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Upper extremity reflex sympathetic dystrophy
Current Orthopaedics (2000) 14, 356–364 © 2000 Harcourt Publishers Ltd doi: 10.1054/ cuor.2000.0128, available online at http://www.idealibrary.com on
Upper Limb
Upper extremity reflex sympathetic dystrophy
P. N. Soucacos, E. O. Johnson
response may involve an unchecked generalized (central) and/or local (peripheral) sympathetic response(s) to an insult. The generalized response includes a centrally-mediated ‘stress response’ or ‘fight or flight’ response, as well as responses stemming from the hypothalamus, suprahypothalamic structures (such as the hippocampus) and the mesencephalon.9 Local reactions include adrenocortical-mediated and somatomotor-mediated responses of the somatosympathetic, viscerosympathetic and viscerovisceral nerve fibers. Secondly, peripheral responses to tissue insult include hyperalgesia, diffuse burning pain, altered blood flow and vasomotor control, as well as changes in the integrity of the bone, joints, muscles subcutaneous tissue and skin. These peripheral responses may be in response to alterations in sympathetic activity induced by receptor blockade, neural blockade or transmitter depletion. There is evidence suggesting that upregulated sensitivity of alphaadrenoreceptors for catecholamines may induce RSD.10 Other theories as to the pathogenesis of RSD have been proposed, including abnormal feedback into the internuncial centers of the spinal cord, periarteritis of the vessels surrounding the injured segment and shortcircuiting effects at the area of injury, which lead to irritation of the sensory afferents by the efferent sympathetic impulses. It has been proposed that activation of the nocioceptors leads to excitation of an internuncial pool of neurons of the spinal cord, with induction of increased activity of the efferent sympathetic system.11 The subsequent vasoconstriction, with ischaemia of the tissue, was believed to stimulate nocioceptors with re-excitation of the spinal cord. Thus, injury produces painful impulses that travel via afferent pathways to the spinal cord where a series of reflexes spread via the interconnecting neuron pool. These reflexes stimulate the anterior and lateral spinal
INTRODUCTION Reflex sympathetic dystrophy (RSD) represents a complex, multifaceted syndrome with a plethora of signs and symptoms which vary both between individuals and with the progression of the disease over time. Various clinical types of RSD are now recognized which present as a modification of the normal pain response. In the upper extremity, RSD usually occurs after trauma to the nerves and/or muscles, surgery (e.g. carpal tunnel release), or other factors (primarily overuse syndromes).1–5 The great variety of treatment regimes used today to manage RSD, appear to provide only temporary relief of symptoms in most cases. It is likely that this may be related not only to mostly unimodal therapeutic paradigms, but to the difficulties faced in diagnosis, as well as classification of the disease.
AETIOLOGY AND PATHOPHYSIOLOGY RSD is believed to be an abnormally severe and prolonged manifestation of the normal physiologic response of the sympathetic nervous system to pain after injury.6–8 The prolongation of a normal sympathetic response disrupts homeostasis, and an otherwise adaptive response, becomes chronic and pathologic. There are two general mechanisms by which the sympathetic system may be involved in an abnormal pain response. First, the abnormal pain
P. N. Soucacos MD FACS Professor and Chairman, Department of Orthopaedic Surgery; E. O. Johnson PhD, Department of Anatomy, University of Ioannina, School of Medicine, Ioannina 45110, Greece. Correspondence to: P. N. S. Tel: +30 651 97 515; Fax: +30 651 46 222.
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Upper extremity reflex sympathetic dystrophy tracts, resulting in stimulation of the efferent pathways that travel via the peripheral nerves, resulting in peripheral manifestations of RSD. Although the sympathetic component of RSD is widely accepted, recent data suggest that the early symptoms closely resemble those of an inflammatory reaction.12 The hypothetical inflammatory agent is believed to be an endogenous pro-inflammatory agent which can induce hyperaemia, such as histamine. However, neuroinflammatory mediators, such as neuropeptide-y, substance P, bradykinin and calcitonin gene-related peptide may have an important role in the pathogenesis of the neurologic components in RSD, and maybe involved in the inflammatory reaction and accompanying motor and sensory disturbances that occur.12 Other similar hypotheses on the pathogenesis of RSD reflect the involvement of the central nervous system in an exaggerated peripheral (neuro)inflammatory response to tissue injury,13 which is related to the tight intercommunication between the nervous system and the inflammatory/ immune system.9 Overall, the various findings regarding pathogenesis underscore the complexity of the disease and its presentations and have led to further refinements of the initial definition, such as sympathetically maintained pain (SMP), sympathetically independent pain (SIP) and complex regional pain syndrome (CRPS) in an effort to delineate more accurately the abnormal pain alterations in response to trauma.14,15 The pathophysiology of the syndrome, however, remains obscure and an appropriate animal model for its in depth investigation is still lacking.
CLINICAL SIGNS AND SYMPTOMS OF RSD RSD of the upper extremity typically involves severe pain, swelling, stiffness and discolouration of the extremity which presents after trauma or disease. The entire symptom complex is rarely observed in one patient and usually only one sign or symptom predominates. Four cardinal signs and symptoms and six secondary signs and symptoms compose the clinical presentation of RSD.8,16,17 The four primary signs and symptoms are pain, swelling, stiffness and discolouration of the extremity, all of which are considerably worse than expected from the initial injury or disease
Table 1 Primary signs and symptoms of RSD Sign
Features
Pain
Paramount feature; often with burning, throbbing, aching, stabbing, bursting pressure or crushing sensations First physical sign; initially local soft oedema then extensive and hard Progressively worsens due to increased fibrosis Red, cyanotic, or pale to grayish; related to vasomotor instability
Swelling Stiffness Discolouration
(Table 1). Secondary symptoms and signs are diffuse osteoporosis, pseudomotor and temperature changes, as well as vasomotor instability, palmar fasciitis and trophic changes (Table 2). Swelling of the hand is normally the first physical sign of the RSD complex observed by the patient and clinician. Initially, the swelling is local and in the form of a soft oedema. It then spreads over the extremity and eventually becomes hard and brawny. Only in the late stages of the disorder, does the swelling increase dramatically around the joints producing profound peri-articular thickening. The resultant stiffness of the hand and extremity is much worse than expected from the initial injury. It increases significantly if untreated, and is usually attributed to an increase in fibrosis and adhesion formation. Excruciating burning pain is the most characteristic clinical feature of RSD. It is usually described as burning, and is often combined with a throbbing, aching, stabbing, bursting pressure or crushing sensation. The magnitude of the pain is generally out of proportion to the severity of the trauma or disease. The pain frequently begins immediately after injury and in the early stages of the disease the pain is constant and increases notably with all attempts at motion. Most patients complain of paraesthesiae, even with light touch.17,18 The pain is usually located in the anatomical region corresponding to the cutaneous distribution of the nerve affected. Some discolouration is always present in RSD. Discolouration of the hand varies from a more common notable redness to pale or grayish or a cyanotic colour, which appears initially over the dorsal surface of the metacarpophalangeal joints and then spreads over the interphalangeal joints. In late RSD, the discolouration often spreads over the entire dorsal surface of the hand. The colour of the extremity appears to be directly related to the presence of vasomotor
Table 2 Secondary signs and symptoms of RSD Sign
Features
Osteoporosis
Ranges from speckled appearance to marked osteopaenia in polar regions; it may be marked on X-ray examination Hyperhidrosis is observed early, and progressively becomes dry in late stages Related to vasomotor changes; warm extremity in early stages and cool skin later Vasospasm common Acute nodules and thickening of long bands of palmar fascia Glossy appearance due atrophy of skin and subcutaneous tissue
Pseudomotor changes Temperature changes Vasomotor instability Palmar fasciitis Trophic changes
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instability.4,5 Specifically, redness is usually associated with dilation of both the arterial and venous systems, while pallor is associated with vasoconstriction of both the arterial and venous systems. On the other hand, a blue or cyanotic extremity is normally seen with vasospasm of the venous system only. A purple discolouration is often observed in the flexor creases, particularly when palmar fasciitis is present. Osteoporosis, as a secondary change in patients with RSD, is much more severe than expected from the initial injury or disease and is usually quite marked upon radiographic examination. Early in the course of the disease, demineralization is speckled and is commonly referred to as ‘Sudeck’s atrophy’.19 Subsequently, demineralization is located primarily in the polar regions and in the final stages of the disease osteoporosis can be profound, spreading homogeneously throughout the affected area. Sudomotor changes observed in RSD are not as noteworthy. Commonly, hyperhidrosis is observed early on. The sweating slowly diminishes over the course of the disease and dryness is observed in the last stages. Although most patients are able to recall a significant temperature difference in their affected limb when their symptoms were first noted, the temperature of the affected extremity of RSD patients is variable. At the onset of RSD, during which time the extremity increases in redness, the temperature is usually elevated. When pallor or cyanosis are present, the perceived temperature tends to be low. Thermoregulatory changes have been found to be directly related to vasospasm,4,5 and vasomotor instability is commonly noted. In general, vasomotor instability in the later stages can be verified with a prolonged capillary refill time upon pinching of the finger tip or nail bed. Palmar fasciitis can be observed in a number of patients with RSD with the formation of acute nodules and thickening of the long bands of palmar fascia. Finally, trophic changes are noteworthy in RSD patients and are most commonly associated with a glossy and shiny skin appearance. Initially, the glossy smooth skin surface of the affected hand is attributed to the severe oedema which smoothes out the normal creases in the skin’s surface. Later, however, the glossy skin appearance is a result of the significant atrophy of the skin and subcutaneous tissue which progressively increases with time.
