CRPS I (RSD) with pictures. Differential Diagnosis
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CRPS I (RSD) with pictures. Differential Diagnosis

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This presentation is a summary of several lectures given by the past president of the Reflex Sympathetic Dystrophy of America. The Power Point presents the appropriate way to diagnose CRPS (RSD), and ...

This presentation is a summary of several lectures given by the past president of the Reflex Sympathetic Dystrophy of America. The Power Point presents the appropriate way to diagnose CRPS (RSD), and has pictures of CRPS compared to nerve entrapment syndromes, mistakenly diagnosed as CRPS. A list of appropriate medical testing is included, as is an explanation of the pathophysiology. See www.DiagnoseMyPain.com to take a test to clarify the diagnosis.

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CRPS I (RSD) with pictures. Differential Diagnosis CRPS I (RSD) with pictures. Differential Diagnosis Presentation Transcript

  • Lecture 6 RECOGNIZING CRPS I (RSD) Nelson Hendler, MD, MS Former Assistant Professor of Neurosurgery Johns Hopkins University School of Medicine Past president –American Academy of Pain Management Past president- RSD Association of America www.DiagnoseMyPain.com
  • Definitions • Allodynia- a painful response to a normally non-painful stimulus. • CRPS I –complex regional pain syndrome type I, which used to be called reflex sympathetic dystrophy. • CRPS II – complex regional pain syndrome type II, which used to be called causalgia.
  • IASP Definition of CRPS I (International Association for the Study of Pain) • Pain in excess of what is expected. (This is a very subjective definition, and not scientific) • Swelling or edema • Coldness or heat in limb • Loss of hair • Nail growth • Can spread to other side View slide
  • Diagnostic Criteria for CRPS I The Sine Qua Non of Dx. • Thermal allodynia (Raja, Campbell, Meyers- American Pain Society abstract,’96) • Circumferential pain (Raja and Hendler, Current Practices in Anesthesiology, ‘90 ) • Not a cold limb –also found in radiculopathy, nerve entrapment, CRPS II. • Not mechanical allodynia – also seen in nerve entrapment and radiculopathy as well. • Not skin changes- also seen in CRPS II, N. entrap. • Not edema – also seen in lymphatic damage,sprains View slide
  • Flaws with Research Design • Symptoms change over time, following three stages (Schwartzman, and Payne) • These stages are based on severity or clusters of symptoms, not temporal staging. • Many errors in literature, due to failure to report the stage, or list the clinical diagnostic criteria for the patient selection, resulting in difficulty doing meta analysis research, with highly variable outcome results, i.e. 12%-97% success rate for sympathectomy (Payne).
  • Definitions • Circumferential: a location which described a circumference, i.e. all the way around something, like a tree or a limb. • Tinel: a response to a tap on a nerve that sends a sensation in the anatomical distribution of that nerve, like hitting your “funny bone” (the ulnar nerve).
  • Anatomy of Spinal Cord • Sympathetics Sympathetic Chain Dorsal Horn of Spinal Cord Wide Dynamic Range Neurons
  • Concepts • The sympathetic nerves have origin in the thoracic spinal cord. • They form ganglion outside the spinal cord. • Their activity is controlled by the wide dynamic range neurons of the posterior horn. • Sympathetics control functions such as blood vessel diameter, sweating, heart rate. • The wide dynamic range neurons have neuronal plasticity, i.e. they can change activity over time.
  • MEDICAL FACTS ABOUT CRPS I (RSD) • In early stages, CRPS I (RSD) is a disorder of sympathetic nerves. • In later stages, CRPS I (RSD) is a disorder of the spinal cord, of the wide dynamic range neurons, in lamina II and V of the dorsal horn, and NMDA • CRPS I (RSD) may spread to the countralateral limb, or ipsilateral limb, due to neuronal plasticity • 71% of patients diagnosed with CRPS I (RSD) actually have just nerve entrapments, 27% have both (Hendler, Pan Arab Journal of Neurosurgery,’02) • 80% of patiet diagnosed with CRPS I have nerve entrapments which respond to surgery (Dellon,et al, J. Brachial Plex Peripher Nerve Inj, 2009)
  • Theories about the Etiology of CRPS I • Auto-immune - Knobler • Central - angry back firing C fibers - Ochoa • Wide Dynamic Range neurons - Roberts • Neuronal plastisity- Dubner • Hyperpathia- Bennett • Ephaptic connections - Sweet • In reality, no-one really knows the cause.
