence of a Warm CRPS subtype common in patients with acute (<6 months) CRPS and a relatively distinct Cold CRPS subtype most common in chronic CRPS. The pattern of clinical features suggests that inflammatory mechanisms contribute most prominently to the Warm CRPS subtype but that these mechanisms diminish substantially during the first year postinjury.
This double-blind, randomized, multicenter study was designed to determine the short-term and long-term efficacy of intravenous regional block with guanethidine in patients with reflex sympathetic dystrophy (RSD)/causalgia. Sixty patients were enrolled to receive four intravenous regional blocks at 4-day intervals with either guanethidine or placebo in 0.5% lidocaine. Each patient was randomized to receive either one, two, or four blocks with guanethidine. Follow-up visits were scheduled for 4 days, 1 mo, 3 mo, and 6 mo after their final block. At 4 days after the initial block, the group treated with placebo experienced a greater decrease in pain scores than those treated with guanethidine, although this difference was not statistically significant. On long-term followup there was no difference in pain scores between groups receiving one, two, or four guanethidine blocks. Overall, only 35% of patients experienced clinically significant relief on long-term followup even though all were treated early in the evolution of RSD.
(Anesth Analg 1995;81:718-23)
Objective:
To study the clinical and epidemiological characteristics of complex regional pain syndrome (CRPS) in children.
Patients and methods:
All children and adolescents under 16 years of age with a new diagnosis of CRPS who were reported to the Scottish Paediatric Surveillance Unit were included. Patients' recruitment ran between 1 November 2011 and 31 October 2015. Information was collected on patients' demography, clinical features, investigations, management and impact of disease on child and family. The diagnosis of CRPS was made on fulfilling the clinical criteria of the International Association for the Study of Pain.
Results:
26 cases of CRPS were reported over 4 years, giving a minimum estimated incidence of 1.16/100 000 (95% CI 0.87 to 1.44/100 000) children 5-15 years of age. Nineteen patients were female (73%) and mean age at diagnosis was 11.9 (range 5.5-15.4 years). The median interval between onset of symptoms and diagnosis was 2 months (range 1-12). The majority of children have single site involvement, with legs been more often affected than arms and the right side is more often affected than the left. There was a clear trauma at onset of the illness in 19 children and possible nerve injury in one. All investigations were normal and several treatment modalities were used with variable success. The disease had significant impacts on the patients' education and family lives.
Conclusions:
The estimated incidence of CRPS is 1.2/100 000 children 5-15 years old. The diagnosis of CRPS is often delayed. CRPS has a significant impact on children and their families.
Introduction:
Complex regional pain syndrome type 1 (CRPS I) in children differs from its adult counterpart and relevant literature is scarce. Our aim was to investigate potential risk factors and to assess midterm outcome and quality of life.
Material and methods:
Medical records of patients diagnosed with CRPS I between 2004 and 2012 were analyzed. Patients and parents were called for a phone interview including the PEDS Quality of Life 4-0 questionnaire. Results were compared to a control group matched for age, gender and socio-economic status.
Results:
Seventy-three patients were included (64 girls, 9 boys). Mean age at diagnosis was 11.5 years and mean time to diagnosis was 14.2 months. The lower limb was affected in 89% of cases. Allodynia, coldness and cyanosis were noted in 95%, 81% and of 74% of cases, respectively. Forty-nine percent of patients reported a physical injury. Multivariate analysis showed a strong association with being anxious (OR = 44.9, 95% CI [7.4-273]), presence of an atopic background (OR = 25.0, 95% CI: [4.6-135]), being good to excellent school performers (OR = 8.4 95% CI [1.3-52.1]), and having trouble falling asleep (OR = 5.3, 95% CI [1.6-17.0]). At a mean 37 months' follow-up (12-102), PEDS QL 4-0 score was significantly lower in CRPS patients compared to controls. Fifty-seven percent of patients acknowledged healing and 55% had presented a relapse.
Conclusion:
Childhood onset CRPS I affects predominantly preadolescent girls at the ankle. The present study highlights the relatively poor outcome, especially its physical and emotional aspects and the large role of psychology.
Level of evidence:
IV.