CLINICAL COURSE OF RSD The excessive prolongation of the sympathetic response constitutes the basis of the major symptoms of reflex sympathetic dystrophy. Of the signs and symptoms, usually only one is paramount in a single patient. Compounding the significant variation due to individual differences, it is well recognized that these signs and symptoms also vary with the time course of the disease. The course of RSD can be divided into three stages according to the evolution of the symptoms.20,21 The clinical picture varies significantly as the patient passes through early, intermediate and late stages of the disorder (Table 3). Early RSD: ‘the traumatic stage I’ In the early, acute stage (stage I), pain, vasomotor instability and sympathetic overactivity are evident. This is the so-called ‘traumatic stage’, which extends from the first to the third month, and is characterized by an intermittent burning pain, swelling and lack of motion, as well as redness and sweating of the extremity. Very soon after injury vasodilation produces hyperaemia and oedema. Swelling also starts early in this stage and is usually soft. Initially, pain is the most pronounced feature. There is usually painful paraesthesia to light touch, but pain with motion is not normally noted until later. Stiffness is predominantly related to the pain upon movement and becomes progressively worse throughout stage I. Discolouration is variable. The extremity tends to be red and warm at the onset, although throughout the later part of stage I, the extremity becomes cool and cyanotic due to vasoconstriction and vasospasm. Osteoporosis does not appear until the end of stage I and usually not until after the 3rd to 5th week. At its onset, osteoporosis in RSD takes on a patchy appearance. Intermediate RSD: ‘the dystrophic stage II’ The second or ‘dystrophic’ stage begins from approximately the third month and lasts until about the 9th to 12th month. During this stage, the extremity begins to cool and the skin undergoes atrophy and becomes shiny. Characteristic of stage II is the marked stiffening of the extremity (related to the hardening of the swollen extremity), increased pain and a decrease in
Table 3 Clinical course of RSD Primary Signs
Early RSD (0–3 months)
Intermediate RSD (3–9 or 12 months)
Late RSD (9 or 12 months–years)
Pain
Paraesthesia
Notable increase, pain with motion
Swelling Stiffness
Soft local oedema Pain-related
Hard, brawny oedema over extremity Increased stiffening due to fibrosis
Discolouration Principle feature of the stage
Red then cyanotic Traumatic
Cyanotic, with redness over joints Dystrophic
Diminished pain, but severe with motion Periarticular (minimal) Peak stiffening, contractures common Pallor Atrophic
Upper extremity reflex sympathetic dystrophy sweating and redness. Swelling changes from the soft oedema observed in stage I to anoedema which is brawny and hard, as fluid and protein deposits begin to appear in the capsular structures. Increased redness is noted, particularly around the joints, as well as an increased warmth and dryness of the extremity. There is decreased sweating as the vasomotor instability persists. Peripheral vasospasm resulting from persistent vasoconstriction is more evident. Polar demineralization is usually severe and more widespread. An increasingly glossy appearance of the hand is also noteworthy, as the skin and subcutaneous tissue undergoes atrophy. Flexion creases are eventually lost and palmar fasciitis may also be present. Late RSD: ‘the atrophic stage III’ Stage III or the ‘atrophic stage’ begins around the 9th or 12th month and may last for several years. The pain, which peaked in stage II, may slowly begin to diminish. Although pain may subside, in the majority of patients it remains quite severe with motion. On the other hand, stiffness peaks in late RSD and the extremity often resembles a Volkmann’s ischaemic contracture. The swelling changes from brawny, hard oedema to a pronounced periarticular thickening of the joints. In general, the extremity is pale, dry and cool. Osteoporosis is often profound and diffuse. The most striking feature of the last stage of RSD is the glossy appearance of the hand, which is related to the significant degree of skin and subcutaneous tissue atrophy which has taken place.
DIAGNOSTIC CRITERIA OF RSD Although RSD applies to an extremity with excruciating burning pain, swelling, stiffness and discolouration which present after trauma or disease, its presentation varies greatly. RSD of the upper extremity can range from minor involvement including one or more fingers (segmental RSD) to involvement of large anatomic regions or of the entire extremity (regional RSD). RSD also presents with widely varying degrees of pain and dysfunction. Due to these significant variations in the signs and symptoms, diagnosis is often difficult.
Table 4 Diagnosis of RSD Diagnostic criteria Persistent diffuse pain Loss of hand function Autonomic dysfunction Evidence for autonomic dysfunction Altered blood flow Altered temperature Altered sweating Skin and subcutaneous tissue atropy Oedema
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In general, the three pre-requisites underlying the development of RSD are required for diagnosis: a persistent lesion or injury, a predisposing constitution, and an abnormal sympathetic reflex arc (Table 4).4–6,21 The persistent lesion is often a complication of trauma to the nerves and/or soft tissues, surgery or other factors, such as disease or overuse. In this regard, RSD has been observed after a wide range of injuries and operations. The majority of patients are females, with a female-to-male ratio of 3:1. The primary aetiological factor associated with the expression of RSD is trauma (57.4%), followed by surgery (22.9%) and, overuse syndromes (19.7%).4 RSD has been associated with various diseases, including stroke and diabetes mellitus. Although not definitive, most clinicians believe that a significant number of RSD patients have a predisposing or susceptible personality.22 The inherent constitutional traits of the patient appear to make them more vulnerable to develop RSD after injury. Some traits frequently associated with RSD patients include anxiety, dependency, insecurity, an overpowering sense of dissatisfaction and other personality disturbances.23,24 Finally, central to the diagnosis of RSD, is an abnormality in the normal sympathetic response arc. Most of the signs and symptoms characteristic of RSD, such as swelling, stiffness, vasomotor and thermoregulatory instability, can be attributed at least in part to sympathetic dysfunction. Initially, other conditions such as Dupuytren’s disease, hypertrophic osteoarthritis, psoriatic arthritis, carpal tunnel syndrome and stenosing tenosynovitis, which are associated with pain, swelling, stiffness and dysfunction, should be ruled out. Thereafter a presumptive diagnosis can be made on the presence of some or all of the signs and symptoms.6,17,20,21 Exact diagnostic criteria remain controversial, although the presence of all four cardinal signs and symptoms and at least one secondary sign and symptom is generally sufficient to presume RSD. However, a presumptive diagnosis can be made with fewer signs and symptoms if other diseases have been effectively ruled out and when the observed condition is severe. Confirmation of the presumed diagnosis is achieved only after disruption of the sympathetic nerve reflex produces a significant decrease in the symptom complex expressed by the patient.17 Although RSD is confirmed if interruption of the sympathetic reflex produces relief of symptoms, failure of relief after sympathetic block does not necessarily rule out RSD. RSD should not be excluded until after at least three or more blocks have been carried out with no amelioration of the symptoms.4,5 Today, diagnosis of RSD takes into consideration the criteria established in the Consensus report of an ad hoc committee of the American Association for Hand Surgery on the definition of RSD syndrome, as well as the criteria outlined by Kozin.23,25 Diagnostic criteria included persistent diffuse pain, the loss of
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hand function and autonomic dysfunction. Evidence for autonomic dysfunction can be based on changes in skin and/or soft tissue blood flow, as reflected by an increase or decrease in temperature and sweating. Three-phase bone scans can also be performed as a supplemental diagnostic means for confirmation of RSD.4,5 Although the specificity and sensitivity of the method remain somewhat debatable in so far as information on the patient’s angiogram (phase I) and blood pool (phase II) are questionable, the findings from the third or delayed metabolic phase appear to be valuable in evaluating the patient.26–28 The criteria for diagnosis of segmental versus regional dystrophy are similar, with the exception that the anatomic involvement is less than the entire hand for patients with segmental dystrophy.4,5
PRINCIPLES OF TREATMENT Treatment of RSD is primarily geared towards disruption of the abnormal sympathetic response. However, the clinician should also attempt to ameliorate the other components of the diagnostic triad: the initiating injury and predisposing personality. In this regard, initial efforts should attempt to eliminate the injury which propagated the pain complex. This may range from the simple removal of a cast to a surgically demanding revision of an amputation or removal of a painful neuroma. Secondly, various approaches can be applied to help modify the patient’s inherent diathesis, including therapy or medications. The major component of treatment is interruption of the abnormal sympathetic reflex which can be achieved using an individually tailored regime of stellate ganglion blocks, somatic nerve blocks and sympatholytic drugs (Table 5). The sympatholytic and antihypertensive drug, guanethidine, has been given successfully to a selected number of patients.4,5 Guanethidine acts by blocking catecholamine storage in sympathetic nerve terminals, inhibiting pre-synaptic release of noradrenaline. This results in reduction of sympathetically-induced vasoconstriction of the peripheral vessels.29–31
