summary Complex Regional Pain Syndrome, also known as reflex sympathetic dystrophy, is an idiopathic condition caused by an aberrant inflammatory response that leads to sustained sympathetic activity in a perpetuated reflex arc. Patients present with extremity pain out of proportion to physical exam findings Diagnosis is made clinically with the Budapest diagnostic criteria and can be confirmed by pain relief with sympathetic block. Treatment is usually physical therapy, pain medication, nerve stimulation or nerve blockade. Surgical sympathectomy is indicated in cases of progressive pain that fails nonoperative management. Epidemiology Incidence US incidence is 5.5 per 100,000 person-years Demographics females more commonly affected (4:1) incidence increases with age up until 70 years old Anatomic location 60% occurs in the upper extremities 40% occurs in the lower extremities Risk factors trauma with an exagerrated response to injury most common reason for a poor outcome following a crush injury to the foot surgery prolonged immobilization anxiety or depression use of ACE inhibitors at the time of trauma history of migraines or asthma smoking fibromyalgia Etiology Pathophysiology aberrant inflammatory response vasomotor dysfunction maladaptive neuroplasticity Genetics thought to have a genetic predisposition siblings of young-onset CRPS patients have an increased risk of developing CRPS associated with polymorphisms in TNF-alpha and ACE genes Prevention vitamin C 500mg daily x 50 days in distal radius fractures treated conservatively 200mg daily x 50 days if impaired renal function vitamin C also has been shown to decrease the incidence of CRPS (type I) following foot and ankle surgery avoid tight dressings and prolonged immobilization Classification Lankford and Evans Stages of RSD Stage Onset Exam Imaging Acute 0-3 months Burning pain, redness, swelling, warmth, hyperhidrosis, hyperesthesia, cold intolerance, joint stiffness Normal x-rays, positive three-phase bone scan Subacute (dystrophic) 3-12 months Worsening pain, cyanosis, dry skin, stiffness, skin atrophy Subchondral osteopenia on x-ray Chronic (atrophic) > 12 months Diminished pain, glossy skin, fibrosis, joint contractures, loss of hair and nails Extreme osteopenia on x-ray International Association for the Study of Pain Classification International Association for the Study of Pain Classification Type I CRPS without demonstrable nerve damage Most common CRPS without demonstrable nerve damage Type II CRPS with evidence of identifiable nerve damage Minimal positive response with sympathetic blocks Presentation Cardinal signs exaggerated pain swelling stiffness skin discoloration Physical exam vasomotor disturbance trophic skin changes hyperhidrosis "flamingo gait" if the knee is involved equinovarus defomity if the ankle is involved Imaging Radiographs findings osteopenia affects the patella if the knee is involved soft tissue swelling subperiosteal bone resportion preservation of joint spaces Three-phase bone scan indications can help to rule out CRPS type I (has high negative predictive value) phases phase I (2 minutes) shows an extremity arteriogram phase II (5-10 minutes) shows cellulitis and synovial inflammation phase III (2-3 hours) shows bone images phase IV (24 hours) can differentiate osteomyelitis from adjacent cellulitis findings increased uptake in all phases phase III is most sensitive Thermography used to quantify temperature differences between the limbs questionable utility EMG/NCV may demonstrate slowing in known nerve distribution (e.g. slowing of median nerve conduction for CRPS type II in the forearm) Studies Diagnosis usually clinical but can be confirmed by pain relief with sympathetic block early diagnosis is critical for a successful outcome Budapest diagnostic criteria 1. Continuing pain that is disproportionate to any inciting event 2. Must report at least one symptom in three (clinical diagnostic criteria) or four (research diagnostic criteria) of the following categories: sensory: hyperesthesia or allodynia vasomotor: temperature asymmetry, skin colour changes, or skin colour asymmetry sudomotor/edema: edema, sweating changes, or sweating asymmetry motor/trophic: decreased range of motion, motor dysfunction (weakness, tremor, or dystonia), or trophic changes (hair, nails, or skin) 3. Must display at least one sign at time of diagnosis in two or more of the following categories: sensory: hyperalgesia (to pinprick) or allodynia (to light touch, deep somatic pressure, or joint movement) vasomotor: temperature asymmetry, skin colour changes or asymmetry sudomotor/edema: oedema, sweating changes, or sweating asymmetry motor/trophic: decreased range of motion, or motor dysfunction (weakness, tremor, or dystonia), or trophic