C5omplex Regional Pain Syndrome

(Reflex Sympathetic Dystrophy)

Complex regional pain syndrome (CRPS) is a condition of intense burning
pain, stiffness, swelling, and discoloration that most often affects the hand.
Arms, legs, and feet can also be affected by CRPS.
This condition was previously known as reflex sympathetic dystrophy,
Sudeck's atrophy, shoulder-hand syndrome, or causalgia.
Description
There are two types of CRPS:
Type 1 occurs after an illness or injury that did not directly damage a nerve
in the affected area
Type 2 follows a distinct nerve injury
Although the triggers vary, both types of CRPS have the same symptoms and
go through the same three stages of disease.
Stage I: Acute
Stage I may last up to 3 months. Burning pain and increased sensitivity to
touch are the most common early symptom of CRPS. This pain is different
more constant and longer lasting than would be expected with a given
injury. Swelling and joint stiffness usually follow, along with increased warmth
and redness in the affected limb. There may be faster-than-normal nail and
hair growth and excessive sweating.

Acute stage CRPS, 2 months after injury
Stage II: Dystrophic
Stage II can last 3 to 12 months. Swelling is more constant and skin wrinkles
disappear. Skin temperature becomes cooler. Fingernails become brittle. Pain
is more widespread, stiffness increases, and the affected area becomes more
sensitive to touch.
Stage III: Atrophic
Stage III occurs after 1 year. The skin of the affected area becomes pale, dry,
tightly stretched, and shiny. The area is stiff and there is less hope of getting
motion back. Pain may decrease and the condition may spread to other areas
of the body.
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Cause
Although the two types of CRPS can be tied to injury or illness, the exact
cause of CRPS is unknown. One theory is that a "short circuit" in the nervous
system is responsible. This "short circuit" causes overactivity of the
sympathetic (unconscious) nervous system which affects blood flow and
sweat glands in the affected area.
Symptoms most commonly occur after injury or surgery. Other causes include
pressure on a nerve, infection, cancer, neck problems, stroke, or heart attack.
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Doctor Examination
After discussing your medical history and symptoms, your doctor will carefully
examine your hand or affected limb.
People with CRPS are unusually protective of the involved limb. Even a light
touch may evoke expressions of severe pain.
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Tests
There is no single test that can make the diagnosis of CRPS. Some imaging
studies, such as x-rays, bone scans, and magnetic resonance imaging (MRI)
scans can help your doctor make a firm diagnosis.
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Treatment
Early diagnosis and treatment are important in order to prevent CRPS from
developing into the later stages.
It is also important that these patients not be told that the pain is "in their
heads." CRPS is a physiological condition. Even though it is not fully
understood, CRPS is treatable.

After 6 months of treatment, this patient's hands have regained normal color and are
no longer swollen.
Nonsurgical Treatment
Medications. Non-steroidal anti-inflammatory drugs (NSAIDs), oral
corticosteroids, anti-depressants, blood pressure medications, anti-
convulsants, and opioid analgesics are medications recommended to relieve
symptoms.
Injection therapy. Injecting an anesthetic (numbing medicine) near the
affected sympathetic nerves can reduce symptoms. This is usually
recommended early in the course of CRPS in order to avoid progression to
the later stages.
Biofeedback. Increased body awareness and relaxation techniques may help
with pain relief.
Therapy. Active exercise that emphasizes normal use of the affected limb is
essential to permanent relief of this condition. Physical and/or occupational
therapy are important in helping patients regain normal use patterns.
Medications and other treatment options can reduce pain, allowing the patient
to engage in active exercise.
Surgical Treatment
If nonsurgical treatment fails, there are surgical procedures that may help
reduce symptoms.
Spinal cord stimulator. Tiny electrodes are implanted along your spine and
deliver mild electric impulses to the affected nerves.
Pain pump implantation. A small device that delivers pain medication to the
spinal cord is implanted near the abdomen.
Results from surgical procedures may be disappointing. Many patients with
chronic CRPS symptoms benefit from psychological evaluation and
counseling.