Complex regional pain syndrome (CRPS) is a chronic pain condition typically involving a limb, which is characterized by neuropathic pain, sensory abnormalities and neurovascular findings. The exact cause of CRPS is unknown; however, proposed theories include alterations in the sympathetic and central nervous system (CNS), small fibre changes in the peripheral nervous system and psychological factors. Although this condition was previously considered rare among children and adolescents, it has been increasingly recognized in paediatric patients and can result in significant disability.
The diagnosis of paediatric CRPS is based upon clinical criteria obtained from a thorough history and physical examination. Other possible causes, such as orthopaedic, infectious, vascular and rheumatologic disorders, should be ruled out prior to making the diagnosis. Treatment focuses on a rehabilitative strategy consisting of physical therapy, occupational therapy and cognitive-behavioural therapy with an overall focus on return to functioning.
CRPS in children and adolescents is characterized by a painful, mottled appearing, swollen limb with allodynia and hyperalgesia. For most patients, pain is severe, resulting in significant functional disability. More recent evidence suggests that a rehabilitative programme results in improvement in both pain and functional measures.
Background:
Local anesthetic blockade of the sympathetic chain is widely used to treat reflex sympathetic dystrophy (RSD) and causalgia. These two pain syndromes are now conceptualized as variants of a single entity: complex regional pain syndrome (CRPS). A recent meta-analysis of the topic has been published. However, this study only evaluated studies in English language and therefore it could have overlooked some randomized controlled trials.
Objectives:
This systematic review had three objectives: to determine the likelihood of pain alleviation after sympathetic blockade with local anesthetics in the patient with CRPS; to assess how long any benefit persists; and to evaluate the incidence of adverse effects of the procedure.
Search strategy:
We searched the Cochrane Pain, Palliative and Supportive Care Register, the Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE, LILACS, and conference abstracts of the World Congresses of the International Association for the Study of Pain. Bibliographies from retrieved articles were also searched for additional studies.
Selection criteria:
We considered for inclusion randomized controlled trials that evaluated the effect of sympathetic blockade with local anesthetics in children or in adult patients to treat RSD, causalgia, or CRPS.
Data collection and analysis:
The outcomes of interest were the number of patients who obtained at least 50% of pain relief shortly after sympathetic blockade (30 minutes to 2 hours) and 48 hours or later. We also assessed the presence of adverse effects in each treatment arm. A random effects model was used to combine the studies.
Main results:
Two small randomized double blind cross over studies that evaluated 23 subjects were found. The combined effect of the two trials produced a relative risk (RR) to achieve at least 50% of pain relief 30 minutes to 2 hours after the sympathetic blockade of 1.17 (95% CI 0.80-1.72). It was not possible to determine the effect of sympathetic blockade on long-term pain relief because the authors of the two studies evaluated different outcomes.
Authors' conclusions:
This systematic review revealed the scarcity of published evidence to support the use of local anesthetic sympathetic blockade as the 'gold standard' treatment for CRPS. The two randomized studies that met inclusion criteria had very small sample sizes, therefore, no conclusion concerning the effectiveness of this procedure could be drawn. There is a need to conduct randomized controlled trials to address the value of sympathetic blockade with local anesthetic for the treatment of CRPS.
This review aimed to synthesize the current evidence on the effectiveness of invasive treatments for Complex Regional Pain Syndrome (CRPS) in children and adolescents. Studies on children and adolescents with CRPS that evaluated the effects of invasive treatment were identified in PubMed (search March 2013).
Thirty-six studies met the inclusion criteria. Articles reported on a total of 173 children and adolescents with CRPS. Generally, many studies lack methodological quality. The invasive treatments applied most often were singular sympathetic blocks, followed by epidural catheters and continuous sympathetic blocks. Rarely, spinal cord stimulation and pain directed surgeries were reported. An individual patient frequently received more than one invasive procedure. Concerning outcome, for nearly all patients an improvement in pain and functional disability was reported. However, these outcomes were seldom assessed with validated tools. In conclusion, the evidence level for invasive therapies in the treatment of CRPS in children and adolescents is weak.
This is an update of the original Cochrane review published in The Cochrane Library, 2005, Issue 4, on local anaesthetic blockade (LASB) of the sympathetic chain used to treat complex regional pain syndrome (CRPS).
To assess the efficacy of LASB for the treatment of pain in CRPS and to evaluate the incidence of adverse effects of the procedure.
