No full-text available
To read the full-text of this research,
you can request a copy directly from the authors.
Technetium 99m-methylene diphosphonate bone scans in children with reflex neurovascular dystrophy
Abstract
Eleven children with reflex neurovascular dystrophy were investigated by technetium-labeled methylene diphosphonate bone scanning. Eight of 12 scans demonstrated abnormal findings, four showing diffusely decreased uptake and four diffusely increased uptake of the radionuclide in the affected site. Three scans showed normal findings initially, as did one previously abnormal scan when repeated in the asymptomatic patient 6 months later. Diffusely abnormal findings can be helpful in the diagnosis of childhood reflex neurovascular dystrophy, but a normal scan does not exclude the diagnosis.
... Veldman et al. 6 estudaram 829 pacientes com esta doença, e, dentre os mesmos, havia somente uma paciente abaixo dos 9 anos de idade. Em nossa casuística, a média de idade dos pacientes foi de 11,5 anos, compatível com os dados de literatura 7-10 , mas não se observou o predomínio do sexo feminino que ocorre na infância 7,8,11,12 . ...
... Na faixa etária pediátrica, há menor probabilidade de eventos precipitantes, como trauma 8,[11][12][13] , e também é rara a incidência de alterações tróficas em relação aos adultos 8,11,13,14 . Sua maior raridade na infância pode ser devido à menor duração da doença ou à imobilidade menos prolongada 8 . ...
... Na faixa etária pediátrica, há menor probabilidade de eventos precipitantes, como trauma 8,[11][12][13] , e também é rara a incidência de alterações tróficas em relação aos adultos 8,11,13,14 . Sua maior raridade na infância pode ser devido à menor duração da doença ou à imobilidade menos prolongada 8 . ...
To describe eight patients with reflex sympathetic dystrophy in terms of clinical and laboratory characteristics and treatment.
Eight children (four girls) with reflex sympathetic dystrophy were retrospectively analyzed. The diagnosis of reflex sympathetic dystrophy was based on the presence of pain in the distal extremities, local edema, vasomotor instability and impairment of sensibility. Two patients had associated systemic lupus erythematosus, one had juvenile idiopathic arthritis and one had Glanzmanńs thrombasthenia. Mean age was 11.5 years. Most of the patients had lower extremity involvement (7/8). The most important clinical signs were pain, edema and vasomotor instability in the affected extremity (8/8), functional impairment (7/8), and impaired sensibility (3/7). The erythrocyte sedimentation rate was abnormal in three patients and the bone scans in five. All patients received non-steroidal anti-inflammatory drugs and physical therapy with improvement of the symptoms in seven patients, until six months of treatment. Three patients were submitted to acupuncture with good response. One patient had a severe disease and received tricyclic antidepressants, with improvement more than one year after.
Reflex sympathetic dystrophy should be included as part of the differential diagnosis of limb pains of childhood, so that physicians can make an earlier diagnosis and prevent functional impairment.
... C hildhood-onset complex regional pain syndrome (CRPS) differs from adult-onset CRPS in significant ways, including more typically lower limb involvement, decreased bone uptake on scintigraphy, and generally a better outcome. [1][2][3][4][5][6][7][8][9] Severe trauma, such as a fracture, is much more commonly mentioned as a preceding event before the onset of adult CRPS. 7 The Budapest criteria for adult CRPS requires disproportionate pain to an inciting event, usually a fracture or crush injury. ...
Objective:
To determine if differences exist between children with complex regional pain syndrome (CRPS) who identify an inciting physical traumatic event (Group T) versus those without such history (Group NT).
Methods:
We performed a single center, retrospective study of children diagnosed with CRPS ≤18 years old presenting between April 2008 and March 2021 and enrolled in a patient registry. Abstracted data included clinical characteristics, pain symptoms, functional disability inventory, psychological history, pain catastrophizing scale for children (PCS-C). Charts were reviewed for outcome data.
Results:
We identified 301 children with CRPS, 95 (64%) reported a prior physical trauma. There was no difference between the groups regarding age, sex, duration, pain level, function, psychological symptoms, and scores on the PCS-C. However, those in group T were more likely to have had a cast (43% vs. 23%, P<0.001). Those in group T were less likely to experience complete resolution of symptoms (64% vs. 76%, P=0.036). There were no other outcome differences between the groups.
Discussion:
We found minimal differences in children with CRPS who report a prior history of physical trauma to those who do not. Physical trauma may not play as significant a role as does immobility, such as casting. The groups mostly had similar psychological backgrounds and outcomes.
... In the early acute stage (<6 weeks), the uptake is normal or minimally increased in all three phases [8]. An atypical pattern of TPBS, which is the decreased uptake in all three phases in the affected extremity, is frequently observed in children with CRPS and can also be seen in adults with chronic CRPS [11,12]. The diagnostic performance of TPBS varies according to the clinical stage, diagnostic criteria, location, and age at onset of CRPS [1,2]. ...
Complex regional pain syndrome (CRPS) is a chronic pain condition caused by a variety of diseases. The pathophysiology of CRPS is uncertain, and the diagnosis depends on clinical criteria. Three-phase bone scintigraphy (TPBS) using 99mTc-diphosphonates is helpful in documenting the symptoms and signs of CRPS and in the differential diagnosis of extremity pain. The typical pattern of TPBS in patients with CRPS is diffusely increased blood flow, blood pool, and delayed periarticular bone uptake in the affected extremity. In this chapter, we introduce several cases to help understand the findings and clinical usefulness of TPBS in patients with CRPS.