  • PATIENT HISTORY • There is no way to predict who will get CRPS I (RSD) • Very often, a minor trauma will trigger CRPS I (RSD) • Post-operatively, if there is a painful limb, the more likely diagnoses are nerve injury or compression, due to surgery or to the use of a tourniquet, infection, or an occult fracture • Immediate post-op pain is not CRPS I (RSD) • A tight cast may trigger CRPS I (RSD)
  • SYMPTOMS of CRPS I (RSD) • Thermal allodynia is almost always present • Pain is constant, but varies in intensity • Pain is circumferential. The pain is not in a peripheral nerve distribution • Change of position of the limb does not worsen the pain • Other “classic” signs are highly variable
  • SIGNS of CRPS I (RSD) • Pain is circumferential, around entire foot or arm. • Pressure on the ulnar, radial, median, tibial, sural, superficial and deep peroneal nerves will be no more or less painful than pressure any where else on the limb. • “Classic” edema may or may not be present • “Classic” mottled skin and shiny skin may or may not be present. • “Classic” hair/nail growth may or may not be present.
  • “CLASSIC” DIFFUSE SWELLING OF CRPS I (RSD)
  • “CLASSIC” PITTING EDEMA OF CRPS I (RSD) Pitting Edema
  • “CLASSIC” MOTTLED SKIN OF CRPS I (RSD) Mottled skin
  • Not RSD- Residual After Twisted Ankle Note: Stocking distribution of swelling and edema. No mechanical nor thermal allodynia. 3 + pitting edema. Marked bruising and discoloration. Tender over 4th and 5th metatarsal.
  • Not RSD (CRPS I)- Pre-Op Skin Discoloration Not RSD-Burning pain was in the top of the foot, shin, and sole of foot after severe auto accident, requiring L knee replacement. Negative bone scan, negative Indium scan, sed rate not elevated, no thermal nor mechanical allodynia, pain was not circumferential. Pain was in distribution of tibial nerve, and superficial peroneal nerve, and 100% relief with tibial nerve blocks, and superficial peroneal nerve blocks.
  • Not RSD (CRPS I)- Post-Op Not RSD-Burning pain was in the top of the foot, shin, and sole of foot after severe auto accident, requiring L knee replacement. 100% relief with left tibial, common peroneal and saphenous nerve decompression, with improved skin coloration, bilaterally. Tibial Decompression
  • THERMOGRAPHY OF CRPS I (RSD) The left leg is so cold it doesn’t show.
  • BONE SCAN OF CRPS I (RSD) Localized inflammation of gout Diffuse hyperemia of CRPS I
  • “CLASSIC” SHINY SKIN AND NAIL GROWTH OF STAGE III CRPS I (RSD)
  • CRPS I (RSD) vs CRPS II (CAUSALGIA) CRPS I (RSD) • Circumferential in distribution • Good response to sympathetic blocks • No response to nerve blocks • Both thermal and mechanical allodynia CRPS II (CAUSALGIA) • Follows discrete nerve distribution • Variable response to sympathetic blocks • Responds well to nerve blocks • Mechanical allodynia
  • CRPS I (RSD) vs Nerve Entrapment CRPS I (RSD) • Circumferential in distribution • Good response to sympathetic blocks • No response to peripheral nerve blocks • Both thermal and mechanical allodynia Nerve Entrapment • Follows discrete nerve distribution • Variable response to sympathetic blocks • Responds well to peripheral nerve blocks • Mechanical allodynia
  • DIFFERENTIAL DIAGNOSIS OF CRPS I (RSD) (Hendler, Pan Arab Journal of Neurosurgery, ’02) • N = 38 patients referred to Mensana Clinic with the diagnosis of CRPS I (RSD). • 1/38 (3%) had pure CRPS I (RSD) without any other illness. • 10/38 (26%) had CRPS I (RSD) with nerve entrapment. • 37/38 (97%) had nerve entrapment confirmed by electrophysiological (CPT) testing, and nerve blocks but missed by the referring doctor. • 27/38 (71%) had no signs or symptoms compatible with CRPS I (RSD). They had just nerve entrapment(s). • Prior to admission, only 7/38 (21%) patients had bone scans and only 22/38 (58%) had sympathetic blocks.