We updated searches of the Cochrane Pain, Palliative and Supportive Care Group Specialised Register, the Cochrane Central Register of Controlled Trials (CENTRAL) on the Cochrane Library (Issue 11 of 12, 2012), MEDLINE (1966 to 22/11/12), EMBASE (1974 to 22/11/12), LILACS (1982 to 22/11/12), conference abstracts of the World Congresses of the International Association for the Study of Pain (1995 to 2010), and various clinical trial registers (inception to 2012). We also searched bibliographies from retrieved articles for additional studies.
We considered for inclusion randomised controlled trials (RCTs) that evaluated the effect of sympathetic blockade with local anaesthetics in children or adults with CRPS.
The outcomes of interest were reduction in pain intensity levels, the proportion who achieved moderate or substantial pain relief, the duration of pain relief, and the presence of adverse effects in each treatment arm.
We included an additional 10 studies (combined n = 363) in this update. Overall we include 12 studies (combined n = 386). All included studies were assessed to be at high or unclear risk of bias.Three small studies compared LASB to placebo/sham. We were able to pool the results from two of these trials (intervention n = 23). Pooling did not demonstrate significant short-term benefit for LASB (in terms of the risk of a 50% reduction of pain scores).Of two studies that investigated LASB as an addition to rehabilitation treatment, the only study that reported pain outcomes demonstrated no additional benefit from LASB.Eight small randomised studies compared sympathetic blockade to another active intervention. Most studies found no difference in pain outcomes between sympathetic block and other active treatments.Only five studies reported adverse effects, all with minor effects reported.
This update has found similar results to the original systematic review. There remains a scarcity of published evidence to support the use of local anaesthetic sympathetic blockade for CRPS. From the existing evidence it is not possible to draw firm conclusions regarding the efficacy or safety of this intervention but the limited data available do not suggest that LASB is effective for reducing pain in CRPS.
While complex regional pain syndrome (CRPS) is a well-established entity in adults, its occurrence in children was doubted for a long time. However, in the last few years several case reports and some comparative studies have described CRPS in children and adolescents.
In contrast to adults most of the involved children are female, suffering from CRPS after an initial event that is typically a minor trauma. Furthermore, CRPS occurs more frequently in the lower extremity than in the upper extremity when compared to adults. Since neither radiological findings nor laboratory parameters are able to confirm CRPS, the diagnosis is made from a detailed description of the clinical signs and symptoms characterized by a remarkable intra- and inter-individual variability.
The treatment concept is comprised of intensive physical therapy, pain management and psychological therapy, physical therapy having the major impact. In spite of a sometimes long and variable clinical course the prognosis in most cases is excellent and usually results in complete functional restoration. Nevertheless, relapses are described in up to 30% and can involve other locations or present in a different fashion regarding clinical symptoms.
This topical update reports recent progress in the international effort to develop a more accurate and valid diagnostic criteria for complex regional pain syndrome (CRPS). The diagnostic entity of CRPS (published in the International Association for the Study of Pain’s Taxonomy monograph in 1994; International Association for the Study of Pain [IASP]) was intended to be descriptive, general, and not imply etiopathology, and had the potential to lead to improved clinical communication and greater generalizability across research samples. Unfortunately, realization of this potential has been limited by the fact that these criteria were based solely on consensus and utilization of the criteria in the literature has been sporadic at best. As a consequence, the full potential benefits of the IASP criteria have not been realized. Consensus-derived criteria that are not subsequently validated may lead to over- or underdiagnosis, and will reduce the ability to provide timely and optimal treatment. Results of validation studies to date suggest that the IASP/CRPS diagnostic criteria are adequately sensitive; however, both internal and external validation research suggests that utilization of these criteria causes problems of overdiagnosis due to poor specificity. This update summarizes the latest international consensus group’s action in Budapest, Hungary to approve and codify empirically validated, statistically derived revisions of the IASP criteria for CRPS.
