A very dangerous dysfunction of sympathetic reflexes causing serious impairment from performing daily activities.

1. REFLEX SYMPATHETIC
DYSTROPHY OR
COMPLEX REGIONAL PAIN
SYNDROME
Radhika Chintamani

2. Contents
 Definition
 Introduction
 History
 Types
 Stages
 Management

3. Definition
 Reflex Sympathetic Dystrophy (RSD) is a diseased state of an extremity that is
characterized by very severe pain, swelling, stiffness and discoloration. It usually
occurs after a trauma or disease of an extremity, and is generated by an abnormal
sympathetic reflex which is characteristically treated by abolition of this increased
sympathetic nerve stimulation.
 RSD is defined as a pain state maintained by sympathetic efferent activation by
circulating catecholamines or by neurochemical action – By Harris Gellman MD
& Andrew Markewtiz MD.

4. Normal sympathetic reflex
Pain from extremity due to any cause
Transmitted to spinal cord via afferent nerves
Synapse in the posterior root ganglion, posterior horn and finally the lateral
horn
signal is transferred to sympathetic nerve cell bodies.
Efferent sympathetic signals sent out the anterior horn cell through anterior root
ganglion to the sympathetic chain, the white ramus into the sympathetic
ganglion where a synapse occurs.
The postganglionic sympathetic fibre then leave the ganglia goes distally into
extremity to produce vasoconstriction of the small vessels.

5.  Vasoconstrictive reflex prevents excessive bleeding in the injured tissue and
after few hours it gives way to vasodilation which is part of the reparation
process.
Intense degree of sympathetic nerve activity.
The increased vasoconstriction
Ischemia in the tissue
Increased afferent pain impulses sent centrally thus activating sympathetic arc.
This abnormal sympathetic reflex causes RSD.
 In short, normally the sympathetic stimulation secondary to injury results in
vasoconstriction which decreases blood loss and swelling. Sympathetic tone
then decreases after injury allowing increased blood flow. In abnormal
sympathetic activity inappropriate continuation of sympathetic activity
results in edema with capillary collapse and ischemia causing continued
pain.
Abnormal sympathetic reflex

6. Introduction
 RSD is generally considered to be synonymous with vasomotor and trophic
disorder.
 There are several clinical types of RSD, ranging from a minor involvement of
one or more fingers to the severest form which is major causalgia.
 Injury or disease in hand that has tendency towards excessive fibrosis most
likely will produce a greater amount of pain, swelling, stiffness and dysfunction.
Conditions that fall in this category are the Dupuytren’s palmar fasciitis,
hypertropic arthritis, psoriatic arthritis, carpal tunnel syndrome, stenosing
tenosynovitis etc. when patients with these fibrosis producing tendencies have
injuries or surgeries a condition called flare reaction may result.

7.  It is frequently diagnosed as RSD. One must not be deluded into
thinking that all bad results and stiff hands are caused by RSD.

8. History
 Mitchell (1864) is generally credited with the first accurate description of the condition that we now
call RSD. During same time Raget described trophic changes & a glossy appearance of the hand &
finger with severe pain and dysfunction after a nerve injury.
 Sudecks in 1900 described a condition which he called inflammatory bone atrophy that produced an
RSD appearance. In 1937, Takals described a similar condition and called Reflex dystrophy and later
he called it causalgia state of hand.
 Homans in 1945 called it minor causalgia. After this many authors felt the need to recognize the role
of trauma and vasomotor dysfunction and called it with various names like – Post traumatic
osteoporosis, Post traumatic causalgia, Sympathetic neurovascular dystrophy, Post traumatic
vasomotor disorder etc. Even recentely Evans called it as RSD syndrome. Also now it is called as
Complex Regional Pain Syndrome, which are of two types CRPS I & CRPS II. CRPS I is not linked
to a specific process (RSD), type II is related to a known nerve injury.

9. FACTS
 Women are affected with RSD three times as often as men
 Most common history given by the RSD patient is a minor initial injury accompanied by
disproportionate pain persisting long after healing.
 25% patients with Colle’s fracture displayed RSD features – by Atkins et al. 1989
 28% patients had residual features of RSD as late as 10 years after Colle’s fracture by Field et al.
1992, also reported that elevated intracast pressure, indicative of tight cast and swelling
correlated with 60% likelihood of developing symptoms of RSD.
 Richards reported that 46% patients with causalgia, 83% had injuries that involved median nerve.
 Compression neuropathy at any level can be complicated by RSD (CRPS II) by Grundberg &
Regan in 1991.

