Complex regional pain syndrome in children

INTRODUCTION — Complex regional pain syndrome (CRPS) is an amplified musculoskeletal pain syndrome that is characterized by extreme pain in a limb out of proportion to the history and physical findings, accompanied by one or more signs of autonomic dysfunction [1,2]. Although CRPS was first reported in 1864 [3], it was not until 1971 that CRPS was described in a child [4]. Since the initial pediatric report, CRPS is increasingly recognized in pediatric pain, rheumatology, and neurology clinics [5-7].

The clinical manifestations, diagnosis, and management of CRPS in children are reviewed here. CRPS in adults is discussed separately. (See "Complex regional pain syndrome in adults: Pathogenesis, clinical manifestations, and diagnosis" and "Complex regional pain syndrome in adults: Treatment, prognosis, and prevention".)

CATEGORIZATION — Alternative names for CRPS in the literature include reflex sympathetic dystrophy (RSD), algodystrophy, causalgia, Sudeck atrophy, transient osteoporosis, and acute atrophy of bone. In 1995, a consensus conference grouped these disorders under a single heading of CRPS [8].

CRPS is divided into two categories:

●Type I occurs in patients with CRPS without a definable nerve lesion.

●Type II, referred to as causalgia, occurs in cases where a definable nerve lesion is present.

Children with CRPS overwhelmingly have type I disease.

Childhood versus adult CRPS — CRPS in children substantially differs from that seen in adults [5-7,9-17].

●Children are less likely than adults to have a history of an inciting injury or noxious insult.

●The leg is more often involved, and the skin temperature of the involved extremity is more often cooler in children than adults.

●Neurologic symptoms are less pronounced in children.

●Psychologic issues play a more prominent role in children than adults.

●Children typically have a better outcome and are much more responsive to physical and occupational therapy alone than are adults.

PATHOGENESIS — The pathogenesis of CRPS is unknown but likely involves the local and central nervous system (CNS), as well as psychologic, immune, and genetic factors.

Local factors — Significant preceding trauma is not a common event in most pediatric series, but CRPS can follow surgery, fractures, motor vehicle accidents, soft tissue injury, and injections [18,19]. CRPS that follows trauma usually starts within days or sometimes a few weeks in the healing phase of the injury. The average time between injury and onset of CRPS was four days in one series of 78 children with CRPS [9]. Limb immobility has been associated with the risk for the development of CRPS after physical trauma [20].

Peripheral nerve ischemia can be identified by technetium bone scintigraphy or magnetic resonance imaging (MRI) and has been implicated in the development of CRPS [21]. Ischemic nerve injury may lead to small fiber neuropathy and subsequent amplification of pain by central sensitization [21-23].

Role of the central nervous system — Data supporting the role of the CNS in the pathogenesis and spread of pain in CRPS include the following:

●In adults, magnetoencephalography demonstrates extensive reorganization of the S1 cortex on the contralateral side of the affected limb, which is reversed once the pain subsides [24].

●Functional MRI in children demonstrates changes in basal ganglia and parietal lobe activation that persist even after CRPS resolution and findings suggestive of pain-induced activation of the endogenous pain modulatory system [25].

●Significant changes were seen in the gray matter in multiple areas of the brain and functional connectivity of brain networks associated with sensory, motor, emotional, cognitive, and pain regions of the brains of children when symptomatic compared with controls [26,27]. These changes rapidly improved after treatment with intense physical and occupational therapy.

Psychologic issues — Psychologic stress and other comorbid psychologic disorders are commonly reported in pediatric CRPS [5,11,15,28-30]. It is unclear whether these psychologic disorders are due to CNS factors that play a role in the etiology of CRPS or the result of the stress and anxiety related to CRPS. As in adults, children with CRPS experience a greater number of stressful life events [31,32].

Children with CRPS are often described as overly mature, with an incongruent affect (eg, cheerful appearance while reporting severe pain). In addition, conversion (functional neurologic symptom) disorder is not uncommon. The author has cared for children with CRPS who have developed nonepileptic seizures, conversional blindness, paralysis, gait impairment, muscle spasms (both stiff limbs or movement disorders), and incapacitating dizziness. Other psychologic conditions seen in children with CRPS include disordered eating, suicidal gestures, and self-injury (such as cutting) behavior [33,34]. Data from the author's center suggest that children with the highest rate of a second occurrence are those who have a prior suicide attempt. (See "Suicidal ideation and behavior in children and adolescents: Evaluation and management" and "Eating disorders: Overview of epidemiology, clinical features, and diagnosis".)