Knowledge about the pathophysiology underlying the complex regional pain syndrome (CRPS) has increased over the last years. Classically, CRPS has been considered to be mainly driven by sympathetic dysfunction with sympathetically maintained pain being its major pathogenetic mechanism. Currently, the disease is understood as result of a complex interplay between altered somatosensory, motor, autonomic and inflammatory systems. Peripheral and central sensitization is a common feature in CRPS as in other neuropathic pain syndromes. One important mechanism is the sensitization of spinal dorsal horn cells via activation of postsynaptic NMDA-receptors by chronic C-fiber input. Differential activity of endogenous pain modulating systems may play a pivotal role in the development of CRPS, too. Neuronal plasticity of the somatosensory cortex accounts for central sensory signs. Also the motor system is subject to central adaptive changes in patients with CRPS. Calcitonin-gene related peptide (CGRP) and substance P mediate neurogenic inflammation. Additionally other proinflammatory cytokines involved in the inflammatory response in CRPS have been identified. In terms of the sympathetic nervous system, recent evidence rather points to a sensitization of adrenergic receptors than to increased efferent sympathetic activity. Particularly the expression of alpha (1)-adrenoceptors on nociceptive C-fibers may play a major role. These pathophysiological ideas do not exclude each other. In fact they complement one another. The variety of the involved systems may explain the versatile clinical picture of CRPS.
Georg Thieme Verlag KG Stuttgart, New York.
The outcome of complex regional pain syndrome (CRPS) is relatively unknown. High disease resolution rates have been reported, but also long-lasting impairments in many patients. This study aims to assess CRPS outcome in a population-based cohort of CRPS patients.
CRPS patients were retrospectively identified (1996 to 2005) in a Dutch general practitioners database, the integrated primary care information project, and included if at onset (ie, in the past) they had complied with the International Association for the Study of Pain (IASP) diagnostic criteria. The disease status at minimum of 2 years since onset was assessed during visits using questionnaires, interviews, and physical examination. Symptoms and signs were compared with reference patients with an identical past injury but without CRPS. Actual fulfillment of the IASP criteria, treatment status, self-reported recovery, and working status were recorded. Moreover, to identify the potential prognostic factors, baseline patient characteristics were compared across subgroups according to the CRPS outcome. These subgroups were derived by cluster analysis on actual symptoms and signs.
One hundred and two CRPS patients were assessed at on average 5.8 years (range: 2.1 to 10.8) since onset. CRPS patients displayed higher symptom and sign prevalences in all categories (sensory, vasomotor, sudomotor, and motor/trophic) than controls. Sixteen percent (95% CI: 9-22) reported the CRPS as still progressive, whereas 31% (95% CI: 19-43) were incapable of working. Patients in the poorest outcome cluster more often had their upper extremity affected, event other than a fracture, and cold CRPS.
Severe CRPS outcome is rare, but a majority of patients has persistent impairments at 2 or more years since onset.
Complex Regional Pain Syndrome type 1 (CRPS1) is a complication after trauma or surgery. Its pathophysiology is still a matter of debate, and psychological factors have been suggested to play a role, although their influence is unclear. The aim of this study was to investigate the evidence for the influence of psychological factors on the onset and maintenance of CRPS1 in adults. In a systematic review, articles were selected using Cochrane, Pubmed/Medline, Psychinfo, and Cinahl since 1980. Only original articles and empirical studies were included. Based on these selection criteria, 31 articles were identified. Studies were evaluated and weighted using a quality assessment instrument. The few prospective studies do not report a relationship between CRPS1 and depression, anxiety, neuroticism, or anger. The results of the retrospective/cross-sectional studies yield contradictory results regarding psychological problems in patients with CRPS1. A majority show no association, and studies with a higher methodological quality lean to a conclusion of no relationship between psychological factors and CRPS1. The majority of included studies (N=24; 77%) had only a poor to moderate methodological quality. Although many patients with CRPS1 are stigmatized as being psychologically different, this literature review identified no relationship between CRPS1 and several psychological factors. Only life events seemed to be associated with CRPS1: patients who experienced more life events appeared to have a greater chance of developing CRPS1. More studies with greater methodological quality and more participants should be performed on the association between psychological factors and the development and course of CRPS1.
The clinical presentation of Complex Regional Pain Syndrome type I (CRPS I) in children differs compared to the presentation in adults. Reported results of treatment of CRPS I in children are usually more favourable and seem better than the reported treatment of adults with CRPS I. We investigated the quality of life (QoL) in adults who have been treated for childhood-onset CRPS I.
We performed a retrospective chart review on signs, symptoms and treatment of all patients, seen and treated for CRPS I in childhood (age <16 years). At one time point a survey was sent by mail to all adult patients with onset CRPS I in childhood with a postal reminder after one month. The first part of the survey consisted of questions focused on the experience of chronic pain and other current complaints in the affected extremity. The second part consisted of a generic-health-related quality of life instrument (SF-36).
