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Complex regional pain syndrome (type I, RSD; type II, causalgia): controversies.

Introduction of the term complex regional pain syndromes (CRPS) as a replacement of the older terminology, reflex sympathetic dystrophy (RSD) and causalgia, has achieved two goals: it has focused attention on the diagnosis and treatment, and sent basic scientists back to their laboratories. The relation of sympathetically maintained pain and sympatholysis is examined, particularly as a neuropathic process that is found in many conditions, including CRPS. This review also focuses on recent observations proposing a pathologic basis in support of diagnosis and treatment of these disorders.

Causalgia↗

[A multidisciplinary approach to algodystrophy of the hand].

A multidisciplinary approach for the management of reflex sympathetic dystrophy at SOS Main of Strasbourg has been progressively adopted due to the severe functional sequelae of this disease. The TC99 bone scan in three phases has allowed us after a one week duration of the symptoms to make an early diagnosis and to start dynamic splinting in flexion. Our study compares two groups of patients, one with an early treatment and the other with delayed treatment. It shows a correlation between the stiffness of the affected hand and the isotopic findings. The benefit from an early dynamic splinting in flexion as evidenced by a clinical improvement is corroborated by the bone scan.

Combined Modality Therapy↗

Spinal cord stimulation for chronic pain.

BACKGROUND: Spinal cord stimulation (SCS) is a form of therapy used to treat certain types of chronic pain. It involves an electrical generator that delivers pulses to a targeted spinal cord area. The leads can be implanted by laminectomy or percutaneously and the source of power is supplied by an implanted battery or by an external radio-frequency transmitter. The exact mechanism of action of SCS is poorly understood. OBJECTIVES: To assess the efficacy and effectiveness of spinal cord stimulation in relieving certain kinds of pain, as well as the complications and adverse effects of this procedure. SEARCH STRATEGY: We searched MEDLINE and EMBASE to September 2003; the Cochrane Central Register of Controlled Trials (CENTRAL) (Issue 3, 2003); textbooks and reference lists in retrieved articles. We also contacted experts in the field of pain and the main manufacturer of the stimulators. SELECTION CRITERIA: We included trials with a control group, either randomized controlled trials (RCTs) or non-randomized controlled clinical trials (CCTs), that assessed spinal cord stimulation for chronic pain. DATA COLLECTION AND ANALYSIS: Two independent reviewers selected the studies, assessed study quality and extracted the data. One of the assessors of methodological quality was blinded to authors, dates and journals. The data were analysed using qualitative methods (best evidence synthesis). MAIN RESULTS: Two RCTs (81 patients in total) met our inclusion criteria. One was judged as being of high quality (score of 3 on Jadad scale) and the other of low quality (score of 1 on Jadad scale). One trial included patients with Complex Regional Pain Syndrome Type I (reflex sympathetic dystrophy) and the other patients with Failed Back Surgery Syndrome. The follow-up periods varied from 6 to 12 months. Both studies reported that SCS was effective, however, meta-analysis was not undertaken because of the small number of patients and the heterogeneity of the study population. REVIEWERS' CONCLUSIONS: Although there is limited evidence in favour of SCS for Failed Back Surgery Syndrome and Complex Regional Pain Syndrome Type I, more trials are needed to confirm whether SCS is an effective treatment for certain types of chronic pain. In addition, there needs to be a debate about trial designs that will provide the best evidence for assessing this type of intervention.

Chronic Disease↗

Clinical features of algodystrophy: is the sympathetic nervous system involved?

There is a spectrum of conditions which have in common burning pain, often with hyperpathia, hyperalgesia, vasomotor and sudomotor changes. When due to major nerve damage, the condition is known as causalgia; when due to minor nerve damage or other factors, various terms such as algodystrophy or reflex sympathetic dystrophy are employed. Radiography and isotopic bone scanning may be helpful investigations, and procedures which interrupt the local sympathetic nervous system are those most likely to help the pain. The classical view that the peripheral sympathetic nervous system is involved in generation of pain is questioned, and the possible roles of the central nervous system and of non-adrenergic mechanisms are summarised. That pain could be due to dysfunction of the polymodal nociceptor neurone is considered.

Humans↗

[Treatment of Sudeck's syndrome with human calcitonin].