In order to carry out a guanethidine block, 10 mg guanethidine in 25 ml of normal saline is administered using the Bier double-tourniquet block technique. The tourniquet is released after 20 min.4,5,30 Stellate ganglion blocks can be applied both therapeutically and diagnostically. Stellate blocks inhibit efferent impulses to the extremity, interrupting the abnormal sympathetic reflex without blocking normal somatic nerve function. By preventing sympathetic activity in the extremity, the patient’s skin becomes warmer and dryer, returns to a normal colour and pain is reduced. Technically, stellate blocks are much more demanding to perform than IV regional blocks, and employ either bupivacaine (Marcaine, 15–20 ml of 0.25%) or lignocaine (Xylocaine, 20 ml of 1%). Amelioration of the symptoms is usually noted after about 30 min, and may last up to a few hours. Three to 10 sessions can be performed at 10–14-day intervals. The block is considered successful when a Horner’s sign develops and warming of the affected hand is observed. We have reserved the application of stellate ganglion blocks mainly for treating patient’s with shoulder–hand syndrome.4,5 Local anaesthetic blocks of somatic nerves can be performed using lignocaine (Xylocaine). Somatic nerve blocks are aimed at interrupting the abnormal reflex through the somatic nerves, by blocking afferent nerve impulses. Blocks can be administered two to three times per week without producing local irritation. It is well recognized that steroids, in moderation, can help to reduce pain, swelling, stiffness and dysfunction.32–34 Corticosteroids, specifically, are effective in the treatment of RSD.25 Thus, lidocaine can be applied regionally along with corticosteroids (5 ml of 2% lidocaine in 15 ml of normal saline and 80 mg of prednisone in 5 ml of normal saline). Together, these prevent increased sympathetic stimulation from reaching the effected area.35 Various adjunctive modes of therapy are also applied to the management of RSD. Elimination of the factors causing pain is primarily achieved by antiinflammatory drugs and vasodilators, which reduce swelling and stiffness, as well as corticosteroids which can be administered with local anaesthetic. Calcitonin
Table 5 Treatment of RSD Method Agent Alternatives Purpose
Stellate ganglion block Bupivacaine (Marcaine) or lignocaine (Xylocaine) Continuous blockage with catheter Blocks efferent impulses to extremity. Not somatic nerves Alleviates symptoms due to sympathetic dysfunction
Method Agent Alternatives Purpose
Somatic nerve blocks Lignocaine (Xylocaine) Bupivacaine (Marcaine)—longer acting agent Blocks afferent pain impulse and interupts abnormal somatic nerve reflex Blocks sympathetic fibres for short time, assists in diagnosis Alleviates pain and other symptoms
Method Agent Alternatives Purpose
Sympatholytic drugs Guanethidine Alpha-adrenergic blocking agents (phentolamine) or catcholamine depleting agents (reserpine) Catecholamine storage blocker at sympathetic neuroeffector junction Alleviates symptoms due to autonomic dysfunction
Upper extremity reflex sympathetic dystrophy nasal spray (100 IU per day), which has been reported to significantly decrease pain and improve the range of motion, is also given.36 Warm gloves can be used to reduce muscle spasm. The loss of hand function can be effectively managed following Watsons’ hand stress rehabilitation program by using both ‘scrub’ and ‘carry’ exercises.37 Hand therapy is also effective in eliminating pain. In general, the programme couples stress loading with fundamental modalities of hand therapy. Stress is provided by active traction and compression exercises which avoid the use of joint motion. The programme is progressive, starting with short repeated session of ‘scrub’ exercises, progressing to ‘carry’ exercises, which increase in difficulty as the tolerance of the patient increases. Other exercises and range of motion activities are initiated once a notable depreciation in pain has occurred. Night splints can be used to return the hand to a normal resting position and relieve pain during motion. Caffeine and smoking are restricted; this is of particular significance, since smoking has been implicated in the pathogenesis of RSD.38 Reconstructive procedures and psychological support, either with medication or therapy, are applied as indicated by the patient’s needs. In summary, although the specific treatment protocol should be individualized according to the patient’s needs, successful treatment requires a multimodal regime aimed at interrupting the abnormal sympathetic reflex arc, eliminating the initiating pain and managing the loss of hand function.4,5 Management of the abnormal sympathetic reflex can be achieved using stellate ganglion blocks, and the regional application of guanethidine, lignocaine and glucocorticoids.
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FEATURES AND MANAGEMENT OF THE CLINICAL TYPES OF RSD Five clinical types of RSD are now recognized.17,20,21 These are minor causalgia, minor traumatic dystrophy, shoulder–hand syndrome, major traumatic dystrophy and major causalgia. The term ‘causalgia’ (burning pain) is usually reserved to reflect RSD arising from nerve injury which often involves a mixed nerve.21 However, RSD more frequently involves injury to soft tissues, joints or bone, rather than damage to a specific nerve, and in these cases is referred to as ‘traumatic dystrophy’. Specific nerve injury or causalgia can be minor, with less severe symptoms arising from trauma to peripheral or digital nerves, or major when damage to a major nerve, such as the median nerve, is involved and very severe symptoms are produced. The most common form of RSD, traumatic dystrophy, can be either minor or major depending on the severity of the trauma to the soft tissues, joint or bone. Finally, shoulder–hand syndrome arises usually from trauma to the proximal part of the body or from visceral disease (Table 6). MINOR CAUSALGIA Minor causalgia is the least severe clinical type of RSD and is generally associated with less pain, swelling, stiffness, discolouration and osteoporosis than other types. Minor causalgia is generally attributed to injury of a purely sensory cutaneous nerve. It tends to involve only the nerves of the wrist and hand and thus only a small region of the hand, usually one or
Table 6 Prominent features of the five clinical types of RSD Type of RSD
Major features
Minor causalgia
Associated with cutaneous nerve injury Involves nerves of hand or wrist Affects one or two fingers or small part of hand Less pain, swelling, stiffness and osteoporosis
Minor traumatic dystrophy
Most common, usually involves one or few fingers Initial trauma = crush, fx/dl, sprain, puncture wound Red dorsum over joints, mild palmar fasciitis Stiff digits, flexion contracture
Shoulder–hand syndrome
Pain stiffness in the shoulder Swelling hand, wrist, arm Initial trauma proximal Diffuse redness, warm, dry extremity Palmar fasciitis
Major traumatic dystrophy
Increased pain and symptoms Flexion contracture of digits common Limited wrist motion, carpal tunnel syndrome common Major initial trauma
Major causalgia
Extreme pain, associated with major mixed nerve injury Damage usually proximal Slight redness, sweating, coolness Extreme stiffness, flexion contracture of fingers
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two fingers, are affected. The injured nerve is usually purely sensory and is commonly the dorsal superficial sensory branch of the radial nerve, palmar cutaneous branch of the median nerve, dorsal superficial branch of the ulnar nerve or digital nerves. The injured nerve is frequently trapped by fibrous tissue or adhesions distal to the joints which leads to neurodesis and pain when the joint is moved. It is the least severe form of RSD and is associated with less pain, swelling, stiffness, discolouration and osteoporosis. We have reported that minor causalgia is predominantly related to overuse syndromes and more rarely is a complication of trauma or surgery.4,5 Pain is the most prominent symptom in these patients, although the intensity of pain tends to be moderate and significantly lower than that experienced by patients with shoulder–hand sydrome.4,5 Most patients with minor causalgia pursue treatment after bearing the symptoms for a relatively long period of time. The majority of patients do not demonstrate problems with swelling or present a hard, brawny oedema. Stiffness is frequently related to pain during movement and not to fibrosis.4 Of the secondary signs and symptoms those most frequently observed are osteoporosis (in about 12% of patients), Sudomotor changes (36%), thermoregulatory abnormalities (20%), vasomotor instability (20%) and trophic changes (20%).4,5 Treatment of patients with minor causalgia primarily consists of somatic nerve blocks with the local administration of lignocaine, plus corticosteroids, for two to eight sessions. Some patients may require stellate ganglion blocks or guanethidine. The majority of patients with minor causalgia exhibit excellent results following treatment. Of significance, the quality of the results is associated with early onset of treatment. Specifically, patients with excellent results sought treatment significantly earlier than to those whose results were fair.4 Minor traumatic dystrophy Minor traumatic dystrophy is the most common of the clinical types of RSD. Frequently this form of dystrophy is related to a definite, but mild injury, such as a mild crushing of the hand, a fracture/dislocation, sprain or puncture wound, rather than a specific nerve injury. It expresses with redness dorsal to the joints, a mild palmar fasciitis and stiff digits, usually with a flexion contracture. Often minor traumatic dystrophy is overlooked, particularly if it involves only a small part of the hand or one or more fingers. The majority of patients with minor traumatic dystrophy have segmental involvement.5 Minor traumatic dystrophy is primarily attributed to trauma (53.6%) followed by surgery (32.1%). Pain is usually moderate and significantly less than the pain scored for shoulder–hand syndrome and major traumatic dystrophy. The majority of patients have soft oedema over the affected extremity and stiffness is
