Definition- Complex Regional Pain Syndrome (CRPS) is a chronic neuropathic pain condition that
usually develops after trauma or surgery, characterized by pain disproportionate to the initial injury,
along with sensory, motor, autonomic, and trophic disturbances.
Epidemiology
Incidence: ~5–26 per 100,000 annually.
More common in females (3–4:1).
Typically affects adults aged 30–60 years.
Most commonly involves the upper limb, especially after distal radius fractures or soft tissue
injuries.
Classification
CRPS is classified into two types:
Type I (Reflex Sympathetic Dystrophy): No confirmed nerve injury (90% of cases).
Type II (Causalgia): Identifiable peripheral nerve injury.
Clinical Presentation
CRPS has three overlapping phases (not strictly chronological):
1. Acute (Warm phase):
o Burning pain
o Swelling
o Redness
o Hyperesthesia
o Increased sweating
2. Dystrophic (Cold phase):
o Decreased temperature
o Cyanosis
o Skin dryness
o Progressive stiffness
3. Atrophic phase:
o Muscle wasting
o Joint contractures
o Brittle nails
o Osteopenia
Common features include:
Allodynia: Pain from non-painful stimuli
Hyperalgesia: Exaggerated response to painful stimuli
Autonomic symptoms: color/temp changes, edema
Motor: tremor, weakness, dystonia
Pathophysiology
Multifactorial and not fully understood. Key mechanisms:
Abnormal inflammatory response
Autonomic nervous system dysfunction
Central sensitization of the spinal cord
Peripheral nerve damage (especially in CRPS-II)
Sympathetic overactivity and dysregulation
Diagnosis
Clinical diagnosis; no single definitive test.
Diagnostic criteria: Budapest Criteria (gold standard):
o Continuing pain disproportionate to any inciting event
o At least 1 symptom in 3 of 4 categories (sensory, vasomotor, sudomotor/edema,
motor/trophic)
o At least 1 sign in 2 of 4 categories on examination
Diagnostic Tests
Used to support diagnosis or rule out other conditions:
Triple-phase bone scan (may show increased uptake)
X-rays: Spotty osteopenia (late)
MRI: Soft tissue changes and edema
Thermography: Detects temperature asymmetry
Quantitative sensory testing: Evaluates nerve function
Management of CRPS
1. Drug Therapy
NSAIDs: Pain and inflammation (early phase)
Corticosteroids: Acute phase; reduce inflammation
Anticonvulsants: Gabapentin, pregabalin for neuropathic pain
Tricyclic antidepressants: Amitriptyline
Bisphosphonates: Improve bone pain and osteopenia
Calcitonin: For pain relief
Opioids: Reserved for severe, unmanageable pain
2. Physical Therapy
Mainstay of treatment
Gentle, graded exercises to maintain range of motion
Desensitization techniques
Mirror therapy and functional rehabilitation
3. Psychological Therapy
Cognitive Behavioral Therapy (CBT)
Helps address anxiety, depression, and fear-avoidance behaviors
Important for long-term coping and compliance
4. Invasive Therapies
Sympathetic nerve blocks (stellate ganglion, lumbar sympathetic block)
Intrathecal drug delivery: For refractory pain
Spinal cord stimulation: Effective in chronic CRPS
IV ketamine infusions: For severe, refractory cases
5. Surgical Decompression
Indicated in CRPS II with confirmed nerve entrapment or compressive neuropathy
Nerve release or decompression surgeries
Outcomes are variable and should be reserved for select cases
CRPS is a debilitating, multifactorial condition with complex pathogenesis and variable progression.
Early diagnosis and a multidisciplinary management approach are crucial to prevent chronic
disability. Long-term outcomes depend on timely intervention, compliance, and psychosocial
support.