CRPS

CRPS is a chronic progressive disease characterized by severe pain, swelling and changes in the skin

Type I, also known as RSD, Sudeck’s atrophy, reflex neurovascular dystrophy (RND) or algoneurodystrophy, does not have demonstrable nerve lesions.

Type II, also known as causalgia, has evidence of obvious nerve damage.

Pathophysiology

�                     The pathophysiology is not fully understood. �Physiological wind-up� and central nervous system (CNS) sensitization, are key neurologic processes that appear to be involved in the induction and maintenance of CRPS

�                     There is compelling evidence that the N-methyl-D-aspartate (NMDA) receptor has significant involvement in the CNS sensitization process.

Diagnosis
(IASP CRITERIA)

CRPS types I and II share the common diagnostic criteria

�                     Spontaneous pain or allodynia is not limited to the territory of a single peripheral nerve, and is disproportionate to the inciting event.

�                     The presence of an initiating noxious event or a cause of immobilization

�                     Continuing pain, allodynia (perception of pain from a nonpainful stimulus), or hyperalgesia disproportionate to the inciting event

�                     Evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the area of pain

�                     The diagnosis is excluded by the existence of any condition that would otherwise account for the degree of pain and dysfunction

How will you recognize them

�                      They are terrified of any stimuli that will increase their pain,

�                     They don�t like their bodies, they are socially isolated, they have lost all confidence in themselves

�                     And their physicians are stymied.

�                     They have severe muscle wasting and joint and dystrophic changes. They need intensive psychiatric care, physical therapy, and vocational rehabilitation to restore their lives. There is a new beginning for a great number of our patients

CRPS-II

�                     The presence of continuing pain, allodynia, or hyperalgesia after a nerve injury, not necessarily limited to the distribution of the injured nerve

�                     Evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the region of pain

�                     The diagnosis is excluded by the existence of any condition that would otherwise account for the degree of pain and dysfunction.

STAGES

�                     Stage one : lasts from 1 to 3 months and is characterized by severe, burning pain, along with muscle spasm, joint stiffness, rapid hair growth, and alterations in the blood vessels that cause the skin to change color and temperature.

�                     Stage two: from 3 to 6 months, characterized by intensifying pain, swelling, decreased hair growth, cracked, brittle, grooved, or spotty nails, softened bones, stiff joints, and weak muscle tone.

�                     stage three: the syndrome progresses to the point where changes in the skin and bone are no longer reversible..

TREATMENT MODALITIES

�                     The nomenclature, pathophysiology, and treatment modalities of complex regional pain syndrome (CRPS) are controversial. Thus far there are no specific, scientifically valid treatments for the management of CRPS.

�                     The numerous modalities of treatment range from sympathetic ganglion blocks, intravenous regional blocks, administration of a multitude of pharmacologic agents and behavioral interventions, to surgical sympathectomy.

�                     Minimally invasive radiofrequency lesioning for managing CRPS is a modality in its developmental stages.

Treatments for CRPS type 1 supported by evidence of efficacy and little likelihood for harm are:

Topical DMSO cream,

IV bisphosphonates  and limited courses of oral corticosteroids .

Despite some contradictory evidence, physical therapy and calcitonin (intranasal or intramuscular) are likely to benefit patients with CRPS type 1 .

Invasive Treatment of CRPS

�                     Due to modest benefits and the invasiveness of the therapies,

�                     Epidural clonidine injection

�                     Intravenous regional sympathetic block with bretylium and

�                     Spinal cord stimulation  should be offered only after careful counseling (B).

�                     Evidence for intervention less than compelling

Ketamine Inpatient Protocol

�                     In more severe patients that have been refractory to all modes of standard therapy, an inpatient protocol is employed. This includes 40 mg of ketamine per hour for five days. The concentrations of ketamine in the bloodstream must reach 250 to 300 � g/L to be effective. Patients are awake, and if they become dysphoric, are given 2 mg of midazolam supplemented with 1 to 2 mg of lorazepam every four hours.

