New Zealand Survey of the Diagnosis and Treatment Experiences of People With Upper Limb Complex Regional Pain Syndrome.

COMPLEX regional pain syndrome (CRPS), previously known as reflex sympathetic dystrophy (RSD), is used to describe a syndrome of pain and sudomotor or vasomotor instability. This pain syndrome usually has an initiating noxious event in the periphery, is not limited to the distribution of a single nerve, and is disproportionate to the inciting event. The Consensus Conference of the International Association for the Study of Pain has subclassified CRPS into two forms: CRPS I (formerly RSD) and CRPS II (formerly causalgia). According to the International Association for the Study of Pain, the diagnosis of CRPS I requires (1) continuing pain, allodynia, or hyperalgesia disproportionate to the injury; (2) evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the region of pain; and (3) no other conditions that would otherwise account for the degree of pain and dysfunction. Motor disturbances and trophic changes, such as altered nail and hair growth, may be observed in some cases. CRPS II is a pain syndrome that starts after a nerve injury and is not necessarily limited to the distribution of the injured nerve. The diagnostic criteria are the same as those of CRPS I. Patients with CRPS I or CRPS II can have sympathetically maintained pain or sympathetically independent pain. Sympathetically maintained pain, a term introduced in 1986 by Roberts, is pathologic pain that is supported by sympathetic efferent activity, circulating catecholamines, and/or increased sensitivity of -adrenergic receptors. Sympathetically maintained pain is identified by the ability to lessen the pain by sympatholytic blocks or interventions. Sympathetically independent pain has components of pain from sources other than sympathetic innervation and is believed to be most commonly observed in advanced cases of CRPS that do not respond to sympathetic blocks. Patients with CRPS may present with components of only sympathetically maintained pain or sympathetically independent pain or, more commonly, a combination of pain from each. Despite increasing research interest, little is known regarding which patients are at increased risk for development of postoperative CRPS and what the optimal perioperative treatment strategy is for those patients undergoing surgery who have a previous history of CRPS. This review outlines the surgical procedures that are believed to increase risk for development of CRPS and describes pharmacologic and regional analgesic techniques that may be of benefit for preventing the development of CRPS after surgery.

IntroductionThis paper describes the epidemiology and clinical presentation of complex regional pain syndrome (CRPS) in a large, integrated health care delivery system; and CRPS incidence rates (IRs) over a time period spanning human papillomavirus (HPV) vaccine licensure and published case reports of CRPS following HPV vaccination.MethodsThe authors examined CRPS diagnoses in patients aged 9–30 years between January 2002 and December 2017 using electronic medical records, excluding patients with lower limb diagnoses only. Medical record abstraction and adjudication were conducted to verify diagnoses and describe clinical characteristics. CRPS IRs were calculated for 3 periods: Period 1 (2002–2006: before HPV vaccine licensure), Period 2 (2007–2012: after licensure but before published case reports), and Period 3 (2013–2017: after published case reports).ResultsA total of 231 individuals received an upper limb or unspecified CRPS diagnosis code during the study period; 113 cases were verified through abstraction and adjudication. Most verified cases (73%) were associated with a clear precipitating event (eg, non–vaccine-related injury, surgical procedure). The authors identified only 1 case in which a practitioner attributed CRPS onset to HPV vaccination. Twenty-five incident cases occurred in Period 1 (IR = 4.35/100,000 person-years (PY), 95% confidence interval (CI) = 2.94-6.44), 42 in Period 2 (IR = 5.94/100,000 PY, 95% CI = 4.39-8.04), and 29 in Period 3 (IR = 4.53/100,000 PY, 95% CI = 3.15-6.52); differences between periods were not statistically significant.ConclusionThese data provide a comprehensive assessment of the epidemiology and characteristics of CRPS in children and young adults and provide further reassurance about the safety of HPV vaccination.

Complex Regional Pain Syndrome (CRPS) is most common in the upper limb and associated with high disability. The purpose of this review was to critically appraise and synthesise literature exploring non-pharmacological treatment for upper limb CRPS, to guide upper-limb-specific management. Using an integrative review methodology, 13 databases were searched to identify all published studies on non-pharmacological management of upper limb CRPS. The Crowe Critical Appraisal Tool was used to provide quality ratings for included studies, and analysis employed a qualitative descriptive approach. From 236 abstracts reviewed, 113 full texts were read, and 38 articles selected for data extraction. Designs included single case (n = 14), randomised controlled trial (n = 8), prospective cohort (n = 8), case series (n = 4), retrospective (n = 3), and mixed methods (n = 1). Interventions were categorised as sensory retraining (n = 13), kinesiotherapy (n = 7), manual therapies (n = 7), physical modalities (n = 6), and interdisciplinary treatment programmes (n = 5). All studies measured pain intensity, and most (n = 24) measured physical parameters such as strength, movement, or perceptual abilities. Few measured patient-rated function (n = 13) or psychological factors (n = 4). Quality ratings ranged from 30% to 93%, with a median of 60%. Methodological quality of non-pharmacological treatment approaches for upper limb CRPS is overall poor. Movement, desensitisation, and graded functional activity remain the mainstays of intervention. However, despite the impact of CRPS on wellbeing and function, psychological factors and functional outcomes are infrequently addressed. Further robust research is required to determine which aspects of treatment have the greatest influence on which symptoms, and when and how these should be introduced and progressed.

