Abstract
Complex regional pain syndrome is a syndrome that can present in a variety of ways. It is characterized by pain and can also be threatening to both quality of life and function. Patients are also frequently depressed and anxious as a result of this disease. A logical and systematic approach to address the many facets of treatment must be utilized. The initial treatment plan therefore must be continuously reassessed and updated based on patient progress from acute injury to a potential chronic life-altering disease.
Keywords
Alternative treatment, CRPS, Desensitization, Edema, Interdisciplinary, Mirror visual feedback, Mobilization
Introduction
This chapter is meant to complement the other chapters in this book on complex regional pain syndrome (CRPS). Its goal is not to review all of the treatments that are available. Rather, it is an attempt to create a progressive treatment approach to what is often an arduous and potentially chronic problem. There is also a discussion of evidence base surrounding rehabilitative approaches to treatment.
A syndrome is defined as “a group of signs and symptoms that occur together and characterize a particular abnormality or condition or a set of concurrent things (as emotions or actions) that usually form an identifiable pattern.” CRPS is a syndrome that can present in a variety of ways. It is characterized by varying degrees by pain and vasomotor, sudomotor, and motor changes. It is not only painful but also threatening to both quality of life and function. Patients are frequently depressed and anxious as a result of the severity of the impact of this disease on their lives. Further complexity is added to the diagnosis and treatment by the fact that it can be a complication of nociceptive and/or neuropathic injuries.
With all of these variables, coupled with the fact that it is a relatively infrequent syndrome (see introductory chapter on CRPS), it becomes challenging to objectively evaluate treatments. Diagnostic criteria have also changed over the years, and outcome measures vary between studies. Thus, there is a dearth of evidence base surrounding its treatments. Recent treatment guidelines depend on systemic reviews, randomized and controlled studies, retrospective studies, open-label trials, pilot studies and anecdotes, case reports, and clinical experience to arrive at recommendations.
Furthermore, there is a paucity in disease-specific outcome measures that are designed to specifically evaluate CRPS. The Radboud skills questionnaire was constructed to analyze disability in patients with upper extremity reflex sympathetic dystrophy. A multitude of scales and questionnaires have been used in studies on CRPS. The neuropathic pain scale is designed to assess specific pain qualities associated with neuropathic pain. Nonspecific unidimensional scales, such as the visual analogue scale, numeric rating scale, and verbal rating scale, have been used to evaluate pain intensity. The McGill Pain is a general questionnaire that evaluates pain intensity as well as the sensory, affective, and evaluative dimensions of pain. Other examples of common questionnaires used in the assessment of depression include the Minnesota Multiphasic Personality Inventory and the Beck Depression Inventory. The State Trait Anxiety Inventory is a questionnaire that evaluates anxiety. Further work needs to be done to create questionnaires that specifically evaluate function, pain, quality of life, depression, anxiety, and costs associated with treatment so that we are better able to evaluate treatment interventions.
Although there is a suboptimal literature that supports many of our proven treatments, it is incumbent on the practitioner to formulate a logical approach to address the many facets of treatment that must be utilized to treat the physiologic and functional challenges that beset the patient. Pain, swelling, range-of-motion deficits, weakness and dystonias, functional deficits, depression and anxiety, and social and financial problems must all be addressed in the treatment plan. The initial treatment plan must be continually reassessed and updated based on the progress or lack thereof in the individual case in light of new and changing signs and symptoms that may develop as the syndrome passes from an acute injury to a potential chronic life-altering disease.
Interdisciplinary Treatment
There are many specialties of healthcare practitioners who treat pain. Physicians specializing in surgery (neurosurgery, orthopedics, vascular, general), anesthesia, neurology, psychiatry, family medicine, and rehabilitative medicine may have special interest in pain management. Other healthcare professionals, such as physical therapists, occupational therapists, psychologists, social workers, nurses, recreational therapists, and vocational therapists, may play a part in treatment. These practitioners may treat patients as individuals or as part of a multidisciplinary or interdisciplinary team of professionals.