Human calcitonin (Cibacalcin), at a dose of 0.5 mg daily for 15 days followed by 0.5 mg every other day for 4-6 months, was administered to 11 patients (eight men and three women, aged 36-74 years) with posttraumatic reflex sympathetic dystrophy of the lower limbs (stage I-II). Within one month there was significant lessening of pain, improved mobility and less oedema. Biochemical tests were within normal limits before and after treatment, while the pre-treatment raised bone retention of 99mTc-methylene-diphosphonate and increased blood flow in the affected area became normal during treatment. In nine patients healing occurred in the course of four to six months on treatment, in two patients after more than six months. There were no serious side-effects requiring interruption of treatment. These results indicate that human calcitonin should be tried in the treatment of this condition.

Adult↗

Laser Doppler perfusion imager (LDPI)--for the assessment of skin blood flow changes following sympathetic blocks.

A laser Doppler perfusion imager (LDPI) for evaluation of skin blood flow is presented and its use illustrated in two cases with a presumptive diagnosis of reflex sympathetic dystrophy (RSD) treated with local anesthetic sympathetic blocks. Among the advantages are the good spatial resolution over the area measured (12 x 12 cm) and that measurements can be done without contact with the skin. Generating a complete image takes 4.5 min but if higher temporal resolution is needed continuous measurements at one point are possible by use of another software routine.

Adult↗

Reflex sympathetic imbalance. Response to epidural blockade.

Eleven patients (two male, nine female) were treated with epidural sympathetic blockade for reflex sympathetic imbalance, an incomplete manifestation of reflex sympathetic dystrophy. Each had developed severe pain, sensitivity, and disability disproportionate to associated trauma. One patient injured an ankle, and the remaining 10 patients one or both knees (12 knees). Seven patients had undergone previous surgery. All but one had a favorable response to initial blockade. This individual eventually failed treatment despite surgical sympathectomy. Seven have required readministration of a block for clinical relapse. Mean followup was 22 months (range, 10 to 41 months). Five underwent extensive psychological testing. All have required adjunctive forms of therapy including physical therapy, transcutaneous electrical nerve stimulation (TENS), antiinflammatory or other nonnarcotic agents. Recovery is typically prolonged, particularly if the diagnosis is delayed. Close attention to, and therefore prevention of, situations that trigger its recurrence is essential for successful rehabilitation.

Adolescent↗

[Significance of distal motor latency after carpal tunnel operation].

Motor distal latency (MDL) is one of the most important parameters in the electrodiagnosis of carpal tunnel syndrome (CTS). In a retrospective study of 1816 open surgical decompressions for CTS, a total of 612 postoperative motor nerve conduction measurements on 485 hands could be evaluated. In patients with good or satisfactory results after carpal tunnel release, an average improvement of MDL of 1.0 ms after 9-13 days, and of 2.2 ms after 1 year and longer was found. The individual motor nerve conduction improvement was in close correlation with the extent of preoperative prolongation of the MDL. Whereas relief of symptoms can be noted almost immediately, prolonged latencies often do not return to normal, even when the study is done a year later. Of the 10 patients with persisting symptoms, four had a new postoperative impairment of MDL, and also four had a marked improvement, whereas it remained unchanged in two. Patients with severe recurrent CTS presented in 11 out of 31 cases with an improved MDL from 0.3 ms to 3.0 ms in comparison to the initial preoperative evaluation; in 6 hands MDL was unchanged, further prolongation up to 2.0 ms was seen in 6 cases and marked worsening with new loss of motor response had to be noted in 8 hands at repeat electrodiagnosis. In 33 cases of postoperative reflex sympathetic dystrophy, an improvement of MDL from 0.3 ms to 3.3 ms (mean 1.7 ms) was observed in 22 hands and dissolution of a preexisting motor conduction block in three others, whereas two remained unchanged (without motor response) and an electrophysiological impairment was found only in six hands. Three of them presented with a further prolongation of MDL from 0.3 to 1.0 ms and a new loss of response was noted in the remaining three. In conclusion, postoperative motor nerve conduction studies may assess a favorable course following carpal tunnel release. However, they are often not helpful when surgical results are unsatisfactory, and indication for repeat surgical decompression should be based merely on clinical symptoms.

Carpal Tunnel Syndrome↗

Intravenous regional guanethidine in the treatment of reflex sympathetic dystrophy/causalgia: a randomized, double-blind study. Guanethidine Study Group.

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.

Adult↗

[Pain of autonomic nervous system origin].

Reflex sympathetic dystrophy (algodystrophy) is a descriptive term used to designate a clinical phenomenology, which appears after a trauma, often a minor one; it is thought that the sympathetic nervous system plays an important role in its physiopathology. The same applies to sympathetic maintained pain appearing after a lesion of a peripheral nerve (causalgia). There is no agreement as to which are the criteria necessary to make the diagnosis and as to what is the physiopathology of these syndromes. Research in this field is very active today.

