Reflex Sympathetic Dystrophy / Complex Regional Pain Syndrome


- Definition:
       - “RSD” is an imprecisely used term; has been used to describe changes in soft tissue and bone;
       - RSD may not involve the sympathetic nervous system and may not be the consequence of a reflex;
       - 1995: IASP Consensus Conference: “Complex regional pain syndrome” replaced RSD
       - CRPS Type 1: noxious event; Type 2: nerve injury (causalgia)
       - pain/hyperalgesia not limited to single nerve distribution, disproportionate to inciting event
       - described as abnormal pain, swelling, vasomotor instability, contracture, and osteoporosis following injury or noxious stimulus.
       - CRPS: other names
              - reflex sympathetic dystrophy, Sudeck’s atrophy, causalgia (minor and major), algodystrophy, shoulder-hand
                        syndrome, post-traumatic pain syndrome, painful post-traumatic osteoporosis, transient migratory osteoporosis
       - incidence:
               - female > male
               - no correlation with age
               - adults: upper extremity > lower extremity
               - chronic, severe CRPS rare (< 2%)
               - 25% of Colles’ fractures with CRPS at 9 weeks, most pain resolved
               - 25-50% of Colles’ fractures with residual symptoms (stiffness, vasomotor instability) at 10 years.


- CRPS: Clinical features
       - patients often demonstrate bizarre behavior
       - wrapping extremity in damp towels, cutting clothing to prevent touching skin, refusal to sleep on sheets, refusal to touch slick objects, etc.
       - historically attributed to psychiatric disorders
       - CRPS: Stages
               - Stage 1, 0-3 months:
                       - puffy swelling, redness, warmth, hyperhydrosis, decreased ROM, usually normal radiographs, positive bone scan
                       - aggressive treatment at stage 1 yields best outcome
               - Stage 2, 3-6 months:
                       - worse pain, hard edema, cyanosis, dry skin, worse stiffness, atrophy of skin, osteopenia on radiographs;
                       - palmar faciitis may be present with nodules.
               - Stage 3, > 6-12 months:
                       - skin pale, cool, dry, tight and glossy; joint contractures
                       - severe osteopenia with spindling of fingers; diminished pain; nails become rigid; hair becomes fragile
       - clinical findings supported by:
               - begins within 30 days of injury
               - hand and foot most common; also knee (esp. following arthroscopy); hip (during pregnancy); rare in elbow
               - pain: burning, stabbing, stinging, searing
               - constant, worsening
               - intolerance to motion of involved joints
               - pts often do not permit exam
               - increased uptake on bone scan (early CRPS)
               - radiographic evidence of osteoporosis (late CRPS)

- Clinical Presentation:
    - acute clinical presentation:
           - may result from trivial injury or may result from a peripheral nerve injury;
           - pt notes pain out of proportion w/ magnitude of initiating injury which may or may not follow an anatomic distribution;
           - even in cases in which the CRPS is clearly a result of a peripheral nerve lesion, the trophic changes may extend beyond the
                  nerve's anatomic distribution (sympathetically mediated pain does not follow anatomic distributions);
                  - it is essential to document whether the pain is or is not confined to the autonomous zone (i.e., whether there has been
                            autonomic disregulation);
           - swelling is often the most common symptom;
           - intolerance to cold may be an early symptom, and may herald especially severe future symptoms;
           - the limb becomes swollen, stiff, and painful;
           - color changes range from dark red to blue;
           - sensitivity to slightest touch (allodynia) is most common physical finding and is seen in almost 90 per cent of the patients;
    - chronic clinical presentation:
           - trophic changes in the skin which becomes shiny and smooth;
           - nails become brittle and fast growing;
           - osteoporosis (Sudek's osteoporosis);
           - especially severe changes occur in about 1% of patients (more common in young females who present with early cold intolerance);
                  - these include chronic edema, ulcers, infection, and in some cases need for amputation;
    - RSD in children 
    - diagnostic criteria:
           - 4 major criteria: intense and prolonged pain, swelling, stiffness discoloration (vasomotor disturbances);
           - minor criteria: trophic changes, osseous demineralization, temperature changes, and palmar fibromatosis;


- Diagnostic Studies:
    - diagnostic sympathetic block 
    - bone scans in CRPS 
    - radiographs: focal patchy demineralization and osteoporosis may be seen;


