The treatment goals for pediatric CRPS are pain relief and restoration of function. Therefore all multidisciplinary treatment programs are believed to be optimal.
To date, the consensus of clinical experts is that a multidisciplinary approach, which provides noninvasive interventions including education, physical and occupational therapy, analgesics and psychotherapy and/or Cognitive-Behavioral Therapy (CBT) is the mainstay of treatment. (7) The invasive procedures used are intravenous administration of adrenergic inhibitors, peripheral nerve blocks, epidural analgesia, sympathetic nerve blocks, and surgical sympathectomy.
As with all chronic pain syndromes, CRPS should be managed based on the biopsychosocial model, either on an outpatient or an inpatient basis if required. (70)
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The earliest possible mobilization and behavioral support can provide an essential reversal of the presenting signs and symptoms. (7, 73, 162)
Pharmacological treatments:
A large amount of the treatment of pediatric CRPS has been extrapolated from adult data and standard therapies for nociceptive or neuropathic pain.
The standard treatments for nociceptive pain are Paracetamol, nonsteroidal anti-inflammatory drugs (NSAIDs), glucocorticoid drugs, and opiates. The standard treatments for neuropathic pain are
antidepressants, anticonvulsants, and topical analgesic patches. These treatments are also prescribed to facilitate the child’s participation in functional rehabilitation therapy.
Although trials of antidepressants, anticonvulsants, and nonsteroidal medications are often used, there is little high-quality efficacy data among pediatric patients. Because of the lack of such studies in the pediatric population with CRPS, the restrained efficacy of these drugs, the risks of adverse effects and the scarcity of long-term effects information, some authors consider that these drugs should not be advocated or that they should be discontinued if used.
A retrospective study by Wilder et al. of 70 pediatric patients between 5 and 17 years old, investigated the efficacy of nonsteroidal anti-inflammatory, steroid, a tricyclic antidepressant, anticonvulsant, and opioid medications. Their authors reported that 60% of patients found that NSAIDs were ineffective to relieve pain. Anticonvulsants were ineffective in the treatment of pain in 58%, whereas opioids in 60%
of patients. A hundred percent of patients who received steroids had no improvement in pain or function. Tricyclic antidepressants led to an improvement in both pain and sleep disturbances in 50%
of patients. (161)
Low et al. reported that 70% of children with CRPS received amitriptyline, gabapentin or both as adjuvant treatment to facilitate mobilization during physiotherapy sessions and these drugs were reduced and when possible discontinued when symptoms subsided. (55, 67, 78)
Low-dose tricyclic antidepressants are also sometimes used for patients who have sleep disturbances due to pain. A baseline ECG is recommended to rule out cardiac abnormalities before starting tricyclic antidepressants. (16, 70, 155)
These drugs, tricyclic antidepressants (TCAs, such as amitriptyline) and anticonvulsants (gabapentin), as well as serotonin-norepinephrine reuptake inhibitors (SNRIs), modulate the pathways leading to central sensitization act by increasing the level of biogenic amines and reducing the spontaneous ectopic discharges. The use of antidepressant drugs may also be necessary when the child has
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psychological comorbidities such as depression or anxiety. These may be preexisting or may result from the chronic pain itself. The presence of depression is known to have a considerable impact on the outcomes of CRPS treatment as well as anxiety and the fear of pain.
Many other drugs (ketamine, immunoglobulins, baclofen, botulinum toxin, angiotensin-converting enzyme inhibitors) have been used in case-reports based on different pathophysiological hypotheses without consensus to date to recommend them. (70)
Physical rehabilitation:
Functional rehabilitation therapy is a cornerstone treatment of pediatric CRPS patients and has excellent results. It includes physiotherapy, occupational therapy, sensory rehabilitation, Scottish baths, and elastic taping. (70)
For some children with chronic pain, the pain leads to significant disability, often resulting in missed school, extracurricular activities, and poor socialization with peers. Physical therapy and occupational therapy aim to help the patient become more physically active as well as return to age-appropriate activities. Thus, the focus is primarily on improving the patient return to age-appropriate functioning rather than reducing pain. Therefore, the physiotherapy sessions are focused on restoring the function of the affected limb by increasing loads, strength, and joint motion range. This frequently contradicts the patient’s logic, where rest is viewed as a means to alleviate the pain.
