Download Reflex Sympathetic Dystrophy in the Upper Extremity

Reflex Sympathetic Dystrophy
in the Upper Extremity
Harris Gellman, MD, and David Nichols, MD
Abstract
The diagnosis and treatment of pain are among the most challenging problems
facing orthopaedic surgeons, and reflex sympathetic dystrophy is probably the
most frustrating and difficult pain syndrome to manage. Pain, swelling, and
autonomic dysfunction are cardinal signs of the condition. Although the pathogenesis is still unclear, many theories have been proposed. Because reflex sympathetic dystrophy is sympathetically mediated, diagnosis can be confirmed on
the basis of response of the pain to sympathetic blockade. Treatment may
include an appropriate exercise program, α-adrenergic blocking agents, moodelevating drugs, calcium channel blockers, intravenous regional blocks, and
stellate ganglion blocks. Recent additions to therapy include electroacupuncture, transcutaneous electrical nerve stimulation, and biofeedback. Prognosis
is, at best, guarded with this perplexing condition, but the best response is
obtained when diagnosis is made early (within the first 2 or 3 weeks after
injury) and treatment is initiated during the first stage of the disease.
J Am Acad Orthop Surg 1997;5:313-322
Reflex sympathetic dystrophy
(RSD) has been defined as a sympathetically mediated pain syndrome.1 This active, progressive
process is characterized by pain,
edema, and autonomic dysfunction, often exacerbated by emotional factors. Swelling is the most
constant physical finding, which if
not treated early is often followed
by the rapid onset of stiffness.
Secondary signs, which are variably present, include osseous demineralization, movement disorder, skin discoloration, palmar
fibrosis, hyperhydrosis, and sudomotor, temperature, trophic, and
vasomotor changes. 1 Clinicians
experienced in the treatment of
RSD agree that early diagnosis and
treatment are of paramount importance.
Vol 5, No 6, November/December 1997
Pathophysiology
Although the etiology of RSD is not
yet clearly defined, several factors
appear to be involved. However,
the exact mechanism whereby the
sympathetic response becomes abnormal is not known. Normally,
an injury activates the sympathetic
nervous system. Sympathetic outflow (initiated at least in part by
the pain of injury) causes vasoconstriction in the limb, leading to
decreased blood loss and swelling.
Sympathetic tone decreases and
blood flow to the limb increases,
allowing ingress of the constituents
of repair as well as egress of waste
products from the site of injury. If
sympathetic tone persists inappropriately, an abnormal feedback
mechanism and an atypical sympa-
thetic reflex result. This causes tissue edema, resulting in capillary
collapse and ischemia, which in
turn cause local pain in the injured
limb. This pain signal re-excites
the sympathetic nerves, and thus a
positive feedback circuit becomes
established. Both surgical and
chemical sympathectomy have
been recommended as means of
interrupting this positive-feedback
loop.2
Sympathetic stimulation has
been shown to prolong and enhance abnormal ectopic pain afferents in various experimental conditions. Experimentally produced
neuromas demonstrate ectopic discharge responsive to sympathetic
stimulation and hypersensitivity to
chemical, mechanical, and thermal
stimuli. Sympathetic stimulation
normally suppresses C-fiber noci-
Dr. Gellman is Professor and Chief, Hand and
Upper Extremity Surgery Services,
Department of Orthopaedic Surgery,
University of Arkansas for Medical Sciences,
Little Rock. Dr. Nichols is Hand Surgery
Fellow, Department of Orthopaedic Surgery,
University of Arkansas for Medical Sciences.
Reprint requests: Dr. Gellman, Department of
Orthopaedic Surgery, University of Arkansas
for Medical Sciences, Slot 531, 4301 West
Markham Street, Little Rock, AR 72205.
Copyright 1997 by the American Academy of
Orthopaedic Surgeons.
313
Upper-Extremity Reflex Sympathetic Dystrophy
ceptor activity. After injury, ectopically firing C fibers are stimulated by sympathetic activity. 3
Ectopic discharge has been noted
in the dorsal root ganglion in
experimentally produced chronic
constriction of the sciatic nerve. 4
Central sensitization to increased
amplitude and frequency of C-fiber
nociceptor activity occurs, hardening the abnormal sympathetic
reflex into an irreversible pattern.5
Successful treatment of RSD is contingent on interruption of this
abnormal continuous-feedback
loop.
Recent evidence suggests a possible genetic diathesis in RSD patients resistant to treatment. Mailis
and Wade6 reported the possibility
that RSD is a neuroimmune disorder linked to multiple sclerosis and
narcolepsy.
Diagnosis
The diagnosis of RSD is based primarily on the patient’s history and
clinical characteristics. There is
usually a history of recent or remote trauma, with persistent burning, aching, or throbbing pain. One
or more of the following signs may
be found: vasomotor or sudomotor
disturbances (e.g., mottling discoloration of the extremity), edema,
stiffness with loss of joint motion,
sensitivity to cold, autonomic dysfunction (e.g., the limb is cold to
the touch), and skin changes.
Often there is muscle weakness or
atrophy. Relief of pain and modification of signs after regional sympathetic blockade are virtually
diagnostic of the disorder.
Many subtle cases of RSD are
accompanied by only one or two of
the signs and symptoms, or the
entire symptom complex may be
vague and confusing, making differential diagnosis difficult. Certain medical problems, such as
314
brain injury, may make obtaining a
detailed history impossible. In
these cases, advanced radiologic
techniques and thermography may
help to confirm the diagnosis.