Autonomic Nervous System Diseases↗

Somatic versus sympathetic mediated chronic limb pain. Experience and treatment options.

It has been helpful in our practice to separate somatic from sympathetic-mediated peripheral nerve pain. We would recommend application of the new nomenclature of type I complex regional pain (sympathetic dystrophy) and type II complex regional pain (causalgia) (see Table 1). We believe it is essential that both of these conditions be separated into their early and late phases and that the treatment alternatives be customized for the individual patient and the peripheral nerve involved. If a cast, pin, or external fixation apparatus is associated with peripheral nerve pain, the offending apparatus must be removed immediately and other forms of treatment initiated for the underlying injury. For acute injury and postsurgical pain, narcotic pain medications should be used no longer than 72 hours and careful patient re-examination must be performed if pain persists. Prescription of narcotic pain medications on a continuing basis is often the primary reason for the development of chronic pain syndromes. Physical therapy for the patient with chronic peripheral limb pain must be performed in a pain-free environment. "No pain, no gain" does not apply in the treatment of chronic limb pain-rather the reverse: "Only gain with no pain." In differentiating between sympathetic pain and somatic pain, the use of the reflex sympathetic dystrophy (RSD) score can be helpful (Table 4). If the pain is somatic, treatment options include: Somatic Pain: Treatment Isolated nerve block Continuous nerve block TENS (external) Direct electrical nerve stimulation (internal) Nerve ablation If the pain is sympathetic in origin, treatments to be considered are: Sympathetic Nerve Pain: Treatment Protection of limb (garment or splint) Combine with active use Sympathetic blocks single continuous Sympathectomy In addition, the treatment of each of those conditions must be directed at the primary condition. Once the two conditions are separated, a careful program of pain management is required. In patients who present with late pain dysfunction, the more commonly observed phenomenon at our institution, the combination of physician, surgeon, and anesthesiologist is essential. The role of physical therapist in restoring function to the injured limb must be discussed and planned carefully. Initial pain management is organized through a qualified anesthesiologist dedicated to this field. Physical therapy follows but only in a pain-free environment. The surgeon's role is to assist and direct the pain management program. Surgeons can be involved in the placement of percutaneous catheters, as well as isolated peripheral nerve blocks. Surgical intervention is limited to the release of compressive neuropathies, nerve transfers, and revascularization of the peripheral nerve bed. The surgeon occasionally may be involved in the manipulation and pinning of contracted joints, as well as release of muscle or joint contractures, followed by a supervised program of early range of motion. Finally, it is important that both physician and surgeon serve as patient advocates when questions of workers' compensation intervene that could deter proper treatment programs or when the patient needs the encouragement and guidance to continue with treatments that don't always initially appear to have immediate results. Finally, requests to the surgeon to find an operative cure must be resisted while continued psychological encouragement is provided.

Arm↗

The role of Tc-99m bone imaging in the management of pain after complicated total hip replacement.

A case of reflex sympathetic dystrophy (RSD) after total hip replacement (THR) is reported. It is a very unusual cause of pain after any arthroplasty procedure. Three-phase bone imaging diagnosed the condition and sympathetic blockade was used for confirming and treating the condition. The role of the bone scan in differentiating RSD from other causes of pain after THR is highlighted.

Ankle Joint↗

Histopathology of the vessels of the femoral heads in specimens of osteonecrosis, osteoarthritis and algodystrophy.

The authors studied by light microscopy the vessels of the femoral head and neck in 38 well preserved specimens from core biopsy. There were 17 cases of osteonecrosis (ON), 11 cases of osteoarthrosis (OA) and 10 cases of reflex sympathetic dystrophy, so called algodystrophy (AD). Mean age of the patients was respectively 44, 45 and 42 years. The sex ratio, M/F, was respectively 12/5, 6/5 and 9/1. Types of staining used were hematoxylin-eosin, Masson Trichrome, P.A.S. and Verhoeff. In counting, thick-walled and thin-walled vessels were distinguished. There was a significant reduction in the number of the thick- and thin-walled vessels, in the ON group, by comparison with the OA and AD groups. An increased number of thin-walled vessels in the AD group were also observed. Morphological study showed an abnormal frequency of fibrosis of the media in the arteries of the ON group, i.e. arteriosclerosis. These data were compared with the few other histopathological studies previously published. The authors recommend further studies in order to precise their frequency and their significance.