Reflex sympathetic dystrophy (RSD) is a clinical syndrome of variable
course and unknown cause characterized by pain, swelling, and
vasomotor dysfunction of an extremity. This condition is often the result
of trauma or surgery. In 1864, Mitchell referred to this malady as
causalgia, a Greek word meaning burning pain. Newer taxonomy refers
to RSD as a type of complex regional pain syndrome (CRPS), which
may develop after an initiating event such as trauma or surgery or may
occur spontaneously.[1] Under this classification, causalgia is a type of
CRPS that develops after nerve injury. In patients with either of these
conditions, sympathetic mediation of the pain (ie, improvement with
sympathetic blockade) may or may not be evident.

The pathogenesis of RSD is unknown. Three conditions are deemed
important in the development of RSD, including a persistent painful
lesion, a predisposition or susceptibility to developing RSD, and an
abnormal sympathetic reflex. Susceptibility factors are unknown and
may include genetic predisposition (HLA typing)[2, 3, 4] and, in some
patients, a tendency toward increased sympathetic activity. This
includes cold hands, hyperhidrosis, or a history of fainting.
Healthy individuals undergo a sympathetic response to injury, with
vasoconstriction designed to prevent blood loss and swelling. This initial
response soon subsides and gives way to vasodilatation and increased
capillary permeability, allowing tissue repair.
In patients with RSD, this sympathetic response continues unabated.
The reasons for the perpetuation of the response are unknown but may
be related to central dysregulation of nociceptive impulses. This
dysregulation may be mediated by wide dynamic range neurons in the
spinal cord. Prolonged ischemia caused by the vasoconstriction
produces more pain, establishing a reflex arc that promotes further
sympathetic discharge and vasospasm. This is compounded by the
local response to trauma, with liberation of substantial amounts of
proinflammatory mediators, such as histamine, serotonin, and
bradykinin. The result is a swollen, painful, stiff, nonfunctioning
extremity. At least partial sympathetic mediation of this phenomenon is
likely because of the ability of sympathetic nerve blockade to relieve
pain and other features of RSD in some patients. The pathogenesis of
RSD is unknown. Three conditions are deemed important in the
development of RSD, including a persistent painful lesion, a
predisposition or susceptibility to developing RSD, and an abnormal
sympathetic reflex. Susceptibility factors are unknown and may include
genetic predisposition (HLA typing)[2, 3, 4] and, in some patients, a
tendency toward increased sympathetic activity. This includes cold
hands, hyperhidrosis, or a history of fainting.
Healthy individuals undergo a sympathetic response to injury, with
vasoconstriction designed to prevent blood loss and swelling. This initial
response soon subsides and gives way to vasodilatation and increased
capillary permeability, allowing tissue repair.
In patients with RSD, this sympathetic response continues unabated.
The reasons for the perpetuation of the response are unknown but may
be related to central dysregulation of nociceptive impulses. This
dysregulation may be mediated by wide dynamic range neurons in the
spinal cord. Prolonged ischemia caused by the vasoconstriction
produces more pain, establishing a reflex arc that promotes further
sympathetic discharge and vasospasm. This is compounded by the
local response to trauma, with liberation of substantial amounts of
proinflammatory mediators, such as histamine, serotonin, and
bradykinin. The result is a swollen, painful, stiff, nonfunctioning
extremity. At least partial sympathetic mediation of this phenomenon is
likely because of the ability of sympathetic nerve blockade to relieve
pain and other features of RSD in some patients.

Epidemiology
Frequency
United States
An estimated 5% of patients who experience trauma to the upper
extremity develop RSD, although this figure is not known with certainty
because of confusion over the diagnosis. Extremity immobilization can
trigger RSD. Without prophylactic measures (active physical therapy),
RSD can develop in 12-20% of people who experience a hemiplegic
stroke.
Mortality/Morbidity
RSD causes essentially no mortality.
Race
No racial predilection exists.
Sex
Sexual distribution is equal.
Age
The age of onset in most patients with RSD is 30-60 years,
and the mean age is 49 years.[5]
RSD affects children and carries a much better prognosis
than in adults