equally attributed to pain and fibrosis.4 Vasomotor instability in the form of vasoconstriction or vasodilation can be observed, which is compatible with the temperature differences noted in these patients. Hyperhidrosis is frequently observed and a few patients also show radiographic signs of osteoporosis. A glossy appearance of the skin over the affected region can be observed in about 50% of the patients.4 The majority of cases with minor traumatic dystrophy can be effectively treated with somatic nerve blocks with lignocaine plus steroid over two to 10 sessions. Rarely guanethidine or stellate ganglion blocks may be required, although when used they are effective. A high rate of excellent results (71.4%) can be anticipated. Good results obtained after treatment are dependent upon the initiation of early treatment.4,5 The shoulder–hand syndrome The shoulder–hand syndrome is characterized by pain and stiffness in the shoulder and/or entire upper extremity with significant swelling of the hand and wrist, if not the entire extremity. Characteristically it starts with pain and stiffness in the shoulder area which then spreads to the entire extremity. The initiating trauma is usually proximal (e.g. injury to shoulder, neck or rib cage). It is also frequently associated with visceral disorders such as myocardial infarction, stroke, gastric ulcer, cervical osteoarthritis and even hemiplegia.39 Limited or minor hand trauma may also predispose the patient to the onset of shoulder–hand syndrome. The signs of the shoulder–hand syndrome include a diffusely red, warm and dry extremity which exhibits palmar fasciitis. Swelling is usually fusiform, with the fingers in extension and not flexion. Vasomotor disturbances, including both vasoconstriction and vasodilation, are common. Mottled osteoporosis of the humeral head and wrist are characteristic radiographic findings. Patients can be treated with two to five stellate blocks and with lignocaine and corticosteroids administered locally. Although the excellent outcome observed for the other types of RSD is not as often seen, fair to good results can be anticipated with early treatment.4 Major traumatic dystrophy Major traumatic dystrophy presents with more severe pain and symptoms than does minor traumatic dystrophy. It is usually attributed to a major injury, such as a severe crush injury, Colles’ fracture, or severe wrist fracture/dislocation. It has also been associated with elective surgery such as carpal tunnel syndrome. A number of patients with Colles’ fracture and RSD have also been noted to have compressive neuropathy of the median nerve secondary to carpal tunnel syndrome. Flexion contractures are common. Wrist motion, as well as rotary motion of the forearm tend to be limited in these patients.
Upper extremity reflex sympathetic dystrophy Almost all patients should undergo treatment with somatic nerve blocks. Stellate ganglion blocks may also be required in a few selected cases. Good to excellent results can be anticipated for more than half of the patients. Moreover, patients treated for major traumatic dystrophy early in the course of their disease, achieve significantly better results; patients with an excellent outcome received therapy by 4–5 months, compared to those with fair results who did not seek therapy until after about 19–20 months.4,5
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oedema, stiffness and discolouration) are prominently expressed in each clinical type, while secondary signs and symptoms vary significantly across the different types of RSD. Despite the wide range in disease expression, all clinical types of RSD respond equally well to the multimodal treatment regime. More importantly, however, the available data overwhelmingly support that treatment is most effective if commented early in the disease process. REFERENCES
Major causalgia Major causalgia is relatively infrequent and is characterized by severe pain. It is usually attributed to an injury of a major mixed nerve, such as the median nerve, which has a larger number of sympathetic fibres. Patients usually complain of increased pain from light touch or vibration. The extremity manifests a slight redness, which is somewhat less than that observed with other types of RSD, and in early stages may be pale or cyanotic. Sweating and coolness tend to be prominent. Flexion contractures are known to occur, although they may not be as extreme as observed for major traumatic dystrophy. A hard, brawny oedema is often noted with, cyanotic discolouration and stiffness related to fibrosis. However, mild oedema with stiffness related to pain and diffuse red discolouration may be present instead. Vasomotor instability can be observed with vasoconstriction or vasodilation, and a corresponding decrease or increase in the temperature of the affected hand. Severe osteoporosis and sudomotor dysfunction is also common. Excellent results can be achieved with stellate ganglion blocks and guanethidine or local somatic nerve blocks.
CONCLUSIONS RSD is a complex clinical entity which applies to an extremity with severe pain, swelling, stiffness, discolouration and a variety of other symptoms mostly attributed to autonomic dysfunction in the limb. The entire symptom complex is rarely found in any single patient, but rather one sign or symptom predominates. The signs and symptoms of the disorder demonstrate considerable individual variation, and significant variations occur with the progression of the disease over time. However, it is generally agreed that the most striking feature of RSD is a marked, burning pain, which also varies significantly with the evolution of RSD. Upper extremity RSD occurs more frequently in young adult women, with a female:male ratio which approaches 3:1. Sex differences vary however with each clinical type of RSD. Minor traumatic dystrophy is observed most frequently, whilst the shoulder–hand syndrome and major causalgia are relatively rare. The primary signs and symptoms (pain,
1. Fitzcharles M A, Esdaile J M. Carpal tunnel syndrome complicated by reflex sympathetic dystrophy syndrome. Br J Rheumatol 1991; 30: 468–470. 2. Grundberg A B, Reagan D S. Compression syndromes in reflex sympathetic dystrophy. J Hand Surg 1991; 16A: 731–736. 3. Monsivais J J, Baker J, Monsivais D. The association of peripheral nerve compression and reflex sympathetic dystrophy. J Hand Surg 1993; 18B: 337–338. 4. Soucacos P N, Diznitsas L A, Beris A E, Xenakis T A, Malizos N. Reflex sympathetic dystrophy of the upper extremity: Clinical features and response to multimodal management. Hand Clinics 1997; 13: 339–354. 5. Soucacos P N, Diznitsas L, Beris A E, Malizos K N, Xenakis T A, Papadopoulos G S. Clinical criteria and treatment of segmental versus upper extremity reflex sympathetic dystrophy. Acta Orthopaedica Belgica 1998; 64: 314–321. 6. Dzwierzynski W W, Sanger J R. Reflex sympathetic dystrophy. Hand Clinics 1994; 10: 29–44. 7. Goldner J L. Pain: general review and selected problems affecting the upper extremity. J Hand Surg 1983; 8: 740–745. 8. Koman L A, Smith T L, Poehling G G, Smith B P. Reflex sympathetic dystrophy. Curr Opinion Orthop 1993; 4: 85–88. 9. Johnson E O, Kamilaris T C, Chrousos G P, Gold P W. Mechanisms of stress: a dynamic overview of hormonal and behavioral homeostasis. Neurosci Biobehav Rev 1992; 16: 115–130. 10. Arnold J M, Teasell R W, MacLeod A P et al. Increased venous alpha-adrenoceptor responsiveness in patients with reflex sympathetic dystrophy. Ann Intern Med 1993; 118: 619–621. 11. Livingston W K. Pain mechanisms: Aphysiological interpretation of causalgia and its related states. London: MacMillan, 1944. 12. van der Laan L, Goris R J A. Reflex sympathetic dystrophy. an exaggerated regional inflammatory response. Hand Clinics 1997; 13: 373–386. 13. Oyen W J, Arntz I E, Claessens R M et al. Reflex sympathetic dystrophy of the hand: An excessive inflammatory response? Pain 1993; 55: 151–157. 14. Veldman P H J M, Reynen H M, Arntz I E, Goris R J A. Signs and symptoms of reflex sympathetic dystrophy: prospective study of 829 patients. Lancet 1993; 342: 1012–1016. 15. Roberts W J. A hypothesis on the physiological basis for causalgia and related pains. Pain 1986; 24: 297–311. 16. Inhofe P D, Garcia-Moral C A. Reflex sympathetic dystrophy: a review of the literature and long-term outcome study. Orthop Rev 1994; 655–661. 17. Lankford L L. Reflex sympathetic dystrophy. In: Hunter J M, Schneider L H, Mackin E J et al. (eds). Rehabilitation of the hand—surgery and therapy. St. Louis: CV Mosby, 1990; 763–786. 18. Rosenthal A K, Wortmann R L. Diagnosis, pathogenesis and management of reflex sympathetic dystrophy syndrome. Compr Ther 1991; 17: 46–50. 19. Sudeck P H M. Uber die acute entzundliche Knockenatrophie. Arch Klin Chir 1900; 62: 147. 20. Lankford L L, Thompson J E. Reflex sympathetic dystrophy, upper and lower extremity: diagnosis and management. In: Instructional course lectures: The American Academy of Orthopaedic Surgeons. St. Louis: C V Mosby, 1977: 163–178.