  • Missed Diagnoses-CRPS I (Hendler, N, Differential Diagnosis of CRPS I, Pan Arab Journal of Neurosurgery, ’02) • Of the 38 patients referred to Mensana Clinic with the diagnose of RSD (CRPS I): • 42 % never had a sympathetic block. • 79 % never had a bone scan. • 100% never have a peripheral nerve block. • 71% had pain in a peripheral nerve distribution, not circumferentially. • The word “allodynia” was not found in 100% of the charts.
  • Discharge Diagnosis in Patients From Mensana Clinic, referred with only CRPS I (Hendler, N, Differential Diagnosis of CRPS I, Pan Arab Journal of Neurosurgery, ’02) • Discharge Diagnosis • N =10 - CRPS I • N = 2 - CRPS II • N = 9 - Disrupted Disc • N = 37 - Nerve entrapments • N = 9 - Radiculopathy • N = 16 - Thoracic Outlet
  • Testing Done in Patients (number done) From Mensana Clinic, referred with only CRPS I (Hendler, N, Differential Diagnosis of CRPS I, Pan Arab Journal of Neurosurgery, ’02) • Test # of pts # of tests/pt + tests finding* • Sympathetic blocks 11 pts (1-5) 10/11 • Bone Scans 38 pts (1-1) 11/38 • Phentolamine I.V. 7 pts (1-3) 4/7 • EMG/NCV 38 pts (1-2) 37/38 • Peripheral N. block 35 pts (1-6) 35/35 • Root blocks 10 pts (3-10) 9/10 • Provocative discog. 10 pts (1-3) 9/10 • Dopplers of arms 17 pts (1-2) 16/17 * not published
  • CRPS I (RSD) PRESENTS WITH A COLD LIMB, BUT NERVE INJURIES MAY ALSO BE COLD (Uematsu, Hendler,Hungerford, Long and Ono, Electromyogr. Clin. Neurophysiol. #21, pp165-182, 1981) N = 803 cases
  • Thermography and Electromyography in the Differential Diagnosis of Chronic Pain Syndromes and Reflex Sympathetic Dystrophy • Uematsu, Hendler, Hungerford, Long and Ono, Electromyogr Clin. Neurophysiology, ’81 • Review of 803 patients with chronic limb and axial pain. • 431 had abnormal skin (>1 C) temperature in the affected limb. • 140 had increase temperature: 291 had lower temperature. • In 73 cases of patients with abnormal neurological examination, 89% of patients had thermography abnormalities > 1 C. • In 56 cases with abnormal EMG/NCV, 89% also had thermography abnormalities > 1 C, 42 % > 2 C. • In 42 patients with RSD, 92 % had thermography abnormalities >1 C, 67% > 2 C.
  • Degree of Coldness in Limb with CRPS I Measured by Thermography 1-1.9 C 2-2.9 C > 3 C
  • Degree of coldness, measured by thermography, in a limb with nerve injury, confirmed by EMG/NCV 1-1.9 C 2-2.9 C > 3 C
  • Hendler Alcohol Drop and Swipe Test (Hendler, Complex Regional Pain Syndrome I and II, Chapter 20, in Pain Management, Edited by Richard Weiner, Ph.D, CRC Press, 2002) • Squeeze an alcohol swab, and let a drop fall on the affected area. • If the patient immediately responds with severe pain, this is thermal allodynia. • Let the alcohol remain on the foot for 2 minutes. If there is pain, this is chemical allodynia. • Use the swab and gently swipe the affected area. • Immediate pain is mechanical allodynia.
