You are in: eMedicine Specialties > Orthopedic Surgery > TRAUMA Reflex Sympathetic DystrophyArticle Last Updated: Jul 2, 2008AUTHOR AND EDITOR INFORMATIONSection 1 of 12
  Author: Satishchandra Kale, MD, FRCS(Edin), FRCS Ed(UK), Dip Sports Med, Honorary Assistant Professor of Trauma and Orthopedics, University of Bombay; Consultant, Department of Orthopedics, Dr R N Cooper Hospital, Brahmakumaris Global Hospital, India Satishchandra Kale is a member of the following medical societies: British Orthopaedic Association Editors: James F Kellam, MD, Vice-Chair, Department of Orthopedic Surgery, Director of Orthopedic Trauma and Education, Carolinas Medical Center; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Samuel Agnew, MD, FACS, Associate Professor, Departments of Orthopedic Surgery and Surgery, Chief of Orthopedic Trauma, University of Florida at Jacksonville; Consulting Surgeon, Department of Orthopedic Surgery, McLeod Regional Medical Center; Dinesh Patel, MD, FACS, Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital; Mary Ann E Keenan, MD, Professor, Vice Chair for Graduate Medical Education, Department of Orthopedic Surgery, University of Pennsylvania School of Medicine; Chief of Neuro-Orthopedics Program, Department of Orthopedic Surgery, Hospital of the University of Pennsylvania Author and Editor Disclosure Synonyms and related keywords: reflex sympathetic dystrophy, RSD, algodystrophy, Sudeck atrophy, painful dysfunction syndrome, reflex neurovascular dystrophy, shoulder-hand syndrome, chronic regional pain syndrome, CRPS, causalgia, complex regional pain syndrome, sympathetically mediated pain syndrome, SMP, algodystrophy complex regional pain syndrome, chronic pain syndrome INTRODUCTIONSection 2 of 12
  BackgroundReflex sympathetic dystrophy (RSD) is a condition that is often described under various synonyms that point to its incompletely understood etiology. In 1864, Weir Mitchell coined the term causalgia to designate severe pain following nerve injury. In 1900, Sudeck described regional demineralization accompanying posttraumatic pain. In 1923, Leriche described vasomotor disequilibrium.1 In 1947, Evans introduced the term reflex sympathetic dystrophy. In 1993, the International Association for the Study of Pain renamed algodystrophy complex regional pain syndrome (also known as chronic regional pain syndrome or CRPS). Reflex sympathetic dystrophy, or RSD, is type 1 CRPS. RSD can be considered an excessive sympathetic reaction of joints and periarticular soft tissues to any insult, traumatic or unknown. This is quite different from causalgia (type 2 CRPS), in which the etiology is a partial nerve injury. RSD is characterized by pain, regional edema, joint stiffness, muscular atrophy, vasomotor disturbances (including temperature changes), trophic skin changes, and regional skeletal demineralization seen on radiographs. These changes are aggravated by activity and extend over a larger area than the primary injury or surgery, including the area distal to this focus.2, 3, 4 Because pathognomonic criteria are lacking for RSD, a taxonomic system based on clear definitions and objective quantification is desirable. Therefore, the current terminology of CRPS is increasingly being used as an umbrella to replace the myriad empirical descriptions used previously. No apparent relationship exists between the degree of initial trauma and severity of RSD, but RSD generally is more frequent following minor trauma or operations. PathophysiologyReflex sympathetic dystrophy (RSD, algodystrophy, complex regional pain syndrome [CRPS]) usually follows minor trauma or surgery. It also has been associated with various clinical conditions (eg, diabetes, parkinsonism). RSD begins with spontaneous pain associated with vasomotor and sudomotor disturbances. Bonica described the progress of severe cases in 3 stages.5 The acute stage of RSD is marked by pain, swelling, and warmth. Neurologic changes, such as hyperesthesia (glove and stocking distribution), incoordination, tremor, muscle spasms, and paresis, may be seen. The second dystrophic stage is characterized by cold skin with trophic changes. The final atrophic stage is manifested by muscle wasting and joint contractures. Symptoms usually are disproportionate to the cause and reflect disturbance of autonomic, sensory, and motor function. FrequencyUnited StatesAccording to various researchers, incidence of reflex sympathetic dystrophy (RSD) may be 2-17% following minor trauma or surgery. If causalgia is included in the broad definition, incidence can be as high as 32-35%. In the past, many subtle forms of RSD were missed, but with increased awareness of the condition, actual incidence may be much higher than initially thought. InternationalThere are