�                     This protocol has not �cured� anyone, but it does offer approximately 70% relief for three to six months. A few patients have had sustained relief for eight months.

�                     Reference

�                     1. Schwartzman RJ, Alexander GM, Grothusen J. Pathophysiology of complex regional pain syndrome. Expert Rev Neurother. 2006;6(5):669-681.

Sympathetic Blocks (SB)

�                     Many physicians consider sympathetic blocks as a mainstay treatment for CRPS. Current data does not support the long-term effectiveness of sympathetic block for the treatment of CRPS. The advocates for SB believe that it decreases the abnormal hyperactive sympathetic tone and thus decreases the pain.  The opponents of SB argue that several studies have shown no correlation between sympathetic dysfunction and pain relief obtained from SB.

STELLATE GANGLION BLOCK

�                     The mechanism of action of the stellate ganglion block (SGB) is still uncertain; however it has been used successfully in treatment of chronic regional pain syndrome (CRPS) for many years.

Inverse relationship exists between hand perfusion and the duration of symptoms of CRPS I, On the other hand, a positive correlation exists between SGB efficacy and how soon SGB therapy is initiated.

Duration of symptoms greater than 16 weeks before the initial SGB and/or a decrease in skin perfusion of 22% between the normal and the affected hands adversely affects the efficacy of SGB therapy.

Guided SB blocks

�                     in our experience if a stellate ganglion block performed blindly utilizing standard technique, injection at cervical 6 vertebra level, fails to relief CRPS pain. We repeat the injection under fluoroscopic guidance at lower level either at Thoracic 1 or 2 level, which very often relieves pain. The reason behind this is, in 10 to 20% of human, the sympathetic nerve supply to the upper extremity comes from the lower thoracic ganglions instead of higher cervico-thoracic ganglion.

Sympathectomy

�                     Depending on the series and the duration of follow-up, the success rate of sympathectomy varies from 12% to 97%. Radiofrequency neurolysis (RFN) is becoming a popular method of sympathectomy among pain specialists.

�                     The reasons for the failure of sympathectomy are incomplete sympathectomy, extensive interconnection of chains of sympathectomy ganglia cause rerouting of sympathetic impulse after removal of short chain of ganglia, and hypersensitization of adenoreceptors in the sympathectomized area.

The role of SCS in CRPS

�                     Currently the mechanisms of Complex Regional Pain Syndrome (CRPS) are poorly understood and stratification of either diagnosis or therapy is very weak. .

�                     Spinal Cord Stimulation (SCS) has been used as the last resource to alleviate pain and re-establish function in CRPS patients.

�                     However, there is a disagreement over how it works  An underlying concept is that it works by modulating autonomic nervous system (ANS) activity.

�                     Some studies indicated that SCS has also an effect on blood pressure regulation and improves the CRPS patients’ response to Valsalva maneuver -a test of autonomic function.

Mechanism of action

�                     Gate control theory:stimulation of A-� fibers closes the dorsal born�gate� and reduces the nociceptive input from periphery

�                     Increased dorsal horn inhibitory action of neurotransmitters such as GABA, adenosine A-1,substance P

�                     Activation of descending analgesia pathways by serotonin,norepinephrine


Stimulation trials

�                     Evaluate the SCS analgesic activity in everyday surroundings

�                     Criteria for a successful trial:>50% reduction in pain, decrease in analgesic intake, significant functional improvement

�                     Predict a long-term outcome in 50-70% of cases

�                     Trial time:1-5days

Lead tip positioning

�                     Upper extremity:C2-C5(shoulder area can be difficult to cover)

�                     Foot:T11-L1

�                     Lower extremity:T9-T10

�                     Low back:T8-T10

�                     Chest:T1-T2

�                     Occipital neuralgia:C1-C2

�                     Pelvic pain:S2-S4

A systematic review of the clinical
and cost-effectiveness literature and assessment of prognosis