In addition to the physical
examination, the most reliable aid
to making the diagnosis of RSD is
the three-phase bone scan.7 Before
the use of this modality, the finding
of periarticular or diffuse mottled
demineralization on plain radiographs was used. Because calcium
content must be altered 30% to 50%
before becoming evident on conventional radiographs, the threephase bone scan will be positive
earlier than plain films will. Patchy
demineralization is not specific for
RSD, reportedly occurring in 30%
to 80% of patients. 8 Demineralization may also result from disuse or atrophy associated with
muscle paralysis. Thus, radiographs of quadriplegic or hemiplegic patients generally are not
helpful in differentiating atrophy
due to disuse from RSD. Spasticity
in the brain-injured patient may
mask or prevent the development
of osteoporotic changes.
The first phase of the three-phase
bone scan consists of a radionuclide
angiogram (sequential 5-second
images of both hands obtained over
a span of 40 seconds). This is followed by a blood-pool phase involving 500,000-count images. The
third-phase (delayed-phase) images
are obtained 3 to 4 hours after injection. For a scan to be considered diagnostic of RSD, the delayed-phase
scan must show diffusely increased
activity in the involved hand and
wrist joints.7 Periarticular accentuation in the delayed phase of the
three-phase bone scan has been a
characteristic finding of RSD, occasionally even preceding clinical
symptoms (Fig. 1).
Mackinnon and Holder 7 have
found the delayed bone-uptake
phase of the three-phase bone scan
Fig. 1
A positive delayed-phase bone
scan of a patient with bilateral RSD. Note
periarticular accentuation.
to have a 96% sensitivity and a 98%
specificity in detecting RSD. Using
stricter criteria for the definition of
RSD, Werner et al9 found a sensitivity of 50% and a specificity of
92% for the bone-uptake phase.
The sensitivity and specificity of
the study were found to be higher
when done within 6 months of
onset and when performed on
patients older than 50 years.
A positive bone scan alone does
not necessarily correlate with the
vascular autonomic dysfunction
seen in RSD. Using cold-stress testing, Pollock et al10 found that vasomotor response patterns to cold
stress were the same whether or
not a patient had a positive bone
scan. O’Donoghue et al 11 found
that marked asymmetry can be
seen in all three phases of bone
scanning in asymptomatic persons
as well as in those with RSD, especially in the two early phases. At
present, there is no truly reliable
method to correlate bone scintigraphic findings with RSD staging
or to predict outcome on the basis
of the results of scanning.
Thermography has also been
reported to have a role in confirming the diagnosis in the more subtle
cases of RSD. Low et al12 examined
121 patients with chronic pain and
reported a 21.5% prevalence of pre-
Journal of the American Academy of Orthopaedic Surgeons
Harris Gellman, MD, and David Nichols, MD
viously undiagnosed RSD. Hendler
et al13 studied patients with chronic
pain for whom there was no definitive diagnosis. Nineteen percent of
the patients had abnormal thermographs of the affected limb. In this
subset, 74% had some clinical signs
consistent with RSD. The diagnosis was then confirmed by sympathetic block. Sympathectomy was
performed in some cases, which
resulted in permanent pain relief.
Sylvest et al14 have reported on
the use of measurement of resting
blood flow and muscle temperature
as another adjunct in confirming
the diagnosis of RSD. Temperature
and blood flow were measured in
both extremities of 51 patients, 25
of whom were believed to have
RSD. The temperature of the brachioradialis muscle and the resting
blood flow in the same segment
of the forearm were found to be
notably elevated in affected arms as
compared with unaffected arms.
These differences were not seen in
the control group. Blood flow and
muscle temperature measurements
may also prove useful in assessing
response to treatment.
Disease Progression and
Prognosis
The course of RSD can be divided
into three stages (Table 1). Stage 1
begins immediately or shortly after
injury and usually lasts 3 to 6
months. The patient has severe,
diffuse, deep, burning pain of far
greater severity than would be
expected from the initial injury.
Allodynia (extreme sensitivity to
light touch) is characteristic. Pain
severity may increase during stage
1, usually not following a specific
dermatomal nerve pattern. Pain
may be localized or regional, often
spreading from its original site.
Edema, initially soft and localized,
spreads to include periarticular tis-
Vol 5, No 6, November/December 1997
Table 1
Stages of Reflex Sympathetic Dystrophy
Stage
Duration
Signs and Symptoms
1
3-6 months
Severe, diffuse, deep burning pain (localized or
regional)
Allodynia
Edema spreads to periarticular tissues, causing
stiffness
Erythema, pallor, or cyanosis
Tremor
Dystonic posture of upper extremity (adduction
of shoulder; flexion of elbow, wrist, and fingers)
2
3-6 months
More diffuse severe pain
Hardening of edematous tissue leads to progressive joint stiffness
Thin, cracked nails
Thin, glossy skin
Loss of flexion creases and hair on extremity
3
May last years
or become
permanent
Severe pain may spread or be more closely associated with movement
Fibrous ankylosis
Skin is constantly cool, pale, dry
Subcutaneous tissues disappear, causing narrowing of fingers
Radiographs show severe osteopenia; contracted,
thickened, fibrotic joint tissues
sues, producing stiffness. Altered
cutaneous blood flow regulation
due to autonomic dysfunction can
be manifested by erythema, pallor,
or cyanosis. The affected upper
extremity is held in a dystonic posture, characterized by adduction of
the shoulder and flexion of the
elbow, wrist, and fingers, which
grows more severe with advancing
disease. A fine (3- to 6-Hz) tremor
is often seen in the affected extremity.15 The tremor can often be reduced or eliminated by sympathetic block in the early phase. Trophic
changes usually indicate that the
condition has progressed to the
second stage.