- Management:
    - keys to successful treatment 
           - early clinical suspicion and treatment 
           - late CRPS is highly refractory to treatment and results in permanent disability 
           - early mobilization
    - it is first essential to address any direct cause or associated cause of the pain;
    - examples include:
           - an over-distracted external fixator placed across the wrist for a distal radius frx;
           - distal radius fracture w/ persistent swelling (from dependent position) w/ MP joints  left in an extended position sets up a
                       swollen, painful, and stiff wrist and hand;
           - saphenous neuroma resulting from meniscal repair;
           - meniscal tear, osteoid osteoma ect...
           - if further surgery is required, use epidural anesthesia (lower extermity) or a continuous regional anesthetic block for the upper extremity;
    - active ROM;
           - goal of physical therapy is to attain early active ROM;
           - often pts can be helped with lidocaine and cortisone blocks w/ immediate physical therapy and ROM;
           - this is especially true in pts that have this therapy within the first 6 months of their injury;
           - passive exercises may make things worse by increasing pain at point of discomfort;
    - sympathetic blockade:
           - successful stellate block would be noted by Horner's syndrome, rapid relief of pain, and a cool, dry hand;
           - stellate ganglion blocks using lidocaine or marcaine confirm diagnosis and give relief; may be curative early in disease
           - consider alternate diagnosis in patient who does not respond
           - can be repeated with a max of 12 blocks
           - treatment alternatives include:
                       - epidural anesthetic (allows continuous infusion):
                       - regional sympathetic blocks (repeated qod for 2 weeks):
                       - continuous regional sympathetic blocks;
                       - surgical sympathectomy
                               - good for patient who has return of symptoms after relief from stellate block.
                               - curative in 80% of patients who were refractory to other treatment
    - bier block 


- Medications: 
   - Vitamin C:
           - Can vitamin C prevent complex regional pain syndrome in patients with wrist fractures?
    - NSAIDs often give better relief than opiates
    - centrally acting analgesics: 
           - cymbalta 
           -
neurontin 
           - lyrica 
           - amitryptilene and carbamazepine 
    - fosamax: based on premise that pain results from osteoporosis; 
    - tricyclics 
    - resistant symptoms: add steroids, calcitonin, beta blockers, vasodilators, and other indicated medications; 
    - vasodilators: 
           - prazosin: 10 mg PO tid; 
           - terazosin10 mg PO tid; 
           - clonidine
           - guanethidine leads to vasodilation (vasomotor conditions) (reserpine not effective)
           - calcium channel blockers: vasodilation without interfering with SNS
           - alpha-blocker: phenoxybenzamine, leads to vasoconstriction
    - corticosteroids are controversial
                 - Wilder, et al (1992) demonstrated efficacy
                 - prednisone given in pulse fashion (start high, taper quickly)
                 - methylprednisolone dose pack is helpful early in disease
                 - side effects of steroids known 
                 - Reflex sympathetic dystrophy in children. Clinical characteristics and follow-up of seventy patients


    - alternative methods:
                 - transcutaneous electrical stimulation: very effective in children
                 - electroacupuncture
    - surgical treatment:
           - most indicated when there is an identifiable and localized nerve lesion, which has not yet started autonomic disregulation (i.e.,
                       pain is mainly confined to the autonomous zones);
           - goal is to free the nerve from a bed of avascular scar tissue the surrounding abnormal epineurium;
           - amputation:
                       - CRPS may recur in stump unless amputation is performed higher than level of skin changes; 
                       - references:
                                - Amputation for Long-Standing, Therapy-Resistant Type-I Complex Regional Pain Syndrome
                                - Amputation in patients with crps: comparative study between amputees and non-amputees with intractable disease
           - surgery (carpal tunnel, neuromas, etc) should be put off until pain resolves 
           - spinal cord stimulator:
                 - references:
                       - The efficacy of spinal cord stimulation for chronic pain.
                       - Spinal Cord Stimulation in Patients with Chronic Reflex Sympathetic Dystrophy



Thoracoscopic sympathectomy in the management of vasomotor disturbances and complex regional pain syndrome of the hand.

Complex regional pain syndrome

Reflex sympathetic dystrophy in children. Clinical characteristics and follow-up of seventy patients.

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.

Reflex sympathetic dystrophy after operative procedures on the lumbar spine.

Sympathetic maintained pain (causalgia) associated with a demonstrable peripheral-nerve lesion. Operative treatment.

Effect of sympathetic block demonstrated by triple-phase bone scan

Segmental reflex sympathetic dystrophy: clinical and scintigraphic criteria

Reflex sympathetic dystrophy of the knee. Causes, diagnosis, and treatment

Reflex sympathetic dystrophy of the knee: Treatment using continuous epidural anesthesia

Silas Weir Mitchell, MD: the physician who discovered causalgia.

Complex Regional Pain Syndromes: Causalgia and Reflex Sympathetic Dystrophy from the Orthopaedic Care Textbook

The "kick-off" position: a new sign for early diagnosis of complex regional pain syndrome in the leg.

Images in clinical medicine. A white hand and a red hand—erythromelalgia.

Four Treatment Strategies for Complex Regional Pain Syndrome Type 1

Reflex sympathetic dystrophy in the foot: clinical and scintigraphic criteria.

Allergy/Hypersensitivity Reactions as a Predisposing Factor to Complex Regional Pain Syndrome I in Orthopedic Patients




Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Thursday, May 19, 2016 10:22 am