An interdisciplinary team is a group of healthcare professionals from complementary fields who work in tandem to treat a patient. The core team frequently consists of a physician (often a physiatrist), physical therapist, occupational therapist, and psychologist. Social work, nursing, recreational therapist, and vocational therapist may be involved. The team meets on a regular basis to discuss progress and treatment adjustments. The team leader is often the physician or psychologist. Although these are practitioners with different backgrounds and expertise, they have the individual and complementary skills and the team approach helps to foster communication.
Perhaps the most important part of the team is the patient. The patient meets with these various team members to establish goals, including pain management and relief, function (activities of daily living [ADLs], ambulation, recreation, vocation, childcare, sexuality, etc.), mood, and anxiety. The patient must be motivated to achieve these goals, or the program cannot succeed.
Harden and Cole suggested that interdisciplinary treatment “always be considered early in every complicated case” with a neuropathic pain syndrome. Interdisciplinary pain treatment has long been a standard of care for treatment of chronic nonmalignant pain. Peters et al. reported that 68% of inpatients and 61% of outpatients who received multidisciplinary treatment had improvements in pain rating, analgesic use, and activity levels. A meta-analysis of 65 studies evaluating multidisciplinary treatments for chronic back pain tended to support the improvement of pain, mood, return to work, and use of the healthcare system in a stable fashion. Caution was urged because of limitations in the quality of the studies. Guzman et al. also reviewed multidisciplinary treatment of back pain and concluded that there was moderate evidence that intensive biopsychosocial rehabilitation with functional restoration improves function relative to nonmultidisciplinary rehabilitation. Contradictory evidence existed regarding vocational outcomes with these programs.
Singh et al. treated a group of 12 patients with CRPS type I for 4 weeks with physical therapy, occupational therapy, water therapy, psychotherapy, stellate ganglion blocks, and drug therapy. Anxiety levels remained stable and function improved. At 2-year follow-up, 75% of patients were working. Daly et al. rated this study as good-quality evidence that an outpatient treatment program is effective for CRPS.
The concept behind treating patients with complicated biopsychological syndromes in a multidisciplinary way is to reset the altered central processing and normalize the distal environmental level. There have been three consensus meetings that have gradually molded a therapeutic approach to the treatment of CRPS. Treatment addresses function immediately. ADLs and mobility issues should be addressed. Medication, procedural treatment, and psychological treatments are gradually worked into the treatment as the clinical course unfolds.
The initial step in treating the patient involves a comprehensive evaluation. A thorough history and physical examination may lead to an underlying problem that can be treated effectively, such as an unstable fracture or a compressed nerve. The patient’s concurrent medical problems must be understood, because these other diagnoses may have an impact on pain or on the potential treatments that may be considered for pain. Underlying psychological issues and the presence of depression or anxiety must be addressed. Inability to perform simple tasks, such as washing and dressing, homemaking, toiletry, childcare, vocational activities, and recreational activities, must be a part of the history. The functional requirements of the patient must also be understood, and goals should be created to address those specific activities whenever feasible. Strife in relationships with a spouse, parent, child, or coworker or boss may be factors in care.
Therapeutic Treatment
The initial therapeutic treatment begins with the activation of presensorimotor cortices via motor imagery, visual and tactile discrimination, and desensitization. Treatment guidelines have been created, but these are conceptual and flexible and should flow in the context of the individual’s specific needs (see Fig. 3.1 ). It progresses to edema control, range of motion, and isometric strengthening. Treatment of posture and secondary positional deviations ensues. As the patient tolerates more, isotonic and isokinetic strengthening can be added in combination with stress loading. Aerobic conditioning and postural strategies are utilized. More advanced strategies are added as the patient makes progress, including ergonomics, movement strategies, and normalization of use, and vocational and functional rehabilitation progresses to hierarchical goals as the syndrome improves. The progression of the treatment should not be based on a time sequence because of the variability in the course of the syndrome. When there is lack of progress, medication and psychological treatment and procedural interventions may be considered if appropriate.