... However, conversion disorder lacks true physical symptomatology. CRPS can be differentiated from conversion disorder by a supporting history, positive physical examination, and suggestive findings on triple phase bone scan, although there is still some controversy concerning the diagnostic value of bone scans [11][12][13]. ...
Objective:
This self-directed learning module focuses on the role of accurate diagnosis, psychological support, and family integration of children who have chronic impairments such as pain, spasticity, or cognitive disability. It is part of the study guide on pediatric rehabilitation in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation and pediatric medicine. The role of therapeutic, medical (traditional and nontraditional) and psychological interventions that improve family and individual function are emphasized. The goal of this article is to refine a learner's knowledge of the impact family-centered care can have on the medical, psychological, financial, and functional capabilities of families to improve treatment decisions in the context of children with disability.
... Recent reports have observed increased, decreased, and normal radionucleotide uptake in children with RSD. 15 These variations seem to depend on the duration of illness before scanning. The investigations are used early, and are helpful in the search for unrecognised organic disease. ...
Reflex sympathetic dystrophy (RSD), an unusual diagnosis in general paediatrics, is well recognised by paediatric rheumatologists. This study reports the presentation and the clinical course of 46 patients (35 female, age range 8-15.2) with RSD. The patients saw professionals from an average of 2.3 specialties (range 1-5). Twenty five (54%) had a history of trauma. Median time to diagnosis was 12 weeks (range 1-130). Many children had multiple investigations and treatments. Once diagnosis was made, treatment followed with physiotherapy and analgesics. Median time to recovery was seven weeks (range 1-140), with 27.5% relapsing. Nine children required assessment by the child and adolescent psychiatry team. This disease, though rare, has significant morbidity and it is therefore important to raise clinicians' awareness of RSD in childhood. Children with the condition may then be recognised and referred for appropriate management earlier, and spared unnecessary investigations and treatments which may exacerbate the condition.
Pediatric patients with complex regional pain syndrome (CRPS) differ from their adult counterparts in several ways. The lower limb is disproportionately affected and there is a higher percentage of cases that present after no known trauma. While many pediatric CRPS patients have psychological comorbidities, evidence does not suggest a psychological origin of pediatric CRPS. Adult criteria for diagnosis is often used, although many recognize the need for pediatric-specific criteria. Multidisciplinary therapy is the foundation of treatment. There is not good evidence to support the use of medications in pediatric CRPS. Invasive procedures are not recommended as first-line therapy, but may be helpful for cases that are refractory to conservative treatment. The prognosis is generally favorable, though recurrence is very common. Despite a tremendous growth in research on pediatric CRPS in the recent decades, there is a still a great need for more high-quality evidence in the treatment of pediatric CRPS.
Complex regional pain syndrome (CRPS) is a disorder that describes a variety of chronic pain states. CRPS usually follows a noxious stimulus such as trauma and is usually focal and distal. CRPS can lead to sensory, motor, and autonomic dysfunction. It is also accompanied by central dysfunction and sensitization. CRPS can be classified into CRPS type 1 and CRPS type 2 (causalgia). If left untreated, immobilization and disuse of the affected extremity can occur.
Complex regional pain syndrome type I (CRPS-I) is a complex disorder characterised by pain, autonomic dysfunction and decreased range of motion. The syndrome was believed as a well-recognized disorder in adults, but less commonly recognized in children. In this article, literature was reviewed for assessing clinical features, diagnostic methods and treatment modalities of the syndrome in children. Some of the clinical features that we reviewed from some case reports on CRPS-I, ware different from the features given in the textbooks or some other literature. Changing concepts about diagnosis and treatment methods of this disorder was also observed. This syndrome was thought to be well-recognized in recent years in children. However, further studies are necessary for a better understanding and effective treatment of this disorder.
Reflex sympathetic dystrophies (RSD) are taken to be polyetiological diseases with monopathogenic nature. Data on the latter have been collected over centuries, but regardless of in-depth researches into the problem, and accumulation of vast factological material, there are still a lot of not well enough clarified issues interfering greatly with the build up of a versatile theory of the pathophysiological mechanism involved. This is also the major obstacle hindering the creation of an effective mechanism for adequate therapeutic approach. The basic historical stages of accumulated information on posttraumatic reflex dystrophies, their systematization and elaborating pathophysiological hypotheses are outlined. The current trends of RSD diagnosis and treatment, and the contribution of Bulgarian and foreign authors along this line are comprehensively discussed.
Pediatric Rheumatology in Clinical Practice is authored by a trio of experts from the UK, US and Canada. The book presents the physician with key points and summary boxes about various symptoms, making the book a useful quick reference guide. 79 illustrations, most of them in color, enhance the text. Pediatric Rheumatology in Clinical Practice will be of particular interest to rheumatologists in practice, residents on rotation through the rheumatology clinic, primary care physicians, hospital doctors, as well as to senior nursing staff.
