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Reflex Sympathetic Dystrophy (Complex Regional Pain Syndrome)
In 1598, Pare first recorded awareness of the condition. Then, later
during the American Civil War, RSD was recognized and described. The
hallmark of Complex Regional Pain Syndrome (CRPS) is severe,
spontaneous pain associated with local features of autonomic
dysfunction that occurs after trauma or operation to a limb. It is
defined as a disordered response by an extremity to a noxious
(hurtful) stimulus. The exact cause is unknown.
The five major signs and symptoms are:
- Pain out of proportion to the injury
- Edema
- Autonomic dysfunction
- Movement disorder, and
- Trophic changes
Causes of CRPS include accidental trauma. Injury may be the result
of fractures, dislocations or sprains, amputations, crush injuries
or even minor cuts of the toes or feet. Other etiologic (causative)
factors include surgical procedures, diabetes mellitus, hemiparesis,
venipuncture, and infections. It usually results from incomplete
nerve trauma or soft tissue injury, and most commonly affects the
hands and feet.
Genant proposed criteria for diagnosis of RSD which included pain
and tenderness in the extremity, soft-tissue swelling, decreased
motor function, trophic skin changes, vasomotor instability and
patchy oteoporosis.
Histological findings on synovial biopsies characteristic for RSD
that Kozin noted include proliferation and disarray of synovial
lining cells, increased numbers of small blood vessels, mild
perivascular inflammatory infiltrate and synovial edema.
Stages of RSD (CRPS)
- STAGE I occurs between 1-3 months. The foot displays soft, puffy
edema, antalgic guarding of the part, pronounced pain,
allodynia(hyperesthesia to light touch-pressure),
hyperhidrosis(excessive perspiration); spotty osteoporosis on
radiographic inspection; pain aggravated by movement and emotional
stress.
- STAGE II occurs between 3-6 months. Edema is still present,
range of motion decreases, dystonia (impairment of muscle tone),
mottled or cyanotic coloration, and progressive spotty bone
demineralization noted radiographically. Hair growth is decreased,
nails are brittle, and there is muscle wasting causing limited joint
mobility.
- STAGE III occurs between 6-9 months. There is a cool, dry foot
with pallor;with the skin slightly taut, waxy and thin; stiffness
and disability are pronounced, marked disuse atrophy of bone
(Sudek's atrophy).
Techniques to confirm clinical diagnosis
Plethysmography(the determination of changes in volume by using an
instrument for recording variations in volume of an organ, part, or
limb) and venous blood gas measurement for determination of
vasoconstriction.
Thermography or videothermogram to evaluate near-surface blood flow.
A positive thermogram will show significant coolness in the affected
extremity.
Xenon clearance or laser Doppler to measure peripheral blood flow.
Serologic tests, such as antinuclear antibody and rheumatoid factor
(are usually negative).
Plain film radiographic examination reveals patchy osteoporosis,
which may progress to a diffuse ground-glass appearance.
Three-phase Technetium bone scan, showing diffuse uptake in the
blood flow, pool and delayed phase. In the delayed phase,
periarticular uptake is noted in the affected part.
Other studies include electromyography(the recording and study of
the electrical properties of skeletal muscle) and nerve conduction
velocity studies, arteriography(radiography of an artery or arterial
system after injection of a contrast medium into the blood stream),
and erythrocyte sedimentation rate. The results of these studies are
negative. Quantitative bone density testing is also used.
Pharmaceutical treatment for CRPS is Prednisone, Elavil, Procardia,
NSAIDS(Non-Steroidal Anti-Inflammatory Drugs), Neurontin, Dilantin
PHYSICAL THERAPY treatment may be administered including, massage,
ultrasound, ROM(range of motion) exercises, splint, hydrotherapy and
contrast baths, transcutaneous electrical nerve stimulation (TENS),
trigger point electrical stimulation, acupuncture.
Other forms of treatment include chemical sympathectomy, epidural
block, lidocaine, morphine pump, and amputation
References
- Banks, Alan S., et al McGlamry's Comprehensive Textbook of Foot
and Ankle Surgery. Philadelphia:Lippincott Williams and Wilkins,
2001.
- Kozin F. Reflex sympathetic dystrophy syndrome:a review. Clin
Exp Rheumatol 1992;10:401-409.
- Schwartzman RJ, McLellan TL. Reflex sympathetic dystrophy.Arch
Neurol 1987;44:555-561.
- Schwartzman RJ. Reflex sympathetic dystrophy. Curr Opin Neurol
Neurosurg 1993;6:531-536.
- Stanton-Hicks MD. Upper and lower extremity pain. In: Raj P, ed.
Practical Management of pain. St. louis:Mosby-Year-Book,
1992:312-328.
- Bej MD, Schwartzman RJ. Abnormalities of cutaneous blood flow
regulation in patients with reflex sympathetic dystrophy as measured
by laser Dopler fluxmetry. Arch Neurol 1991;48:912-915.
- Amadio PC, Mackinnon SE, Merritt WH, et al. Reflex sympathetic
dystrophy syndrome:consensus report on 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.
- Schwartzman RJ. The diagnosis and staging of RSD: an overview of
the problem. Reflex sympathetic dystrophy: current strategies in
diagnosis and treatment. Paper presented at Jefferson Medical
College, Philadelphia, April 10, 1992.
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