Forty-two patients (75%) responded to our survey. The median follow-up period was 12 years (SD 4.7; range 2-22). Fifty-two percent of all patients complained about pain at the time of follow-up. Of the 12 symptoms and signs, 4 are improved, 1 is worse and the remainder are unchanged. Fifteen patients experienced one or more documented relapses. General health and physical functioning (2 out of 8 scales on the SF 36) were lower in patients compared to those of the literature.
In contrast to the literature, the prognosis of childhood-onset CRPS I seems less favourable than usually reported, and is comparable to the prognosis of the adult-onset CRPS I in view of a decreased quality of life and a large relapse percentage (33%) at long-term follow-up.
We report on the experience with our first seventy patients who had reflex sympathetic dystrophy and were less than eighteen years old (average age, 12.5 years). In our series, the patients were predominantly girls (male to female ratio, 11:59) and the lower extremity was involved most often (sixty-one of the seventy patients). The average time from the initial injury to the diagnosis was one year, which indicates that the syndrome remains under-recognized in patients in this age-group. Conservative treatment with physical therapy, transcutaneous electrical nerve stimulation, psychological therapies including cognitive-behavioral management and relaxation training, and tricyclic anti-depressants was effective in improving the average scores for pain and function for forty patients. Sympathetic blocks were helpful for twenty-eight of thirty-seven patients. Thirty-eight of the seventy patients in the series continued to have some degree of residual pain and dysfunction. Reflex sympathetic dystrophy in children differs in presentation and clinical course from the syndrome in adults. It is best treated in a multidisciplinary fashion.
Reflex sympathetic dystrophy (RSD) is an uncommonly reported entity in children. This paper reports five cases of RSD in children and summarizes 80 cases of pediatric RSD reported in the literature. The diagnosis is based on the clinical findings of pain, dysesthesia, and autonomic instability. Tache cérébrale, not previously described in the diagnosis of RSD, is a helpful sign of vasomotor dysfunction. RSD in childhood frequently affects the lower extremities, in contrast to the adult localization around the shoulders and hands. Noninvasive, nonpharmacologic management is generally successful. A simple outpatient program of massage and mobilization was beneficial in four of the five patients treated in this study.
• Reflex sympathetic dystrophy syndrome is a well-recognized disorder in adults, but it is rarely diagnosed in the pediatric age group. This report summarizes our experience with this condition from 1975 to 1985. We diagnosed, treated, and followed up this condition in 18 children and adolescents. The condition usually followed trauma. The most prominent feature in all patients was a constant limb pain with episodes of paroxysmal exacerbation. The pain was associated with two or more of the following: edema, hyperhidrosis or anhidrosis, cyanosis or erythema, and, in severe cases, dystrophic skin changes and muscle atrophy. Roentgenograms were normal. Bone scans were helpful to exclude other possible causes of bone and joint pain. Reflex sympathetic dystrophy syndrome in children probably often goes unrecognized, sometimes being confused with psychiatric conditions such as conversion reaction and malingering. Reflex sympathetic dystrophy syndrome should always be considered in the differential diagnosis of unexplained persistent limb pain in children: early recognition and proper management may result in the prevention of potentially crippling sequelae.
(AJDC 1988;142:1325-1330)
During the past 6 years, ten children with reflex sympathetic dystrophy were treated. Pain in an extremity was the initial complaint in all patients. The pain was unilateral in 90% of the patients; upper and lower extremities were affected with equal frequency. Tenderness to palpation, extreme hyperesthesia, and dysesthesia were other dominant features. All patients had some evidence of autonomic nervous system dysfunction in the affected extremity (swelling, color change, decreased temperature, and/or hyperhidrosis). The median duration of symptoms prior to referral and diagnosis was 5 months. All children were treated as outpatients with a transcutaneous electric nerve stimulator and home-based physical therapy. With this regimen, seven patients had complete remission within 2 months. Two other patients improved with transcutaneous electric nerve stimulation therapy, and one patient had no response to transcutaneous electric nerve stimulation. Reflex sympathetic dystrophy is frequently underdiagnosed in children. Increased awareness of this syndrome is important because accurate diagnosis is crucial and transcutaneous electric nerve stimulation offers a safe, simple, and effective outpatient therapy for reflex sympathetic dystrophy in children.