Complex regional pain syndrome (CRPS), formerly known as reflex sympathetic dystrophy is a pain syndrome with an unclear pathophysiology and unpredictable clinical course. The disease is often therapy resistant, the natural course not always favorable. The diagnosis of CRPS is based on signs and symptoms derived from medical history and physical examination. Pharmacological pain management and physical rehabilitation of limb function are the main pillars of therapy and should be started as early as possible. If, however, there is no improvement of limb function and persistent severe pain, interventional pain management techniques may be considered.Intravenous regional blocks with guanethidine did not prove superior to placebo but frequent side effects occurred.Therefore this technique receives a negative recommendation (2 A–). Sympathetic block is the interventional treatment of first choice and has a 2 B+ rating. Ganglion stellatum (stellate ganglion) block with repeated local anesthetic injections or by radiofrequency denervation after positive diagnostic block is documented in prospective and retrospective trials in patients suffering from upper limb CRPS. Lumbar sympathetic blocks can be performed with repeated local anesthetic injections. For a more prolonged lumbar sympathetic block radiofrequency treatment is preferred over phenol neurolysis because effects are comparable whereas the risk for side effects is lower (2 B+). For patients suffering from CRPS refractory to conventional treatment and sympathetic blocks, plexus brachialis block or continuous epidural infusion analgesia coupled with exercise therapy may be tried (2 C+). Spinal cord stimulation is recommended if other treatments fail to improve pain and dysfunction (2 B+). Alternatively peripheral nerve stimulation can be considered, preferentially in study conditions (2 C+).

Stellate ganglion (SG) interventions for treating upper limb complex regional pain syndrome (CRPS) have been reported in studies. However, more substantial evidence is required to reach a consensus on its analgesic efficacy. To the best of our knowledge, no systematic review demonstrating the effectiveness of SG intervention for upper limb CRPS has been reported. Hence, this meta-analysis was done to ascertain the efficacy of SG intervention in managing patients with upper limb CRPS. A database search of PubMed, Cochrane, Embase, Scopus and Google Scholar was done for articles published between January 2001 and December 2021. Two independent reviewers extracted data from the included studies, and the studies were evaluated for any potential risk of bias, and a meta-analysis was performed. Eight studies were included in the qualitative synthesis, four were randomised controlled trials (RCTs), and the rest were non-RCTs. Six studies were assessed quantitatively. A significant reduction in pain scores post-intervention, up to two weeks, and four to 28 weeks after the procedure was observed, although the included studies exhibited marked heterogeneity. Qualitative analysis of these studies revealed an overall improvement in disability scores and functionality as assessed by an improved range of motion. Most studies reported no complications or only short-term minor complications after the procedure. Stellate ganglion interventions improve pain and disability scores with self-limiting short-term complications and no long-term complications. However, further studies with a large sample size are required to validate this treatment modality.

Undaunted Complexity

The mechanism of complex regional pain syndrome (CRPS) was reported as being related to both the central and peripheral nervous systems. Recurrence of CRPS was, reportedly, induced by hand surgery in a patient with upper limb CRPS. However, there is no documentation of mechanical allodynia and burning abdominal pain induced by Cesarean section under spinal anesthesia in patients with upper limb CRPS. We report the case of a patient who suffered from burning abdominal pain during Cesarean section under spinal anesthesia 13 years after the occurrence of venipuncture-induced CRPS of the upper arm. The patient's pain characteristics were similar to the pain characteristics of her right arm during her previous CRPS episode 13 years earlier. In addition, mechanical allodynia around the incision area was confirmed after surgery. We provided ultrasound-guided rectus sheath block using 20 mL of 0.4% ropivacaine under ultrasound guidance twice, which resulted in the disappearance of the spontaneous pain and allodynia. The pain relief was probably related to blockade of the peripheral input by this block, which in turn would have improved her central sensitization. Our report shows that attention should be paid to the appearance of neuropathic pain of the abdomen during Cesarean section under spinal anesthesia in patients with a history of CRPS.