However, there is no consensus about the duration, frequency, or content of the physical rehabilitation treatment. Lee et al. made a prospective, randomized, single-blind trial of a six-week program with physical therapy (PT) and cognitive-behavioral treatment (CBT) (PT once or 3 times per week with six sessions of CBT in both groups) with 28 children and adolescents with CRPS. He reported reduced pain and improved function (89%) in all patients with no statically significant differences between the two groups at follow-up. (73)
A case series published by Logan et al. examined the success rate of a day-hospital approach with multidisciplinary treatment in pediatric patients with refractory CRPS. The management of CRPS is an initial outpatient treatment plan of physical therapy and cognitive behavioral therapy aimed at active mobilization of the affected limb, desensitization, and strengthening combined with learning active coping strategies. Patients who do not improve with outpatient treatment or those with more severe disabilities go on an inpatient or day-hospital program that integrates more intense active exercise with psychological interventions, reintegration to school, and psychoeducation. In the study, 56 patients, (age 8 to 18, median=14) participated in daily physical therapy, occupational therapy, and
psychological therapy for a median duration of 3 weeks. The authors found statistically significant improvements from admission to discharge in pain intensity, functional disability, subjective reports of
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limb function, and emotional functioning. The majority of patients maintained functional improvement and reduced pain at long-term follow-up. (77)
The second edition UK guidelines for CRPS in adults edited by the Royal College of Physicians in 2018 recommend treatment approaches that therapists should refer as appropriate if there is no improvement after four weeks. However, they make therapists aware that successive use of multiple methods may lead to prolonged treatment. (116)
The therapeutic approaches proposed are:
- patient education and support
- Self-administered tactile and thermal desensitization to normalize touch perception (see appendix 5)
- general exercises and strengthening - functional activities
- mirror visual feedback - gait re-education
- transcutaneous electrical nerve stimulation (TENS) - postural control
- pacing, prioritizing and planning activities - goal setting
- facilitating self-management of the condition - splinting (generally short term, in acute CRPS)
These are treatment approaches for adults, and so are the CRPS-specific rehabilitation techniques proposed:
- graded motor imagery - tactile discrimination
- strategies to correct body perception disturbance, involving looking, touching and thinking about the affected body part
- mental visualization to normalize the altered size and form perception of the involved body Part
- functional movement techniques to improve motor control and awareness of affected limb
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- principles of stress loading
- conflict allodynia re-education to reduce the fear of physical contact with others in community settings
- management of CRPS-related dystonia
Because of the importance of the active participation of the patient, UK guidelines for CRPS in adults propose to give written leaflets to patients about desensitization to help them practice at home. (116)
See Annexe 9: Appendix 5 (Desensitisation), p. 64.
Psychological therapy:
Psychological therapies are a mainstay in the management of pediatric CRPS and are recommended as an integral part of the multidisciplinary treatment approach. Psychological therapies include cognitive-behavioral therapy (CBT), operant conditioning (OC), counseling, pain education, and relaxation techniques. (24)
Psychologic stress and dysfunction are commonly described in children with CRPS and their families.
Furthermore, a substantial number of children with CRPS have comorbid psychologic conditions, such as anxiety, depression, school avoidance, conversion, or an eating disorder, and require consultations with a child psychiatrist. Therefore patients and their families should have a psychological assessment to evaluate potential issues, whether they are individual, familial, social or academic.
Psychological and behavioral factors can exacerbate the pain and dysfunction associated with CRPS in some patients, and psychological therapies address unhelpful pain-related behaviors and beliefs that could help maintain the condition and teach pain coping and management strategies. (24, 105) Cognitive therapy can help the child by targeting thoughts that serve to increase his/her anxiety and discomfort associated with the pain. For example, when the child feels helpless about his/her pain and has catastrophic thinking regarding his/her pain, it can increase his/her disability associated with the pain experience. Cognitive therapy helps the child understand the links between the thoughts, feelings, and behaviors and to become more aware of his/her thoughts and reframe them (in cognitive
behavioral therapy) or accept them (in acceptance and commitment therapy).
Parental Behaviour Therapy focuses on parental cognitions and behaviors which have a significant impact on the pain perception and expression of the child with chronic pain. Parents are taught to minimize their attention to the child’s pain (e.g. no more questions about the pain nor excessive
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reassurance) and instead provide attention for their child’s coping attempts (e.g. attending school, using relaxation). Clear expectations for functioning are established, and parents may be taught to use a reward strategy to encourage their child when succeeding and to remove privileges if their child is unable to meet expectations such as school attendance for example. Positive self-talk is another strategy that is a common component of CBT treatments. Training in relaxation has been a core component of pain management treatment for a long time, and recent meta-analytic reviews strengthen its efficacy for pain reduction. (17)
Although there are no current large, prospective, blinded, placebo-controlled studies of the efficacy of cognitive and behavioral strategies for treating CRPS in children nor in adults, cognitive-behavioral therapies (CBT) have been widely used since decades and are the only psychiatric treatments reported to date in published studies about CRPS. Furthermore, the benefit of psychological therapies has been demonstrated in many studies of pediatric patients with chronic pain.
