Complex regional syndrome Flashcards
what are synonyms of crps?
sudeck’s dystrophy
reflex sympathetic dystrophy
what is crps?
this is term give to a group of vague painful conditions observed as a sequelae of trauma.
the trauma is sometimes relatively minor and hence symptoms & sign are out of proportion to the trauma
what are the type?
type 1: (90%) occurs when there is injury/illness with no direct evidence of nerve damage of the affected limb
(after surgery)
type 2:(10%) occurs when there is a distinct evidence of injury to a nerve
(after traumatic injuries)
C/f
Early stage: pain hyperaesthesia tenderness swelling skin becomes red shiny skin warm
As disease progressive (later stage)
atrophy of the skin
muscle atrophy
atrophy of nails
which part is more commonly affected?
wrist is most frequently affected after distal radial fractures
Prevalence
CRPS affects approximately 26 out of every 100,000 people. It is more common in females than males, with a ratio of 3.5:1.[4] CRPS can affect people of all ages, including children as young as three years old and adults as old as 75 years, but typically is most prevalent in the mid-thirties. CRPS Type I occurs in 5% of all traumatic injuries, with 91% of all CRPS cases occurring after surgery.[5]
Clinically Relevant Anatomy
CRPS can affect any part of the body, but occurs most often in the extremities. The wrist is most frequently affected after distal radial fractures.[6]
The central and peripheral nervous systems are connected through neural and chemical pathways, and can have direct control over the autonomic nervous system. It is for this reason that there can be changes in vasomotor and sudomotor responses without any impairment in the peripheral nervous system. Pain, heat, and swelling are usually not located at the site of injury and there may be no clear damage. Central sensitisation is seen as a main cause for developing CRPS.[7][8]
Etiology
Sprains and strains Surgeries Fractures Contusions Crush injuries Nerve lesions Stroke
Sometimes the provoking injury can occur spontaneously or can not be determined.[10][11][12][13][14][15] One study found that 56% of patients felt their CRPS was due to an ‘on the job’ injury, with the most common type of work-related injury occurring in service employments, such as in restaurants, bakeries, and police offices.[16]
location
The location of CRPS varies from person to person, often affecting the extremities, occurring slightly more in the lower extremities (+/- 60%) than in the upper extremities (+/- 40%). It can also appear unilaterally or bilaterally [17][18][19] and as mentioned above, occurs regularly in young adults and is more frequent in females than male
onset
The onset is mostly associated with a trauma, immobilisation, injections, or surgery, but there is no relation between the grade of severity of the initial injury and the following syndrome. A stressful life and other psychological factors may be potential risk factors that impact the severity of symptoms in CRPS
Type I
Definition
Formerly “Reflex Sympathetic Dystrophy (RSD) “
Occurs after a trauma remote from the affected extremity, with or without minor nerve damage
Etiology
Minor, soft tissue trauma (sprains, bruises and skin lesions)
Bone fracture or surgery
Frostbite or burns
Stroke
Myocardial infarction or lesion of the central nervous system
Immobilisation
Sensory disturbances
Allodynia and hyperalgesia
Hypoesthesia and hypoalgesia
Strange, disfigured, or dislocated feelings in limbs[4] 4]
Autonomic disorders + inflammatory symptoms
Swelling and edema [4][33]
Changes of sweating (especially hyperhidrosis)
Abnormal skin blood flow
Trophic changes Thick, brittle, or rigid nails Increased or decreased hair growth Fibrosis Thin, glossy, clammy skin[4] Osteoporosis (chronic stage)
Motor dysfunction Weakness of all muscles[4][33] Inability to move the extremity Stiffness Tremor Reduced range of motion Severe impairment of complex movements Atrophy
Pain (sympathetic nervous system)
Burning and spontaneous
Disproportionate in the intensity to the inciting event
Increase when the extremity is in a dependent position
Elicited by movement and pressure at the joints
Not in all cases (pain may not be present in 7% of CRPS sufferers)[4][33]
Deep, unpleasant, sensitive, surface, dull
Colour changes (redness or pale) Temperature changes[4]
Type II
Formerly “Causalgia”
Occurs after injury to a major peripheral nerve
Sensory disturbances
Allodynia and hyperalgesia
Hypersensitivity of the skin to light mechanical stimulation - some patients report intolerance to air moving over skin[4]
Autonomic disorders + inflammatory symptoms
Limb is cold and sweaty Distal extremity swelling Changes of sweating Abnormal skin blood flow Temperature changes
Trophic changes