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21. Schwartzman R J, McLelland T L. Reflex sympathetic dystrophy: a review. Arch Neurol 1987; 44: 551–561. 22. Boyes J H. Trophic and vascular conditions. In: Bunnell’s surgery of the hand, 5th edn. Philadelphia: J B Lippincott, 1978; 643–651. 23. Amadio P C, Mackinnon S E, Merritt W H, Brody G S, Terzis J K. Reflex sympathetic dystrophy syndrome: Consensus report of an ad hoc committee of the American Association for Hand Surgery on the definition of reflex sympathetic dystrophy syndrome. Plast Reconstr Surg 1991; 87: 371–375. 24. Hardy M A, Merritt W H. Psychological evaluation and pain assessment in patients with reflex sympathetic dystrophy. J Hand Therapy 1988; 3: 155–164. 25. Kozin F, Genant H K, Bekerman C, McCarty D J. The reflex sympathetic dystrophy syndrome. II. Roentgenographic and scintigraphic evidence of bilaterality and of periarticular accentuation. Am J Med 1976; 60: 332–338. 26. Kline S C, Beach V, Holder L E. Segmental reflex sympathetic dystrophy: Clinical and scintigraphic criteria. J Hand Surg 1993; 18A: 853–859. 27. Mackinnon S E, Holder L E. The use of three-phase radionuclide bone scanning in the daignosis of reflex sympathetic dystrophy. J Hand Surg 1984; 9A: 556–563. 28. Verner R, Davidoff G, Jackson D, Cremer S, Ventocilla C, Wolf L. Factors affecting the sensitivity and specificity of the three-phase technetium bone scan in the diagnosis of reflex sympathetic dystrophy syndrome in the upper extremity. J Hand Surg 1989; 14A: 520–523.
29. Eulry F, Lechevalier D, Pats B et al. Regional intravenous guanethidine blocks in algodystrophy. Clin Rheumatol 1991; 10: 377–383. 30. Hannington-Kift J G. Intravenous regional sympathetic block with guanethidine. Lancet 1974; 1: 1019. 31. Hannington-Kift J G. Pharmacological target block in hand surgery and rehabilitation. J Hand Surg 1984; 9B: 29–36. 32. Glick E N. Reflex dystrophy (algoneurodystrophy): results of treatment by corticosteroids. Rheumatol Rehabil 1973; 12: 84–88. 33. An H S, Hawthorne K B, Jackson W T. Reflex sympathetic dystrophy and cigarette smoking. J Hand Surg 1988; 13A: 458. 34. Rosen P S, Graham W. The shoulder–hand syndrome: historical review with observations on 73 patients. Can Med Assoc J 1957; 77: 86–91. 35. Ross J P: Causalgia. St Barthol Hosp Rep 1932; 65: 103–188. 36. Tountas A A, Noguchi A. Treatment of posttraumatic reflex sympathetic dystrophy syndrome (RSDS) with intravenous blocks of a mixture of corticosteroid and lidocaine: A retrospective review of 17 consecutive cases. J Orthop Trauma 1991; 5: 412–419. 37. Bickerstaff D R, Kanis J A. The use of nasal calcitonin in the treatment of posttraumatic algodystrophy. Br J Rheumatol 1991; 30: 291–294. 38. Watson H K, Carlson L. Treatment of reflex sympathetic dystrophy of the hand with an active ‘stress loading’ program. J Hand Surg 1987; 12A: 779–785. 39. Steinbrodker O, Argyros T G. The shoulder–hand syndrome: present status as a diagnostic and therapeutic entity. Med Clin North Am 1958; 42: 1533–1553.
Upper Limb
Upper extremity reflex sympathetic dystrophy
P. N. Soucacos, E. O. Johnson
response may involve an unchecked generalized (central) and/or local (peripheral) sympathetic response(s) to an insult. The generalized response includes a centrally-mediated ‘stress response’ or ‘fight or flight’ response, as well as responses stemming from the hypothalamus, suprahypothalamic structures (such as the hippocampus) and the mesencephalon.9 Local reactions include adrenocortical-mediated and somatomotor-mediated responses of the somatosympathetic, viscerosympathetic and viscerovisceral nerve fibers. Secondly, peripheral responses to tissue insult include hyperalgesia, diffuse burning pain, altered blood flow and vasomotor control, as well as changes in the integrity of the bone, joints, muscles subcutaneous tissue and skin. These peripheral responses may be in response to alterations in sympathetic activity induced by receptor blockade, neural blockade or transmitter depletion. There is evidence suggesting that upregulated sensitivity of alphaadrenoreceptors for catecholamines may induce RSD.10 Other theories as to the pathogenesis of RSD have been proposed, including abnormal feedback into the internuncial centers of the spinal cord, periarteritis of the vessels surrounding the injured segment and shortcircuiting effects at the area of injury, which lead to irritation of the sensory afferents by the efferent sympathetic impulses. It has been proposed that activation of the nocioceptors leads to excitation of an internuncial pool of neurons of the spinal cord, with induction of increased activity of the efferent sympathetic system.11 The subsequent vasoconstriction, with ischaemia of the tissue, was believed to stimulate nocioceptors with re-excitation of the spinal cord. Thus, injury produces painful impulses that travel via afferent pathways to the spinal cord where a series of reflexes spread via the interconnecting neuron pool. These reflexes stimulate the anterior and lateral spinal
INTRODUCTION Reflex sympathetic dystrophy (RSD) represents a complex, multifaceted syndrome with a plethora of signs and symptoms which vary both between individuals and with the progression of the disease over time. Various clinical types of RSD are now recognized which present as a modification of the normal pain response. In the upper extremity, RSD usually occurs after trauma to the nerves and/or muscles, surgery (e.g. carpal tunnel release), or other factors (primarily overuse syndromes).1–5 The great variety of treatment regimes used today to manage RSD, appear to provide only temporary relief of symptoms in most cases. It is likely that this may be related not only to mostly unimodal therapeutic paradigms, but to the difficulties faced in diagnosis, as well as classification of the disease.
AETIOLOGY AND PATHOPHYSIOLOGY RSD is believed to be an abnormally severe and prolonged manifestation of the normal physiologic response of the sympathetic nervous system to pain after injury.6–8 The prolongation of a normal sympathetic response disrupts homeostasis, and an otherwise adaptive response, becomes chronic and pathologic. There are two general mechanisms by which the sympathetic system may be involved in an abnormal pain response. First, the abnormal pain
P. N. Soucacos MD FACS Professor and Chairman, Department of Orthopaedic Surgery; E. O. Johnson PhD, Department of Anatomy, University of Ioannina, School of Medicine, Ioannina 45110, Greece. Correspondence to: P. N. S. Tel: +30 651 97 515; Fax: +30 651 46 222.
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Upper extremity reflex sympathetic dystrophy tracts, resulting in stimulation of the efferent pathways that travel via the peripheral nerves, resulting in peripheral manifestations of RSD. Although the sympathetic component of RSD is widely accepted, recent data suggest that the early symptoms closely resemble those of an inflammatory reaction.12 The hypothetical inflammatory agent is believed to be an endogenous pro-inflammatory agent which can induce hyperaemia, such as histamine. However, neuroinflammatory mediators, such as neuropeptide-y, substance P, bradykinin and calcitonin gene-related peptide may have an important role in the pathogenesis of the neurologic components in RSD, and maybe involved in the inflammatory reaction and accompanying motor and sensory disturbances that occur.12 Other similar hypotheses on the pathogenesis of RSD reflect the involvement of the central nervous system in an exaggerated peripheral (neuro)inflammatory response to tissue injury,13 which is related to the tight intercommunication between the nervous system and the inflammatory/ immune system.9 Overall, the various findings regarding pathogenesis underscore the complexity of the disease and its presentations and have led to further refinements of the initial definition, such as sympathetically maintained pain (SMP), sympathetically independent pain (SIP) and complex regional pain syndrome (CRPS) in an effort to delineate more accurately the abnormal pain alterations in response to trauma.14,15 The pathophysiology of the syndrome, however, remains obscure and an appropriate animal model for its in depth investigation is still lacking.