no regions or population groups that have a predilection for reflex sympathetic dystrophy. Mortality/MorbidityMortality associated with reflex sympathetic dystrophy (RSD) is negligible, though morbidity is extremely high. Despite good results following intravenous sympathetic blockade and intensive mobilization techniques, weakness of the extremity resulting from RSD is seen in almost 50-65% of patients, even 18-24 months following initial diagnosis. Full range of movement accompanying the above aggressive therapies is seen in 60-74% of patients. Prolonged morbidity is observed in about 50% of patients with psychiatric diathesis, workers' compensation claims, and lawsuits.6 RaceNo particular race has a predilection for reflex sympathetic dystrophy. SexReflex sympathetic dystrophy is more common in women than in men; the male-to-female ratio is approximately 3:7. The ratio of upper extremity to lower extremity involvement is approximately 2:1. Even in children, girls are affected more frequently than boys, but peculiarly, the lower extremity is involved more frequently than the upper extremity.7 AgeMost patients with reflex sympathetic dystrophy (RSD) are aged 30-55 years, and the mean age is 45 years. With increasing awareness, RSD is being diagnosed in children more often; however, no studies exist pointing to a particular age distribution. CLINICALSection 3 of 12
HistoryPatients with reflex sympathetic dystrophy have a history of trauma, minor rather than major (eg, Colles fracture), in about 50-65% of cases. The condition may also follow a surgical procedure. Physical
CausesIatrogenic causes of reflex sympathetic dystrophy (RSD) following surgery, such as carpal tunnel decompression or Dupuytren release, can be diagnosed easily.8 No clear etiology (including trauma) can be identified in 25-35% of cases. A detailed history can be useful to pinpoint uncommon causes of RSD.
Related Medscape topics: DIFFERENTIALSSection 4 of 12
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| Drug Name | Reserpine (Serpalan) |
|---|---|
| Description | Inhibits vesicular uptake of noradrenaline and thus stops excitation of sympathetic nervous system; inhibits beta-hydroxylation of dopamine to noradrenaline. Lewis et al reported good results in 90% of patients at 18 months, with 93% of patients experiencing pain relief. |
| Adult Dose | 1.25 mg PO for upper extremity and 2.5 mg PO for lower extremity, diluted in isotonic sodium chloride solution; 1-2 blocks needed |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity; diagnosed mental depression |
| Interactions | Tricyclic antidepressants may decrease antihypertensive effects of reserpine when used concurrently; cardiac arrhythmias may occur when either digitalis or quinidine is administered concurrently with reserpine |
| Pregnancy | D - Unsafe in pregnancy |
| Precautions | Procedure must be performed in facility with resuscitation equipment; nasal congestion, abdominal cramps, and orthostatic hypotension may occur |
May exert effect by causing blockade of postganglionic synapses.
| Drug Name | Phenoxybenzamine (Dibenzyline) |
|---|---|
| Description | May have effect through long-lasting noncompetitive alpha-adrenergic blockade of the postganglionic synapses in smooth muscle. |
| Adult Dose | 10 mg PO bid, increasing by 10 mg qod until optimum dose achieved Dose range: 20-40 mg PO bid/tid |
| Pediatric Dose | 1-2 mg/kg/d PO divided q6-8h |
| Contraindications | Documented hypersensitivity; those in whom a fall in blood pressure would be undesirable |
| Interactions | Used concurrently, alpha-adrenergic agonists decrease effects of medication; beta-blockers increase toxicity |
| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus |
| Precautions | Caution in cerebral or coronary arteriosclerosis and renal impairment; can worsen symptoms of respiratory tract infections |
| Drug Name | Phentolamine (Regitine) |
|---|---|
| Description | Alpha-1 and alpha-2 adrenergic blocking agent that blocks circulating epinephrine and norepinephrine action. |
| Adult Dose | 5-20 mg IV/IM; repeat prn q2-4h until hypertension controlled |
| Pediatric Dose | 0.05-0.1 mg/kg/dose IV/IM; not to exceed 5 mg/dose; repeat prn q2-4h until hypertension controlled |
| Contraindications | Documented hypersensitivity; coronary or cerebral arteriosclerosis and renal impairment |
| Interactions | Concurrent administration of epinephrine or ephedrine may decrease phentolamine effects; ethanol increases phentolamine toxicity |
| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus |
| Precautions | Caution in tachycardia, peptic ulcer, and gastritis; cerebrovascular occlusions and myocardial infarctions can occur following phentolamine administration |
May act to decrease muscle tone.