�                     SCS appears to be an effective therapy in the management of patients with CRPS type I(level A evidence)

�                     And for type CRPS II(level D evidence)

�                     There is evidence to demonstrate that SCS in a cost-effective treatment for CRPS type I

European Journal of Pain 10(2006)91-101

Is Scs Cost-Effective

�                     RCT reported a significant long-term benefit of SCS plus physical therapy over physical therapy alone for pain relief in CRPS type I

�                     Data from the case series included in the review demonstrate the consistent benefit of SCS for CRPS. On average 67% of implanted patients experienced at least 50% pain relief.


Grades of recommendation

�                       At least one meta-analysis, systematic review,of RCT rated as 1++, and directly applicable to the target population, or a systematic review of RCTs or a body of evidence rates as 1+, directly applicable to the target population, and demonstrating overall consistency of results.

�                     D Evidence level 3 or 4, or extrapolated evidence from studies rates as 2+.

Reference

�                     The Massachusetts General Hospital Handbook of Pain Management 3rd  P.354-365;P193-203

�                     Michael Stanton-Hicks MD.Complex regional pain syndrome:manifestations and the role of neurostimulation in its management. Journal of Pain and Symptom Management 2006;Vol.31 No.4S20-24

�                     Rod S.Taylor,Jean-Pierre Van Buyten. Spinal cord stimulation for complex regional pain syndrome:A systematic review of the clinical and cost-effectiveness literature and assessment of prognostic factors.European Journal of Pain 10(2006)91-101

REFERENCES

�                     (1.) Stanton-Hicks MD, Burton AW, Bruehl SP, et al. An updated interdisciplinary clinical pathway for CRPS: Report of an expert panel. Pain Practice 2002; 2:1-16.

�                     (2.) Commentary on RSD focus article. Bandolier 2002. Available at: (3.) Petchkrua W, Weiss D J, Patel RR. Reassessment of the incidence of complex regional pain syndrome type 1 following stroke. Neurorehabil Neural Repair 2000; 14:59-63.

�

what is the sense of doing SCS treatment?

�                     The spinal cord stimulator is effective in the SMP phase of the CRPS, not the SIP. The recent research has shown that the later in the course of the disease spinal cord stimulator (SCS) is started, the less likelihood SCS will relieve the pain

As a rule of Thumb, if the stellate ganglion nerve blocks have lost their effect, about the same ti

� me the SCS loses its effect. This is because both treatments aim at the sympathetic system. With passage of time, as the pain gradually changes to SIP, such treatment cannot be expected to help.

� Reference : . Rosenblum, JA: Spinal abdominal myoclonus. The Neurologist. 1996; 2: 784-787.

Failure Of Repetitive Sympathetic Nerve Blocks

� A patient came to see me today because of persistence of pain in all four extremities. He  is very upset because he  has been told that she needs to have the following course of treatment:

1. Sympathetic nerve blocks. he had no objections to it and I welcome it.

2. Surgery for carpal tunnel syndrome.

3. Post operatively he is to be scheduled to see a psychologist to follow her course.

�
The architectural design of the nerve block-surgery-psychotherapy is very similar to the work of an architect in a small town in Florida who designed a dead-end street to start with the police department, followed by the fire department, followed by hospital, followed by nursing home, followed by mortuary, followed by grave yard. The grave yard was quite large so it blocked the road and the sign declared “dead-end”.

� An old wise farmer once said “there is no lesson to be learned from the second kick of a mule”.

� surgery for the secondary carpal tunnel or tarsal tunnel syndrome results in disastrous acceleration and deterioration of RSD.

Treatment course

� The same patient after undergoing a few nerve blocks is then exposed to treatments such as spinal stimulator or infusion pump. We have already discussed the futility of the use of the spinal stimulator in CRPS

� Even a treatment as powerful as infusion pump is apt to fail if the patient is already loaded with intake of strong narcotics.