Physical therapy and occupational therapy often form a complementary team with separate but potentially overlapping roles. It has been stated that “physical therapy is the cornerstone and first line treatment for CRPS.” In addition to mobilization, the physical therapist addresses functional activities, including transfers and ambulation on levels, steps, and uneven surfaces. The occupational therapist may help with some of the transfer activities but concentrates more on upper extremity mobilization and strengthening as well as functional activities such as ADLs, homemaking, childcare, vocational activities, and any other functional goals that are important to the individual patient. “Functional restoration” is at the core of treatment of CRPS. Several studies have supported the benefits of these disciplines in CRPS.
Mirror Visual Feedback and Graded Motor Imagery
Mirror visual feedback (MVF) was first used by Ramachandran and Roger-Ramachandran in amputees to lessen phantom pain It has also subsequently been used to treat phantom limb pain, stroke, CRPS, and persistent pain following wrist fracture and hand surgery. In 2003, McCabe et al. performed a pilot study with MVF in eight patients with CRPS type 1. After 6 weeks, patients with disease that was present for less than 1 year had normalization of pain and improved thermal changes. No improvements were found in patients with chronic CRPS.
Mechanism of action for MVF is unclear, but proposed mechanisms revolve around correcting or improving the mismatch between the motor and sensory systems in CRPS, improving attention to the painful limb, lessening kinesiophobia, and improving body perception disturbance. Moseley et al. demonstrated that a limb could be cooler if that limb was perceived as less important than the contralateral limb. Moseley also demonstrated that imagined movements can also cause pain and swelling in a patient with CRPS. Patients with CRPS have a distorted body image. Patients with chronic pain develop shrunken or enlarged changes in the primary motor and sensory maps with representations of the affected body part, as seen on functional MRI. Changes are also seen in the thalamus, visual cortex, and brainstem. Pain reduction may cause reversal of these changes.
McCabe emphasizes that MVF “is not a stand-alone therapy but should be used within a multidisciplinary rehabilitation program and may instead of pay be a useful adjunct to desensitization therapy.” As in most treatments in CRPS (not unlike many other pain syndromes) there is a limited evidence base supporting MVF use and even less information supporting any specific protocol. O’Connell et al. concluded that there was low-quality evidence that mirror therapy reduced pain and improved upper limb function in poststroke CRPS compared with covered mirror therapy and the effect may have moderate clinical significance.
The patient is generally introduced to the idea of mirror therapy by having them create an awareness of the limbs in their body and trying to help them to understand the difference in how their brain perceives the affected limb versus the contralateral unaffected limb. The concept may be explained to the patient as an attempt to “trick” the brain into improving the image of the damaged extremity. Imagined movements may be rehearsed before the initiation of MVF. McCabe suggests the “Bath MVF Treatment Protocol.” The affected limb is placed on the nonreflective side of the mirror, whereas the unaffected limb is seen in the reflective surface of the mirror ( Fig. 3.2 ). The patient is asked to gradually begin movements bilaterally and synchronously. The patient is asked to perform this technique “little, but often” up to five or six times per day for no more than 5 min. Treatment should be discontinued if pain levels increase, if tremor and/or dystonia worsens significantly, and if the patient cannot understand the treatment. Some patients may not be able to move the hidden affected extremity when it is hidden from their vision, even though the limb is capable of movement when it is not hidden; this is called motor extinction. It may be related to deficits in attention in patients with impaired sensory feedback, leading to difficulty with motor function. This represents a severe disturbance in body image. MVF should not be used in these patients, as it may increase pain and motor deficits.
Moseley hypothesized that activating the premotor cortex might be improved by starting out with a treatment that does not initially involve movement through the use of motor imagery. Part of the theory was that pain would be less likely to occur with imagined movements and that pain and motor function are so closely related that movement execution commands may induce pain. In 2004 a standardized motor imagery plan was evaluated in 26 patients with chronic upper extremity CRPS I over 6 weeks. Patients were treated with three stages, each of 2 weeks’ duration. Thirteen of the patients received the motor imagery program and were compared with 13 patients who received normal management; the control group then crossed over to the motor imagery program.
Stage one consisted of identification of hand laterality. Forty-two pictures of the right and left hand (total of 82 pictures) in various postures were presented in a random order on a monitor in front of a seated patient. The patient pressed a button as rapidly as possible to identify which hand was on the screen. This activity was performed on a computer and was to be performed three times per each waking hour. It took approximately 10 min.