To study the reaction of water vapour with uranium, we have exposed clean liquid uranium surfaces to HâO under UHV conditions. We have measured the surface concentration of oxygen as a function of exposure, and determined the maximum attainable surface oxygen concentration X/sup s//sub O/ as a function of temperature. We have used these measurements to estimate, close to the melting point, the solubility of oxygen (X/sup b//sub O/, < 10â»â´) and its surface segregation coefficient ..beta../sup s/(> 10³). 11 refs., 5 figs.
Unter einem idiopathischen muskuloskelettalen Schmerzverstärkungssyndrom versteht man einen Symptomenkomplex, bei dem es zu
inadäquat stark empfundenen Schmerzen kommt. Dieser Zusammenhang drückt sich in der Bezeichnung Schmerzverstärkungssyndrom
aus. Im Gefolge treten Hautveränderungen, funktionell-muskuloskelettale Störungen sowie im Spätstadium auch eine Dystrophie
auf. Die Beeinträchtigung des allgemeinen, sozialen und psychischen Befindens kann dabei erheblich sein (Konijenber et al. 2005; Murray et al. 2000). Von einer Chronifizierung ist auszugehen, wenn die Symptomatik länger als 3 Monate anhält.
Adult patients with reflex sympathetic dystrophy (RSD), also known as complex regional pain syndrome, of the foot and ankle characteristically have a 3-phase bone scan pattern consisting of diffusely increased tracer uptake with juxtaarticular accentuation of tracer uptake on late-phase images. The authors present an uncommon case of RSD in an adult in whom decreased activity was demonstrated on early and late phase images of bone scintigraphy in the affected limb.
Musculoskeletal scintigraphy has excellent sensitivity for the evaluation of benign disease in children. Using illustrative cases, a spectrum of techniques and applications of nuclear medicine studies for benign bone diseases are presented. An approach to the use and evaluation of bone density evaluation in children also is discussed.
Eight patients with dysesthetic pain were treated in an open trial with capsaicin 0.075%, including five patients with reflex sympathetic dystrophy, and one each with meralgia paresthetica, thalamic pain syndrome, and multiple sclerosis. Pain relief and functional impairment were assessed by visual analogue and functional capacity scales respectively. At the end of the study period, pain relief in the RSD group averaged 73%; impairment score declined by 60%. Abnormalities of pretreatment bone scintigraphy reversed in patients with symptoms of less than eight months duration. In patients with RSD and other dysesthetic pain syndrome topical capsaicin may specifically relieve superficial, burning, dysesthetic pain, a major factor in the physical and emotional disability seen in this syndrome. The spectrum of capsaicin's current clinical use is discussed. On the basis of the results of this study we recommend larger, controlled and blinded studies to assess further the efficacy of topical capsaicin in dysesthetic pain syndromes.
Reflex neurovascular dystrophy (RND) is less common in children than in adults, and differences in onset, clinical course, response to treatment, and degree of disability suggest a different pathogenesis. We have assessed the usefulness of nuclear imaging in 15 children with RND who were evaluated from March 1983 to September 1985. Abnormal findings on 3-phase bone scans were observed in 14 children, with diffusely decreased bone uptake at the symptomatic site being the most common observation. This contrasts sharply with previous reports of diffusely increased uptake in most adults with RND.
A case report is presented of a 15-year-old girl with reflex sympathetic dystrophy (RSD). She was referred to hospital because of left upper limb pain. Her left upper limb was cold, edematous and blue with a limited active range of movement. The serum concentration of noradrenaline was lower on the painful side than on the healthy side, and neurotropin, which has an antinociceptive effect to hyperalgesia, was clearly effective. Early diagnosis and management is essential in the treatment of RSD and administration of neurotropin is a useful and non-invasive treatment without severe adverse effects.
A three-phase bone scan, before conventional radiography shows morphological changes in CRPS 1, represents a valuable diagnostic tool thanks to the depiction of pathognomonic findings and the localisation ability in all 3 phases. It must be interpreted within the clinical context. In late or subsequent stages the lack of scintigraphic uptake seems to represent the end of the active disease process. The role of three-phase bone scintigraphy in CRPS I is to support or even confirm the diagnosis, given its various presentations. Furthermore, it enables exclusion of other diagnoses such as arthritis, benign or malignant bony lesions, or even metabolic bone diseases such as Paget's disease, particularly if an integrated SPECT/CT is added. Moreover, the earlier the diagnosis is finally established and treatment of CRPS I is initiated, the better the prognosis. Therefore, bone scintigraphy potentially has a major impact on patient management of this disorder.
© Georg Thieme Verlag KG Stuttgart · New York.
Diphosphonate bone scintigraphy is used since the 1970s for studying bone pathology; it is increasingly common because of increased physical activity of the population. The disphosphonate uptake in bone requires the preservation of bone vascularity and reflects osteoblastic activity; it allows studying the viability of bone. Using data from the literature and our experience, we tried to take stock of its main uses in routine emphasizing the study of vascular and describe its use in fractures of the scaphoid and in the study of vascularised bone transfers. If the value of scintigraphy is well known, the use made by means of pinhole collimator and the market entry of new and more sensitive detectors with better resolution could allow the side of the imaging anatomy of improving the study of the vascularisation of small bone transfers which are increasingly used, particularly at wrist and hand.
Copyright © 2010. Published by Elsevier SAS.