Genetic predisposition — Genetic factors may be involved in the pathogenesis of CRPS:

●In a study of patients recruited through the Dutch Association of CRPS, 31 families were identified who had two or more affected members [35]. Patients with a family history of CRPS developed the disease at a younger age and appeared to have more severe findings than sporadic cases.

●In a study from a tertiary metabolic genetic practice, eight children who had dysautonomic conditions secondary to maternally inherited mitochondrial disease also met the diagnostic criteria for CRPS [36].

●The author cares for three sets of identical twins with CRPS and multiple families with more than one member affected.

EPIDEMIOLOGY — The incidence of CRPS in children is estimated at 1 per 100,000 children [37,38] and appears to be higher in females and children age 12 or older [37,39]. In a large pediatric series, CRPS is more common in females (70 percent), and the mean age at diagnosis is 13 years of age (range of 5 to 17 years) [6,7,11]. Although the youngest reported child with CRPS was two years of age [40], CRPS is unusual under the age of seven years.

There is an association between CRPS and immunization [19,41-43]. However, the possibility that the immunization is causal has been questioned on several fronts. Different immunizations have preceded CRPS. An alternative explanation is that the development of CRPS is related to the injury associated with the needle jab since CRPS seems to develop within minutes to hours of immunization; however, postimmunization reaction is less common in younger children even though they receive more vaccines in a single visit. In a Danish national registry involving nearly 1.4 million females aged 10 to 44 years, no elevated risk of CRPS over the subsequent year was seen in those who received quadrivalent human papillomavirus vaccine (risk rate ratio 1.31, 95% CI 0.91-1.90), suggesting no causal association [41]. Similarly, a United States study of more than 1.2 million 11-year-old females receiving the HPV vaccine found no elevated risk of CRPS in the subsequent 30-, 90-, and 180-day intervals post-immunization [39].

CLINICAL MANIFESTATIONS — CRPS is defined as pain that is out of proportion to the history and physical findings of the patient and is associated with at least one sign of autonomic dysfunction (temperature asymmetry, skin color change, edema, sweating asymmetry, dystrophic changes) and one symptom (allodynia, hyperalgesia, motor dysfunction). Pain typically occurs in a single extremity, most commonly a lower extremity, but pain at multiple sites can occur.

In one large case series of 70 children with CRPS, the following findings and their incidences were observed in the affected extremity [7]:

●Pain – 100 percent

●Allodynia (pain due to normally non-noxious stimuli) – 86 percent

●Edema – 77 percent

●Cyanosis – 73 percent

●Hyperhidrosis (excessive sweating) – 31 percent

Pain — Pain typically occurs in a lower extremity but may occur in an upper extremity or at multiple sites. Most patients will experience allodynia and are unable to tolerate light touch or clothing on the limb. The borders of allodynia vary by 4 to 6 cm with retesting [6]. Children with CRPS frequently hold the involved extremity in an unusual position, and the limb is cool and cyanotic (picture 1A and picture 1B).

Patients typically have an incongruent affect, that is they appear cheerful while reporting pain that they score as a 10 out of 10 on a pain scale (10 is the maximum score for pain).

The history may elicit the following findings:

●Minor trauma

●Increasing pain over time

●Pain that is no better or worsens with immobilization

●Pain that may spread beyond the initial site with new areas that may or may not have overt signs of autonomic dysfunction

●Migratory pain

●A greater degree of disability than expected

Autonomic dysfunction — Signs of autonomic dysfunction include:

●Decreased skin temperature

●Cyanosis

●Edema (picture 1D)

●Increased sweating

Sleep disturbances — Disturbed or interrupted sleep commonly occurs in children with CRPS. The vast majority will complain of difficulty getting to sleep and frequent awakenings, but generally they do not fall asleep during the day. We do not recommend treating the sleep complaint per se.

Psychologic findings — Psychologic stress and dysfunction are common in children with CRPS and their families [15]. Children with CRPS are often described as bright, high achieving, and accomplished and whose pain becomes the central focus of the family, leading to significant stress within the family.