Psychosocial factors in 21 families with children affected by reflex neurovascular dystrophy were studied. Each family was interviewed and given a battery of standardized psychologic tests. Two distinct types of families were identified. Fifteen families showed high internal cohesion, expressiveness, and organization and low levels of conflict. Six families showed high overt conflict with low levels of family cohesion, expressiveness, and organization. In all families parental enmeshment with the patient was present. Marital discord was present in 12 families. Thirteen patients had significant school problems (ten had learning disabilities). Although most of the children were described as especially bright, only four had above average intelligence test scores. Four had a history of sexual abuse. The patients and their mothers perceived the health problem as significantly worse than did children with arthritis from whom similar scores had been obtained. Possible role models with similar symptoms were reported by ten patients. These data support the concept that childhood reflex neurovascular dystrophy is frequently a stress-related disease; the therapeutic approach to treating these children and their families must take these psychosocial factors into account.
A crossover study was performed for patients with RSD to evaluate the therapeutic efficacy of the hydroxyl radical scavenger DMSO. All patients were given DMSO locally 5 times a day during one week, and a placebo during one week. Before and after each treatment, subjective evaluation was performed by both the patient and the examinor as to clinical activity of RSD, and measurement was performed of the range of motion (ROM) of all joints in the affected extremity. DMSO was the most effective treatment as to improvement of ROM (p = 0.035) and as to overall improvement (p = 0.001). The efficacy of the hydroxyl radical scavenger DMSO indicates that RSD primarily involves an inflammatory process rather than a sympathetic reflex. As during the last 20 years no single report was published studying RSD in terms of inflammation, it is suggested that such studies are urgently needed to elucidate the real nature of RSD.
The pathogenesis of reflex sympathetic dystrophy--variously known as Sudeck's atrophy, causalgia, algodystrophy, and peripheral trophoneurosis--is not yet understood, and diagnosing and treating patients is difficult. We have prospectively studied 829 patients, paying particular attention to early signs and symptoms. In its early phase, reflex sympathetic dystrophy is characterised by regional inflammation, which increases after muscular exercise. Pain was present in 93% of patients, and hypoaesthesia and hyperpathy were present in 69% and 75% respectively. With time, tissue atrophy may occur as well as involuntary movements, muscle spasms, or pseudoparalysis. Tremor was found in 49% and muscular incoordination in 54% of patients. Sympathetic signs such as hyperhidrosis are infrequent and therefore have no diagnostic value. We found no evidence consistent with the presence of three consecutive phases of the disease. Early symptoms are those of an inflammatory reaction and not of a disturbance of the sympathetic nervous system. These data support the concept of an exaggerated regional inflammatory response to injury or operation in reflex sympathetic dystrophy.
To design diagnostic criteria for reflex sympathetic dystrophy (RSD) and to initiate a prospective treatment protocol, we reviewed our experience with 49 episodes of RSD in 36 children. There were 24 females and 12 males; mean age at diagnosis was 13.4 years (range: 8 to 19); mean time from pain onset to correct diagnosis was 9.2 months (range: 1 to 53). Lower extremity involvement predominated. Pain was "severe" in 61%, and skin color changes, swelling, hyperesthesia, abnormal skin temperatures, muscle weakness, and decreased range of motion were all present in at least 75% of cases. Osteopenia was observed in 15 of 38 radiographs; of 24 bone scans, 7 were normal, 11 showed increased uptake, and 6 demonstrated decreased uptake. Of the 23 children who had psychological evaluations, 83% revealed some type of significant emotional dysfunction. Analgesic and antiinflammatory medications were not helpful, nor were local injections or regional blockades effective. An inpatient diagnostic and rehabilitation program for treating chronic pain, including orthopedics, rheumatology, psychology, and twice-daily physical therapy was most likely to lead to resumption of age-appropriate activities. Despite extensive physiological testing, physician, parent, and/or patient reluctance to accept absence of a primary organic disease was common. We present diagnostic criteria for pediatric RSD.