OBJETIVO: Descrever as características clínicas, laboratoriais e terapêuticas de oito crianças com distrofia simpático-reflexa. DESCRIÇÃO: Foram analisados retrospectivamente oito casos de distrofia simpático-reflexa. O diagnóstico foi baseado na presença de dor no segmento distal de um membro, edema local, alterações vasomotoras e de sensibilidade. Dentre os oito pacientes com distrofia simpático-reflexa, dois eram portadores de lúpus eritematoso sistêmico, um de artrite idiopática juvenil e um de trombastenia de Glanzmann. Quatro eram meninas, e a média de idade foi de 11,5 anos. Os membros inferiores foram acometidos na maioria dos pacientes (7/8), e as características clínicas mais marcantes foram dor, edema e alterações vasomotoras da extremidade afetada (7/8), incapacitação funcional (7/8) e alterações de sensibilidade (3/8). A velocidade de hemossedimentação esteve alterada em três pacientes, e a cintilografia músculo-esquelética em cinco. Todos os pacientes receberam antiinflamatórios não-hormonais e fisioterapia, com melhora clínica observada em sete pacientes em até 6 meses de tratamento. Três pacientes foram submetidos à acupuntura, com boa resposta. Um paciente teve curso de difícil controle, tendo recebido antidepressivos tricíclicos e apresentando melhora após mais de 1 ano de tratamento. COMENTÁRIOS: A distrofia simpático-reflexa é uma doença que deve ser incluída no diagnóstico diferencial das dores em membros na infância, para que se estabeleça o diagnóstico precocemente, evitando-se, assim, prejuízo funcional a médio e longo prazo.
Thermography is a noninvasive imaging technique that visualizes extremely small but significant cutaneous sensory segment temperature alterations, which reflect physiologic changes not depicted by static techniques including CT and MR imaging. The equipment in current use, techniques of upper extremity examination, criteria for interpretation, a classic body of thermographic patterns associated with sympathetic nerve dysfunction, and theories relating to their mechanisms have been summarized. The particular contributions of thermography in diagnosing and corroborating acute, recurrent, and chronic clinical symptoms related to the hand and wrist have been illustrated and discussed.
Reflex sympathetic dystrophy syndrome (RSDS) is a well-recognized entity associated with a wide variety of precipitating factors, most commonly with a history of trauma. The classical signs - painful swelling in an extremity, fluctuating vasomotor changes, and dystrophic skin and muscle changes - have been given different names such as minor causalgia, Sudeck's atrophy, and hand/shoulder syndrome. This paper will deal with the clinical manifestations of RSDS and the possible connection between this condition and anorexia nervosa.
We reviewed the medical records and technetium bone/joint scans of 160 children presenting to the inpatient Pediatric Rheumatology service over a 3-year period. When the scan result (normal versus abnormal) was considered for each patient as a whole, scan sensitivity and specificity were both approximately 75%. However, when each joint was considered individually, sensitivity decreased to 37%, while specificity rose to more than 95% when compared to clinical examination. Reasons for these variations and their clinical correlation are discussed. Overall, radionuclide bone/joint scanning was found to be very useful in the evaluation of monoarticular and nonrheumatic disorders, but it did not alter therapy in children with known connective tissue disorders or other polyarticular diseases.
We report 3 children with reflex sympathetic dystrophy syndrome, review the literature, and discuss current concepts of diagnosis and management. In this disorder, pain, tenderness, swelling, vasomotor instability, and dystrophic skin changes frequently develop after minor injury. The clinical diagnosis is supported by osteopenia detected on radiographs and either increased or decreased radionuclide uptake on bone scan of the affected extremity. Treatment with a graduated program of physical therapy and transcutaneous electrical nerve stimulation is beneficial in almost all patients. In contrast to adults, the prognosis of childhood reflex sympathetic dystrophy syndrome is favorable; most children recover completely after one episode.
Radionuclide imaging with Tc-99m diphosphonates is not an effective method for detecting or ruling out most osteoporotic diseases including senile osteoporosis or accelerated postmenopausal osteoporosis, and the slow loss of bone tissue generally remains undetected by this modality. Nonetheless, it frequently surpasses or supplements radiographic findings in evaluating the focal complications of metabolic bone disease, including fractures, microfractures, stress fractures, vertebral compressions, Milkman-Looser zones, aseptic necrosis, and acute infarction. In contrast to its secondary role in osteoporosis, bone imaging is of prime importance in investigating hypercalcemia, because the major cause of this abnormality is skeletal metastatic malignancy. In defective bone mineralization due to hyperparathyroidism or osteomalacia, a general increase in diphosphonate skeletal uptake is detected more frequently than radiographic abnormalities. However, normal skeletal images do not rule out metabolic bone disease. Biochemical testing is more reliable in detecting primary hyperparathyroidism. On the other hand, in renal osteodystrophy, biochemical abnormalities are variable and bone imaging is helpful in assessing the severity of skeletal involvement, but not its etiology. Many methods of quantitating the kinetics of Tc-99m diphosphonates have been explored, such as plasma clearance, bone-to-soft-tissue ratios, 24-hour total body retention and 24-hour urinary excretion. None of these have been widely accepted. The value of bone imaging is established in other systemic diseases, most notably in Paget's disease, hypertrophic pulmonary osteoarthropathy, sickle cell disease, fibrous dysplasia, and sympathetic dystrophy.