Stage 2 begins about 3 to 6
months after the onset of pain and
lasts from 3 to 6 months. Pain
becomes more diffuse with wors-
ening severity. Tissue edema
changes from soft to hard, with
progressive joint stiffness. Vascular autonomic dysfunction continues, becoming less responsive to
sympathetic blockade. Trophic
changes become apparent, manifested as thin, cracked nails and
thin, glossy skin with loss of flexion creases and hair.
Stage 3 starts approximately 6 to
12 months after injury and may last
years or become a permanent condition. Pain reaches a plateau of
severity and may show spontaneous improvement while spreading to wider areas. Pain also becomes more closely associated with
movement. Fibrous ankylosis occurs as edema continues to harden.
Autonomic dysfunction becomes
fully stabilized, giving a constantly
315
Upper-Extremity Reflex Sympathetic Dystrophy
cool, pale, dry appearance to the
extremity. Trophic changes spread
to the deeper tissues; subcutaneous
tissue disappears, with narrowing
of the fingers. Radiographs show
severe osteopenia; contracted,
thickened, fibrotic joint tissues; and
muscle atrophy.
Reflex sympathetic dystrophy in
children shows the same spectrum
of clinical signs and symptoms.
Wilder et al16 followed up 70 pediatric patients with RSD. Girls predominated, and the lower extremity
was affected most often. The prognosis for recovery or improvement
was better in children than in
adults. However, limb-length discrepancy can develop in children
secondary to altered blood flow
and trophic changes.17
Etiology
Injury
Reflex sympathetic dystrophy
can occur after many types of injury.
Atkins et al18 found clinical evidence
of early RSD in 25% of patients with
Colles’ fractures 9 weeks after injury. Field et al19 found that of 55
patients reviewed 10 years after
Colles’ fracture, 14 (26%) still had
some residual features of RSD.
Symptoms included tenderness in
the fingers, persistent swelling, stiffness, and vasomotor instability. Of
these 14 patients, 6 had one feature,
7 had two, and 1 had three diagnostic features of the disease.
Field et al20 studied the association between cast tightness and the
onset of RSD symptoms in patients
with Colles’ fractures. Pressures
were measured in air bladders
inserted between the cast and the
forearm. Finger tenderness, joint
stiffness, swelling, and vasomotor
instability were considered signs of
RSD. Of 23 patients, 6 (26%)
showed all three features of RSD 9
weeks after fracture. These 6 pa-
316
tients had cast pressures greater
than the 99% upper limit in the
control group. The authors concluded that when intracast pressure is normal 2 weeks after fracture, it is unlikely that RSD will
occur. However, an elevated intracast pressure correlated with a 60%
likelihood that symptoms of RSD
would develop.
Bickerstaff and Kanis21 prospectively studied the data on 274
patients with Colles’ fractures.
Reflex sympathetic dystrophy,
diagnosed on the basis of the presence of diffuse tenderness, vasomotor instability, swelling, and stiffness, was noted in 76 (28%). Pain,
tenderness, and especially stiffness
persisted in 38 (50%) of these symptomatic patients 1 year after injury.
In that study, the most commonly
identified risk factors for RSD were
more severe fractures, fracture
manipulation, and involvement of
the ulnar styloid.
The association of nerve injuries,
particularly multiple injuries, and
RSD is well recognized. Richards22
analyzed 461 cases of causalgia
from the World War II experience
and found that 382 (83%) were in
patients with median or tibial nerve
injury. Injury to more than one
nerve occurred in 244 (53%). In
88% of nerve injuries complicated
by RSD, the injury was proximal to
the elbow or knee.
Partial nerve injuries are also
associated with RSD, especially in
the hand, which often causes an
atypical presentation of RSD. This
is frequently seen in patients with
partial median nerve laceration,
who present with classic atrophic
and sudomotor changes in only the
thumb, index, and long fingers
while the remainder of the hand
remains unaffected.
Neurologic Disorders
Nerve compression syndromes,
such as carpal tunnel syndrome,
cubital tunnel syndrome, and herniated cervical disk, can be complicated by RSD, with the nerve compression serving as the persistent
painful stimulus. Stein23 reported
the cases of 6 patients with RSD
associated with carpal tunnel syndrome. Grundberg and Reagan24
found that of 93 patients with RSD,
22 patients who were unresponsive
to treatment had carpal tunnel syndrome, 5 had cubital tunnel syndrome, 1 had compression of the
ulnar nerve at Guyon’s canal, and 1
had a herniated cervical disk.
Reflex sympathetic dystrophy
occurs in approximately 10% of
patients with a spinal cord injury, a
traumatic brain injury, or stroke.
Of 60 consecutive patients with
spinal cord injuries evaluated by
Gellman et al,25 7 had diffuse hand
pain, swelling, and stiffness; 4 had
bilateral involvement; and 6 had
bone-scan abnormalities. Three of
the 6 patients with abnormal bone
scans were treated with stellate
ganglion blockade and improved
enough to resume occupational
therapy. In patients with spinal
cord injuries, the bone scan pattern
can be useful in differentiating pain
of central origin from pain due to
either unrecognized trauma or
RSD.