CLINICAL SIGNS AND SYMPTOMS OF RSD RSD of the upper extremity typically involves severe pain, swelling, stiffness and discolouration of the extremity which presents after trauma or disease. The entire symptom complex is rarely observed in one patient and usually only one sign or symptom predominates. Four cardinal signs and symptoms and six secondary signs and symptoms compose the clinical presentation of RSD.8,16,17 The four primary signs and symptoms are pain, swelling, stiffness and discolouration of the extremity, all of which are considerably worse than expected from the initial injury or disease
Table 1 Primary signs and symptoms of RSD Sign
Features
Pain
Paramount feature; often with burning, throbbing, aching, stabbing, bursting pressure or crushing sensations First physical sign; initially local soft oedema then extensive and hard Progressively worsens due to increased fibrosis Red, cyanotic, or pale to grayish; related to vasomotor instability
Swelling Stiffness Discolouration
(Table 1). Secondary symptoms and signs are diffuse osteoporosis, pseudomotor and temperature changes, as well as vasomotor instability, palmar fasciitis and trophic changes (Table 2). Swelling of the hand is normally the first physical sign of the RSD complex observed by the patient and clinician. Initially, the swelling is local and in the form of a soft oedema. It then spreads over the extremity and eventually becomes hard and brawny. Only in the late stages of the disorder, does the swelling increase dramatically around the joints producing profound peri-articular thickening. The resultant stiffness of the hand and extremity is much worse than expected from the initial injury. It increases significantly if untreated, and is usually attributed to an increase in fibrosis and adhesion formation. Excruciating burning pain is the most characteristic clinical feature of RSD. It is usually described as burning, and is often combined with a throbbing, aching, stabbing, bursting pressure or crushing sensation. The magnitude of the pain is generally out of proportion to the severity of the trauma or disease. The pain frequently begins immediately after injury and in the early stages of the disease the pain is constant and increases notably with all attempts at motion. Most patients complain of paraesthesiae, even with light touch.17,18 The pain is usually located in the anatomical region corresponding to the cutaneous distribution of the nerve affected. Some discolouration is always present in RSD. Discolouration of the hand varies from a more common notable redness to pale or grayish or a cyanotic colour, which appears initially over the dorsal surface of the metacarpophalangeal joints and then spreads over the interphalangeal joints. In late RSD, the discolouration often spreads over the entire dorsal surface of the hand. The colour of the extremity appears to be directly related to the presence of vasomotor
Table 2 Secondary signs and symptoms of RSD Sign
Features
Osteoporosis
Ranges from speckled appearance to marked osteopaenia in polar regions; it may be marked on X-ray examination Hyperhidrosis is observed early, and progressively becomes dry in late stages Related to vasomotor changes; warm extremity in early stages and cool skin later Vasospasm common Acute nodules and thickening of long bands of palmar fascia Glossy appearance due atrophy of skin and subcutaneous tissue
Pseudomotor changes Temperature changes Vasomotor instability Palmar fasciitis Trophic changes
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instability.4,5 Specifically, redness is usually associated with dilation of both the arterial and venous systems, while pallor is associated with vasoconstriction of both the arterial and venous systems. On the other hand, a blue or cyanotic extremity is normally seen with vasospasm of the venous system only. A purple discolouration is often observed in the flexor creases, particularly when palmar fasciitis is present. Osteoporosis, as a secondary change in patients with RSD, is much more severe than expected from the initial injury or disease and is usually quite marked upon radiographic examination. Early in the course of the disease, demineralization is speckled and is commonly referred to as ‘Sudeck’s atrophy’.19 Subsequently, demineralization is located primarily in the polar regions and in the final stages of the disease osteoporosis can be profound, spreading homogeneously throughout the affected area. Sudomotor changes observed in RSD are not as noteworthy. Commonly, hyperhidrosis is observed early on. The sweating slowly diminishes over the course of the disease and dryness is observed in the last stages. Although most patients are able to recall a significant temperature difference in their affected limb when their symptoms were first noted, the temperature of the affected extremity of RSD patients is variable. At the onset of RSD, during which time the extremity increases in redness, the temperature is usually elevated. When pallor or cyanosis are present, the perceived temperature tends to be low. Thermoregulatory changes have been found to be directly related to vasospasm,4,5 and vasomotor instability is commonly noted. In general, vasomotor instability in the later stages can be verified with a prolonged capillary refill time upon pinching of the finger tip or nail bed. Palmar fasciitis can be observed in a number of patients with RSD with the formation of acute nodules and thickening of the long bands of palmar fascia. Finally, trophic changes are noteworthy in RSD patients and are most commonly associated with a glossy and shiny skin appearance. Initially, the glossy smooth skin surface of the affected hand is attributed to the severe oedema which smoothes out the normal creases in the skin’s surface. Later, however, the glossy skin appearance is a result of the significant atrophy of the skin and subcutaneous tissue which progressively increases with time.
CLINICAL COURSE OF RSD The excessive prolongation of the sympathetic response constitutes the basis of the major symptoms of reflex sympathetic dystrophy. Of the signs and symptoms, usually only one is paramount in a single patient. Compounding the significant variation due to individual differences, it is well recognized that these signs and symptoms also vary with the time course of the disease. The course of RSD can be divided into three stages according to the evolution of the symptoms.20,21 The clinical picture varies significantly as the patient passes through early, intermediate and late stages of the disorder (Table 3). Early RSD: ‘the traumatic stage I’ In the early, acute stage (stage I), pain, vasomotor instability and sympathetic overactivity are evident. This is the so-called ‘traumatic stage’, which extends from the first to the third month, and is characterized by an intermittent burning pain, swelling and lack of motion, as well as redness and sweating of the extremity. Very soon after injury vasodilation produces hyperaemia and oedema. Swelling also starts early in this stage and is usually soft. Initially, pain is the most pronounced feature. There is usually painful paraesthesia to light touch, but pain with motion is not normally noted until later. Stiffness is predominantly related to the pain upon movement and becomes progressively worse throughout stage I. Discolouration is variable. The extremity tends to be red and warm at the onset, although throughout the later part of stage I, the extremity becomes cool and cyanotic due to vasoconstriction and vasospasm. Osteoporosis does not appear until the end of stage I and usually not until after the 3rd to 5th week. At its onset, osteoporosis in RSD takes on a patchy appearance. Intermediate RSD: ‘the dystrophic stage II’ The second or ‘dystrophic’ stage begins from approximately the third month and lasts until about the 9th to 12th month. During this stage, the extremity begins to cool and the skin undergoes atrophy and becomes shiny. Characteristic of stage II is the marked stiffening of the extremity (related to the hardening of the swollen extremity), increased pain and a decrease in
Table 3 Clinical course of RSD Primary Signs
Early RSD (0–3 months)
Intermediate RSD (3–9 or 12 months)
Late RSD (9 or 12 months–years)
Pain
Paraesthesia
Notable increase, pain with motion
Swelling Stiffness
Soft local oedema Pain-related
Hard, brawny oedema over extremity Increased stiffening due to fibrosis
Discolouration Principle feature of the stage
Red then cyanotic Traumatic
Cyanotic, with redness over joints Dystrophic
Diminished pain, but severe with motion Periarticular (minimal) Peak stiffening, contractures common Pallor Atrophic
Upper extremity reflex sympathetic dystrophy sweating and redness. Swelling changes from the soft oedema observed in stage I to anoedema which is brawny and hard, as fluid and protein deposits begin to appear in the capsular structures. Increased redness is noted, particularly around the joints, as well as an increased warmth and dryness of the extremity. There is decreased sweating as the vasomotor instability persists. Peripheral vasospasm resulting from persistent vasoconstriction is more evident. Polar demineralization is usually severe and more widespread. An increasingly glossy appearance of the hand is also noteworthy, as the skin and subcutaneous tissue undergoes atrophy. Flexion creases are eventually lost and palmar fasciitis may also be present. Late RSD: ‘the atrophic stage III’ Stage III or the ‘atrophic stage’ begins around the 9th or 12th month and may last for several years. The pain, which peaked in stage II, may slowly begin to diminish. Although pain may subside, in the majority of patients it remains quite severe with motion. On the other hand, stiffness peaks in late RSD and the extremity often resembles a Volkmann’s ischaemic contracture. The swelling changes from brawny, hard oedema to a pronounced periarticular thickening of the joints. In general, the extremity is pale, dry and cool. Osteoporosis is often profound and diffuse. The most striking feature of the last stage of RSD is the glossy appearance of the hand, which is related to the significant degree of skin and subcutaneous tissue atrophy which has taken place.
DIAGNOSTIC CRITERIA OF RSD Although RSD applies to an extremity with excruciating burning pain, swelling, stiffness and discolouration which present after trauma or disease, its presentation varies greatly. RSD of the upper extremity can range from minor involvement including one or more fingers (segmental RSD) to involvement of large anatomic regions or of the entire extremity (regional RSD). RSD also presents with widely varying degrees of pain and dysfunction. Due to these significant variations in the signs and symptoms, diagnosis is often difficult.