Proximal myofascial pain in patients with distal complex regional pain syndrome of the upper limb

Distorted representations of the body and peripersonal space are common in complex regional pain syndrome (CRPS), and might modulate its symptoms (eg, asymmetric limb temperature). In pain-free people, such representations are malleable, and update when we interact with objects in our environment (eg, during tool-use). Distortions are also common after immobilisation, but quickly normalise once movement is regained. We tested the hypothesis that people with CRPS have problems updating bodily and spatial representations, which contributes to the maintenance of their distorted representations by preventing normalization. We also explored spatially defined modulations of hand temperature asymmetries, and any influence of updating bodily and spatial representations on this effect. Thirty-six people with unilateral CRPS (18 upper limb and 18 lower limb) and 36 pain-free controls completed tool-use tasks considered to alter body and peripersonal space representations (measured using tactile distance judgements and a visuotactile crossmodal congruency task, respectively). We also tested how the arrangement (crossed and uncrossed) of the hands and tools affected hand temperature. In upper-limb CRPS, the nonaffected arm representation updated normally, but the affected arm representation updated in the opposite to normal direction. A similar pattern was seen in lower-limb CRPS, although not significant. Furthermore, people with CRPS showed more pronounced updating of peripersonal space than the controls. We did not observe any modulation of hand temperature asymmetries by the arrangement of hands or tools. Our findings show enhanced malleability of bodily and spatial representations in CRPS, which may suggest that central mechanisms are altered in this condition.

Impairments and activity limitations in subjects with chronic upper-limb complex regional pain syndrome type I

Complex Regional Pain Syndrome (CRPS) is characterised by pain, autonomic, sensory and motor abnormalities. It is associated with changes in the primary somatosensory cortex (S1 representation), reductions in tactile sensitivity (tested by two-point discrimination), and alterations in perceived hand size or shape (hand perception). The frequent co-occurrence of these three phenomena has led to the assumption that S1 changes underlie tactile sensitivity and perceptual disturbances. However, studies underpinning such a presumed relationship use tactile sensitivity paradigms that involve the processing of both non-spatial and spatial cues. Here, we used a task that evaluates anisotropy (i.e., orientation-dependency; a feature of peripheral and S1 representation) to interrogate spatial processing of tactile input in CRPS and its relation to hand perception. People with upper limb CRPS (n = 14) and controls with (n = 15) or without pain (n = 19) judged tactile distances between stimuli-pairs applied across and along the back of either hand to provide measures of tactile anisotropy. Hand perception was evaluated using a visual scaling task and questionnaires. Data were analysed with generalised estimating equations. Contrary to our hypotheses, tactile anisotropy was bilaterally preserved in CRPS, and the magnitude of anisotropic perception bias was comparable between groups. Hand perception was distorted in CRPS but not related to the magnitude of anisotropy or bias. Our results suggest against impairments in spatial processing of tactile input, and by implication S1 representation, as the cause of distorted hand perception in CRPS. Further work is warranted to elucidate the mechanisms of somatosensory dysfunction and distorted hand perception in CRPS.

Cortical reorganization of the healthy hand in upper-limb complex regional pain syndrome (CRPS): Is reorganizations of common beliefs about CRPS necessary?

Cellular and behavioural models to predict responses to immunoglobulin G treatment in complex regional pain syndrome.

Background: Complex regional pain syndrome (CRPS) has been reported following spinal surgery, but its frequency after spinal surgery is unknown. The aim of this study was to determine the frequency of spinal surgery preceding CRPS and to examine these patients regarding the course of the disease and prognostic factors. Methods: We examined 35 CRPS patients regarding the symptoms and signs of CRPS, the type of CRPS (I or II), the origin and grade of the disease, the type of surgeries prior to CRPS onset, the course of the disease, and the therapies following diagnosis of CRPS. Results: In 6 patients, CRPS began during the postoperative course (lumbar spine surgery, n = 5; cervical spine surgery, n = 1). Four of these patients suffered from CRPS II. The course of the disease in the 6 patients was not different from that of patients with CRPS of other origins. First symptoms of CRPS could be observed 1–14 days after surgery. Conclusions: CRPS is a rare complication after spinal surgery, but spinal surgery precedes the onset of CRPS of the lower limb in almost one-third of the cases. The first typical symptoms of CRPS emerge within 2 weeks after spinal surgery.

Exploring the Relationships Between Altered Body Perception, Limb Position Sense, and Limb Movement Sense in Complex Regional Pain Syndrome