We present a revised taxonomic system for disorders previously called reflex sympathetic dystrophy (RSD) and causalgia. The system resulted from a special consensus conference that was convened on this topic and is based upon the patient's history, presenting symptoms, and findings at the time of diagnosis. The disorders are grouped under the umbrella term CRPS: complex regional pain syndrome. This overall term, CRPS, requires the presence of regional pain and sensory changes following a noxious event. Further, the pain is associated with findings such as abnormal skin color, temperature change, abnormal sudomotor activity, or edema. The combination of these findings exceeds their expected magnitude in response to known physical damage during and following the inciting event. Two types of CRPS have been recognized: type I, corresponds to RSD and occurs without a definable nerve lesion, and type II, formerly called causalgia refers to cases where a definable nerve lesion is present. The term sympathetically maintained pain (SMP) was also evaluated and considered to be a variable phenomenon associated with a variety of disorders, including CRPS types I and II. These revised categories have been included in the 2nd edition of the IASP Classification of Chronic Pain Syndromes.
One hundred patients who had sustained a Colles' fracture were observed for features of algodystrophy at 1, 5, 9 and 12 weeks following injury. The diagnosis of algodystrophy was possible as soon as 1 week after fracture. Early diagnosis has important clinical implications: the aetiological factors may become apparent and different treatment modalities be identified; furthermore, early treatment can be started, limiting the morbidity of the condition. It is proposed that patients with features of algodystrophy require physiotherapy after a Colles' fracture. Those without features may not.
The systemic inflammatory response syndrome (SIRS) and acute reflex sympathetic dystrophy syndrome (RSD) share clinical signs of severe inflammation, a protracted course, and a similar problem of impaired oxygen utilization. The difference is that SIRS patients have these signs and symptoms systemically and are severely ill in the intensive care unit (ICU), whereas acute RSD patients are in good health and their problems are limited to one extremity. Both conditions seem to be the result of an exaggerated inflammatory response. As RSD patients have a healthy contralateral extremity, they may be their own control in various flux studies. It is hypothesized that this situation is exquisitely suitable for studying the pathophysiology of severe inflammatory responses in humans. Only a few patients are required to perform studies of, for example, oxygen metabolism and cytokine or oxygen radical production. Assessment methods may be utilized, such as nuclear magnetic resonance spectroscopy, which cannot easily be performed in ICU patients.
To report the initial and long-term outcome after an intensive exercise therapy program for childhood complex regional pain syndrome, type I (CRPS).
Prospective follow-up.
A children's hospital.
We followed 103 children (87 girls; mean age = 13.0 years) with CRPS. Forty-nine subjects were followed for more than 2 years (mean = 5 years 3 months).
An intensive exercise program (most received a daily program of 4 hours of aerobic, functionally directed exercises, 1-2 hours of hydrotherapy, and desensitization). No medications or modalities were used. All had a screening psychological evaluation, and 79 (77%) were referred for psychological counseling.
Outcomes included pain, presence of physical dysfunction, or recurrent episodes of CRPS or other disproportional musculoskeletal pain.
The mean duration of exercise therapy was 14 days, but over the past 2 years has decreased to 6 days. Ninety-five children (92%) initially became symptom free. Of those followed for more than 2 years, 43 (88%) were symptom free (15, or 31 %, of these patients had had a reoccurrence), 5 (10%) were fully functional but had some continued pain, and 1 (2%) had functional limitations. The median time to recurrence was 2 months; 79% of the recurrences were during the first 6 months after treatment.
Intense exercise therapy is effective in initially treating childhood CRPS and is associated with low rate of long-term symptoms or dysfunction.
This study tested for evidence supporting the clinical lore of three sequential stages of complex regional pain syndrome (CRPS) and examined the characteristics of possible CRPS subtypes. A series of 113 patients meeting IASP criteria for CRPS underwent standardized history and physical examinations to assess CRPS signs and symptoms in four domains identified in previous research: pain/sensory abnormalities, vasomotor dysfunction, edema/sudomotor dysfunction, and motor/trophic changes. K-Means cluster analysis was used to derive three relatively homogeneous CRPS patient subgroups based on similarity of sign/symptom patterns in these domains. The resulting CRPS subgroups did not differ significantly regarding pain duration as might be expected in a sequential staging model. However, the derived subgroups were statistically-distinct, and suggested three possible CRPS subtypes: (1) a relatively limited syndrome with vasomotor signs predominating, (2) a relatively limited syndrome with neuropathic pain/sensory abnormalities predominating, and (3) a florid CRPS syndrome similar to "classic RSD" descriptions. Subtype 3 showed the highest levels of motor/trophic signs and possible disuse-related changes (osteopenia) on bone scan, despite having directionally the briefest pain duration of the three groups. EMG/NCV testing suggests that Subtype 2 may reflect CRPS-Type 2 (causalgia). Overall, these results are consistent with limited previous work that argues against three sequential stages of CRPS. However, several distinct CRPS subtypes are suggested, and these could ultimately have utility in targeting treatment more effectively.