Smoothness and mottling of the skin
Acute arthritis
Motor dysfunction
Inability to initiate movement of the extremity Stiffness[4] Tremor Dystonia Reduced range of motion
Pain (sympathetic nervous system)
Ongoing
Neuropathic
Spontaneous
Triggered by movement, loud noises, or strong emotions
Deep, unpleasant, sensitive, surface, dull
stage 1
Stage I: acute inflammation: denervation and sympathetic hypoactivity
Time Period
Begins 10 days post injury;
Lasts 3-6 months
Classic Signs and Symptoms
Pain: more severe than expected; burning or aching; increased with position, physical condition, or emotional disturbances
Hyperalgesia, allodynia, hyperpathia: lower pain threshold, increased sensitivity, all stimuli are perceived as painful, increased pain threshold then increased sensation intensity (faster and greater pain)
Oedema: soft and localised
Vasomotor/Thermal Changes: warmer
Skin: hyperthermia, dryness
Other: increased hair and nail growth
stage 2
Stage II: dystrophic: paradoxic sympathetic hyperactivity
Begins 3-6 months after onset of pain;
Lasts about 6 months
Pain: worsens, constant, burning, aching
Hyperalgesia, allodynia, hyperpathia: present
Oedema: hard, causes joint stiffness
Vasomotor/Thermal Changes: none
Skin: thin, glossy, cool due to vasoconstriction, sweaty
Other: thin and rigid nails, osteoporosis and subchondral bone erosion noted on x-rays
stage 3
Stage III: atrophic
Begins 6-12 months after onset of pain;
Lasts for years, or may resolve and reappear
Pain: spreads proximally and occasionally to entire body, may plateau
Oedema: hardening
Vasomotor/Thermal Changes: decreased SNS regulation, cooler
Skin: thin, shiny, cyanotic, dry
Other: fingertips and toes are atrophic, thick fascia, possible contractures, demineralisation and ankylosis seen on x-rays
Associated Co-morbidities
CRPS may also be associated with:
Arterial Insufficiency[36] Asthma[4] Bone Fractures[33] Cellulitis[36] Central Pain Syndromes[36] Conversion Disorder[36] Depression/Anxiety Factitious Disorder [36] Lymphedema[36] Malignancy[36] Migraines[4] Nerve Entrapment Syndromes[36] Osteomyelitis[36] Osteoporosis[4][33] Pain Disorder[36] Peripheral Neuropathies[36] Rheumatoid Arthritis[36] Scleroderma[36] Septic arthritis[36] Systemic Lupus Erythematosus[36] Tenosynovitis[36] Thrombophlebitis[36]
Differential Diagnosis
Bony or soft tissue injury Peripheral neuropathy, nerve lesions Arthritis Infection Compartment syndrome Arterial insufficiency Raynaud’s Disease Lymphatic or venous obstruction Thoracic Outlet Syndrome (TOS) Gardner-Diamond Syndrome Erythromelalgia Self-harm or malingering Cellulitis Undiagnostic fracture
Diagnostic Procedures
The Budapest Criteria
The Budapest criteria (also known as the IASP) has been developed for the diagnosis of CRPS, but improvements still need to be made.[39][40][41] Clinical diagnostic criteria for complex regional pain syndrome are[42][43][44][45][46][47]:
Constant pain, higher than the normally perceived pain
Minimum one symptom in three of the following four symptoms. Categories must be reported:
Vasomotor: temperature asymmetry and/or skin colour changes/asymmetry
Sensory: hyperalgesia and/or allodynia
Sudomotor/edema: changes in sweating
Motor/trophic: smaller range of motion, motor dysfunction and/or changes in hair, nails and skin
Additionally, the patient must also show signs of developing symptoms in at least two symptom categories
No other illness could explain the set of symptoms the patient is presenting with.
Other Tests
Infrared Thermography
Infrared thermography (IRT) is an effective mechanism to find significant asymmetry in temperature between both limbs by determining if the affected side of the body shows vasomotor differences in comparison to the other side. It is reported having a sensitivity of 93% and a specificity of 89%.[48] This test is hard to obtain so it is not often used for diagnosing of CRPS.[49][50][51]
Sweat Testing
Determining if the patient sweats abnormally. Q-sweat is an adequate instrument to measure sweat production. The sweat samples should be taken from both sides of the body at the same time.[52][53][54]
Radiographic Testing
Irregularities in the bone structure of the affected side of the body can become visible with the use of X-rays. If the X-ray shows no sign of osteoporosis, CRPS can be excluded if the patient is an adult.[55][56][57]
Three Phase Bone Scan
A triple phase bone scan is the best method to rule out Type I CPRS[58]. With the use of technetium Tc 99m-labeled bisophosphonates an increase in bone metabolism can be shown. Higher uptake of the substance means increased bone metabolism and the body part could be affected.[59][60] The triple-phase bone scan has the best sensitivity, NPV, and PPV compared to MRI and plain film radiographs.