Other psychologic conditions seen in children with CRPS include motor symptoms of a functional neurologic disorder (eg, conversion gait, stiffness [sometimes labeled as dystonia]), somatization, disordered eating, and self-injury behaviors [29,30,33,34,44].

Other findings — Less common clinical findings in children with CRPS include dystrophic skin changes (picture 1C), bony edema, osteoporosis, and increased hair or nail growth over the affected area.

DIAGNOSIS — CRPS is diagnosed clinically, and there are no uniform criteria for children. However, most experts include the following criteria:

●Pain that is out of proportion to the history and physical findings

●Accompanied by at least one sign of autonomic dysfunction (temperature asymmetry, skin color change, edema, sweating asymmetry, dystrophic changes) and one symptom (allodynia, hyperalgesia, motor dysfunction)

●No other cause or, if there is an underlying condition, the pain and dysfunction are not in proportion to the degree of biologic damage

Laboratory studies — There are no laboratory studies that confirm or exclude the diagnosis of CRPS. Laboratory evaluation, which includes blood studies and imaging, is primarily performed to determine whether another pathologic process is present early in the disease course when the characteristic findings of CRPS are not present and the diagnosis is less certain. (See 'Differential diagnosis' below.)

Blood tests — The following blood tests generally are normal in children with CRPS:

●Complete blood count (CBC)

●Serum electrolytes, blood urea nitrogen (BUN), and creatinine

●Erythrocyte sedimentation rate (ESR)

Other conditions in the differential diagnosis for CRPS may have abnormal blood tests (eg, elevated ESR in osteomyelitis).

Imaging studies — Radiographs and other imaging modalities (ie, magnetic resonance imaging [MRI], bilateral thermography, and bone scans) do not reliably differentiate CRPS from other disease processes, such as trauma.

Radiographs of the affected extremity are normal in patients with CRPS and are generally obtained to exclude the presence of a fracture or, uncommonly, a tumor. However, a normal radiograph does not entirely exclude the presence of a fracture. Radiographs may show evidence of osteoporosis in cases of prolonged CRPS [4,45].

In children with CRPS, technetium bone scintigraphy usually shows decrease uptake, but a spotty increased pattern may be seen (the pattern more common in adult CRPS) or the study may be normal [46,47]. A normal bone scan excludes osteomyelitis, osteoid osteoma, or a stress fracture, but it does not exclude the diagnosis of CRPS. An abnormal scan is subject to differing interpretations, especially if the findings are subtle [48].

Magnetic resonance scans in children with CRPS can show bony edema and small joint effusions, but it is difficult to distinguish these findings from trauma [49,50]. Such edema should not dissuade one from the diagnosis of CRPS if the history is not suggestive of trauma, the clinical findings are typical of CRPS, or the duration is such that trauma should be healed.

Doppler ultrasound should only be performed in patients in whom a diagnosis of thrombus is highly suspected as the cause of pain.

Other studies — Once the clinical diagnosis of CRPS is made, it is counterproductive to do further studies as they delay treatment and put doubts about the diagnosis in the mind of the patient and family. The following studies have been used to diagnose CRPS in children, but generally they are not helpful. The author does not recommend their routine use in the evaluation of pediatric CRPS.

●Electromyographic or nerve conduction studies are usually normal in children with CRPS, although nonspecific, mild slowing of the nerve conduction may be found.

●Autonomic testing includes measurements of resting sweat output, resting skin temperature, and quantitative sudomotor axon reflex test (QSART). Although these tests are used in adult patients, they play no useful role in the diagnosis of children with CRPS. (See "Complex regional pain syndrome in adults: Pathogenesis, clinical manifestations, and diagnosis", section on 'Autonomic testing'.)

●Tilt table testing is generally normal in children with CRPS and is not helpful in the diagnosis [51].

●Thermography is not helpful in the diagnosis of pediatric CRPS [52].

DIFFERENTIAL DIAGNOSIS — The differential diagnosis of CRPS includes the following:

●Trauma, which is distinguished from CRPS by history and imaging studies.

●Inflammatory conditions (eg, juvenile idiopathic arthritis [JIA] or osteomyelitis [bacterial or nonbacterial]), which may be differentiated from CRPS by local findings of swelling, enthesitis, erythema, and systemic findings of fever. Laboratory findings often include elevations of inflammatory markers, such as white blood cell count, erythrocyte sedimentation rate, and C-reactive protein. (See "Classification of juvenile idiopathic arthritis" and "Hematogenous osteomyelitis in children: Evaluation and diagnosis".)

