Complex Regional Pain Syndrome

Complex Regional Pain Syndrome Felipe D. Villena Jr. D.O. US Army, CPT (P), MC National Capital Consortium 3/31/2008 3/31/2008 OBJECTIVES  History of RSD/CRPS  Definition  Clinical Features/Diagnosis  Etiology  Treatment – Medical, Sympathetic Blocks, SCS  Future/Prognosis 3/31/2008 PATIENT  37 y/o AD male involve in a roll-over accident while serving in Afghanistan (Sep 2001). No major injuries sustained but noticed pain /discomfort on his R hand which progressively worsen over time. Pt presented to WRAMC pain clinic about 5 months later with R hand burning/tingling, throbbing pain. Physical exam revealed allodynia throughout R hand, edema, erythema, and temperature difference in relation to L hand. Hair loss was also noted on his R hand. 3/31/2008 Civil War Silas Weir Mitchell MD (1829-1914) “Sensory Hallucination” “The Case of George Dedlow” Causalgia “Kausos” – Heat “Algos” - Pain 3/31/2008 Silas Weir Mitchell  “…..the most amiable grow irritable, the bravest soldier becomes a coward, and the strongest man is scarcely less nervous than the most hysterical girl. …..Causalgia, the most terrible of all tortures which a nerve wound may inflict.” 3/31/2008 Injuries of Nerves and Their Consequences (Philadelphia: J.B. Lippincott) Civil War 3/31/2008 World War I (1914-1919) Rene Leriche, M.D. (1879-1955) Sympathectomy “Surgery of Pain” (1937) 3/31/2008 Rene Leriche  I saw the patient on the 20th June: the upper limb was completely paralyzed-arm, forearm, hand and fingers….dominating everything, was an intense burning pain, concentrated particularly in the palm of the hand…On the 27th August I exposed the brachial artery, which I found small and contracted. I removed its adventitia for a distance of 12 cm…By the next day it was obvious that the patient had less pain. 3/31/2008 World War II (1941-1944) William K. Livingston, M.D. (1892-1966) Lieutenant Commander Oakland Naval Hospital “Vicious Circle” “Mirror image” 3/31/2008 Livingston's Case Notes from a WWII Peripheral Nerve Injury Patient, 1945 3/31/2008 Different Names Sudeck’s atrophy  Shoulder-Hand Syndrome  Acute atrophy of bone  Traumatic vasospasm  Reflex Neurovascular Dystrophy  Sympathetically maintained pain & complex regional pain syndrome  3/31/2008 NEW TERMINOLOGY Dr. Evans – RSD (1947)  The International Association for the Study of pain (1995)  – Dr. Michael Stanton-Hicks  Complex Regional Pain Syndrome I  Complex Regional Pain Syndrome II 3/31/2008 Stanton-Hicks et al, Pain: 63:127133, 1995 Complex Regional Pain Syndrome I (RSD)  Develops after an initiating noxious event  Spontaneous pain and/or allodynia/hyperalgesia occurs, is disproportionate to the inciting event in severity, duration and distribution. and is beyond the territory of a single peripheral nerve.  There is or has been evidence of edema, skin blood flow abnormality (i.e. temperature and/or color change), or abnormal sudomotor (sweat gland) activity in the region of the pain since the inciting event.  This diagnosis is excluded by the existence of conditions that would otherwise account for the degree of pain and dysfunction. 3/31/2008 Complex Regional Pain Syndrome II    Develops after a nerve injury. Spontaneous pain and/or allodynia/hyperalgesia occurs, is disproportionate to the inciting event in severity and duration, and is not necessarily limited to the territory of the injured nerve. There is or has been evidence of edema, skin blood flow abnormality, or abnormal sudomotor (sweat gland) activity in the region of the pain since the inciting event. This diagnosis is excluded by the existence of conditions that would otherwise account for the degree of pain and dysfunction. 3/31/2008 EPIDEMIOLOGY  Frequently in young adults  Females:Male (2.3-3:1)  Onset linked to history of trauma, immobilization or procedures.  No correlation between severity of initial injury and ensuing painful syndrome  Precipitating event unknown in ~10% 3/31/2008 ETIOLOGY        Trauma (often minor) Ischemic heart disease and MI Cervical spine or spinal cord disorder Cerebral lesions Infections Surgery Repetitive motion disorder or cumulative trauma, causing conditions such as carpal tunnel. 