10. Pathophysiology
Following are the 3 factors which must occur concurrently before a
patient can develop RSD
 Persistent painful lesion (due to trauma or disease)
 Diasthesis ( includes: Predisposition, susceptibility or inherent trait)
 Abnormal Sympathetic Reflex

11. Persistent painful lesion
 A significant characteristic of RSD is that a magnitude of the inciting
trauma or disease is not necessarily equivalent to the severity of the
patient’s symptoms and signs.
 That is; minor traumas will produce the minor traumatic dystrophy
clinical type of RSD & major traumas will produce the major traumatic
dystrophy.
 Most persistent painful lesion that initiate RSD are of traumatic origin.
In some cases pain from disease will also be sufficient to initiate the
process like tissue ischemia, nerve entrapment, heart attack or stroke.

12. Diasthesis
 Not all patients develop RSD.
 There appears to be a predisposition, inherent characteristic, natural tendency, body
constitutional trait or diathesis that allows some patients to develop RSD.
 Two different types of diathesis are recognized:
I. The first is the tendency of patient to be a hyper sympathetic reactor. These patients
have increased sympathetic nerve activity. This is confirmed if patient always have
sweaty palms or hyper hydrosis or history of excessive coolness of fingers & toes
when they are exposed to colder temperature.
II. Diathesis has to do with the psychologic make up of the patient. This is difficult to
determine the hyper sympathetic reactor. This can be recognized by certain
psychologic traits like fear, suspicion, emotion, chronic complainer, dependent
personality. These Patients also have the tendency to develop low pain threshold.
These type of patients usually are un-co-operative and do not follow the doctors or
therapists advice.

13. Abnormal Sympathetic Reflex
 RSD could not take place unless a grossly abnormal situation arose in the
sympathetic nerve reflex to perpetuate the extreme increase in sympathetic
nerve activity for a prolonged period of time. If already a person has existing
abnormal hyper sympathetic reflexia, on occurrence of any trauma may lead
to this disorder.
 This abnormal sympathetic reflex is therefore the 3rd etiological factor that
must be present before RSD can take place.

14. Pathogenesis
 Livingstone et al have furthere developed a concept first proposed by
Lorence de NÓ which states that
Trauma/ injury
Abnormal feedback mechanism occurring in poster and lateral horns and spinal
cord
An abnormal sympathetic reflex generated
Increase in sympathetic nerve activity
Produced tissue ischemia
Generates more pain, turns into viscous circle which can certainly accentuate
for the severe pain and increased sympathetic nerve activity seen in RSD.

15. Sign and Symptoms
1. Pain: Paramount symptom of RSD is pain and it is characteristically
described as burning pain. Gradually pain turns to pressure crushing,
binding, searing, aching, cutting and cramping pain. Pain is constant and is
aggravated by either active or passive attempts of motion. Another
distinguishing feature is the dysaesthesia and paraesthesia that’s produced
by even a light touch of the affected skin. The initial pain is in the expected
nerve distribution, area of the injury, but it soon spreads to the whole hand,
wrist; forearm and the entire extremity.

16.  Swelling: swelling is usually the first physical sign, occurs initially in
the involved area and slowly spreads to other area so that eventually
it may encompass the hand, wrist and distal part of the forearm. At
first soft edema develops but in time it becomes a hard, brawny
edema and certainly is one of the factors that produce loss of motion.

17. 3. Stiffness: stiffness increases with time, because any attempt at either active
or passive motion is exquisitely painful. Initial lack of motion is attributable
to the enforced immobility to escape further aggravation of the pain.
Subsequently, the stiffness is attributable to increased fibrosis in ligamentous
structures and adhesion formation around the tendon. All fibrous structures
about the joint are thickened and quite hard and inelastic. This will add to the
severity of flexion contractures in the digit.

18.  Discoloration: The discoloration takes the form of redness and
initially is most commonly located over the dorsum of
metacarpophalengeal joints and interphalangeal joints of the fingers.
A purplish discoloration often may be seen in the flexor creases of
the fingers in the palm. Pallor present, where there is
vasoconstriction of both arterial and venule systems. Redness,
present when there dilation of both sides of the vascular tree, but
blueness or cyanosis is usually present when there is
vasoconstriction of the venous system.