Implications:
We report our experience with ketorolac/lidocaine IV regional anesthesia (Bier block) (IVRA) in two adolescents with complex regional pain syndrome 1. IVRA resulted in complete resolution of symptoms.
Because complex regional pain syndrome--type I usually occurs following minor trauma, clients will most likely be encountered in community settings. Nurses will come into contact with them as family members, friends, neighbors, and coworkers. It is important that nurses become knowledgeable about this syndrome to assist in early recognition, diagnosis, and treatment. The latest research findings have relevance for current practice, but many questions remain unanswered.
This work was undertaken to identify scintigraphic patterns obtained in patients suspected of having reflex sympathetic dystrophy (RSD), now often referred to as complex regional pain syndrome, whose interpretations could be difficult.
Ten patients had bone scans because of clinical suspicion of RSD in the lower legs. They were selected retrospectively to have a wide sample of scintigraphic patterns. The radionuclide images and a multiple-choice questionnaire were presented as a PowerPoint file that was sent electronically on the Internet to 54 Belgian nuclear medicine physicians. They had to determine whether the images were in favor of the diagnosis of RSD.
Twenty-eight answers (52%) were received. There was near-complete interobserver agreement for perfectly normal scans, for scans showing diffuse uptake with enhancement of periarticular activity, and for scans showing only focal hyperactivity at the site of previous trauma. Results were more discordant when the hyperactivity was mild and when there was a diffuse hypoactivity, with or without focal hyperactivity.
This study shows that using very simple methodology, it is possible to identify some scintigraphic patterns in which there is disagreement among observers and whose interpretations vary. As the results are returned to the participants, they can compare their own interpretations with those of their peers. This aspect could be useful in continuing education in medical imaging.
Recurrent complex regional pain syndrome I is not rare in the pediatric population. The authors conducted this study to evaluate the efficacy of continuous peripheral nerve blocks with elastomeric disposable pumps associated with initial Bier blocks for the treatment of recurrent complex regional pain syndrome I in children.
After parental informed consent, 13 children who did not respond to conventional complex regional pain syndrome treatment were included (mean age, 13 yr; range, 9-16 yr). After general anesthesia, peripheral nerve block was performed using 0.5 ml/kg lidocaine, 1%, with epinephrine and 0.5% ropivacaine injected in the peripheral nerve block catheter. Then, a 20-min Bier block was performed using a tourniquet and 0.2 ml/kg lidocaine, 1%; 3 ml/kg hydroxyethyl starch 130/06; and 5 mg/kg buflomedil injected intravenously. A solution of 0.1 ml . kg . h continuous ropivacaine, 0.2%, was infused through the catheter using an elastomeric pump for 96 h. Need for rescue analgesia, occurrence of side effects, and status of motor and sensory block were recorded at hours 1, 6, 12, 24, 48, 72, and 96. Children and parents completed a satisfaction assessment. All of the children had follow-up visits after 2 months.
Postoperative analgesia was excellent. The median pain score was 0 for each period studied. Motor blockade was minimal before 12 h (median, 1) and absent thereafter. One child needed rescue analgesia. All children were able to walk easily after the initial 24-h period (walking score, > 4). Children and parents were all satisfied. Children returned home under parental surveillance beginning in the 24th hour. Neither peripheral nerve block nor Bier block caused side effects. After 2 months, none of the children exhibited any clinical symptom of recurrent complex regional pain syndrome.
Ambulatory continuous peripheral nerve block associated with an initial Bier block seems to be a significant and novel contribution to treat recurrent pediatric complex regional pain syndrome I. It allows complete pain relief, early mobilization, and rapid return home, representing a psychological advantage for these children.
Complex regional pain syndrome type I (CRPS-I) is a complex disorder characterised by pain, autonomic dysfunction, and decreased range of motion. The syndrome was believed as a well-recognized disorder in adults but, less commonly recognized in children. CRPS-I after vaccination has been rarely reported. We reported an 11-year-old young girl with CRPS-I due to rubella vaccine. © 2004 European Federation of Chapters of the International Association for the Study of Pain. Published by Elsevier Ltd. All rights reserved.