| Drug Name | Clonidine (Catapres) |
|---|---|
| Description | Stimulates alpha2-adrenoreceptors in brain stem, activating an inhibitory neuron, which in turn results in reduced sympathetic outflow. |
| Adult Dose | Initial: 0.1 mg PO bid Maintenance dose: 0.2-1.2 mg/d PO in 2-4 divided doses; not to exceed 2.4 mg/d |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity |
| Interactions | Tricyclic antidepressants inhibit hypotensive effects of clonidine; coadministration of clonidine with beta-blockers may potentiate bradycardia; tricyclic antidepressants may enhance hypertensive response associated with abrupt clonidine withdrawal; hypotensive effects of clonidine are enhanced by narcotic analgesics |
| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus |
| Precautions | Caution in cerebrovascular disease, coronary insufficiency, sinus node dysfunction, and renal impairment |
| Drug Name | Guanethidine (Ismelin) |
|---|---|
| Description | Acts on postganglionic fibers at the presynaptic level, releasing noradrenaline and inhibiting reuptake and rerelease. Fiscat et al reported good results in 63% of cases; Bensigner et al reported good results in 58.6% of cases. For administration, IV access is gained as close as possible to the involved part. A BP cuff is tied well above the site of pain and inflated above the systolic pressure; a second BP cuff is tied below the first cuff as in a Bier block. Pressure is maintained for 20 min, the limb manipulated, and pressure is reduced while alternating between the 2 cuffs. Six blocks are performed on alternate days with rigorous rehabilitation; repeated blocks even if the first block fails. |
| Adult Dose | Up to 30 mg in 30 mL isotonic sodium chloride solution IV |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity; pheochromocytoma; MAO inhibitor use within the last 14 d |
| Interactions | Tricyclic antidepressants, methylphenidate, thioxanthenes, phenothiazines, sympathomimetics, anorexiants, and haloperidol may reduce effects of guanethidine; minoxidil, epinephrine, and norepinephrine may increase the toxicity of guanethidine |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| Precautions | Procedure must be performed in facility with resuscitation equipment; clonic movements, orthostatic hypotension, and minor sexual problems possible |
Inhibit calcium ions from entering slow channels; select voltage-sensitive areas, or vascular smooth muscle.
| Drug Name | Nifedipine (Adalat, Procardia) |
|---|---|
| Description | May have a relaxant effect on certain muscles. Inhibits transmembrane influx of calcium ions into smooth muscle, which, in turn, inhibits contraction of the muscle fibers. |
| Adult Dose | 10-30 mg SL 30 min ac; hs prn if nocturnal regurgitation and cough are prominent |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity |
| Interactions | Fentanyl and alcohol may increase hypotensive effects; calcium channel blocker may increase cyclosporine levels; H2 blockers (cimetidine), erythromycin, nafcillin, and azole antifungals may increase toxicity (avoid combination or monitor closely); carbamazepine may reduce bioavailability (avoid this combination); rifampin may decrease levels (monitor and adjust dose of calcium channel blocker) |
| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus |
| Precautions | May cause lower extremity edema; allergic hepatitis has occurred but is rare |
| Drug Name | Amlodipine (Norvasc) |
|---|---|
| Description | May have a relaxant effect on certain muscles. Inhibits transmembrane influx of calcium ions into smooth muscle, which, in turn, inhibits contraction of the muscle fibers. |
| Adult Dose | 2.5-5 mg PO qd; 10 mg PO qd maximum |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity |
| Interactions | Fentanyl and alcohol may increase hypotensive effects; calcium channel blocker may increase cyclosporine levels; H2 blockers (cimetidine), erythromycin, nafcillin, and azole antifungals may increase toxicity (avoid combination or monitor closely); carbamazepine may reduce bioavailability (avoid this combination); rifampin may decrease levels (monitor and adjust dose of calcium channel blocker) |
| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus |
| Precautions | Adjust dose in renal/hepatic impairment; may cause lower extremity edema; allergic hepatitis has occurred but is rare |
Have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.