To design diagnostic criteria for reflex sympathetic dystrophy (RSD) and to initiate a prospective treatment protocol, we reviewed our experience with 49 episodes of RSD in 36 children. There were 24 females and 12 males; mean age at diagnosis was 13.4 years (range: 8 to 19); mean time from pain onset to correct diagnosis was 9.2 months (range: 1 to 53). Lower extremity involvement predominated. Pain was "severe" in 61%, and skin color changes, swelling, hyperesthesia, abnormal skin temperatures, muscle weakness, and decreased range of motion were all present in at least 75% of cases. Osteopenia was observed in 15 of 38 radiographs; of 24 bone scans, 7 were normal, 11 showed increased uptake, and 6 demonstrated decreased uptake. Of the 23 children who had psychological evaluations, 83% revealed some type of significant emotional dysfunction. Analgesic and antiinflammatory medications were not helpful, nor were local injections or regional blockades effective. An inpatient diagnostic and rehabilitation program for treating chronic pain, including orthopedics, rheumatology, psychology, and twice-daily physical therapy was most likely to lead to resumption of age-appropriate activities. Despite extensive physiological testing, physician, parent, and/or patient reluctance to accept absence of a primary organic disease was common. We present diagnostic criteria for pediatric RSD.
The classical presentation of acute reflex sympathetic dystrophy (RSD) or triple-phase bone scintigraphy usually consists of increased periarticular uptake in each phase. The authors present a rare case of acute adult RSD characterized by bone hypofixation of Tc-99m MDP. Reflex sympathetic dystrophy in adults and children is reviewed.
"Reflex sympathetic dystrophy" and "causalgia" are now classified by the International Association for the Study of Pain as Complex Regional Pain Syndromes I and II. Sympathetically maintained pain is a frequent but variable component of these syndromes, as the sympathetic and somatosensory pathways are no longer functionally distinct. Pain is the cardinal feature of CRPS, but the constellation of symptoms and signs may also include sensory changes, autonomic dysfunction, trophic changes, motor impairment and psychological changes. Diagnosis is based on the clinical picture, with additional information regarding the presence of sympathetically maintained pain or autonomic dysfunction being provided by carefully performed and interpreted supplemental tests. Clinical experience supports early intervention with sympatholytic procedures (pharmacological or nerve block techniques), but further scientific data is required to confirm the appropriate timing and relative efficacy of different procedures. Patients with recurrent or refractory symptoms are best managed in a multi-disciplinary pain clinic as more invasive and intensive treatment will be required to minimize ongoing pain and disability.
The authors describe a 12-year old girl with a painful syndrome at the distal side of the left leg, resulting in limping, incapacity and severe muscle atrophy. Full investigation - no inflammatory laboratory signs, diffuse osteoporosis at the left leg, decreased bone mineral content at the same place, marked hypofixation on bone and vascular scintigraphy - suggested pseudodystrophy (5), which is often induced by psychological factors. Successful treatment was obtained by physiotherapy, hydrotherapy, slight doses of NSAID and psychological assistance. With regard to recent literature, the authors believe that reflex sympathetic dystrophy (RSD) in children is often over-diagnosed, since there are no recognised criteria for diagnosing RSD. Besides the clinical picture, changes on radiography (focal osteoporosis) and on scintigraphy (disturbed vascular scintigraphy with increased pooling in the initial phase and hyperfixation on bone scintigraphy) are necessary. When these are not available, pseudodystrophy is a more correct diagnosis.
Pseudodystrophy is a condition that in the past was regarded incorrectly as a kind of reflex sympathetic dystrophy. The difference between both diseases can be ascertained easily by a bone scintigraphy. Other clinical features of pseudodystrophy are discussed. Infrequent presentations of reflex sympathetic dystrophy are described: radial, parcellar, and zonal cases; vertebral localization; and other misleading conditions.
A case report is presented of a 15-year-old girl with reflex sympathetic dystrophy (RSD). She was referred to hospital because of left upper limb pain. Her left upper limb was cold, edematous and blue with a limited active range of movement. The serum concentration of noradrenaline was lower on the painful side than on the healthy side, and neurotropin, which has an antinociceptive effect to hyperalgesia, was clearly effective. Early diagnosis and management is essential in the treatment of RSD and administration of neurotropin is a useful and non-invasive treatment without severe adverse effects.
In this presentation, the common clinical pediatric problems (appendicular growth aberrations, hip pain, back pain, some aspects of trauma, and inflammatory and infectious processes that can be addressed by nuclear imaging will be discussed. The accurate interpretation of bone scintigraphy in the evaluation of pediatric orthopedic conditions depends on the use of appropriate radiopharmaceuticals and imaging techniques specific for the pediatric population, a knowledge of physiologic changes in the growing skeleton, as well as the different disease processes peculiar to each age bracket. The physes (growth-plates) will be discussed in detail because they are the most active portion of the pediatric skeleton. Their activity (potential for growth) can be altered by physiologic, developmental aberrations (varus and valgus) and pathologic conditions (trauma, infection, etc.).