Braus et al 26 followed up 132
hemiplegic patients following
stroke. Symptoms of RSD developed in the shoulder in 27%, in
most cases in the second or third
month after the onset of hemiplegia. Risk factors included the presence of shoulder subluxation,
marked upper-extremity weakness
for at least 2 weeks after the stroke,
and visual field deficit.
Weiss et al27 prospectively studied the prognostic value of bone
scanning in predicting the development of RSD after stroke. Twentytwo patients underwent threephase bone scintigraphy after
stroke. Sixteen studies were con-
Journal of the American Academy of Orthopaedic Surgeons
Harris Gellman, MD, and David Nichols, MD
sistent with RSD, and five of the
examined extremities were symptomatic at the time of bone scanning. Of the 11 asymptomatic
patients with positive scans, 7 subsequently had symptoms of RSD.
No patient with a negative scan
subsequently had RSD. The authors concluded that bone scintigraphy can be useful in predicting
RSD in stroke patients.
Treatment
Early recognition allows prompt
initiation of treatment. Therefore, a
high index of suspicion is essential.
Delay in treatment not only prolongs the rehabilitation period but
also may allow the pain and physical alterations to become established and refractory to treatment.
The most important factor in predicting improvement with treatment has been reported to be a
short interval (less than 6 months)
between the onset of RSD and the
initiation of treatment. 28 In the
authors’ experience, if diagnosis is
delayed until 6 months after onset,
patients have a much poorer prognosis than those treated acutely or
within the first 3 weeks of the onset
of symptoms. Although many
argue that a patient who has
unremitting pain and swelling
within the first 3 weeks after injury
or surgery does not yet have RSD,
early intervention may halt the
progression of symptoms. Early
treatment can include pulsed-dose
corticosteroid therapy, nonsteroidal anti-inflammatory agents,
analgesics for pain, and aggressive
occupational or physical therapy.
Occupational and Physical
Therapy
Occupational or physical therapy
should be directed at maintaining
active range of motion, preventing
contractures by static splinting,
Vol 5, No 6, November/December 1997
relaxing muscle spasm, and encouraging daily compliance with
the exercise program. Passive
movement is not performed, thereby avoiding painful stimuli by the
therapist.
Watson and Carlson29 reported
the results of use of a “stressloading” program that consisted of
traction and compression exercises
providing stressful stimuli to the
extremity with minimal joint
motion. The patients were told
that specific stressful exercises
were necessary to remedy their
problem and that light activity or
active motion alone would not
work. Stress loading was used as
the only form of treatment for 41
patients, many of whom had had
previous treatment, including sympathetic block, range-of-motion
exercises, transcutaneous electrical
nerve stimulation, and splinting.
At follow-up, an average of 24
months after trauma (16 months
after the beginning of the stressloading program), pain scores had
improved from an average of 7.7
(on a 10-point scale) before the program to 2.7. At final follow-up, 18
patients were pain-free, 18 had
experienced improvement, 4 had
no improvement, and 1 was worse.
Over a 3-year period, 88% had lessening or relief of pain, 95% had
improved range of motion, all had
improved grip strength, and 84%
had returned to the same occupation.
The earlier therapy is initiated,
the better the prognosis. Physical
therapy alone is probably more
important than most drug treatments.
Drugs
Several drugs have shown promise in relieving symptoms of RSD.
Of the many actions of the sympathetic nervous system, the α-adrenergic action is the most important in
its effect as a vasoconstrictor in the
skin and subcutaneous tissues. 2
Phenoxybenzamine is the most
effective α-blocking agent and has
the fewest undesirable side effects.
Ghostine et al 30 reported 40
cases of RSD after nerve injuries
from missile and shrapnel wounds.
Treatment was started with oral
phenoxybenzamine administered
in doses of 10 mg every 8 hours.
The dosage was increased by 10
mg/day every 2 days until the pain
was relieved or postural hypotension occurred. The maximum dose
in that study was 80 mg/day.
Most patients were treated for 6
weeks. In follow-up ranging from
6 months to 6 years, all 40 patients
obtained complete relief.
The usual recommended starting dose for phenoxybenzamine is
10 mg/day. This dose should be
maintained for at least 5 days
before increase. 2 Patients treated
with phenoxybenzamine must be
followed up closely for postural
hypotension.
Phentolamine is an alternative
α-blocking agent. However, its use
is contraindicated for several types
of cardiac conditions; therefore, its
routine use is not recommended.2
Oral guanethidine in a single
dose of 20 to 30 mg/day for 8
weeks has been suggested by
Tabira et al.31 This drug depresses
the function of the postganglionic
adrenergic nerves, thus blocking
sympathetic nerve-mediated impulses. Disadvantages of the drug
include the possibility of inciting
mental depression, loss of appetite,
despondency, and impotence. This
drug has also been associated with
orthostatic hypotension.
Mood-modifying drugs, such as
chlorpromazine, chlordiazepoxide,
trifluoperazine, diazepam, and amitriptyline, have been reported to be
helpful in the control of RSD.32 It
should be noted that their role is
only adjunctive to the primary
treatment.
317
Upper-Extremity Reflex Sympathetic Dystrophy
Calcium channel blockers are a
recent addition to the drug armamentarium for the treatment of
RSD. Calcium entry blockers represent a means of inducing peripheral vasodilatation without
specifically interfering with the
peripheral sympathetic nervous
system. This class of drugs inhibits the movement of calcium
ions into cells. The resulting inhibition of excitation-contraction
coupling causes relaxation of the
arteriolar smooth muscle and
vasodilatation. There is very little
effect, however, on the smooth
muscle of veins.