Table 4 Diagnosis of RSD Diagnostic criteria Persistent diffuse pain Loss of hand function Autonomic dysfunction Evidence for autonomic dysfunction Altered blood flow Altered temperature Altered sweating Skin and subcutaneous tissue atropy Oedema
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In general, the three pre-requisites underlying the development of RSD are required for diagnosis: a persistent lesion or injury, a predisposing constitution, and an abnormal sympathetic reflex arc (Table 4).4–6,21 The persistent lesion is often a complication of trauma to the nerves and/or soft tissues, surgery or other factors, such as disease or overuse. In this regard, RSD has been observed after a wide range of injuries and operations. The majority of patients are females, with a female-to-male ratio of 3:1. The primary aetiological factor associated with the expression of RSD is trauma (57.4%), followed by surgery (22.9%) and, overuse syndromes (19.7%).4 RSD has been associated with various diseases, including stroke and diabetes mellitus. Although not definitive, most clinicians believe that a significant number of RSD patients have a predisposing or susceptible personality.22 The inherent constitutional traits of the patient appear to make them more vulnerable to develop RSD after injury. Some traits frequently associated with RSD patients include anxiety, dependency, insecurity, an overpowering sense of dissatisfaction and other personality disturbances.23,24 Finally, central to the diagnosis of RSD, is an abnormality in the normal sympathetic response arc. Most of the signs and symptoms characteristic of RSD, such as swelling, stiffness, vasomotor and thermoregulatory instability, can be attributed at least in part to sympathetic dysfunction. Initially, other conditions such as Dupuytren’s disease, hypertrophic osteoarthritis, psoriatic arthritis, carpal tunnel syndrome and stenosing tenosynovitis, which are associated with pain, swelling, stiffness and dysfunction, should be ruled out. Thereafter a presumptive diagnosis can be made on the presence of some or all of the signs and symptoms.6,17,20,21 Exact diagnostic criteria remain controversial, although the presence of all four cardinal signs and symptoms and at least one secondary sign and symptom is generally sufficient to presume RSD. However, a presumptive diagnosis can be made with fewer signs and symptoms if other diseases have been effectively ruled out and when the observed condition is severe. Confirmation of the presumed diagnosis is achieved only after disruption of the sympathetic nerve reflex produces a significant decrease in the symptom complex expressed by the patient.17 Although RSD is confirmed if interruption of the sympathetic reflex produces relief of symptoms, failure of relief after sympathetic block does not necessarily rule out RSD. RSD should not be excluded until after at least three or more blocks have been carried out with no amelioration of the symptoms.4,5 Today, diagnosis of RSD takes into consideration the criteria established in the Consensus report of an ad hoc committee of the American Association for Hand Surgery on the definition of RSD syndrome, as well as the criteria outlined by Kozin.23,25 Diagnostic criteria included persistent diffuse pain, the loss of
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hand function and autonomic dysfunction. Evidence for autonomic dysfunction can be based on changes in skin and/or soft tissue blood flow, as reflected by an increase or decrease in temperature and sweating. Three-phase bone scans can also be performed as a supplemental diagnostic means for confirmation of RSD.4,5 Although the specificity and sensitivity of the method remain somewhat debatable in so far as information on the patient’s angiogram (phase I) and blood pool (phase II) are questionable, the findings from the third or delayed metabolic phase appear to be valuable in evaluating the patient.26–28 The criteria for diagnosis of segmental versus regional dystrophy are similar, with the exception that the anatomic involvement is less than the entire hand for patients with segmental dystrophy.4,5
PRINCIPLES OF TREATMENT Treatment of RSD is primarily geared towards disruption of the abnormal sympathetic response. However, the clinician should also attempt to ameliorate the other components of the diagnostic triad: the initiating injury and predisposing personality. In this regard, initial efforts should attempt to eliminate the injury which propagated the pain complex. This may range from the simple removal of a cast to a surgically demanding revision of an amputation or removal of a painful neuroma. Secondly, various approaches can be applied to help modify the patient’s inherent diathesis, including therapy or medications. The major component of treatment is interruption of the abnormal sympathetic reflex which can be achieved using an individually tailored regime of stellate ganglion blocks, somatic nerve blocks and sympatholytic drugs (Table 5). The sympatholytic and antihypertensive drug, guanethidine, has been given successfully to a selected number of patients.4,5 Guanethidine acts by blocking catecholamine storage in sympathetic nerve terminals, inhibiting pre-synaptic release of noradrenaline. This results in reduction of sympathetically-induced vasoconstriction of the peripheral vessels.29–31
In order to carry out a guanethidine block, 10 mg guanethidine in 25 ml of normal saline is administered using the Bier double-tourniquet block technique. The tourniquet is released after 20 min.4,5,30 Stellate ganglion blocks can be applied both therapeutically and diagnostically. Stellate blocks inhibit efferent impulses to the extremity, interrupting the abnormal sympathetic reflex without blocking normal somatic nerve function. By preventing sympathetic activity in the extremity, the patient’s skin becomes warmer and dryer, returns to a normal colour and pain is reduced. Technically, stellate blocks are much more demanding to perform than IV regional blocks, and employ either bupivacaine (Marcaine, 15–20 ml of 0.25%) or lignocaine (Xylocaine, 20 ml of 1%). Amelioration of the symptoms is usually noted after about 30 min, and may last up to a few hours. Three to 10 sessions can be performed at 10–14-day intervals. The block is considered successful when a Horner’s sign develops and warming of the affected hand is observed. We have reserved the application of stellate ganglion blocks mainly for treating patient’s with shoulder–hand syndrome.4,5 Local anaesthetic blocks of somatic nerves can be performed using lignocaine (Xylocaine). Somatic nerve blocks are aimed at interrupting the abnormal reflex through the somatic nerves, by blocking afferent nerve impulses. Blocks can be administered two to three times per week without producing local irritation. It is well recognized that steroids, in moderation, can help to reduce pain, swelling, stiffness and dysfunction.32–34 Corticosteroids, specifically, are effective in the treatment of RSD.25 Thus, lidocaine can be applied regionally along with corticosteroids (5 ml of 2% lidocaine in 15 ml of normal saline and 80 mg of prednisone in 5 ml of normal saline). Together, these prevent increased sympathetic stimulation from reaching the effected area.35 Various adjunctive modes of therapy are also applied to the management of RSD. Elimination of the factors causing pain is primarily achieved by antiinflammatory drugs and vasodilators, which reduce swelling and stiffness, as well as corticosteroids which can be administered with local anaesthetic. Calcitonin
Table 5 Treatment of RSD Method Agent Alternatives Purpose
Stellate ganglion block Bupivacaine (Marcaine) or lignocaine (Xylocaine) Continuous blockage with catheter Blocks efferent impulses to extremity. Not somatic nerves Alleviates symptoms due to sympathetic dysfunction
Method Agent Alternatives Purpose
Somatic nerve blocks Lignocaine (Xylocaine) Bupivacaine (Marcaine)—longer acting agent Blocks afferent pain impulse and interupts abnormal somatic nerve reflex Blocks sympathetic fibres for short time, assists in diagnosis Alleviates pain and other symptoms
Method Agent Alternatives Purpose
Sympatholytic drugs Guanethidine Alpha-adrenergic blocking agents (phentolamine) or catcholamine depleting agents (reserpine) Catecholamine storage blocker at sympathetic neuroeffector junction Alleviates symptoms due to autonomic dysfunction
Upper extremity reflex sympathetic dystrophy nasal spray (100 IU per day), which has been reported to significantly decrease pain and improve the range of motion, is also given.36 Warm gloves can be used to reduce muscle spasm. The loss of hand function can be effectively managed following Watsons’ hand stress rehabilitation program by using both ‘scrub’ and ‘carry’ exercises.37 Hand therapy is also effective in eliminating pain. In general, the programme couples stress loading with fundamental modalities of hand therapy. Stress is provided by active traction and compression exercises which avoid the use of joint motion. The programme is progressive, starting with short repeated session of ‘scrub’ exercises, progressing to ‘carry’ exercises, which increase in difficulty as the tolerance of the patient increases. Other exercises and range of motion activities are initiated once a notable depreciation in pain has occurred. Night splints can be used to return the hand to a normal resting position and relieve pain during motion. Caffeine and smoking are restricted; this is of particular significance, since smoking has been implicated in the pathogenesis of RSD.38 Reconstructive procedures and psychological support, either with medication or therapy, are applied as indicated by the patient’s needs. In summary, although the specific treatment protocol should be individualized according to the patient’s needs, successful treatment requires a multimodal regime aimed at interrupting the abnormal sympathetic reflex arc, eliminating the initiating pain and managing the loss of hand function.4,5 Management of the abnormal sympathetic reflex can be achieved using stellate ganglion blocks, and the regional application of guanethidine, lignocaine and glucocorticoids.