19.  Osteoporosis: The initial demineralization in the more severe RSD usually takes
place in the carpal bones and procedures ‘‘Inflammatory bone atrophy’’ given by
Sudeck. The demineralization also takes place in the polar regions of the long
bones and the metacarpals and the phalanges.
 Sudomotor Changes: are usually present but may be variable according to several
factors. Early stages of RSD display hyperhydrosis and last stages display dryness.
 Temperature: One most common finding is that
 Temperature decreases: during hyperhydrosis.
 Temperature increases during dryness of hand or redness.
 When pallor or cyanosis is present, the temperature is nearly always diminished.

20.  Palmar fasciitis: A palmar fasciitis with acute nodule and thickening of
longitudinal bands of palmar fascia in the fingers or in the hands may be seen in
several clinical types of RSD.
 Trophic changes: glossy, shiny appearance of the skin is associated with the
RSD. In early stages it is due to the swelling and ironing out of the skin wrinkles
and secondary to los of motion of the joints. Later due to skin and subcutaneous
atrophy. The skin feels tight and there is definite decrease in the mobility of the
skin over the dorsum of the fingers and particularly the IP joints. The ends of the
fingers may take on a pencil-pointing appearance, because of the atrophy of the
tip of the fat pad and the concomitant downward curving of the finger nails.

21. Stages of RSD
 Traumatic stage
 Dystrophic stage
 Atrophic stage

22. Traumatic stage
 Onset: at the time of initial injury or within several weeks.
 Increase in edema: soft and localized and Spreads to particular tissue
resulting in increasing stiffness
 Hyperhydrosis.
 Skin: Cool, pale, cyanotic, mottled. May show erythema and warmth
secondary to an increased superficial blood flow.
 Increased hair and nail growth.
 Pain: increased by movement and weight bearing. Voluntary disuse to
minimize discomfort. Increased in severity overtime. Diffuse in nature.
 Osteopenia: on x rays with 4 to 8 weeks after onset of pain.
 Duration: 3-6months.

23. Dystrophic Stage
 Onset: 3-6months after onset of pain.
 Pain becomes more diffuse, increase in severity.
 Edema changes from soft brawny edema- (creases disappear).
 Warmth followed by cyanosis.
 Trophic changes: - hair loss
 Brittle nails, cracked, grooved
 Glossy skin
 Decreased moisture
 ROM decreased, increased stiffness
 Muscle weakness or atrophy
 Radiographs: osteopenia.
 Duration: 3-6months.

24. Atrophic Phase
 Onset: 6-12months after injury.
 Pain: interactable, proximal spread worsens with motion.
 Joints: decreased ROM no functional motion, rigid, flexor tendon
contracture, joint subluxation, potentially ankylosed.
 Skin: marked trophic changes owing to decreased blood flow, cool
pale, dry, glossy appearance, and subcutaneous fat atrophy.
 Muscle atrophy.
 X ray: disuse osteoporosis/penia.
 Duration: years/ permanent.

25. Clinical types of RSD
I. Minor causalgia
 As this clinical type of RSD involving
 a nerve it is called causalgia
 peripheral nerve with a vast pain & dysfunction the term minor causalgia.
 A nerve injury in the upper extremity is seen much more commonly in the hand and wrist
than in the elbow and upper arm. The symptoms produced are less noxious, involve a
smaller part of the hand and may be limited to only one or two fingers. The degree of
pain, swelling, stiffness, discolouration & osteoporosis is concomitantly less severe.
 Common peripheral nerves involved in this condition are:
i. Dorsal superficial sensory branch of radial nerve
ii. Palmar branch of median nerve just proximal to wrist is
iii. Dorsal superficial sensory branch of the ulnar nerve, particularly over the dorsal ulnar
aspect of the wrist & hand.
 The pain of minor causalgia is constant and the other features of RSD such as pain on
motion, swelling, stiffness & osteoporosis are much greater than would be expected from
a simple painful neuroma.

26. 2. Minor traumatic dystrophy:-
 Minor traumatic dystrophy is the most common clinical type of RSD
producing frequent painful stimuli.
 The precipitating painful lesion is usually trauma or minor proportions such
as a mashed finger, #, dislocation, sprain or even penetrating wound that
doesn’t involve nerve injury.
 The degree of involvement may encompass only few fingers rather than
whole hand but the s/s such as pain, swelling, stiffness, discolouration &
dysfunction are present redness over the dorsum of the MCP & IP joints and
also over collateral ligament.
 Digits are usually stiffened in function.