In stage two the patient performs imagined hand movements with the affected hand that were similar to 28 pictures of the same hand in various postures randomly selected from a picture bank. Patients performed the task three times per hour for the entire time that they were awake. Emphasis was on accuracy and not on speed.
Stage three involves mirror therapy; the patient simulates postures similar to 20 pictures of the unaffected hand that were seen on paper copies. They were told to perform this activity with both hands with the use of mirror therapy 10 times each waking hour with the affected hand concealed behind the nonreflective part of the mirror while they watched the unaffected hand perform these movements in the mirror.
Treatment resulted in significant decrease in the pain intensity and finger circumference, which was maintained for 12 weeks. The control group achieved similar gains after it crossed over to the motor imagery program. At 12 weeks after the program, a total of six patients (four in the initial motor imagery program and two from the control group after it crossed over) no longer fulfilled the criteria for CRPS I. Moseley performed a larger randomized controlled study with 51 patients with CRPS or phantom pain; gains in pain and function were made in the group that was treated with graded motor imagery (GMI).
Moseley confirmed that the sequence of preceding mirror therapy with imagined movement before mirror imagery was critical for successful treatment in 2005 when he randomized the order of treatment. Hand laterality recognition/imagined movements/mirror movements were compared with imagined movements/hand laterality recognition/imagined movements and hand laterality recognition/mirror group/hand laterality recognition. The group of hand laterality recognition/imagined movements/mirror movements made the greatest gains in pain and disability. The hand laterality recognition diminished pain and disability in all groups to a lesser degree, and imagined movements lessened pain and disability only when they followed hand laterality recognition. The mirror therapy reduced pain and disability only when it followed imagined movements. The conclusion was that it is important to sequentially activate the premotor and motor networks before mirror therapy to achieve the best outcome.
Bowering et al. performed a literature review and concluded that GMI and mirror therapy alone may be effective, but there is limited evidence. It was concluded that rigorous studies are needed in a wider population with pain. In a separate review of interventions of CRPS, O’Connell et al. opined that there is low-quality evidence that GMI and medical management are more effective at reducing pain and improving function than medical management alone and that ordered GMI is more effective than unordered GMI in reducing pain in patients with CRPS I.
Desensitization
Desensitization is critical early in CRPS to allow the patient to begin to mobilize the injured limb. Pleger et al. demonstrated that intractable pain in CRPS resulted in shrinkage in cortical maps on the contralateral primary (SI) and secondary somatosensory cortex as demonstrated on functional MRI. Behavioral treatment over 1–6 months with sensorimotor retuning (desensitization and graded sensory and motor tasks) resulted in decreased pain, improved tactile discrimination, and improvement in the map size in the primary and secondary somatosensory cortex. Proprioceptive feedback enhancement via vibratory stimulation also led to less pain and improved proprioception.
Edema Control
Swelling and range of motion of the upper extremity must be addressed in tandem with desensitization. Edema control may be accomplished with training the patient to position the affected limb in an attitude that allows fluid to drain from the swollen extremity. Centripetal massage, Coban wrapping (when tolerated), and pressure garments may also be utilized to combat swelling. Contrast baths improve circulation by alternating vasodilation (heat) with vasoconstriction (cold). The heat and cold are also beneficial in desensitization. Splinting, when tolerated, may be employed to maintain or increase motion but has a limited role, and active motion is encouraged.
Mobilization and Strengthening
The clinician who is attempting to mobilize the affected limb or joint must overcome the patient’s fear of movement (kinesiophobia). This fear is based both on fear of nociception and the actual degree of pain that may be encountered with mobilization. Learned behaviors may result in less activity and secondary weakness and functional deficits as well. In back pain, lowering fear may reduce avoidance of motion and improve function. In an attempt to reduce pain-fears and subsequent functional deficit, de Jong et al. utilized graded exposure in vivo (GEXP) as a treatment modality in CRPS I. Eight patients were taught about the vicious cycle posed by pain, catastrophic thought, fear, and avoidance and lessening function with subsequent worsening of pain. They then were placed into a program in which they were gradually exposed to situations that patients thought were dangerous or threatening to their painful extremity. Patients were initially encouraged to perform these activities while in treatment and then, eventually, on their own. The therapist was gradually withdrawn. Pain-related fear, pain intensity, disability, and physiologic signs and symptoms all improved.