Few series on reflex sympathetic dystrophy syndrome (RSDS) have included children. The present series reviewed 10 affected children. The group consisted of 9 girls and one boy with an average age at onset of 11 years (5 years to 16 years). The diagnosis was based on the clinical findings of pain, dysesthesia and autonomic system dysfunction. All patients underwent x rays and bone scans. Their results showed great variation. Minor trauma was the most common trigger factor. The lower extremities were more often involved. The treatment consisted of pain relief and progressive mobilization. Less conventional treatments in children, such as calcitonin and bisphosphonate were also used. The severity and duration of the disease varied greatly among these children. Moderate pain and sympathetic dysfunction persisted often up to two years after onset. Reflex sympathetic dystrophy is more common in children than previously thought. There are differences with the adult form in presentation and clinical course: the diagnosis is often delayed, the lower extremities are more often involved, girls are affected more often and idiopathic forms are frequent. Significant emotional dysfunction is found in a majority of patients and they are best treated as inpatients by a multidisciplinary team.
Reflex sympathetic dystrophy is a syndrome characterized by pain in one or more extremities, usually associated with vasomotor changes. Its occurrence in childhood has long been thought to be rare. We describe six cases of pediatric reflex sympathetic dystrophy and suggest that this syndrome could be underdiagnosed in children and adolescents. Psychologic problems frequently play a role in this disorder, which often can be treated conservatively. We also point out that the diagnosis is mainly clinical. An early diagnosis can avoid unnecessary tests and potentially can improve response to treatment, and prognosis.
Children may have a wide variety of amplified musculoskeletal pain syndromes that may or may not be associated with overt autonomic signs and may be diffuse or localized to one body part. It is most common in pre- to adolescent girls. Hallmarks of the diagnosis include increasing pain over time, allodynia, an incongruent affect, disproportional dysfunction, and the absence of other causes. Psychological distress within the child or family is apparent in most, but not all, since it also is associated with injury or illness. Once the diagnosis is established, all medicines and testing are stopped. A sympathetically driven pain model is used to explain the pain to make it understandable. Treatment is an intense exercise program; ours is 5 hours daily. We focus on functional aerobic training specifically using the involved body part such as sports related drills, running, play activities, and swimming. Allodynia is treated with desensitization such as towel rubbing. A psychological evaluation is done and specific psychotherapy is recommended if indicated. The average duration of the daily program is 2 weeks with a 1 hour home program being done for another 2 to 8 weeks. After one month roughly 80% of the children have no pain and are fully functional, another 15% are fully functional with mild or recurrent pain; 5% are not better. Significant relapses are infrequent; 15% require retreatment. Five to 10% of the children will develop a different symptom of psychological distress. At 5 years, 90% are doing well.
Juvenile idiopathic arthritis accounts for approximately half of the cases of chronic rheumatic diseases in children. Hand surgeons frequently see these children before the primary diagnosis has been made. Common presentations of juvenile idiopathic arthritis and basic medical and surgical management are described. Other inflammatory connective tissue diseases, such as scleroderma, dermatomyositis, systemic lupus erythematosus, and disorders that can cause joint complications are also considered. An awareness of these diagnoses may facilitate prompt referral and treatment for these children.
Idiopathic musculoskeletal pain syndromes in children have a variety of manifestations; they can be diffuse or well localized, constant or intermittent, with or without autonomic symptoms and signs, completely incapacitating or not limiting activities, and they can tax the physician's diagnostic skill. A careful history and examination is usually all that is needed to make a diagnosis, although the differential diagnosis is large and might require laboratory and radiographic investigation. Pain and functional assessment help track the progress with therapy. Intense exercise therapy is associated with the best outcome. Psychologic issues should be evaluated to determine if further psychologic intervention is indicated. The medium-term outcome is probably good for most of these children, but the long-term prognosis is unknown. One must be aware that other manifestations of psychologic problems might emerge. By the time these children and their families see the rheumatologist they are desperate and can be frustrating to work with due to their difficulty in accepting any kind of psychologic element to the pain and its associated disability. Nevertheless, it is rewarding to help the children understand and work through their pain so they can resume normal lives.
Reflex sympathetic dystrophy (Complex Regional Pain Syndrome type 1) is little known by dermatologists. We report a pediatric case of reflex sympathetic dystrophy with predominant cutaneous involvement.
A 10 year-old girl presented a warm, painful and relapsing right hand edema for seven months (three outbreaks). The hand was cyanotic, pigmented and painful. Routine blood tests were normal. Radiography and radionuclide bone scan were consistent with stage 1 reflex sympathetic dystrophy. Physiotherapy led to dramatic improvement.
Reflex sympathetic dystrophy is known since the XVIIIth century. In the last decade, progress in radiology and bone scan have provided elements for understanding the physiopathology of the disease. Microvascular abnormalities under the control of sympathetic nervous system are characteristic of different stages of reflex sympathetic dystrophy. Recently, neurovascular system experiments showed that sympathetic reflex tonus changes may be controlled by the central nervous system. Dermatologic changes of reflex sympathetic dystrophy are well known: edema and erythema in first stage, cyanosis in second stage, sclerosis and atrophia in third stage, but pediatric cases are rarely reported.
Reflex sympathetic dystrophy is a complex disease, however its physiopathology is now understood. The clinical presentation can be atypical and the dermatologist may be the first to be consulted.
This work was undertaken to identify scintigraphic patterns obtained in patients suspected of having reflex sympathetic dystrophy (RSD), now often referred to as complex regional pain syndrome, whose interpretations could be difficult.