27. 3. Shoulder hand dystrophy:
 Least common form of RSD.
 Starts with considerable pain and progresses to stiffness in the shoulder with
spreading of pain into the whole extremity, producing moderate to marked swelling.
 Causative painful lesion either is a proximal trauma such as a shoulder, neck,
ribcage injury or maybe from visceral source as heart attacks, stroke etc.
 Fusiform swelling and fingers are stiffened in extension rather than flexion. Redness
present over MP & IP joints.
 Hand usually warmer and dry.
 Majority of painful stiff shoulder have their origin in disuse from trauma, rotator
cuff tendinitis or adhesive capsulities and these conditions are diagnosed easily and
managed by physical means.
 But if the degree of pain, swelling, stiffness, dysfunction and especially osteoporosis
is much greater than expected & the vasomotor component is recognized then it can
be considered as possible RSD of shoulder hand dystrophy clinical type

28. 4. Major traumatic dystrophy
 Severe type of RSD.
 The persistent painful lesion that triggers major traumatic dystrophy
is usually major trauma such as crushed hand, colle’s # or severe #
dislocation of wrist. It produces greatest pain,stiffness, swelling and
dysfunction in non-nerve injury cases.
 Flexion contractures of digits are more frequent.
 Wrist ROM is mostly limited.

29. 5. Major casualgia:
 Produces the greatest degree of pain and devastation to the patient.
 Inciting painful lesion may be partial nerve injury or major nerve injury in the
proximal part of the extremity.
 In case of upper extremity medial nerve is usually involved in case of lower
extremity of sciatica nerve is involved.
 Pain is most prominent syndrome, is described as burning
 Patient may seek relief by wrapping his hand and upper extremity in wet towels.
 Allodynia: hypersensitivity (+)
 Less red coloration than other types.
 Early stages are almost pale or cyanotic in colour.
 Sweating and coolness are usually a prominent feature.
 Flexion contracture occurs in fingers. Stiffness is severe.

30. Diagnosis
 Its important to make an early diagnosis in cases of RSD because early
treatment is much more effective than treatment at a later stage
 In normal individual who has been injured the signs & symptoms will
improve but in patients with RSD they get progressively worse.
 Four cardinal symptoms and signs of RSD are:-
1. Pain
2. Swelling
3. Stiffness
4. Discoloration
 An actual confirmation comes after interruption of the sympathetic
nerve has produced same amelioration of the patients condition.

31.  In secondary s/s which are most often present but not necessarily
inevitable
1. Osseous demineralization
2. Sudomotor changes
3. Temperature changes
4. Trophic changes
5. Vasomotor instability

32. Specific test for RSD
 Only reliable specific test for RSD is the presence of amelioration of S/S
(four cardinal signs and secondary signs) after a sympathetic blockade.
 Stellate ganglion block is given for suspected RSD.
 Confirmation of RSD: After sympathetic block is given: At least if some of
the symptoms have been partially relieved after sympathetic block and no
presence of 4 cardinal signs & secondary S/S.

33. Non specific tests
 Phentolamine test
 Intravenous use of phentolamine will relieve some of these S/S
indicating RSD. This test also can be used for screening of RSD and
when found to be positive a confirmation of RSD can be obtained
with a stellate ganglion block.

34.  Thermography- to compare the resting blood flow and muscle
temperature in the affected extremity with those in the normal
extremity may be helpful to confirm the diagnosis of RSD
 Three phase bone scanning for diagnosis: For a scan to be considered
diagnostic of RSD the delayed phase must show diffusely increased
activity in the involved joint with periarticular accentuation in the
delayed phase.
•Radiography- Xrays show periarticular or
diffuse mottled osteoporosis.

35.  Catecholamine test-
 Due to increased degree of sympathetic efferent neuronal activity, there will be
increase in the sympathetic neurotransmitted released peripherally in the nerve
terminals which increases sympathetic tone producing an elevation in plasma levels
of norepinephrine. This plasma level can be used as an index of sympathetic
activity.
 When the sympathetic fibers in the upper extremity are stimulated either electrically
or due to stress like pain, cold there is definite increase in catecholamine plasma
level. Determination of increase level in the antecubital vein following presence of 4
cardinal sign will give some idea about RSD and help in diagnosis.