�
In conclusion, regardless of how extensively and repeatedly the patient has had different independent modalities of CRPS treatment, the patient should be started from scratch with multiple treatments of antidepressant as an analgesic for chronic pain, effective pain control with the addition of non-addicting strong narcotic medications, muscle relaxants especially in the form of Baclofen (Lioresal), and nerve blocks.

�
The nerve blocks should not be just simply limited to a few sympathetic ganglion blocks or Bier block, but they should also include epidural and paravertebral nerve blocks.

The epidural and paravertebral nerve blocks are quite effective as a maintenance form of nerve block. They block not only the somatic nerve, but also the sympathetic nerves as well.

Pain pump

� The infusion pump should not be tried unless the patient is already detoxified for at least 1 week from other forms of narcotics.

�
The infusion pump usually requires small enough dosage of Morphine or Dilaulid so that there would be no suppression of secretion of cerebral endorphines. This is usually between 2 to 8 (up to 9) milligram per kilogram Morphine. On the other hand if one goes above the 9mg dosage, the pain recurs due to the suppression of the endorphine function. This is a frequent cause of failure of infusion pumps.


In some patients the patient is intolerant of even a very small dosage

�                     of Morphine or Dilaulid in the spinal fluid. I such patients an addition of a very small amount of Clonidine in the pump (small doses of 75-125 microgram) help enhance the pain control. As a result, the dosage of the Morphine or Dilaulid can be reduced to quite a small amount and yet effective relief of pain can be achieved without nausea.

Assistive Devices and Narcotics

�                     An extremity suffering from RSD- is not suffering from a simply hyperactive sympathetic system. It suffers from a dysfunctional system. The circulation in the bone and muscle is accelerated at the expense of poor circulation to the skin and nerves

�                     Eventually the x-ray shows practically no calcium in the bones and shows stress fractures

�                     The addictive narcotics are not just suppressing your Endorphins but also ACTH (which prevents inflammation), estrogen (which prevents osteoporosis) and natural cerebral antidepressants.

Difficulties in intervention

�                     I may not feel the pain but I spend my life helping the unfortunate victims. You are frustrated with the pain. I am frustrated with the latest most destructive trend in the management of RSD consisting of spinal stimulators that only stimulate the sympathetic system and make the RSD worse, multiple unnecessary surgeries for fictitious, imaginary conditions secondary to inflammation of RSD. I do not need to name them because practically everyone of you have had these operations. The end result is Stage IV with horrible consequences. All of this is done while you are going through the “Rip Van Winkle” phenomenon, slowing watching yourself deteriorate.

OUTCOME OF RSD

�                     The best study about the outcome of RSD and the advanced stages of RSD has been written by Dr. Poplawski from Canada which was published in 1983. He showed that RSD diagnosed in the first 2 years has a chance of successful treatment in 80% of the patients and after two years each year drops the percentage of the success significantly[1].

By the time the RSD is over 4 to 5 years old and has not responded properly to treatment and by the time such a patient continues to deteriorate on her rapidly downhill course, the only thing that can help the patient is an infusion pump. This is in the form of Morphine or Morphine and Baclofen combination, or Morphine and Clonidine combination.

�                     When the patient is given other narcotics along with the infusion pump, the disease becomes much worse and the patient develops a lot of inflammation, arthritis and rapidly deteriorates.

�                     Blaming the pump is the same as blaming the F-16 fighter jet crashing when the pilot does not know how to operate it.

�                     1. Poplawski ZJ, Wiley AM, Murray JF: Post traumatic dystrophy of the extremities. J Bone Joint Surg [Am] 1983; 65:642-55

�                     2. Hooshmand H.: Chronic pain: Reflex sympathetic dystrophy. Prevention and management  Boca Raton, FL, CRC Press, 1993.