changes (hair, nails, or skin) 4. No other diagnosis better explains the signs and symptoms Differential Soft tissue infection Malingering Psychiatric disease (e.g. Clenched Fist Syndrome) Neuropathic pain Chronic pain Raynaud disease Thoracic outlet syndrome Arterial insufficiency Erythromelalgia Treatment Nonoperative physical therapy and pharmacologic treatment indications first line of treatment nerve stimulation indications symptoms present mainly in the distribution of one major peripheral nerve nerve blockade indications failed initial nonoperative treatment chemical sympathectomy indications acts as another option when physical therapy and less aggressive nonoperative management fails Operative surgical sympathectomy indications failed nonoperative management (including chemical sympathectomy) surgical decompression indications CRPS type II with known nerve involvement (e.g. carpal tunnel release if median nerve involved) best success for CRPS is if you can find an associated nerve problem and treat it Techniques Physical therapy and pharmacologic treatment modalities gentle physiotherapy tactile discrimination training graded motor imagery sequential process consisting of laterality reconstruction, motor imagery, and mirror therapy medications NSAIDs alpha blockers (phenoxybenzamine, prazosin) beta blockers (propranolol) anti-depressants anti-convulsants calcium channel blockers GABA agonists (gabapentin) bisphosphonates anti-arrhythmics corticosteroids calcitonin Nerve stimulation programmable stimulators placed on affected nerves types transcutaneous electrical stimulation (TENS) peripheral nerve stimulation spinal cord stimulation Nerve blockade types sympathetic stellate ganglion (for upper extremity) lumbar spinal (for lower extremity) peripheral nerve neuraxial/epidural agents anesthetics (lidocaine or bupivicaine +/- epinephrine) sympatholytics (bretylium, guanethidine) Chemical sympathectomy types stellate ganglion (for upper extremity) lumbar spinal (for lower extremity) agents phenol alcohol Surgical sympathectomy ideal for patients who have had a response to sympathetic nerve blockade methods excision electrocautery Prognosis Typically responds poorly to conservative and surgical treatments Better prognosis if upper extremity, warm CRPS, children
QUESTIONS 1 of 17 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 Previous Next Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ18.96) A 40-year-old patient sustains the injury in Figures A and B six months ago and underwent the appropriate fixation method. The patient is continuing to experience a tremendous amount of pain in the entire right lower extremity, with sensitivity to cold temperatures. Physical exam demonstrates hyperesthesia of the extremity, thin and shiny appearing skin, cyanotic appearing with skin cool to the touch. What is the likely diagnosis and what is the best diagnostic test? QID: 212992 FIGURES: A B Type & Select Correct Answer 1 Undiagnosed compartment syndrome; compartment pressure checks 2% (34/1912) 2 Complex regional pain syndrome type 2; bone scan 7% (139/1912) 3 Popliteal artery aneurysm; angiogram 1% (23/1912) 4 Complex regional pain syndrome type 1; history and physical exam 79% (1508/1912) 5 Reflex sympathetic dystrophy; electromyography and nerve conduction velocities 10% (196/1912) L 2 Question Complexity A Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ13.176) Vitamin C has been shown to decrease the likelihood of which of the following complications following surgery on the foot and ankle in non-diabetic patients? QID: 4811 Type & Select Correct Answer 1 Nonunion 1% (47/4667) 2 Complex Regional Pain Syndrome, type II 15% (688/4667) 3 Malunion 0% (21/4667) 4 Complex Regional Pain Syndrome, type I 79% (3697/4667) 5 Wound infection 4% (190/4667) L 2 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic (SBQ12FA.96) A 67-year-old woman sustained the injury shown in Figure A approximately 14 months ago, which was managed with closed reduction and casting. She presents with new symptoms of hyperalgesia, allodynia, and hyperhidrosis of her wrist. She denies any recent fevers or chills. Her current radiographs reveal a well-healed fracture without any significant malunion. What is the pathophysiology likely attributable to her current symptoms? QID: 3903 FIGURES: A Type & Select Correct Answer 1 Pre-ganglion brachial plexopathy 4% (73/1773) 2 Aberrant inflammatory and vasomotor response 90% (1603/1773) 3 Exaggerated vasoconstriction of the wrist and digital arteries 3% (58/1773) 4 Incomplete glycosaminoglycan breakdown products causing dysfunction 2% (28/1773) 5 Connective tissue disorder 0% (6/1773) L 1 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ10.253) Level 1 evidence has shown vitamin C