3/31/2008 DIFFERENTIAL DIAGNOSIS  Musculoskeletal – Bursitis, MFPS – Rotator cuff tear – Fracture, sprain  Vascular – Raynaud’s disease – Buerger’s disease – Thrombosis – Traumatic vasospasm  Neurologic – – – – Poststroke pain syndrome Peripheral neuropathy Postherpetic neuralgia Radiculopathy  Rheumatic – Rheumatoid arthritis – SLE  Infectious – Cellulitis – Infectious arthritis  Psychiatric – Factitious disorder – Hysterical conversion reaction 3/31/2008 Clinical Features  Pain  Skin Changes  Swelling  Movement Disorder  Spreading Symptoms  Bone Changes  Duration of CRPS/RSD 3/31/2008 3/31/2008 PAIN      Hallmark – Pain out of proportion to those expected from initial injury Severe, Constant burning and/or deep aching pain Allodynia Hyperpathia Myofascial pain syndrome 3/31/2008 SKIN CHANGES     Dystrophy-atrophy Dry or scaly Hair coarse then thin Nail – Brittle – Grow faster/slow  Vasomotor Changes – Warm/cold to touch – Experience warmth/coolness without touching    Sudomotor Changes – Increased sweating  Skin Color – White mottled to red/blue Pilomotor Changes – “goose flesh” appearances Rashes, ulcers, pustules 3/31/2008  Atrophic Skin  Combination of loss of hair and increased thickness of hair 3/31/2008 SWELLING  Pitting or hard (brawny) edema  Diffuse and localized  Painful and Tender  Occasionally sharply demarcated 3/31/2008  Brawny edema  Reddened thickened skin 3/31/2008 MOVEMENT DISORDER  Painful  Direct inhibitory effect of CRPS on muscle contraction  Disuse Atrophy  Tremors and involuntary severe jerking  Spasms  Dystonia 3/31/2008 3/31/2008 SPREADING SYMPTOMS  Initially localized to the site of injury  Pain and symptoms becomes more diffuse as time progresses  Typically starts in an extremity  Trunk or Face  Entire quadrant of the body 3/31/2008 SPREADING SYMPTOMS “CONTINUITY TYPE” – Symptoms spread upward from the initial site, e.g., from the hand to the shoulder. “MIRROR-IMAGE TYPE” – The spread was to the opposite limb. (15%) “INDEPENDENT TYPE” – Symptoms spread to a separate, distant region of the body. This type of spread may be related to a second trauma. (70%) 3/31/2008 Maleki et al., Pain 2000;88:259-66 BONE CHANGES  XRAY  BONE SCINTIGRAM 3/31/2008 XRAY  Partial Osteoporosis = primary x-ray manifestation  Non-specific finding – periarticular and associated with soft tissue swelling  Erosions of the subchondral bone 3/31/2008 BONE SCAN    Depends on the stage of the disease Only positive for significant changes during subacute (<1yr) Scintigraphy useful guide to prognosis – 90% with Positive scintigrams favorable response to steroids – 34% with Negative scintigrams 3/31/2008 Moriarty J., Reflex Sympathetic Dystrophy Syndrome, May 1990 BONE SCAN Stage 1 2 3 Weeks 0-20 20-60 60-100 BF INC N DEC BP INC INC N DI INC INC N INC=increased, DEC=decreased, N=Normal •Demangeant JL, et al. •181 patients with RSDS of the hand •Blood Flow (BF), Blood Pool (BP), Delayed Images (DI) 3/31/2008 Demangeant JL, et al., JNM 1988;29:26-31 BONE SCAN  Intenzo C. et al. – 32 Patients – Sensitivity – 72%    28 % - Normal 59% - Increased 13% - Decreased   Werner R. et al. – Sensitivity = 50%, Specificity = 92% MacKinnon SE, Holder LE – Sensitivity = 96%, Specificity = 98% 3/31/2008 DURATION OF CRPS  Varies  Mild cases – weeks followed by remissions  Remissions may last for weeks, months, or years 3/31/2008 Stages of CRPS  Three stages (dying concept)  Unpredictability of the disease  Not all of the clinical features may be present  Stage I and II symptoms begin to appear within a year  Large multicenter study identified three subgroups 3/31/2008 Bruehl et al., Pain 2002;95:119-24 STAGE I 1. 2. 3. 4. 5. 6. 7. 8. Onset of severe, pain limited to the site of injury Hyperasthesia Localized swelling Muscle cramps Stiffness and limited mobility Onset skin is warm, red, dry the it may change to blue (cyanotic) and become cold/sweaty Hyperhydrosis Mild – last for few weeks, then subsides or treatment 3/31/2008 STAGE II 1. 