36. Management
 Medical management
 Sympathectomy & sympathetic blocks
 Physiotherapy management

37. Medical Management
 Oral sympathetic drugs
 Sympatholytic medications are:-
1. Alpha adrenergic blocking agents which effectively reduce the sympathetic
vasoconstriction action of the peripheral vessels in the involved extremities.
 Drugs – Phenoxybenzamine, phentolamine, clobidine
2. Beta blocking medication propornolol has been shown to be effective in the relief of pain,
hyperalgesia & hyperpathia.
3. Guanethidine is a postganglionic adrenergic inhibitor that blocks norepinephrine release
thus modulating the effect of sympathetic nervous system on its target organs.
4. Mood modifying drugs like Amitriptyline has been reported to help reduce pain and
complaint in patient with RSD. Other drugs like Chlorpromanine, chlordiasepoxide etc
are used in Rx.
5. Calcium channel blockers have been used in the treatment of RSD because of their ability
to reverse vasomotor instability. These drug induce peripheral sympathetic nerve action
6. NSAID’s are frequently used in the treatment of patients with RSD because they are
thought to inhibit pain and decrease swelling & edema. Indomethacin is commonly used.

38. Surgical Management
 Same technique includes removal of the lower third of the stellate ganglion.
 After successful sympathectomy there is dramatic relief of pain & burning
sensation.
 Patient can exercise easily to improve motion and function.
•Useful in patients with only transient relief from sympathetic
blocks and in younger patients or patients in good physical
conditions.
•First four thoracic sympathetic ganglia are removed for
complete ablation of the abnormal sympathetic reflex.

39. Physiotherapy Management
 Physiotherapy management begins with a meticulous evaluation of
each patient with or without a referring diagnosis of RSD. The
initial evaluation first should include some method of quantifying &
qualifying patients pain

40. Pain
1. TENS:
 Various studies have been done on TENS for pain control in RSD.
 Kesler et al treated 10 children with RSD using TENS, they reposted 9 of
10 improved and 7 of 10 patients had complete remission but they did
not describe their stimulus parameters.
 Loeser et al used TENS with variable width and rates of 10 to 150 hz to
treat 198 patients of which 13 were described to have causalgia painlike.
In this group 9 experienced initial relief, 2 reported long term relief.
 Bohn treated 24 patients with TENS and found it to be most effective in
those patients with increased sympathetic activity.
2. Other modalities like cryotherapy, ultrasound, vibration, biofeedback,
active exercises are helpful in relief and control of pain in RSD.

41. Edema
 The edema may be the result of vasomotor instability and a lack of active motion.
1. Elevation- Elevation of the extremity and hand above the level of heart. It
decreases arterial hydrostatic pressure and assists in lymphatic and venous
drainage, thereby decreasing interstitial volume.
2. Active exercises- AROM should be applied early to all joints to assist in
overcoming the edema as well as the accompanying stiffness.
3. Massage- Mobilizes pitting and brawny edema in the RSD patients.
4. Intermittent Compression- Effective in reducing edema in extremity. In acute
edema 2 hours of compression and in chronic fibrotic lymphatic edema which
may be seen in later stages of RSD, 8 to 72 hours of continuous intermittent
compression may be necessary

42. Joint stiffness
 Active exercises: Structured AROM program and activity directed are
extremely important to maintain the patients level of cooperation and
motivation throughout the course of his care.
 Splinting- prevents or alleviates joint stiffness and contractures. Any
splinting performed should be of a low enough force that it doesn’t
exacerbate the patients pain
 Continous Passive ROM: The CPM device should allow movement through
painfree range for each of the joint being treated.
 Passive ROM- It is most appropriately used only after the patient’s pain has
been controlled or in conjunction with medical intervention. Contraindictaed
if hypersensitivity persists
 Combination of superficial heat and a gentle passive stretch has shown to be
most effective in increasing tissue extensibility. The therapist must remember
to apply the stretch, PROM or AROM while the tissues are still warm.

43. Hypersensitivity
 Also known as allodynia. Best approach is desensitization program.
Desensitization may include use of textures, percussion, pressure and
vibration.
 The therapist should initiate desensitization outside of the area of
hypersensitivity and progressively work towards the area of greater
sensitivity.
 Even retrograde massage performed for edema control is part of the
desensitization program.
 Successful treatment of this syndrome is directly related to active
participation by the patient. The therapist should/must play a
motivational role for the patients by assisting them with resolution of
their symptoms.

44. THANK YOU