Adult↗

[Local intravenous treatment of algodystrophy of the hand: buflomedil versus guanethidine, long term follow-up].

This report compares the results obtained after treatment of reflex sympathetic dystrophies (algodystrophies) of the hand by pharmacological segmental blocks with buflomedil (51 cases) versus guanethidine (30 cases). The results were similar for all the different stages of algodystrophies treated: 65% satisfactory to excellent results with buflomedil, versus 63% with guanethidine. The sooner the algodystrophy is treated after its onset, the better the results. On TPBS, when the technique is effective, both hemovelocity and blood pool return to normal, along with the improvement in the patient's condition. Early and delayed bone fixations evolve independently of the treatment. These techniques should always be associated with active, mild physiotherapy, and in some cases with dynamic splints in order to prevent the development of functional sequelae in the form or capsulo-aponeurotic retraction.

Adult↗

Imaging of peripheral nerve lesions.

The imaging of peripheral nerve lesions remains limited to the radiographic demonstration of secondary skeletal lesions in birth trauma, reflex sympathetic dystrophy, neuropathic arthropathy, leprosy, and congenital indifference to pain. Nerve root avulsions can be imaged directly and the newer imaging modalities now allow delineation of lesions that previously could not be studied using conventional radiography. The ability of ultrasound, CT, and MRI to differentiate soft tissue structures makes it possible, in many instances, to study the primary abnormality in trauma, nerve entrapment syndromes, and tumors. With fractures, the possibility of trauma to adjacent nerves can only be inferred on the radiographs, while the role that peripheral nerve injury plays remains controversial in other entities, such as amputation with replantation. Imaging of peripheral nerve lesions remains in its infancy. With further refinement in the signal-to-noise ratio made possible by advances in MRI technology, we may be optimistic about future imaging of peripheral nerve pathology.

Arthropathy, Neurogenic↗

Painful shoulder in hemiplegia.

Shoulder pain is probably the most frequent complication of hemiplegia. In this study 219 hemiplegia patients were regularly followed up after their cerebrovascular accident (CVA) for one year (166 men, 53 women, with a mean age of 47 years). Criteria and parameters for evaluation of these shoulders were established at the outset. Distinction was made between flaccid and spastic hemiplegia. Other influencing factors were subluxation reflex sympathetic dystrophy syndrome (RSD), isolated tendon lesion cuff rotator tear or association of some of these. Roentgen examinations were done for each patient. In our series of patients, 72% had shoulder pain at least once during the course of their recovery. This problem occurred more often in patients having spasticity (85%) than in those with flaccidity (18%). An evolution towards spasticity was noted in 80% of the patients in this series, whereas 20% remained hypotonic. Among the other possible causes of shoulder pain, anteroinferior subluxation was incontrovertibly the most frequently cited. The RSD syndrome was present in only 23% of all cases but was seen more often in spastic patients, that is 27% compared to 7% among flaccid patients. Whatever the cause, the subluxation with flaccid paralysis should be corrected and spasticity should be combatted as early and as vigorously as possible.

Adolescent↗

[Isolated decalcifying algodystrophy of the hip].

Transitory demineralization of the hip, a special form of reflex sympathetic dystrophy, is a rare condition. It should however be distinguished from more severe conditions such as tuberculosis or neoplastic bone disease. The typical symptoms are illustrated from 3 personally observed cases. The patients, usually men between the ages of 30 and 50 years or women in the last months of pregnancy, suffer severe mechanical pain in the hip, with limping and weakness. They usually need crutches for walking. Clinical examination of the hip reveals only slight restriction of passive movements. The erythrocyte sedimentation rate may be slightly elevated. Bone scan discloses hypercaptation, while other laboratory tests are negative. A few weeks after onset of symptoms, pelvic X-rays show extreme demineralization of the femoral head. On the other hand, the joint space and bony contours remain intact. The prognosis is excellent and the symptoms disappear after a few months or after delivery.

Accidents, Traffic↗

Complex regional pain syndrome.

The term "complex regional pain syndrome" encompasses causalgia and reflex sympathetic dystrophy. Symptoms of burning pain with autonomic and tissue changes begin shortly after an injury, usually to a distal extremity. The diagnosis is based on the history and the clinical findings. No confirmatory tests are available, although plain radiographs or a three-phase bone scan may be helpful in diagnosing some cases. Aggressive treatment, which may include sympathetic blockade, medications, physical therapy and psychotherapy, is essential for a favorable outcome. Despite treatment, many patients are left with varying degrees of chronic pain and disability.

Adult↗