However, JIA may also be a risk factor for childhood CRPS, and some children can develop both JIA and CRPS [53].

●Tumors – The physical finding of soft tissue mass and corresponding radiological findings differentiate bony tumors (eg, osteosarcoma) from CRPS. (See "Osteosarcoma: Epidemiology, pathology, clinical presentation, and diagnosis".)

MANAGEMENT — Treatment goals for pediatric CRPS are, first, to restore function and, second, pain relief. If pain cannot be totally eliminated, the goal is adjusted to assist the child in developing skills to cope with residual pain while maintaining function.

There is no proven definitive treatment, the disease course varies with unpredictable and sometimes rapid remission of symptoms, and the underlying cause of the disease is unknown.

Our approach — Ideally, care is provided by a multidisciplinary team that includes a physical therapist, occupational therapist, child psychologist or behavior therapist, and a primary care or other clinician experienced in treating pediatric CRPS. A social worker helps with school reentry and school issues.

The consensus of expert clinicians in the field and a structured literature-scoping review is that physical and occupational therapy and psychotherapy are the mainstays of treatment for this disorder [5,6,10,11,13,17,18,28,54-58]. Treatment is most effective when coupled with patient and family education. The earlier one starts treatment, the less the child suffers. However, outcomes are associated with psychologic variables (attempted suicide and disordered eating portended subsequent episodes), not duration of symptoms [6]. There has been an increase in the medicalization of these children over time, which can increase the risk of iatrogenic injury and burden families with additional medical costs [59].

The management approach is as follows:

●Education – A complete explanation of CRPS is provided to the patient and family. This includes acknowledging the existence of real pain but that the pain does not signify damage even though it is at times severe (amplified). The most effective therapy, based upon the author's experience, is to use the affected limb despite pain and desensitizing techniques if allodynia is present.

●Physical and occupational therapy – A home exercise and desensitization regimen is developed for each person and encourages the patient to use the affected limb as much as possible. The regimen's goals are 45 minutes of daily aerobic activities and 5- to 10-minute blocks of desensitization (ie, rubbing, brushing, patting, and clothing) performed multiple times throughout the day.

For patients not making significant progress after four to eight weeks with a home-based regimen, a more intense hospital-based day program typically consisting of physical and occupational therapy for five to six hours each day may help restore function regardless of pain [60]. Patients are treated in the ambulatory setting with one-on-one supervision. Parents and caregivers are not allowed in the gym. They are encouraged to return to their normal daily routine and take care of their own needs. If there are significant extenuating circumstances, physical or psychologic, patients are admitted to the inpatient service for more in-depth, functional, behavioral, and psychologic intervention. (See 'Physical and occupational therapy' below.)

●Psychologic and behavioral therapy – All children and their families undergo psychologic screening to detect any undue stress and comorbid psychologic conditions. Patients with significant psychologic issues are referred to a pediatric psychologist or psychiatrist before a formal exercise program can safely begin.

Patients who require the intense physical and occupational program also have scheduled twice-weekly sessions (more if indicated) that address any psychologic issues ("talk time"), as well as music and art therapy sessions. (See 'Psychologic and behavioral therapy' below.)

●Medications – Any medications for pain are stopped or, if needed, are tapered. No further diagnostic tests are ordered unless new symptoms warrant them (radiograph following a significant fall or injury). (See 'Pharmacologic therapy' below.)

●Sleep interventions – Sleep disturbances are managed by nonpharmacologic sleep hygiene recommendations. These include:

•No caffeinated beverages.

•No use of electronic devices one to two hours before bedtime.

•Set bedtime and get-up times.

•Darken room.

•No exercise two hours prior to bedtime.

•If the child is unable to fall asleep within 30 minutes, the child is allowed to get up and do a quiet, non-stimulating activity without disturbing the parents or caregivers.

●School work and transition – Patients who require a more intense physical and occupational therapy regimen are not expected to do schoolwork during this time period. However, academic accommodations and resources are allowed so patients can make up work prior to their return to school. In the transition back to school, any physical accommodations (eg, use of elevator and increased time between classes) are eliminated since the children are functional. In children with significant anxiety about school, school transition days are used so that they go to school one day and return to the program to troubleshoot difficulties. In a few children, academic testing is helpful in identifying previously unidentified or unaddressed learning difficulties that can greatly increase the stress of school [15].