Bone Densitometry
An affected limb often shows less bone mineral density and a change in the content of the bone mineral. During treatment of the CPRS the state of the bone mineral will improve. So this test can also be used to determine if the patient’s treatment is effective.[61]
Magnetic Resonance Imaging (MRI)
MRIs are useful to detect periarticular marrow edema, soft tissue swelling and joint effusions. And in a later stage atrophy and fibrosis of periarticular structures can be detected. But these symptoms are not exclusively signs of CRPS.[62][63][64]
Sympathetic Blocks
If the patient shows vasomotor or sudomotor dysfunction and severe pain, blocking the sympathetic nerves proves to be an effective technique to evaluate if the sympathetic nervous system is causing the pain to remain. This technique requires local anesthetic or ablation and is successful if at least 50% of the pain is reduced.[65]
Outcome Measures
Pain (VAS and McGill)
Skin temperature
Volume
ROM
[66][67][68][69] [70][71][72]
Grip Strength
Dynamometry
Full fist grip, pinch grip
[73][74] Foot function (Radboud skills test) Movements of the foot [75][76] Walking stairs Time, effort, need for assistance [77][78][79] Rising and sitting Getting in and out of car, bed, toilet [80][81] Trend (trauma related neuronal dysfunction) symptom inventory 164 items in 10 subscales (sensory, trophic, autonomic, motor, visceral symptoms) [82][83][84] Neuropathic pain questionnaire 12 items (see figure below) [85][86] Brush allodynia Brush evoked allodynia [87]
Examination
Examination
The diagnosis is based on the clinical presentation described above. Procedures start with taking a detailed medical history, taking into account any initiating trauma and any history of sensory, autonomic, and motor disturbances, as well as how the symptoms developed, the time frame, distribution and characteristics of pain.. Assessment for any swelling, sweating, trophic and/or temperature increases, and motor abnormality in the disturbed area is important. Skin temperature differences may be helpful for diagnosis of CRPS. The original International Association for the Study of Pain criteria required only a history and subjective symptoms for a diagnosis of CRPS, but recent consensus guidelines have argued for the inclusion of objective findings. [92]
The examination of the affected limb is from the neck downwards and should be carried out at rest, during activity, and during ambulation. [93] Sensory, motor and autonomic dysfunctions are investigated. [94][95][96]
Autonomic Dysfunction
The majority of patients with CRPS have bilateral differences in limb temperature and the skin temperature depends of the chronicity of the disease. In the acute stages, temperature increases are often concomitant with a white or reddish colouration of the skin and swelling. where the syndrome is chronic, the temperature will decrease and is associated with a bluish tint to the skin and atrophy. [97]
Motor Dysfunction
Studies have shown that approximately 70% of the patients with CRPS show muscle weakness in the affected limb, exaggerated tendon reflexes or tremor, irregular myoclonic jerks, and dystonic muscle contractions. Muscle dysfunction often coincides with a loss of range of motion in the distal joints. [98]
Sensory Dysfunction
The distal ends of the extremities require attention when examining a patient with CRPS. However, common findings of regional neuropathic and motor dysfunction have shown us that it is important to broaden the examination both proximally and contralaterally.[99]
Light touch, pinprick, temperature and vibration sensation should be assessed for a complete picture of the CRPS.[100] Most assessments are interlinked, for example, when vibration sensation is highly positive, light touch should also be positive.[101]
To help distinguish the findings of a sensory dysfunction, bilateral comparisons are made. The results should be clear and reliable.[102][103]
Physical Therapy Management
TENS[133] Aquatic therapy allows activities to be performed with decreased weight bearing on the lower extremities. Mirror therapy Desensitisation [4] Gradual weight bearing [4] Stretching [4] Fine motor control [4
Acute Phase
Immobilisation and contralateral therapy. Intensive active therapy in the acute phase can lead to deterioration.[136]
Chronic Phase
Passive physical therapy including manipulation, manual therapy[137], massage [138][139] and mobilisations. Lymphatic drainage can be used to facilitate regression of oedema.[140] Tender areas are recommended to be treated in the following order: more severe before less severe, more proximal and medial before more distally and laterally located points and the area of greatest accumulation of tender areas is treated first. When tender areas are located in a row, the middle is treated first.[141]
Therapeutic exercise includes isometric strengthening therapy followed by active isotonic training in combination with sensory desensitisation programmes.[142][143] Strength training includes exercises for all four extremities and the trunk.[144] Desensitisation programmes consist of giving stimuli of different fabrics, different pressures (light or deep), vibration, tapping, heat or cold. The exercises can be stress-loading (i.e. walking, carrying weights), endurance training and functional training.[145] When CRPS occurs in the lower extremities, gait re-training is recommended.[146]
Mirror therapy or mirror visual feedback [147]
Mirror therapy is where both hands are placed into a box with a mirror separating the two compartments and, whilst moving both hands, the patient watches the reflection of the unaffected hand in the mirror [148]
There is some evidence to suggest that mirror therapy has the effect of:
Reducing pain intensity and improving function in post-stroke CRPS [149][150][151] A significant improvement in pain [152] Improving function (low-quality evidence, however) [153] Graded motor imagery/learning is effective. [154][155][156][157]However, further trials are required [158] [159][160] GMI plus medical management is more effective than medical management plus physiotherapy [161] GMI may reduce pain and improve function. [162] Other therapies include relaxation, [163][164] cognitive-behavioral therapy, [165] deep breathing exercises and biofeedback.
other treatment
Other Treatments
Whirlpool bath/ contrast baths[175]
Vocational and recreational rehabilitation[176]
Psychological therapies: cognitive-behavioural therapy (CBT), operant conditioning (OC), counselling, pain education and relaxation techniques[177][178][179]
Acupuncture, electroacupuncture[180]
Tactile sensory discrimination training[181][182]
Weight bearing[183]
Ultrasound therapy
Kinesio taping[184]