Efficacy of Psychological Interventions in CRPS Patients:
Wilder et al. (161) Case series Multidisciplinary treatment N = 70 child/adolescent including relaxation training
and CBT
→ Significantly improved pain and function in 57% of patients
Lee et al. (73) Randomized trial PT 1x week + CBT (N = 14), N = 28 child/adolescent PT 3x week + CBT (N = 14)
→Pain and function improved significantly pre-post for both groups. Recurrence rate = 50%
Sherry et al. (124) Case series Multidisciplinary treatment N = 103 child/adolescent including psychotherapy for
77% of sample
→Complete symptom resolution in 92% of sample at end of treatment, 88% symptom-free at 2 year follow-up
Eccleston et al. (36) Case series Interdisciplinary 3 weeks N= 57 child/adolescent residential programme of
group CBT
→Post-treatment: significant improvement in disability and physical function; 3 mo: significant improvement in disability, physical function, anxiety, and somatic awareness
In another study by Sherry et al. psychotherapy (individual or family) was the psychological
intervention with children with musculoskeletal pain (children with CRPS were not included). (123) But those studies with psychotherapy instead of CBT are scarce in the literature to date.
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Hechler et al. reported in a study positive outcomes in adolescents (N=167, five patients with CRPS) suffering from chronic pain three months after multimodal inpatient pain treatment.
The treatment duration was three weeks and delivered by a team including pediatricians, clinical psychologists, children and adolescent psychiatrists, pediatric nurses, physiotherapists, occupational therapists, and social workers.
The treatment included 3 to 4 single psychotherapy sessions, one therapeutic session with the family of the patient, and two group-therapy each week, operant and cognitive-behavioral principles were incorporated in all aspects of treatment. Besides, trauma-specific interventions (stabilizing
interventions or Eye movement desensitization and reprocessing) were included in the individual sessions if children reported traumatic events and demonstrated severe emotional distress related to that event even though not necessarily fulfilling the criteria of a post-traumatic stress disorder.
Homework was an essential part of each treatment session. Similar to the child, parents were actively engaged in the treatment process by initiating changes in daily routines (e.g., supporting the child to attend school despite the pain). Also, they activated the adolescent through everyday activities such as sport. Music and art therapy was provided by trained music and art therapist every week. Total time in treatment summed up to approximately 5 to 8 hours per day. Reintegration into the adolescent’s daily life was initiated from the second week onwards comprising of visits at home and school.
Treatment modules were: (1) education (eg, on vicious cycle of pain and development of a pain memory) and a realistic goal determination, (2) acquisition of pain coping strategies (eg, attention defocusing techniques, cognitive-behavioral approach), (3) treatment of co-occurring emotional distress (eg, respondent and operant techniques to reduce anxiety), (4) family therapy (eg, reducing pain reinforcing behaviors), (5) adjunctive therapy (eg, analgesia, physiotherapy, or art therapy), and (6) relapse prevention. (57, 59)
Education about the pathology being part of the treatment, the UK guidelines for CRPS in adults propose to provide written information about CRPS to patients, cf Appendix 12: (116)
See Annexe 10: Appendix 12 (Patient Information), p. 66.
In the 4th edition of diagnostic and treatment guidelines for CRPS (2013), Harden et al.suggested a psychological intervention algorithm (55):
See Annexe 11:
Psychological intervention treatment algorithm by Harden et al., p. 68.
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In 2018, the UK guidelines for CRPS in adults proposed key contents of an interdisciplinary specialist rehabilitation program, cf Appendix 13: (116)
See Annexe 12: Appendix 13 (Key contents of an interdisciplinary specialist rehabilitation programme), p. 69.
7. PROGNOSIS
Pediatric CRPS patients have a milder and more benign disease course than adults. Average delays for pediatric CRPS diagnosis were commonly up to a year a decade ago but in recent literature, the length of time varied from as early as one week to 41 weeks, most likely due to increasing awareness of CRPS among pediatric providers. (16)
In a study by Borucki et al., the average delay in diagnosis was about three months. They noted that in children who were diagnosed early (G3 months), symptom resolution occurred much more rapidly than in those diagnosed later (10.6 vs. 21.5 weeks, respectively). (79) Delayed diagnosis is associated with a longer disease course.