Physical and occupational therapy — Several case series have reported excellent outcomes in children with the use of physical and occupational therapy in children with CRPS (picture 1D) [5,6,61]. These programs are geared toward having the child maintain function of the affected limb and work through the pain. The frequency, duration, and intensity of therapy required vary. Some individuals may need five to six hours of daily physical and occupational therapy for several weeks to restore full function [6].

The use of exercise without medication was first reported in 1978 in 23 children in Los Angeles, with reduced disease manifestations and improved function seen in all patients [5]. In one case series of 103 children in Seattle, an initial daily exercise program that included four hours of aerobic, functionally directed exercises, hydrotherapy, and desensitization without medications or pain reduction procedures resulted in complete remission in 92 percent of patients [6]. The mean duration of the exercise program was 14 days. All patients had a screening psychologic evaluation, and 79 (77 percent) were referred for psychologic counseling. Of the patients who were followed for a mean of five years, 88 percent remained symptom free despite one-third of patients having a second episode during the follow-up period. Subsequent episodes were responsive to reinstating the exercise regimen. This experience was replicated by the Portland and Pittsburgh groups, where 89 percent of 32 children had resolution of all pain and 95 percent regained full function [62]. The Boston group has also reported that 95 percent of 56 children with CRPS were significantly better with exercise therapy as the mainstay of treatment [56].

A small trial of 28 children with CRPS compared once-weekly with three-times-weekly physical therapy sessions for six weeks along with weekly cognitive-behavioral sessions [61]. Both groups showed improvement in the five outcome measures of pain and physical functioning at a mean follow-up of 10 weeks. Excellent functional outcome was reported in 21 of the 24 patients who participated in long-term follow-up (mean 2.5 years). The study sample was too small to determine whether the frequency of sessions had an effect on outcome.

The dose of physical and occupational therapy varies from patient to patient. Some children do well with a home exercise and desensitization program, others benefit from a more intensive hospital-based day program, and a few report spontaneous recovery after reassurance and virtually no therapy [6,63].

Psychologic and behavioral therapy — Although there are no clinical trials evaluating the effectiveness of psychologic and behavioral therapy, these interventions are generally used based upon observational reports of their beneficial effect [6,7,61,64].

Psychologic and behavioral therapeutic interventions include [4]:

●Relaxation training

●Biofeedback

●Cognitive-behavioral therapy to improve stress management

●Family intervention to eliminate reinforcement of negative behaviors (avoidance of school or social obligations)

●Family intervention to promote coping techniques

These interventions are probably helpful because they decrease the stress and anxiety that commonly occur in these children and their families. As a rule, family therapy is most useful in younger patients, whereas older children require both individual and family intervention.

A substantial number of children have comorbid psychologic conditions, such as conversion (functional neurologic symptom) disorder, depression, school avoidance, or an eating disorder, and require consultations with a child psychologist or psychiatrist.

Pharmacologic therapy — There are no prospective, randomized clinical trials of pharmacologic agents in the treatment of CRPS in children, although there is one trial of treatment of neuropathic pain that included 20 children with CRPS. In that study, patients showed modest improvement with both gabapentin and amitriptyline, but no difference was seen between these agents, perhaps indicating regression to mean [65]. In addition, there are no trials comparing intensive physical therapy alone or in combination with a pharmacologic agent. As a result, the role of pharmacologic agents in the treatment of children with CRPS remains uncertain and controversial [18]. Some experts, including the author, advocate only intensive physical therapy without medications because of the potential of drug side effects and lack of clear benefit [6,66]. Although case reports or small series report beneficial effects of medications such as gabapentin, amitriptyline, oxcarbazepine, and bisphosphonates, the efficacy of these medications has not been confirmed in clinical trials [67-70]. Glucocorticoid therapy was not effective in reducing the symptoms of CRPS in one case series of six children [28]. Medications are more commonly used in adults with CRPS. This is discussed in detail separately. (See "Complex regional pain syndrome in adults: Treatment, prognosis, and prevention", section on 'Approach to pharmacotherapy'.)

There is no role for opioids in children with CRPS. The evidence for the use of opioids in adults with neuropathic pain is equivocal at best [71], and their side effects far outweigh their uncertain benefit in children with CRPS.