| Drug Name | Prednisolone (Articulose-50, Delta-Cortef, Pediapred) |
|---|---|
| Description | Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reducing capillary permeability. |
| Adult Dose | Oral: 60 mg PO initially, rapidly tapered over 5-10 d Alternatively, 20 mg/mL intra-articularly |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity; viral, fungal, or tubercular skin lesions |
| Interactions | Decreases effects of salicylates and toxoids (for immunizations); phenytoin, carbamazepine, barbiturates, and rifampin decrease effects of corticosteroids |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| Precautions | Caution in hyperthyroidism, osteoporosis, cirrhosis, nonspecific ulcerative colitis, peptic ulcer, diabetes, and myasthenia gravis |
Local anesthetics stabilize the neuronal membrane and prevent the initiation and transmission of nerve impulses.
| Drug Name | Lidocaine (Anestacon, Dermaflex, Lidoderm, Zilactin-L) |
|---|---|
| Description | Decreases permeability to sodium ions in neuronal membranes. This results in the inhibition of depolarization, blocking the transmission of nerve impulses. |
| Adult Dose | Apply to the affected area prn |
| Pediatric Dose | Administer as in adults |
| Contraindications | Documented hypersensitivity; Adams-Stokes syndrome, Wolf-Parkinson-White syndrome |
| Interactions | None reported |
| Pregnancy | B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals |
| Precautions | For external or mucous membrane use only; do not use in eyes |
A complex group of drugs that have central and peripheral anticholinergic effects and sedative effects. They have central effects on pain transmission. They block the active reuptake of norepinephrine and serotonin.
| Drug Name | Amitriptyline (Elavil) |
|---|---|
| Description | Analgesic for certain chronic and neuropathic pain. |
| Adult Dose | 30-100 mg/d mg PO hs |
| Pediatric Dose | Children: 0.1 mg/kg PO hs; increase as tolerated over 2-3 wk to 0.5-2 mg/d hs Adolescents: 25-50 mg/d PO initially; increase gradually to 100 mg/d in divided doses |
| Contraindications | Documented hypersensitivity; MAO inhibitor use in past 14 d; history of seizures, cardiac arrhythmias, glaucoma, and urinary retention |
| Interactions | Phenobarbital may decrease effects; coadministration with CYP2D6 enzyme system inhibitors (eg, cimetidine and quinidine) may increase amitriptyline levels; amitriptyline inhibits hypotensive effects of guanethidine; may interact with thyroid medications, alcohol, CNS depressants, barbiturates, and disulfiram |
| Pregnancy | D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus |
| Precautions | Caution in cardiac conduction disturbances and history of hyperthyroidism, renal or hepatic impairment; avoid using in the elderly |
Pain control is essential to quality patient care. Analgesics ensure patient comfort, promote pulmonary toilet, and have sedating properties, which are beneficial for patients who experience pain.
| Drug Name | Codeine |
|---|---|
| Description | Binds to opiate receptors in CNS, causing inhibition of ascending pain pathways, altering perception and response to pain. |
| Adult Dose | 10-20 mg/dose PO q4-6h prn for cough; not to exceed 120 mg/d |
| Pediatric Dose | <2 years: Not established 2-6 years: Not to exceed 30 mg/d PO 6-12 years: Not to exceed 60 mg/d PO >12 years: Administer as in adults |
| Contraindications | Documented hypersensitivity; high altitude cerebral edema or elevated ICP |
| Interactions | Toxicity increases with concurrent administration of tricyclic antidepressants, MAO inhibitors, neuromuscular blockers, CNS depressants, phenothiazines, and narcotic analgesics |
| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus |
| Precautions | Use to treat cough in patients diagnosed with high altitude pulmonary edema only if absolutely necessary; may depress hypoxic ventilatory rate and respiratory drive during sleep |
Deterrence/Prevention:
Prognosis:
Patient Education:
| Media file 1: Reflex sympathetic dystrophy following surgery for Dupuytren contracture. | |
 | View Full Size Image | Media type: Photo |
| Media file 2: Radiograph of affected extremity, depicting regional osteopenia contrasted with normal radiographic appearance of the opposite extremity. | |
 | View Full Size Image | Media type: X-RAY |
Reflex Sympathetic Dystrophy excerpt
Article Last Updated: Jul 2, 2008