Pediatric CRPS appears to have a good prognosis with generally more favorable outcomes compared to adults. A high percentage of pediatric CRPS patients are expected to have complete resolution of their symptoms and signs with a multidisciplinary strategy treatment approach without the need for any invasive treatment. Wilder et al. found that younger patients had an easier pathway than older patients in their study of 70 CRPS pediatric cases and that "a younger age at the time of the injury correlated with less pain, better function, fewer remaining signs of autonomic dysfunction on follow-up and a shorter total duration of symptoms." (161)
Nevertheless, the condition is not benign, with many patients requiring a long period of treatment, and the relapse rate in pediatric patients has been reported to range between 20 and 50% (79), but actually, CRPS recurrence rates ranged more from 28% to 36%. (16, 73, 99, 124, 135)
Besides the risk of relapse, patients are often scared that their CRPS could also spread, but this is rather rare, occurring in about 7% of all cases, although transient pain in other limbs may be more common. (116)
Kachko et al. reported a case series with 14 pediatric CRPS with a 93 % rate of full or partial recovery with treatment and a 29 % relapse rate. (67)
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In a retrospective review by Brooke et al. of 32 children with CRPS, 89 % had an eventual resolution of pain, and 95% had full restoration of physical function at a median time from the start of treatment of 2 months. The treatment was an intensive inpatient rehabilitation with physical and occupation therapy in conjunction with psychological therapy. Relapses were reported in 7 children (32%) at follow-up, and five were able to resolve the recurrence without further medical intervention. (21) Sherry et al. studied the short- and long-term outcomes of 103 children with CRPS-I treated with an intensive exercise program including exercise therapy, hydrotherapy, and desensitization for all patients and psychological counseling for 81 patients (77%) after psychological assessment. The mean duration of the treatment was 14 days initially and decreased to 6 days over the two years of the study.
In this study 92 % of the children became symptom-free initially, 49 children continued follow-up for a mean of 5 years: 88 % of them were fully symptom-free, and 31 % had had a relapse of symptoms during the period of their follow-up, which resolved with the restoration of an exercise program. The median time to recurrence was two months, with 79% of recurrences occurring during the first six months after treatment. (124)
Other reports assumed that the prognosis of pediatric CRPS is not as favorable as reported. Martin et al. interviewed 143 patients (aged 5 to 23) who experienced chronic pain (9% of the cohort had CRPS), with a mean of 3 years after their last visit at a pediatric pain clinic and found that 62% of the cohort had continuing pain. (88)
Tan et al. investigated the quality of life in adults treated for childhood-onset CRPS-I, with a median age of 13.2 years when diagnosed and the median follow-up period was 12 years (range 2 to 22). At follow-up, 52 % of patients experienced pain. Recurrence of signs and symptoms that could be attributed to CRPS-I was 63 %, and relapse of documented CRPS was found in 33 % of cases. (142)
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8. ELABORATION OF A CHILD PSYCHIATRY CARE PROTOCOL FOR CHILDREN WITH CRPS IN THE DEPARTMENT OF WOMEN, CHILDREN AND ADOLESCENTS AT THE UNIVERSITY HOSPITAL OF GENEVA
In our clinic, we treat children with CRPS with a multidisciplinary approach. The multidisciplinary team consists of orthopedic surgeons for children, anesthesiologists specializing in the treatment of pain in children, physiotherapists, occupational therapists, nurses, and child psychiatrists.
See Annexe 13: for the tables outline the care provided in our clinic, Decision tree for suspected then confirmed complex regional pain syndrome type I (70), p. 70.
As described in this decision tree (Annexe 13), the child psychiatrist meets the young person suffering from CRPS at the beginning of the multidisciplinary care. A psychiatric evaluation of both the youth and the family system is carried out to identify potential associated psychiatric pathologies, to evaluate the functioning of the youth and the family system. Parental guidance is provided in parallel with therapeutic sessions with the young person. If the indication for cognitive-behavioral therapy is indicated, the young person is referred to a CBT therapist.
When the young person does not show any functional improvement during multidisciplinary outpatient care, a short hospital stay is scheduled.
During this hospitalization, the young person benefits from intensive care, as shown in a table with example of the schedule for a patient admitted for 1 week to treat complex regional pain syndrome type I:
See Annexe 14: Example of the schedule for a patient admitted for 1 week to treat complex regional pain syndrome type I (70), p.71.
This table summarizes the multidisciplinary approach with, as recommended as the current treatment of this pathology, both physiotherapy, occupational therapy, occupational activities, school time, visits with the pain management team, art therapy, music therapy, and child psychiatry sessions.
To optimize child psychiatry care in the CRPS clinic, this thesis proposes, following a review of the literature to date, a child psychiatry care protocol for these young people within our hospital. This protocol should help the therapist in the management of these children, whether they are in an ambulatory or hospital care unit.
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The protocol should include several treatment areas, as already described:
- Psychoeducation
- Psychiatric evaluation and psychotherapy +/- medication
- Cognitive interventions addressing dysfunctional cognitions (e.g., catastrophizing cognitions are common in CRPS patients. It is therefore essential to help patients learn to identify their specific dysfunctional cognitions regarding CRPS, and replace them with more adaptive cognitions)
- Behavioral Interventions (eg reframing ; given the consensus guidelines for CRPS management on an active rehabilitation approach, it is necessary to reframe the CRPS
- Behavioral Interventions (eg reframing ; given the consensus guidelines for CRPS management on an active rehabilitation approach, it is necessary to reframe the CRPS