One of the standards suggested by the European Pain Federation task force emphasizes that plans to stop medications that are started, if not effective, need to be in place [57]. Continuing ineffective medication or adding medication to medication is not in the patient's best interest.

Pain-reducing procedures — There are no randomized trials to support the use of transcutaneous electrical nerve stimulation (TENS), regional sympathetic nerve block, epidural and intrathecal anesthetics, sympathectomy, or amputation in children with CRPS, and their risks probably outweigh their benefits in most cases [7,18,66,67,72-77]. Based upon the author's experience, none of these procedures are indicated in the vast majority of children with CRPS.

These procedures are discussed in greater detail separately in adults with CRPS. (See "Complex regional pain syndrome in adults: Treatment, prognosis, and prevention", section on 'Interventional options for refractory pain'.)

PREVENTION — Unlike adults, there is no intervention to prevent the first episode of CRPS in children because pediatric CRPS is uncommon and rarely occurs after bony fractures.

PROGNOSIS — The vast majority of children with CRPS has a favorable prognosis. In previously discussed case series, complete remission approached 90 percent in patients treated with intense exercise and counseling [6,56,62]. Children who have a second episode generally respond to the reintroduction of physical and occupational therapy [6,62]. The psychologic aspects of a second episode must be addressed in affected children as these children are more likely to develop other stress-related conditions, such as non-limb pain (eg, headache or abdominal pain), self-injury, eating disorders, school avoidance, or conversion (functional neurologic symptom) disorder. The children at greater risk of a subsequent episode had more psychologic issues at presentation (ie, prior suicide attempts and eating disorders) [6]. It is generally best to treat it early in the disease course. Although the numbers are quite limited, there are mixed data regarding long-term prognosis and those with delayed treatment [78].

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Complex regional pain syndrome".)

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Here are the patient education articles that are relevant to this topic. We encourage you to print or email these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

●Basics topic (see "Patient education: Complex regional pain syndrome (The Basics)")

SUMMARY AND RECOMMENDATIONS

●Clinical manifestations – The clinical manifestation of complex regional pain syndrome (CRPS) include pain, autonomic dysfunction, sleep disturbances, and psychologic stress and dysfunction. (See 'Clinical manifestations' above.)

•Pain typically presents in a single lower extremity. Most patients experience allodynia (pain due to normally non-noxious stimulation) and are unable to tolerate light touch or clothing on the limb. (See 'Pain' above.)

•Autonomic dysfunction is present in the affected limb. Signs may include decreased skin temperature, cyanosis, edema, or increased sweating (picture 1A-D). (See 'Autonomic dysfunction' above.)

•Psychologic stress and sleep dysfunction are common in children with CRPS and their families. Other psychologic conditions seen in children with CRPS include conversion reactions, school avoidance, eating disorders, and self-injury behavior. (See 'Sleep disturbances' above and 'Psychologic findings' above.)

●Clinical diagnosis – CRPS is diagnosed clinically based on the presence of pain out of proportion to the history and physical findings and one finding of autonomic dysfunction. (See 'Diagnosis' above.)

There are no laboratory or imaging studies that confirm or exclude CRPS. Laboratory and imaging evaluations are primarily performed to exclude other conditions that may present with extremity pain including trauma (eg, fracture), inflammatory conditions (eg, osteomyelitis and juvenile idiopathic arthritis), and tumors (eg, osteosarcoma).

●Treatment approach – The treatment goals are restoration of function and relief of pain. If pain cannot be totally limited, the goal shifts to helping the child cope with pain while maintaining function. Management of pediatric CRPS is challenging because there is no proven definitive treatment and the disease course varies with unpredictable and sometimes rapid remission of symptoms. Options include physical and occupational therapy, psychotherapy, pharmacologic therapy, and pain-reducing procedures. (See 'Management' above.)

•We suggest physical and occupational therapy as initial therapy (Grade 2C).

•We suggest the addition of psychologic and behavioral therapy to address the stress and anxiety that commonly occur in affected patients and their families (Grade 2C).

•We suggest against pharmacologic therapy (Grade 2C). There are no data that medications provide additional benefit over nonpharmacologic measures, and they have potential severe side effects.

•Pain-reducing invasive procedures are reserved for patients with symptoms refractory to other therapies.

●Prognosis – The majority of children with CRPS have a favorable outcome with restored function of the affected limb. (See 'Prognosis' above.)