2. 3. 4. 5. Pain becomes even more severe and more diffuse Swelling tends to spread and it may change from a soft to hard (brawny) type Hair may become coarse and scant, nails may grow faster then slower and become brittle. Spotty wasting of bone (osteoporosis) occurs early but may become severe and diffuse Muscle wasting begins 3/31/2008 STAGE II 3/31/2008 STAGE III 1. 2. Marked wasting of tissue (atrophic) eventually become irreversible Pain becomes intractable and may involve the entire limb. 3/31/2008 STAGE III 3/31/2008 3/31/2008 Sympathetically Mediated Pain (SMP) Sympathetic – “flight or fight”  Abnormal function  Vicious cycle  3/31/2008 Sympathetic System 3/31/2008 Kocher, LF et al. Pain 1987;29:363373 3/31/2008 LABORATORY DIAGNOSTIC AIDS  Thermogram  Three phase radionuclide bone scanning  Sympathetic Blocks  X-rays, EMG, Nerve conduction studies  CT scans  MRI 3/31/2008 THERAPEUTIC GOALS  Educate about therapeutic goals  Encourage normal use of the limb – Physical Therapy  Minimize Pain  Determine the contribution of the sympathetic nervous system to the patient’s pain 3/31/2008 The most important general principle in the treatment of CRPS is to organize a multidisciplinary approach after the onset of the disease. 3/31/2008 3/31/2008 3/31/2008 PHYSICAL THERAPY  Desensitization of the affected region  Mobilization, edema control, and isometric strengthening  Stress loading, isotonic strenghtening, range of motion, postural normalization, and aerobic conditioning  Vocational and functional rehabilitation 3/31/2008 Constant Pain with inflammation NSAIDS (Asa, Motrin, etc.) Constant Pain without inflammation Agents acting on CNS by atypical mechanism (e.g. tramadol) Constant Pain or spontaneous Anti-depressants (paroxysmal) jabs and sleep (e.g. elavil, pamalor, etc.) disturbances Oral LA (mexilitine) Spontaneous jabs Anti-convulsants (e.g. carbamazine, gabapentin 3/31/2008 Widespread, severe CRPS pain, refractory to less aggressive pain Sympathetically maintaned pain (SMP) Oral Opioid-controversy? “Opioid contract” Clonidine patch Muscle cramps (spasms and Klonopin (clonazepam) dystonia) Baclofen Localized pain related to nerve injury Capsaicin cream 3/31/2008 “Rest of the meds….”  NMDA receptors antagonist (e.g. Dextromethorphan and Ketamine) – Management of neuropathic pain  Calcitonin Intranasal spray  IV infusion of Alendronate (Bisphophonate)  Dimethyl sulfoxide (DMSO) 3/31/2008 Epidural/Intrathecal Drugs  Epidural Clonidine  Intrathecal Baclofen – Dystonia 3/31/2008 SYMPATHETIC BLOCKS  Three reasons to consider sympathetic blockade to facilitate the management of RSD/CRPS 1. Provide permanent cure or partial remission of symptoms 2. Gain further diagnostic information to pain etiology 3. Provides prognostic information 3/31/2008 SYMPATHETIC BLOCKS  Stellate Ganglion Block  Lumbar Sympathetic Block 3/31/2008 3/31/2008 3/31/2008 3/31/2008 Efficacy of LSB in CRPS  Saxena et.al, – Pt with symptoms + positive response to LSB will most likely report improvement of their symptoms – Worse response with longer duration of CRPS – Younger patients with shorter duration of CRPS respond well to LSB 3/31/2008 Saxena S. et.al, Reg Anesth Pain Med 2003;28 STELLATE GANGLION BLOCK  Cervicothoracic sympathetic block  Fusion of the inferior cervical and the first thoracic ganglion anterior of C7 body  Horner’s syndrome 3/31/2008 Anatomy 3/31/2008 SGB 3/31/2008 SGB 3/31/2008 Flouroscopy 3/31/2008 SGB Complications Needle in the wrong place  Spread of local anesthetic    Hematoma – Carotid trauma – IJ vein trauma Intravascular injection – Carotid, Vertebral, IJ Neuraxial/Brachial Plexus – Epidural, Intrathecal  Neural Injury – Vagus – Brachial plexus roots Local Spread - RLN  Pulmonary Injury – Pneumothorax – Hemothorax – Chylothorax Infection •Soft Tissue (abscess) •Neuraxial (meningitis)  Other – Esophageal perforation 3/31/2008 PHENTOLAMINE  Alpha-adrenergic antagonist  1 mg/kg over 10 minutes  Only diagnostic  43% False negative  Pt with bleeding disorders, coagulopathy 