Nifedipine relaxes smooth muscle, increases peripheral blood
flow, and antagonizes the effects of
norepinephrine on arteriolar and
venous smooth muscle. Nifedipine
may also reverse signs of vasomotor instability and may be useful in
the treatment of Raynaud’s phenomenon.
Prough et al 33 reported on the
use of oral nifedipine in the treatment of 13 patients with RSD. The
starting dose was 10 mg three
times a day. The dosage was
increased weekly to a maximum of
30 mg three times a day. When a
constant level of pain relief was
obtained for 3 weeks, the dose was
tapered over several days. Of the
13 patients, 7 had complete relief, 2
had partial relief, 3 stopped treatment because of side effects
(headache), and 1 experienced no
improvement.
Topical clonidine transdermal
patches have been used to diminish
hyperalgesia secondary to RSD.34
A 7.0- or 10.5-cm2 patch is used for
2 to 10 days at each site. Hyperalgesia under the patch was eliminated in the patients who had previously experienced relief from
sympathetic blockade. Patients
who had no relief from sympathetic
blocks had no relief with topical
clonidine.
318
Although nonsteroidal antiinflammatory drugs are commonly
recommended in the treatment of
the RSD patient, Wilder et al 16
found that 60% of the 70 patients in
their series had no reduction in
pain with this class of drugs.
Corticosteroid use in RSD
remains controversial. Prednisone
has been the most commonly
administered corticosteroid, typically given in pulse fashion. Initially high doses are given (60 to 80
mg/day in divided doses) and then
quickly tapered off. Some authors
have reported good response to
systemic corticosteroid therapy,
but others have found no improvement. Wilder et al16 and Braus et
al26 believe that pulsed treatment
with a methylprednisolone “dosepack” is very useful in treating
RSD diagnosed before a 6-month
duration of symptoms, but is of
limited effectiveness in cases of
established disease.
Sympathetic Interruption
Sympathetic interruption can be
produced chemically (either locally
or regionally) or surgically. If the
symptoms of RSD are not relieved
promptly by oral medications and
aggressive physical and occupational therapy, stellate ganglion
blocks should be strongly considered. Symptomatic improvement
after sympathetic blockade both
confirms the diagnosis and helps to
break the pain cycle.
Early in the course of disease the
relief of pain may last well beyond
the duration of the block and may
even be curative. Initially, one or
two blocks should be tried to assess
effectiveness. If there is good pain
relief, even temporarily, the blocks
should be repeated every other day
until pain is controlled, to a maximum of 12 blocks. If there is no
response after two stellate ganglion
blocks, surgical sympathectomy is
not indicated, as this would sug-
gest that the pain is not necessarily
due to a sympathetically mediated
pain syndrome. If the patient has
responded to the series of 12 blocks
and has recurrent pain after a short
interval of relief, surgical sympathectomy is indicated.
For sympathectomy in the upper
extremity, the stellate ganglion,
which lies at the level of C7-T1, is
bathed in either 1% lidocaine or
0.25% bupivicaine. Blocks may
also be continuous.
Hobelmann and Dellon35 used
an indwelling axillary-sheath
catheter to provide continuous sensory and sympathetic blockade in
RSD patients during surgery for
concurrent problems in the affected
extremity. None of the patients
experienced exacerbation of their
RSD symptoms.
Linson et al 36 treated 29 RSD
patients with continuous stellate
blockade and vigorous physical
therapy for an average of 7 days.
All but 2 patients had marked relief
of pain and increased range of motion. Two thirds showed improvement in trophic and vasomotor
changes. Four patients achieved a
normal status. Improvement was
not maintained in 7 patients; of
these, 6 had legal, psychiatric, or
disability problems.
In a randomized, double-blind
study, Hord et al 37 treated 12
patients with intravenous regional
bretylium and lidocaine. Bretylium
inhibits norepinephrine release by
adrenergic nerve terminals. When
combined with lidocaine intravenous regional block, bretylium
has been found to prolong the duration of pain relief. Tourniquetproduced ischemia alone may be as
effective as the intravenously
injected medication.38
Continuous blockade, while
probably better than intermittent
blocks, is impractical in many clinical environments. Problems with
indwelling catheters include clot-
Journal of the American Academy of Orthopaedic Surgeons
Harris Gellman, MD, and David Nichols, MD
ting or blockage at the tip, infection, and dislodgment of the
catheter. The use of Bier block anesthesia with lidocaine and a corticosteroid or bretylium is a very
effective method of treatment, with
the added advantage of being able
to manipulate the anesthetized
extremity. Intermittent use of stellate ganglion blockade (three times
per week) is also effective, particularly for patients without marked
stiffness.
treatment is shown in Figure 2. For
acute onset of pain (e.g., traumatic
or perioperative), we initially prescribe a methylprednisolone dosepack for 1 week; sustained-release
indomethacin, 75 mg twice daily
for 2 weeks; and amitriptyline, 25
mg, at bedtime. The amitriptyline
is useful in treating the depression
and sleep disorders commonly
experienced by patients with
severe pain. Elevation, ice, and
aggressive occupational or physical
therapy are used daily. Even after
application of a cast, therapy can
help prevent finger stiffness and
swelling.
Recommended Treatment
A general algorithm that we
have found useful in planning
If improvement is found at the
1-week follow-up examination,
therapy is continued three times
per week until the symptoms
resolve. The nonsteroidal antiinflammatory agent can be
changed if necessary.