A balance in intervention must be achieved that allows the patient to progress in range of motion and strength, which are the predicates of function. Peripheral nociceptive stimulation, which is a part of CRPS, sustains central sensitization. Overaggressive mobilization may exacerbate this process. By normalizing motion and activity, the idea is to normalize the afferent input and lessen the central nervous system hypersensitivity. Improving motion and activity in a given extremity lessens pain, range of motion, osteoporosis, swelling, and autonomic disturbance.
Pain exposure physical therapy (PEPT) directly exposes patients to painful stimuli. Patients are advised to return to activity without medication; they are advised to ignore the pain. Patients and their involved family members are educated about the condition and the program; the patient’s partner is directed to abandon nurturing behavior and is instructed to motivate the patient to be active. The therapists and the patient’s partner are to ignore verbal and nonverbal expressions of pain. Self-massage, range of motion, and progressive-loading exercises are instituted. Positive reinforcement is given for progression of movement and activity. Assistive devices such as crutches or canes are discouraged. The goal is to restore function, with the secondary effect being decrease in pain and secondary consequences of immobilization and inactivity.
PEPT has been studied in CRPS several times with mixed results. Ek and van Gijn retrospectively evaluated 106 patients with chronic CRPS treated with PEPT. Functional improvement was seen in 95 patients and full functional recovery was seen in 49 patients, although 23 of those patients had increased pain. van de Meent et al. studied 20 patients with CRPS treated with PEPT and improvements were noted in pain intensity, muscle strength, upper extremity function, walking speed, and kinesiophobia. On the other hand, Barnhoom et al. found more equivocal results with PEPT. Conventional treatment was compared with PEPT in a randomized controlled study of 56 patients with CRPS I. Patients in both groups made significant gains, but there was no significant difference in gains between the two groups other than greater improvement in active range of motion in the PEPT patients.
As patients improve, more advanced mobilization treatments may be employed. Carlson and Watson describe a stress-loading program that utilizes active exercises that minimize joint motion while placing stress through the upper extremity. The patient utilizes a scrub brush to scrub a plywood board with gradual increase in time and pressure. They also carry a weighted briefcase or bag in the affected hand when the patient stands or walks throughout the day. They studied 41 patients over 2 years and patients reported improvements in pain, range of motion, and grip strength. Of 31 patients who worked, 26 people returned to their normal job and 95% of patients returned to full duty, although 50% of them still had difficulty with heavy lifting, buttons, and handling change. As the patient makes progress, stress loading with ambulation and functional activities important to their everyday function (vocational and recreational activities) can be introduced.
Other options to mobilize patients who have difficulty with weight bearing and pain may include aquatic therapy. In general, water must be warm. The buoyancy of the water allows for lesser forces with weight bearing while the water provides some resistance. It has been used with some success in children. As the patient tolerates more weight bearing in the water, weight bearing on land can gradually be introduced and progressed as tolerated.
Recreational therapy can be utilized to help motivate patients to incorporate their personal interests with their exercise routine. Having patients participate in an activity that they perceive as pleasurable may help them to overcome the pain and fear associated with movement of the injured extremity. Socialization with other patients may also be fostered through recreational activities. One of the goals of the good practitioner is to help patients begin to take responsibility for their own care. Having patients enjoy their movement therapy can certainly provide a bridge to independence with exercise. Group therapies with other patients with disabilities or similar injuries can often provide a great support system. Patients may be willing to try to perform activities that they were loath to perform on their own, with the support of a group that is experiencing similar physical problems. Feldenkrais, yoga, and tai chi are other “mind-body strategies” that can be options for the patient.
Vocational therapy can help to create a program that allows the patient to get back to work. Work conditioning/hardening can be an option for the more advanced patient. The patient may need to regain strength, endurance, and agility to return to their previous job. Vocational counseling can help patients to decide whether or not they can return to their previous job. Patient can be helped to create a new vocational plan that is feasible for the individual with a significant impairment or disability.
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