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FEATURES AND MANAGEMENT OF THE CLINICAL TYPES OF RSD Five clinical types of RSD are now recognized.17,20,21 These are minor causalgia, minor traumatic dystrophy, shoulder–hand syndrome, major traumatic dystrophy and major causalgia. The term ‘causalgia’ (burning pain) is usually reserved to reflect RSD arising from nerve injury which often involves a mixed nerve.21 However, RSD more frequently involves injury to soft tissues, joints or bone, rather than damage to a specific nerve, and in these cases is referred to as ‘traumatic dystrophy’. Specific nerve injury or causalgia can be minor, with less severe symptoms arising from trauma to peripheral or digital nerves, or major when damage to a major nerve, such as the median nerve, is involved and very severe symptoms are produced. The most common form of RSD, traumatic dystrophy, can be either minor or major depending on the severity of the trauma to the soft tissues, joint or bone. Finally, shoulder–hand syndrome arises usually from trauma to the proximal part of the body or from visceral disease (Table 6). MINOR CAUSALGIA Minor causalgia is the least severe clinical type of RSD and is generally associated with less pain, swelling, stiffness, discolouration and osteoporosis than other types. Minor causalgia is generally attributed to injury of a purely sensory cutaneous nerve. It tends to involve only the nerves of the wrist and hand and thus only a small region of the hand, usually one or
Table 6 Prominent features of the five clinical types of RSD Type of RSD
Major features
Minor causalgia
Associated with cutaneous nerve injury Involves nerves of hand or wrist Affects one or two fingers or small part of hand Less pain, swelling, stiffness and osteoporosis
Minor traumatic dystrophy
Most common, usually involves one or few fingers Initial trauma = crush, fx/dl, sprain, puncture wound Red dorsum over joints, mild palmar fasciitis Stiff digits, flexion contracture
Shoulder–hand syndrome
Pain stiffness in the shoulder Swelling hand, wrist, arm Initial trauma proximal Diffuse redness, warm, dry extremity Palmar fasciitis
Major traumatic dystrophy
Increased pain and symptoms Flexion contracture of digits common Limited wrist motion, carpal tunnel syndrome common Major initial trauma
Major causalgia
Extreme pain, associated with major mixed nerve injury Damage usually proximal Slight redness, sweating, coolness Extreme stiffness, flexion contracture of fingers
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two fingers, are affected. The injured nerve is usually purely sensory and is commonly the dorsal superficial sensory branch of the radial nerve, palmar cutaneous branch of the median nerve, dorsal superficial branch of the ulnar nerve or digital nerves. The injured nerve is frequently trapped by fibrous tissue or adhesions distal to the joints which leads to neurodesis and pain when the joint is moved. It is the least severe form of RSD and is associated with less pain, swelling, stiffness, discolouration and osteoporosis. We have reported that minor causalgia is predominantly related to overuse syndromes and more rarely is a complication of trauma or surgery.4,5 Pain is the most prominent symptom in these patients, although the intensity of pain tends to be moderate and significantly lower than that experienced by patients with shoulder–hand sydrome.4,5 Most patients with minor causalgia pursue treatment after bearing the symptoms for a relatively long period of time. The majority of patients do not demonstrate problems with swelling or present a hard, brawny oedema. Stiffness is frequently related to pain during movement and not to fibrosis.4 Of the secondary signs and symptoms those most frequently observed are osteoporosis (in about 12% of patients), Sudomotor changes (36%), thermoregulatory abnormalities (20%), vasomotor instability (20%) and trophic changes (20%).4,5 Treatment of patients with minor causalgia primarily consists of somatic nerve blocks with the local administration of lignocaine, plus corticosteroids, for two to eight sessions. Some patients may require stellate ganglion blocks or guanethidine. The majority of patients with minor causalgia exhibit excellent results following treatment. Of significance, the quality of the results is associated with early onset of treatment. Specifically, patients with excellent results sought treatment significantly earlier than to those whose results were fair.4 Minor traumatic dystrophy Minor traumatic dystrophy is the most common of the clinical types of RSD. Frequently this form of dystrophy is related to a definite, but mild injury, such as a mild crushing of the hand, a fracture/dislocation, sprain or puncture wound, rather than a specific nerve injury. It expresses with redness dorsal to the joints, a mild palmar fasciitis and stiff digits, usually with a flexion contracture. Often minor traumatic dystrophy is overlooked, particularly if it involves only a small part of the hand or one or more fingers. The majority of patients with minor traumatic dystrophy have segmental involvement.5 Minor traumatic dystrophy is primarily attributed to trauma (53.6%) followed by surgery (32.1%). Pain is usually moderate and significantly less than the pain scored for shoulder–hand syndrome and major traumatic dystrophy. The majority of patients have soft oedema over the affected extremity and stiffness is
equally attributed to pain and fibrosis.4 Vasomotor instability in the form of vasoconstriction or vasodilation can be observed, which is compatible with the temperature differences noted in these patients. Hyperhidrosis is frequently observed and a few patients also show radiographic signs of osteoporosis. A glossy appearance of the skin over the affected region can be observed in about 50% of the patients.4 The majority of cases with minor traumatic dystrophy can be effectively treated with somatic nerve blocks with lignocaine plus steroid over two to 10 sessions. Rarely guanethidine or stellate ganglion blocks may be required, although when used they are effective. A high rate of excellent results (71.4%) can be anticipated. Good results obtained after treatment are dependent upon the initiation of early treatment.4,5 The shoulder–hand syndrome The shoulder–hand syndrome is characterized by pain and stiffness in the shoulder and/or entire upper extremity with significant swelling of the hand and wrist, if not the entire extremity. Characteristically it starts with pain and stiffness in the shoulder area which then spreads to the entire extremity. The initiating trauma is usually proximal (e.g. injury to shoulder, neck or rib cage). It is also frequently associated with visceral disorders such as myocardial infarction, stroke, gastric ulcer, cervical osteoarthritis and even hemiplegia.39 Limited or minor hand trauma may also predispose the patient to the onset of shoulder–hand syndrome. The signs of the shoulder–hand syndrome include a diffusely red, warm and dry extremity which exhibits palmar fasciitis. Swelling is usually fusiform, with the fingers in extension and not flexion. Vasomotor disturbances, including both vasoconstriction and vasodilation, are common. Mottled osteoporosis of the humeral head and wrist are characteristic radiographic findings. Patients can be treated with two to five stellate blocks and with lignocaine and corticosteroids administered locally. Although the excellent outcome observed for the other types of RSD is not as often seen, fair to good results can be anticipated with early treatment.4 Major traumatic dystrophy Major traumatic dystrophy presents with more severe pain and symptoms than does minor traumatic dystrophy. It is usually attributed to a major injury, such as a severe crush injury, Colles’ fracture, or severe wrist fracture/dislocation. It has also been associated with elective surgery such as carpal tunnel syndrome. A number of patients with Colles’ fracture and RSD have also been noted to have compressive neuropathy of the median nerve secondary to carpal tunnel syndrome. Flexion contractures are common. Wrist motion, as well as rotary motion of the forearm tend to be limited in these patients.
Upper extremity reflex sympathetic dystrophy Almost all patients should undergo treatment with somatic nerve blocks. Stellate ganglion blocks may also be required in a few selected cases. Good to excellent results can be anticipated for more than half of the patients. Moreover, patients treated for major traumatic dystrophy early in the course of their disease, achieve significantly better results; patients with an excellent outcome received therapy by 4–5 months, compared to those with fair results who did not seek therapy until after about 19–20 months.4,5
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oedema, stiffness and discolouration) are prominently expressed in each clinical type, while secondary signs and symptoms vary significantly across the different types of RSD. Despite the wide range in disease expression, all clinical types of RSD respond equally well to the multimodal treatment regime. More importantly, however, the available data overwhelmingly support that treatment is most effective if commented early in the disease process. REFERENCES
Major causalgia Major causalgia is relatively infrequent and is characterized by severe pain. It is usually attributed to an injury of a major mixed nerve, such as the median nerve, which has a larger number of sympathetic fibres. Patients usually complain of increased pain from light touch or vibration. The extremity manifests a slight redness, which is somewhat less than that observed with other types of RSD, and in early stages may be pale or cyanotic. Sweating and coolness tend to be prominent. Flexion contractures are known to occur, although they may not be as extreme as observed for major traumatic dystrophy. A hard, brawny oedema is often noted with, cyanotic discolouration and stiffness related to fibrosis. However, mild oedema with stiffness related to pain and diffuse red discolouration may be present instead. Vasomotor instability can be observed with vasoconstriction or vasodilation, and a corresponding decrease or increase in the temperature of the affected hand. Severe osteoporosis and sudomotor dysfunction is also common. Excellent results can be achieved with stellate ganglion blocks and guanethidine or local somatic nerve blocks.
CONCLUSIONS RSD is a complex clinical entity which applies to an extremity with severe pain, swelling, stiffness, discolouration and a variety of other symptoms mostly attributed to autonomic dysfunction in the limb. The entire symptom complex is rarely found in any single patient, but rather one sign or symptom predominates. The signs and symptoms of the disorder demonstrate considerable individual variation, and significant variations occur with the progression of the disease over time. However, it is generally agreed that the most striking feature of RSD is a marked, burning pain, which also varies significantly with the evolution of RSD. Upper extremity RSD occurs more frequently in young adult women, with a female:male ratio which approaches 3:1. Sex differences vary however with each clinical type of RSD. Minor traumatic dystrophy is observed most frequently, whilst the shoulder–hand syndrome and major causalgia are relatively rare. The primary signs and symptoms (pain,
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