If there is no response to treatment by the end of the first week of
treatment, two stellate ganglion
blocks are given 2 days apart, and
the methylprednisolone is continued for an additional week. If
there is a good response to the stellate blocks, they are continued for 3
to 4 weeks at the rate of three per
week.
Suspicion of RSD
(swelling, pain, discoloration)
Acute onset
(e.g., traumatic,
perioperative)
Established
pain pattern
Trial of two
stellate blocks
• Methylprednisolone
dose-pack for 1 week
• Amitriptyline at bedtime
• Elevation and ice
• Aggressive occupational/
physical therapy
Good response
Good response but with
finger and wrist stiffness
No response
Follow-up
at 1 week
Improvement
No improvement
• Indomethacin for 2
weeks
• Continue amitriptyline
• Occupational therapy
continued 3 times a
week until symptoms
resolve
Add:
• Two stellate blocks
given 2 days apart
• Methylprednisolone
for 1 week
• Full series of 12 stellate
blocks (3 times a week
for 4 weeks)
• NSAID
• Amitriptyline
• Aggressive occupational/
physical therapy
• Can alternate Bier blocks
with stellate blocks
• NSAID
• Amitriptyline
• Aggressive occupational/
physical therapy
• Manipulate under Bier
block
Probably not sympathetically
mediated pain syndrome;
need to look for another
etiology
If good response to stellate
blocks, continue 3 times a
week for 3-4 weeks
Fig. 2
Algorithm for recommended evaluation and treatment of upper-extremity RSD (NSAID = nonsteroidal anti-inflammatory drug).
Vol 5, No 6, November/December 1997
319
Upper-Extremity Reflex Sympathetic Dystrophy
Patients referred with established pain patterns are initially
treated with a trial of two stellate
blocks. This gives both the physician and the patient a chance to
better assess the cause of the pain.
We then follow the same treatment
protocol used for the patient with
acute-onset pain. If the patient has
a good response to the trial blocks,
the full series of 12 blocks is given,
combined with a nonsteroidal antiinflammatory medication, aggressive occupational and/or physical
therapy, and amitriptyline at bedtime. The amitriptyline can be increased to 150 mg in 25 mg/week
increments if necessary.
For patients with stiffness, Bier
block anesthesia will allow manipulation of the joints. Bier blocks
can be alternated with stellate ganglion blocks, but should probably
not be used in place of the stellate
blocks.
Surgical Sympathectomy
Patients with good but only temporary pain relief after sympathetic
blockade may be considered for
surgical sympathectomy. Using an
open transthoracic or retroperitoneal approach, Olcott et al 39
reported 74% excellent, 17% good,
and 9% poor results in 35 RSD
patients followed up for an average
of 14 months.
A new method, endoscopic surgical sympathectomy, has the
potential advantage of a much
shorter hospital stay. Robertson et
al40 treated eight RSD patients with
video-assisted thoracic ganglionectomy. A pneumothorax was produced for the procedure, and treatment was then administered via a
chest tube. The average hospital
stay was 15.4 hours. Operating
time was 30 minutes per side.
There were no intraoperative complications. Six of the patients had
complete or partial relief of their
symptoms during an average follow-
320
up period of 5 months. However,
this procedure should not be considered benign; the senior author
(H.G.) treated a patient in whom
ulnar nerve resection occurred during this procedure.
Amputation
Dielissen et al 41 studied the
effectiveness of amputation in
relieving the symptoms of RSD in
28 patients (34 amputations in 31
limbs). Only 2 patients were
relieved of pain. Reflex sympathetic dystrophy recurred in the stump
in 28 limbs, so that most patients
were unable to wear a prosthesis.
Recurrence was most common
when amputation was done
through a level that was not free of
symptoms. Despite the poor results in controlling pain, 24 patients
were satisfied with the results of
surgery. Many had considered
their situation hopeless before
surgery; amputation improved
social function and cured persistent
infection, and painful physical contact with the limb was eliminated
despite the persistence of pain.
Nevertheless, amputation is not
recommended for pain relief because its effectiveness cannot be
predicted.
Alternative Treatment
Modalities
Electrical stimulation for the
relief of pain has been advocated
for the past century. This method
fell into disrepute for a long period,
but interest was renewed when
Melzack and Wall 42 popularized
the “gate control” theory of pain.
Varying levels of success have been
reported for the use of transcutaneous electrical nerve stimulation
in the treatment of RSD in adults.43
In children, however, transcutaneous nerve stimulation has been
used very successfully and may be
the second line of defense when
physical therapy and oral medica-
tions fail to control the pain. Kesler
et al44 used physical therapy and
transcutaneous nerve stimulation
to treat 10 children with RSD.
Seven children had complete remission within 2 months, 2 others
improved, and only 1 had no response.
Electroacupuncture is rapidly
gaining acceptance as a treatment
alternative for patients with RSD.
Chan and Chow 45 reported pain
relief in 90% of patients treated.
Hill et al46 reported improvement
in a small series of patients with
RSD refractory to other methods of
treatment, including sympathetic
block. The senior author has found
the improvement in response to
electroacupuncture to be quite
impressive, even in patients with
RSD that has been refractory to
other treatment modalities. Patients not only have alleviation of
pain but also experience vasodilatation in the extremity, with
warming and erythema. Treatments are usually done at low frequencies (less than 10 Hz) for 20
minutes.