  • TESTS YOU CAN DO IN YOUR OFFICE (Hendler, Complex Regional Pain Syndrome I and II, Chapter 20, in Pain Management, Edited by Richard Weiner, Ph.D, CRC Press, 2002) • Interpretation of the Hendler Alcohol Drop and Swipe Test: A patient should have both thermal and mechanical allodynia to have CRPS I (RSD). • Just mechanical allodynia suggests nerve entrapment or causalgia or radiculopathy. • Just thermal or chemical allodynia suggests CRPS I (RSD). • If Tinel is + in sural, tibial, superficial, deep peroneal ulnar, or radial nerve reproduces pain, block this nerve. • If a nerve block gives total relief, then the diagnosis is nerve entrapment not CRPS I (RSD).
  • OUTSIDE LABORATORY STUDIES YOU CAN ORDER WHILE WAITING FOR THE CONSULTANT TO SEE PATIENT • A bone scan can be a helpful diagnostic test, but varies over the stages of the disease. • An effective sympathetic block should warm the limb. When the limb is warm, a patient with CRPS I (RSD) will have 100% relief, for 2-6 hours. • EMG/NCV measures only A beta sensory fibers and motor fibers, but not C & A delta fibers. • Current perception threshold measures A beta, A delta and C sensory nerve fibers.
  • WHERE TO REFER THE PATIENT AND WHAT YOU SHOULD EXPECT • Refer the patient to a multi-disciplinary pain diagnostic and treatment center, not a monomodal center (just blocks, just medicine, etc). • The center should do a bone scan and CPT (next page) • No more than 6 sympathetic blocks should be done. The limb must become warm in response to the block. During the time the limb is warm, the patient should experience 100% relief of all pain. Blocks last 2-6 Hrs. • Nerve blocks should be done if the sympathetic block does not produce 100% relief when the limb is warm. • The treating doctor has an obligation to monitor the progress of the patient.
  • Painless Electrodiagnostic Current Perception Threshold and Pain Tolerance Threshold in CRPS Subjects and Healthy Controls: A Multicenter Study- Texas Tech, Stanford, Mensana Clinic, Mayo Clinic, Cleveland Clinic, Johns Hopkins, Vanderbilt, UC-SD, Uni. of Texas, (P. Raj, H. Chado, R. Dotson, N. Hendler, et al, Pain Practice, 2001) • CPT/PTT uses A.C. sinusoid waveforms at 5 Hz, 250 Hz, and 2,000 Hz (2 kHz), for C, A delta, A beta sensory fiber testing respectively. • Current Perception Threshold (CPT) is the threshold of feeling electrical current. Early nerve entrapments have low CPT (hyperalgesia), later, high CPT (hypoalgesia) • Pain Tolerance Threshold (PTT) is the maximum amount of pain from the current tolerated by the patient. • In normal patients, non-nerve PPT is higher than CPT • In CRPS I (RSD) patients, PPT is close to CPT, which gives objective confirmation of clinical mechanical allodynia.
  • EXPECTED TREATMENT PROTOCOLS • High dose steroid and exercise for 2-4 weeks. • Ca++ blocking agents and phenoxybenzamine • Use Anti-convulsants such as Neurontin 300mg qid up to 900 mg qid. Add Topamax if needed. • Use narcotics if needed, and titrate according to response (5th vital sign -JCAHO). • Use tricyclic antidepressants, not SSRIs. • If the first sympathetic block worked, get a series of 5 more sympathetic blocks. • If sympathetic blocks provided 100% relief, but did not last, do a surgical sympathectomy.
  • CONCLUSIONS • There is no way to predict who will develop CRPS I (RSD). Get early confirmation. • The clinical presentation of CRPS I (RSD) is variable. • The clinical stages are not temporal but symptom related. • Early, accurate diagnosis is essential for successful treatment, before it progresses to the spinal cord level.
  • CONCLUSIONS (continued) • CRPS I (RSD) is misdiagnosed 71% of the time. You should refer the patient only to an expert experienced with CRPS I (RSD). • Early diagnosis of CRPS I (RSD) improves the treatment outcome. • Essential features of CRPS I (RSD) are a positive bone scan, thermal and mechanical allodynia, circumferential pain, and total relief from effective sympathetic blocks • For comprehensive information and a test to provide a proper diagnosis, see www.DiagnoseMyPain.com
  • The 6 blind wise men examining an elephant, in order to describe it to their king. The king can visualize an elephant, only by integrating all the descriptions.