reduces the incidence of reflex sympathetic dystrophy (RSD) or complex regional pain syndrome type I (CRPS) in patients with which of the following? QID: 3356 Type & Select Correct Answer 1 Tarsal tunnel syndrome 0% (11/3363) 2 Distal radius fractures 91% (3059/3363) 3 Carpal tunnel syndrome 4% (131/3363) 4 Cervical radiculopathy from herniated nucleus pulposis 1% (45/3363) 5 Ankle fractures 3% (103/3363) L 1 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic (OBQ07.202) Which of the following modalities has been shown to have a positive effect when treating early stages of complex regional pain syndrome? QID: 863 Type & Select Correct Answer 1 Casting of the involved extremity 16% (333/2031) 2 Plyometric exercises 4% (82/2031) 3 Ultrasound therapy 4% (88/2031) 4 Acupuncture 4% (82/2031) 5 Gentle physiotherapy 71% (1438/2031) L 3 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic (OBQ06.29) A 52-year-old woman falls stepping off the escalator and sustains the wrist fracture shown in Figures A and B. Post-reduction radiographs demonstrate 20 degrees of residual dorsal angulation. The decision is made to proceed with open reduction internal fixation with a volar plate. Which of the following adjuvant interventions has studies in the literature to support an improvement in outcomes? QID: 140 FIGURES: A B Type & Select Correct Answer 1 Application of a bone stimulator within one week following surgery 3% (61/2050) 2 Supplemental percutaneous pin fixation that is removed 4 weeks following surgery 3% (70/2050) 3 Immobilization of the wrist in an extension splint or cast for 3 weeks following surgery 7% (144/2050) 4 Administration of oral vitamin C beginning the first day after surgery 82% (1684/2050) 5 Use of an axillary regional block during the surgery 4% (82/2050) L 1 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic (OBQ05.139) All of the following are clinical features of complex regional pain syndrome (reflex sympathetic dystrophy) of the lower extremity EXCEPT: QID: 1025 Type & Select Correct Answer 1 Swelling 1% (29/2724) 2 Cool and shiny skin 2% (58/2724) 3 Allodynia 1% (26/2724) 4 Crepitus 95% (2575/2724) 5 Hyperpathia 1% (26/2724) L 1 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic (OBQ05.230) A 58-year-old female complains of continued pain and swelling 6 months following total knee arthroplasty. She describes a burning pain that radiates from the knee down the anterior compartment of the leg. The pain arises sporadically and is associated with swelling, sweating, and a purplish hue of the leg. Knee radiographs are provided in Figures A and B. Aspiration is negative for infection. Which of the following is the best management? QID: 1116 FIGURES: A B Type & Select Correct Answer 1 Lumbar spine MRI to evaluate for radiculopathy of the L3 nerve root 3% (41/1187) 2 Alpha-adrenergic blockers, physical therapy, tactile discrimination training, and graded motor imagery 87% (1036/1187) 3 Surgical exploration of the knee 1% (16/1187) 4 Surgical debridement, pulsatile irrigation, tissue sampling for culture/biopsy, and polyethylene exchange 4% (50/1187) 5 Magnetic resonance arthrogram (MRA) with intra-articular contrast and diagnostic steroid injection 3% (38/1187) L 1 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic (OBQ05.269) A 38-year-old patient presents 6 months after intramedullary nailing of a distal third tibia fracture with symptoms consistent with complex regional pain syndrome. During the early stage of the disease he was treated with intermittent splinting, elevation and massage, contrast baths, and transcutaneous electrical nerve stimulation. Despite these modalities, he continues to have severe and debilitating symptoms. Which of the following treatment options is indicated as a second line of treatment? QID: 1155 Type & Select Correct Answer 1 Long leg cast immobilization for 3 months 2% (24/974) 2 Walking boot with non weight bearing for three months 5% (47/974) 3 Exchange nailing to stimulate healing response to the limb 4% (41/974) 4 Epidural spinal cord stimulator 18% (178/974) 5 Surgical sympathectomy of the affected limb 69% (674/974) L 3 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ04.191) What is the most common radiographic finding in reflex sympathetic dystrophy (RSD) or complex regional pain syndrome of the knee? QID: 1296 Type & Select Correct Answer 1 patella baja 2% (16/719) 2 patella alta 4% (29/719) 3 patella osteopenia 42% (303/719) 4 generalized osteopenia 51% (365/719) 5 supracondylar stress fracture 1% (4/719) L 4 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic
All Videos (0) Podcasts (1) Basic Science | Complex Regional Pain Syndrome Basic Science - Complex Regional Pain Syndrome (CRPS) Listen Now 21:4 min 10/18/2019 892 plays 5.0 (4)