It has been theorized that the
action of electroacupuncture is in
part due to the release of endorphins in the central nervous system.47 Partial reversal of the electroacupuncture effect has been
seen after subcutaneous injections
of naloxone. 48 An alternative
explanation may be that large-fiber
transmission during acupuncture
“closes the gate” to pain (according to the gate theory of Melzack
and Wall 42). The lasting physiologic changes seen in patients with
RSD after electroacupuncture
appear to be caused by changes in
neurovascular responses, which
may arise after alterations in central nervous system neurochemistry. Electroacupuncture has the
advantage of being noninvasive,
with only minor, if any, side
effects.
Journal of the American Academy of Orthopaedic Surgeons
Harris Gellman, MD, and David Nichols, MD
Summary
As there is no straightforward
approach to the diagnosis and
treatment of RSD, a high index of
suspicion is critical. Because RSD
is sympathetically mediated, the
diagnosis can often be confirmed
on the basis of the response to sympathetic blockade. Treatment may
include an occupational or physical
therapy program, α-adrenergic
blocking agents, mood-elevating
drugs, calcium channel blockers,
intravenous regional blocks, and
stellate ganglion blocks. Patients
respond best when diagnosis is
made early (within the first 2 or 3
weeks after injury) and treatment is
initiated during the first stage of
the disease.
lar response associated with reflex
sympathetic dystrophy of the hand
and wrist. J Hand Surg [Am] 1993;18:
847-852.
O’Donoghue JP, Powe JE, Mattar AG,
Hurwitz GA, Laurin NR: Three-phase
bone scintigraphy: Asymmetric patterns in the upper extremities of
asymptomatic normals and reflex
sympathetic dystrophy patients. Clin
Nucl Med 1993;18:829-836.
Low PA, Neumann C, Dyck PJ, et al:
Contact thermography in diagnosis of
reflex sympathetic dystrophy: A new
look at pathogenesis. Thermology 1985;
1:106-109.
Hendler N, Uematesu S, Long D:
Thermographic validation of physical
complaints in “psychogenic pain”
patients. Psychosomatics 1982;23:283287.
Sylvest J, Jensen EM, SiggaardAndersen J, Pedersen L: Reflex dystrophy: Resting blood flow and muscle
temperatures as diagnostic criteria.
Scand J Rehabil Med 1977;9:25-29.
Schwartzman RJ, Kerrigan J: The movement disorder of reflex sympathetic
dystrophy. Neurology 1990;40:57-61.
Wilder RT, Berde CB, Wolohan M,
Vieyra MA, Masek BJ, Micheli LJ:
Reflex sympathetic dystrophy in children: Clinical characteristics and follow-up of seventy patients. J Bone
Joint Surg Am 1992;74:910-919.
Rush PJ, Wilmot D, Saunders N,
Gladman D, Shore A: Severe reflex
neurovascular dystrophy in childhood. Arthritis Rheum 1985;28:952-956.
Atkins RM, Duckworth T, Kanis JA:
Algodystrophy following Colles’ fracture. J Hand Surg [Br] 1989;14:161-164.
Field J, Warwick D, Bannister GC:
Features of algodystrophy ten years
after Colles’ fracture. J Hand Surg [Br]
1992;17:318-320.
Field J, Protheroe DL, Atkins RM:
Algodystrophy after Colles fractures is
associated with secondary tightness of
casts. J Bone Joint Surg Br 1994;76:901905.
Bickerstaff DR, Kanis JA: Algodystrophy: An under-recognized complication of minor trauma. Br J Rheumatol
1994;33:240-248.
Richards RL: Causalgia: A centennial
review. Arch Neurol 1967;16:339-350.
Stein AH Jr: The relation of median
nerve compression to Sudeck’s syndrome. Surg Gynecol Obstet 1962;115:
713-720.
Grundberg AB, Reagan DS: Compression syndromes in reflex sympathetic dystrophy. J Hand Surg [Am]
1991;16:731-736.
Gellman H, Eckert RR, Botte MJ,
Sakimura I, Waters RL: Reflex sympathetic dystrophy in cervical spinal
cord injury patients. Clin Orthop 1988;
233:126-131.
Braus DF, Krauss JK, Strobel J: The
shoulder-hand syndrome after stroke:
A prospective clinical trial. Ann
Neurol 1994;36:728-733.
Weiss L, Alfano A, Bardfeld P, Weiss J,
Friedmann LW: Prognostic value of
triple phase bone scanning for reflex
sympathetic dystrophy in hemiplegia.
Arch Phys Med Rehabil 1993;74:716-719.
Poplawski ZJ, Wiley AM, Murray JF:
Post-traumatic dystrophy of the
extremities: A clinical review and trial
of treatment. J Bone Joint Surg Am
1983;65:642-655.
Watson HK, Carlson L: Treatment of
reflex sympathetic dystrophy of the
hand with an active “stress loading”
program. J Hand Surg [Am] 1987;12(5
pt 1):779-785.
Ghostine SY, Comair YG, Turner DM,
Kassell NF, Azar CG: Phenoxybenzamine in the treatment of causalgia: Report of 40 cases. J Neurosurg 1984;60:
1263-1268.
Tabira T, Shibasaki H, Kuroiwa Y:
Reflex sympathetic dystrophy (causalgia) treatment with guanethidine.
Arch Neurol 1983;40:430-432.
References
1. Lankford LL: Reflex sympathetic dystrophy, in Green DP (ed): Operative
Hand Surgery, 3rd ed. New York:
Churchill-Livingstone, 1993, vol 1, pp
627-660.