he 3 clinical stages of reflex sympathetic dystrophy (RSD) are acute,
subacute, and chronic. The acute form lasts approximately 3 months.
Pain, often burning in nature, is one of the first symptoms that initially
limits function. Swelling, redness with vasomotor instability that worsens
with dependency, hyperhidrosis, and coolness to the touch are common
physical findings. Demineralization of the underlying bony skeleton
begins because of disuse.
If the process is not arrested or reversed in the acute phase, the
condition may progress to the subacute stage, which can last for up to 9
months. The patient develops persistent severe pain in the extremity
and fixed edema that would have been reversible with elevation during
the acute phase. The redness of the acute stage gives way to cyanosis
or pallor and hyperhidrosis to dry skin. Loss of function progresses, both
because of increased pain and fibrosis of the joints caused by chronic
inflammation. In the hand, this leads to flexion deformity of the fingers.
The skin and subcutaneous tissues begin to atrophy. Demineralization
of the underlying bony skeleton becomes pronounced.
If the process continues, the chronic phase may develop approximately
1 year after disease onset. This stage may last for many years or can
be permanent. Pain is more variable during this period. It may continue
undiminished or abate. Edema tends to subside over time, leaving
fibrosis around the involved joints. The skin is dry, pale, cool, and shiny.
Flexion and extension creases are absent. Loss of function and
stiffness are marked, and osteoporosis is extreme. In the upper
extremity, this can manifest as a frozen shoulder and claw hand.
A thorough general history is strongly suggested. Maintaining a high
index of suspicion is important because proper treatment requires rapid
diagnosis and prompt therapy.
RSD commonly involves only one extremity. It is bilateral in
approximately 25% of cases but is usually more prominent on
one side.
Pain
Usually constant and disproportionate to the precipitant injury
May be exacerbated by ambient factors such as loud noises
and emotional factors (eg, stress, light touch, active motion,
passive motion)
May be described as burning, cutting, searing, pressure, or
tearing
Usually begins locally but may progress to involve the entire
extremity

Possible evidence of prior increased sympathetic activity
Hyperhidrosis
Cold hands
Fainting

Prior trauma, which may be trivial or significant (eg, Colles fracture),
with or without diagnosable nerve injury
Prior surgery
Recent limb immobilization due to hemiplegic stroke, myocardial
infarction
Systemic disease such as diabetes

Perform a thorough physical examination followed by a focused
examination of the involved extremity. Patients with RSD may present
with suggestive physical findings that point to a presumptive diagnosis.
Edema
Edema is the most consistent physical finding and is always
disproportionate to the severity of the precipitant injury or
event.
Pain, swelling, and color change may be more prominent with
dependency in the early stages.
Edema worsens rather than improves and extends beyond the
region of initial concern.
It evolves into a brawny, nonpitting edema that may progress to
an intense fibrosis in all the joints of the extremity.

Stiffness is more severe than expected and may be very distressing to
the patient.
Discoloration
Varies depending on the stage of disease
May be dusky, cyanotic, pale, or red and may eventually lead to
skin hypopigmentation
Begins as redness over the metacarpophalangeal (MCP) or
proximal interphalangeal (PIP) joint flexion creases early in
the disease and progresses as a streak across the palm

Abnormal skin moisture
Hyperhidrosis (early)
Dry skin (late)

Tenderness is initially localized but may progress to generalized
tenderness. Exquisite tenderness, both periarticular and
interarticular, is often present. Patients may exhibit allodynia (ie,
pain with nonnoxious stimuli) and hyperpathia (ie, persistent pain
after light pressure).
Atrophy of the skin and subcutaneous fat pads
Fibrosis of the palmar fascia
Absence of extensor and flexor creases over joints
Frozen shoulder, flexion deformities of the fingers, claw hand
RSD is usually posttraumatic or postsurgical; however, it can occur in a
previously healthy extremity with no known trigger.
Trauma
Penetrating wounds
Lacerations
Abrasions
Venipuncture
Intramuscular injection of medication or illicit drugs
Gunshot wounds
Crush injuries and blunt trauma
Neck or shoulder injuries
Acute traumatic carpal tunnel syndrome
Chest trauma
Sprain, fracture, or dislocation

Postsurgery
Carpal tunnel release
Dental extractions
Cervical rib resection
Fracture repair (Colles fracture)
Postarthroscopy

Local disease
Nerve compression syndromes
Arthritis
Tissue ischemia
Stenosing tenosynovitis

Systemic disease
Myocardial infarction
Stroke
Pancoast tumor
Pancreatic cancer
Herpes zoster