Ten patients had bone scans because of clinical suspicion of RSD in the lower legs. They were selected retrospectively to have a wide sample of scintigraphic patterns. The radionuclide images and a multiple-choice questionnaire were presented as a PowerPoint file that was sent electronically on the Internet to 54 Belgian nuclear medicine physicians. They had to determine whether the images were in favor of the diagnosis of RSD.
Twenty-eight answers (52%) were received. There was near-complete interobserver agreement for perfectly normal scans, for scans showing diffuse uptake with enhancement of periarticular activity, and for scans showing only focal hyperactivity at the site of previous trauma. Results were more discordant when the hyperactivity was mild and when there was a diffuse hypoactivity, with or without focal hyperactivity.
This study shows that using very simple methodology, it is possible to identify some scintigraphic patterns in which there is disagreement among observers and whose interpretations vary. As the results are returned to the participants, they can compare their own interpretations with those of their peers. This aspect could be useful in continuing education in medical imaging.
Complex regional pain syndrome type I (CRPS-I) is a complex disorder characterised by pain, autonomic dysfunction, and decreased range of motion. The syndrome was believed as a well-recognized disorder in adults but, less commonly recognized in children. CRPS-I after vaccination has been rarely reported. We reported an 11-year-old young girl with CRPS-I due to rubella vaccine. © 2004 European Federation of Chapters of the International Association for the Study of Pain. Published by Elsevier Ltd. All rights reserved.
This review summarizes current information about diagnosis and treatment of complex regional pain syndrome (CRPS) in children. Although it has been widely held that CRPS in children is intrinsically different from adults, there appear to be relatively few differences. However, there is a marked preponderance of lower extremity cases in children. Historically, psychological factors have been invoked to explain the genesis and persistence of CRPS in children, but the evidence is not compelling. Treatment outcome studies are limited but indicate that children generally respond to a primary focus on physical therapy. Multidisciplinary treatment reports are particularly encouraging. The general perception that children have a milder course may relate to the potentially greater willingness of children to actively participate in appropriately targeted treatment rather than to innate differences in the disease process itself. Recurrence rates appear higher than in adults, but response to reinitiation of treatment seems to proceed efficiently. Clinical judgment dictates the extent of medication or interventional therapy added to the treatment to facilitate rehabilitation. In many ways, the approach to the treatment of children mirrors that of adults, with perhaps greater restraint in the use of medications and invasive procedures. The rehabilitation of children with CRPS, like that of adults with CRPS, needs further rigorous investigation.
Reflex neurovascular dystrophy has rarely been recognized in children. During the past eight years we have observed 24 instances of RND in 23 children. Lower extremity involvement was manifested in 20 of them and upper extremity in four. The major complaint was pain; swelling and vasomotor instability were prominent, and exquisite tenderness was characteristic. Chronic trophic changes were not observed. Antecedent illness or trauma could be related to the RND in less than half of the children, but personality factors appeared contributory to the development of RND in most children. Physical therapy was the principal form of treatment; therapy with a corticosteroid or by sympathetic blockade was not employed. Reduction in the evidences of disease, including improvement in function, were present in all children at the termination of therapy; improvement was maintained in all but one child after a mean period of 2.4 years. The excellent response to conservative therapy suggests that RND may be a more benign condition in children than in adults.
In a group of 82 children with focal or generalized skeletal pain of obscure etiology, the radionuclide skeletal scintigraphy was the only, or the most informative, clue to the diagnosis of a variety of benign and malignant conditions. It is strongly recommended that any unexplained bone or joint pain in children be evaluated by this non-invasive technique.
A 6 yr old healthy girl suffered a traction injury to her right sciatic nerve. Within 24 hours after the accident, she complained of well-localized pain in the right knee and reported that the area was exquisitely sensitive to touch. Another well-localized sensitive area developed on the plantar surface of the right foot; this became so severe over the next 2 days that she was unable to bear weight, necessitating the use of crutches. The mother noted that very light touch to either of the areas would awaken the child from a sound sleep. Occasionally, the right leg was observed by the mother to be swollen, blue, and blotchy. This tended to occur after painful stimuli, such as attempts at walking. The condition persisted unchanged to the time of admission, 3 months after her initial injury. On the basis of clinical findings, the presence of positive thermography and temperature differentials, and absence of evidence of infection or fracture, a presumptive diagnosis of reflex sympathetic dystrophy was made. A neurolytic lumbar sympathetic block under general anesthesia (because of the patient's age) was recommended, but the parents were reluctant to have this performed. Therefore, it was decided to initiate a trial of transcutaneous nerve stimulation, using a Neuromod Transcutaneous Nerve Stimulator, Model #3700 (Medtronic). Electrodes were arbitrarily placed over the right femoral triangle and the dorsum of the right foot. The frequency of stimulation was set at 90 Hz and the current at 2.5 ma, at which point a tingling but not painful sensation was noted by the patient. Later, these settings were changed to 50 Hz and 3.5 ma, which produced better pain relief. Within 24 hours after initiation of the stimulation, definite improvement in color and reduced hyperesthesia were noted, although repeat thermograms at 24 hours were essentially unchanged. The patient returned to the Pain Clinic 2 weeks after her initial visit. At the time there were no complaints of pain and no findings of hyperesthesia, edema, or cyanosis of the right leg. There was minimal limp on the right side, but she bore body weight without complaint. Skin temperature was equal in both legs, and thermography revealed no difference between the extremities. She was still pain free 1 month after discontinuing treatment.