3/31/2008 SPINAL CORD STIMULATION (SCS)  Patient with chronic intractable pain  Low intensity, electrical impulses to trigger selected nerve fibers (dorsal column)  Replaces pain with tingling sensation (Paresthesia)  May enhance blood flow to affected extremity 3/31/2008 SCS Trial  Temporary electrode  Relatively invasive  Costly procedure 3/31/2008 Radio Frequency Systems      ADVANTAGES External Power High Frequency settings Multiple electrodes with more contacts Patient control includes all parameters Implant slightly smaller in size and weight less 3/31/2008 Radio frequency Systems      DISADVANTAGES Skin irritation Inability to use system while showering, etc Limit choices of clothing Potential difficulties in communication w/ obese patients Frequent Replacemet of recharging of external battery 3/31/2008 Implantable Pulse Generators ADVANTAGES  No requisite external antenna  Ease of use, fewer and simple controls 3/31/2008 Implantable Pulse Generators     DISADVANTAGES Periodic surgical replacement of device Limitation of frequency levels and power output Fewer electrical points Therapy may be compromised to limitations of power output 3/31/2008 3/31/2008 Treatment modalities  Medications – Clonidine Patch, Lidocaine patch, Opioids, Neurontin, Pamelor  Pain Psychiatry Consult  Stellate Ganglion Block (8/28/02)  Radiofrequency Denervation of SG (9/13/02) 3/31/2008 CASE RESULTS 3/31/2008 CASE RESULTS 3/31/2008 CONCLUSION  CRPS is indeed a Complex Disorder  Not a single clinical entity  Far from having a firm understanding 3/31/2008 BIBLIOGRAPHY        Barash P., Cullen B., Stoelting R. Clinical Anesthesia, 4th ed. Philadelphia: Lippincott Williams & Wilkins, 2001:1445-1447 Yao F.S. Anesthesiology-Problem Oriented Patient Management, 5th ed. Philadelphia: Lippincott Williams & Wilkins, 2003:615-628 Staton-Hicks M, Janis W, Hassenburch S. Reflex sympathetic dystrophy: changing concepts and taxonomy. Pain 1995;63:127-133. Merskey H, Bogduk N. Classification of chronic pain. Task Force on Taxonomy. International Association for the study of Pain: relatively generalized syndromes, 2 nd ed. Seattle, WA:IASP Press, 1994:39-56 Veldman PH, Reynen HM, Arntz IE, et al. Signs and symptoms of reflex dystrophy: prospective study of 829 patients. Lancet 1993;342:1012-1016. Janig W. Baron R. Complex regional pain syndrome: Mystery explained? The Lancet of Neurology 2003;2(11): Kemler MA; Barendse GA; van Kleef M; de Vet HC; Rijks CP; Furnée CA; van den Wildenberg FA. Spinal cord stimulation in patients with chronic reflex sympathetic dystrophy. N Engl J Med 2000; 343(9): 618-24 3/31/2008 BIBLIOGRAPHY         Maleki J, LeBel AA, Bennett GJ, Schwartzman RJ: Patterns of spread in complex regional pain syndrome, type I (reflex sympathetic dystrophy). Pain 2000;88:259-66 Raja, S.N., Grabow T.S., Complex Regional Pain Syndrome I (Reflex Sympathetic Dystrophy), Anesthesiology May 2002;96(5):1254-60 Demangeant JL, et al. Three-Phase bone scanning in RSDS of hand. JMN 1988;29:2631 Moriarty J., Drum D., Reflex Sympathetic Dystrophy Syndrome. Saxena S., Debyshire S, Bernstein C, Rizk N, Cope D. Efficacy of lumbar sympathetic blockade in the management of Complex Regional Pain Syndrome (CRPS) van de Beek WJ; Schwartzman RJ; van Nes SI; Delhaas EM; van Hilten JJ. Diagnostic criteria used in studies of reflex sympathetic dystrophy. Neurolgy 2002; 58(4): 522-6 Cope D. K. Phantom Limb and Causalgia Pain in the three great wars. ASA Newsletter 2002;6 (10); Baron R, Wasner G; Backonja MM; Traumatic neuralgias: complex regional pain syndromes (reflex sympathetic dystrophy and causalgia): clinical characteristics, pathophysiological mechanisms and therapy. Neurol Clin 1998; 16(4): 851-68 3/31/2008 BIBLIOGRAPHY   Bruehl S; Harden RN; Galer BS; Saltz S; Backonja M; Stanton-Hicks M. Complex regional pain syndrome: are there distinct subtypes and sequential stages of the syndrome? Pain 2002; 95(1-2): 119-24 Morgan G.E., Mikhail M.S. Clinical Anesthesiology 2nd ed. Connetticult, Appleton & Lange 1996: 308-309 3/31/2008 QUESTIONS??? 3/31/2008