2. Bonica JJ: The Management of Pain:
With Special Emphasis on the Use of
Analgesic Block in Diagnosis, Prognosis,
and Therapy. Philadelphia: Lea &
Febiger, 1953, pp 913-974.
3. Sato J, Perl ER: Adrenergic excitation
of cutaneous pain receptors induced
by peripheral nerve injury. Science
1991;251:1608-1610.
4. Kajander KC, Wakisaka S, Bennett GJ:
Spontaneous discharge originates in
the dorsal root ganglion at the onset of
a painful peripheral neuropathy in the
rat. Neurosci Lett 1992;138:225-228.
5. Woolf CJ, Shortland P, Coggeshall RE:
Peripheral nerve injury triggers central
sprouting of myelinated afferents.
Nature 1992;355:75-78.
6. Mailis A, Wade J: Profile of Caucasian
women with possible genetic predisposition to reflex sympathetic dystrophy: A pilot study. Clin J Pain 1994;
10:210-217.
7. Mackinnon SE, Holder LE: The use of
three-phase radionuclide bone scanning in the diagnosis of reflex sympathetic dystrophy. J Hand Surg [Am]
1984;9:556-563.
8. Kozin F, Genant HK, Bekerman C,
McCarty DJ: The reflex sympathetic
dystrophy syndrome: II. Roentgenographic and scintigraphic evidence of
bilaterality and of periarticular accentuation. Am J Med 1976;60:332-338.
9. Werner R, Davidoff G, Jackson MD,
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 [Am] 1989;14:520-523.
10. Pollock FE Jr, Koman LA, Smith BP,
Poehling GG: Patterns of microvascu-
Vol 5, No 6, November/December 1997
11.
12.
13.
14.
15.
16.
17.
18.
19.
20.
21.
22.
23.
24.
25.
26.
27.
28.
29.
30.
31.
321
Upper-Extremity Reflex Sympathetic Dystrophy
32. Kleinert HE, Cole NM, Wayne L,
Harvey R, Kutz JE, Atasoy E: Posttraumatic sympathetic dystrophy.
Orthop Clin North Am 1973;4:917-927.
33. Prough DS, McLeskey CH, Poehling
GG, et al: Efficacy of oral nifedipine in
the treatment of reflex sympathetic
dystrophy. Anesthesiology 1985;62:796799.
34. Davis KD, Treede RD, Raja SN, Meyer
RA, Campbell JN: Topical application
of clonidine relieves hyperalgesia in
patients with sympathetically maintained pain. Pain 1991;47:309-317.
35. Hobelmann CF Jr, Dellon AL: Use of
prolonged sympathetic blockade as an
adjunct to surgery in the patient with
sympathetic maintained pain. Microsurgery 1989;10:151-153.
36. Linson MA, Leffert R, Todd DP: The
treatment of upper extremity reflex
sympathetic dystrophy with prolonged
continuous stellate ganglion blockade.
J Hand Surg [Am] 1983;8:153-159.
37. Hord AH, Rooks MD, Stephens BO,
Rogers HG, Fleming LL: Intravenous
322
38.
39.
40.
41.
42.
regional bretylium and lidocaine for
treatment of reflex sympathetic dystrophy: A randomized, double-blind
study. Anesth Analg 1992;74:818-821.
Ramaurthy S, Hoffman J, Walsh N,
Schoenfeld L: Role of tourniquet
induced analgesia in I.V. regional
sympatholysis. Anesthesiology 1986;65
(suppl):A207.
Olcott C IV, Eltherington LG,
Wilcosky BR, Shoor PM, Zimmerman
JJ, Fogarty TJ: Reflex sympathetic dystrophy: The surgeon’s role in management. J Vasc Surg 1991;14:488-495.
Robertson DP, Simpson RK, Rose JE,
Garza JS: Video-assisted endoscopic
thoracic ganglionectomy. J Neurosurg
1993;79:238-240.
Dielissen PW, Claassen ATPM,
Veldman PHJM, Goris RJA: Amputation for reflex sympathetic dystrophy. J Bone Joint Surg Br 1995;77:270273.
Melzack R, Wall PD: Pain mechanisms: A new theory. Science 1965;150:
971-979.
43. Loeser JD, Black RG, Christman A:
Relief of pain by transcutaneous stimulation. J Neurosurg 1975;42:308-314.
44. Kesler RW, Saulsbury FT, Miller LT,
Rowlingson JC: Reflex sympathetic
dystrophy in children: Treatment with
transcutaneous electric nerve stimulation. Pediatrics 1988;82:728-732.
45. Chan CS, Chow SP: Electroacupuncture in the treatment of post-traumatic
sympathetic dystrophy (Sudeck’s atrophy). Br J Anaesth 1981;53:899-902.
46. Hill SD, Lin MS, Chandler PJ Jr:
Reflex sympathetic dystrophy and
electroacupuncture. Tex Med 1991;87:
76-81.
47. Clement-Jones V, McLoughlin L,
Tomlin S, Besser GM, Rees LH, Wen
HL: Increased β-endorphin but not
met-enkephalin levels in human cerebrospinal fluid after acupuncture for
recurrent pain. Lancet 1980;2:946-949.
48. Mayer DJ, Price DD, Rafii A: Antagonism of acupuncture analgesia in
man by the narcotic antagonist naloxone. Brain Res 1977;121:368-372.
Journal of the American Academy of Orthopaedic Surgeons