Reflex sympathetic dystrophy in paediatric patients is a rarely recognized pain syndrome probably of neurovascular origin. The manifectations in two young females consisted of disabling pain and localized hyperesthesia in lower extremities without evident trauma. Sympathetic block followed by active mobilization and, in the patient with atrophic changes, lumbar sympathectomy, resulted in complete recovery. Reflex sympathetic dystrophy should be considered in the differential diagnosis of pain and tenderness in an extremity.
Two pediatric patients had reflex sympathetic dystrophy, which has been well described in adults but is rarely recognized in children. The syndrome consists of continuous pain, hyperesthesia, and autonomic symptoms occurring in an extremity, usually following trauma. The various types of presentation, differential diagnosis, and modes of therapy are discussed.
Sixty-four patients were evaluated prospectively for a reflex sympathetic dystrophy syndrome (RSDS), using quantitative clinical measurements, high-resolution roentgenography and scintigraphy. Five separate groups were identified by their clinical features, allowing us to distinguish patients with definite or incomplete forms of the RSDS as well as 16 patients with other disorders. Scintigraphy was found to be a useful diagnostic study that may also provide a method of predicting therapeutic response. Systemic corticosteroid therapy proved to be a highly effective mode of treatment for up to 90 percent of the patients with the RSDS.
Reflex sympathetic dystrophy (RSD) is a syndrome characterized by persistent, hyperesthetic pain in an extremity with concurrent evidence of autonomic nervous system dysfunction.¹ This syndrome frequently results in a serious functional impairment with associated trophic changes that may eventually develop into permanent dysfunction. Reflex sympathetic dystrophy has been referred to as causalgia, reflex neurovascular dystrophy, post-traumatic pain syndrome, shoulder-hand syndrome, and Sudeck's atrophy. It generally develops after nerve injury, trauma, surgery, cervical osteoarthritis, carcinoma, myocardial infarction, or cerebrovascular accident.¹ The antecedent illness or injury may be relatively minor compared with the severity of the symptoms. In some instances, no antecedent injury or illness can be identified. This syndrome is most prevalent in middle-aged and older adults and is infrequently found in children.1–3 Classical treatments of RSD include active and passive range-of-motion exercises, casting, splinting, administration of corticosteroids, and sympathetic blocks.
Reflex sympathetic dystrophy (RSD) consists of an extremity with (1) burning or causalgic pain, (2) limitation of motion, (3) edema with or without pitting, (4) dystrophic skin changes, (5) vasomotor phenomena and (6) patchy osteoporosis on x ray. This disease is rare in adolescents, but of patients with RSD up to 8% are between 11 and 19 years of age. Most cases in this age group resolve after immobilization, analgesics or steroid therapy, surgical ganglionic blockade or sympathectomy. This case report is of a 14-year-old girl who was treated with all these measures, but continues to have significant residual deformity.
Six patients with reflex sympathetic dystrophy were investigated during the period between 1973 and 1978. Children do not develop the severe, disabling pain nor the patchy osteoporosis (Sudeck's atrophy) which are considered essential features of reflex sympathetic dystrophy in adults. In contrast to the adult variety, reflex sympathetic dystrophy in children is a self-limiting condition which usually responds well to mild analgesics and physical therapy. Frequently it may be necessary to administer the physical therapy in an intensive, inpatient program, both to break the pain-disability cycle and to remove the patient from a stressful family environment that may have initiated or prolonged the syndrome. The use of steroids (dexamethasone) had no appreciable effect on the clinical course in these children.
The correct diagnosis of RSD can be difficult. We have found bone scanning helpful in evaluating 18 patients, making the diagnosis in 13 and excluding it in five. A characteristic pattern of uptake is demonstrated and the results summarized.
Sixty-four consecutive patients were studied for possible reflex sympathetic dystrophy syndrome (RSDS). They were divided into five groups, based upon specific clinical criteria, and the radiographic and scintigraphic findings in each group were examined. Osteoporosis was the most common radiographic abnormality, present in 69% of subjects with definite, probable, or possible RSDS, as compared with 21% opf those with RSDS. Scintigraphic abnormalities were noted in 60% of RSDS patients but in only 7% of the others. These findings included increased blood flow and enhanced periarticular radionuclide activity in the affected extremity. Of 11 patients with serial scintigraphy, six (55%) demonstrated a return to normal, symmetrical patterns following successful therapy. The scan may reflect an active, potentially reversible disorder of local blood flow in RSDS. Furthermore, the scintigraphic patterns may be useful in the diagnosis and in predicting which pattients are likely to respond to systemic steroid therapy.
La place de la scintigraphie osseuse aux pyrophosphate de technetium 99m dans le diagnostic des algodystrophies
- Doury
Doury P, Granier R, Pattin S: La place de la scintigraphie osseuse aux pyrophosphate de technetium 99m dans le diag-nostic des algodystrophies. Ann Med Interne i30:553, 1979.
The reflex sympathetic dystrophy syndrome (RSDD). III. Scintigraphic studies: Further evidence for the therapeutic efficacy of systemic corticosteroids, and proposed diagnostic criteria
- Kozin
Case history number 96: Reflex sympathetic dystrophy in a 6 year old. Successful treatment by transcutaneous nerve stimulation
- Stilz