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INTRODUCTION

AS THE NAME INFERS, COMPLEX REGIONAL PAIN syndrome (CRPS) is a multifarious, painful condition that most commonly affects an extremity following a noxious event. Management of this complicated disorder is typically challenging, as the pathogenesis and natural history of CRPS are incompletely characterized, and a multifaceted biopsychosocial process is invariably involved. Subsequently, an interdisciplinary treatment approach among qualified specialists is mandatory to thoroughly address all aspects of this disease. A coordinated approach targeting biomedical and psychosocial rehabilitation has been shown to augment outcomes. This chapter will review the diagnostic criteria and pathogenesis of CRPS and focus on the multimodal, evidence-based treatments shown to optimize functional restoration.

DEFINITION

CRPS is a chronic and often disabling neuropathic pain syndrome that presents with classic neuropathic pain symptoms in conjunction with a combination of vasomotor effects, including skin color and temperature changes, tissue edema, sensory allodynia or hyperalgesia, decreased range of motion, and motor dysfunction. CRPS manifests itself in two primary forms with the vastly more common subtype CRPS type 1 (formerly known as reflex sympathetic dystrophy) diagnosed without any obvious or known neuronal injury. The much less common CRPS type 2 (formerly known as causalgia) is associated with an identified nerve lesion. CRPS is usually preceded by trauma, with the most common culprits being fractures, sprains, crush injuries, and surgery. Insults including stroke, myocardial infarction, injections, and spinal cord injuries are much less commonly the cause. Occasionally in CRPS type 1, the patient is unable to identify the initiating trauma. CRPS type 2 is usually caused by nerve injury during a procedure such as during an injection or neuronal transection.1,2 Ultimately the signs and symptoms of both CRPS subtypes are nearly indistinguishable, and there is no evidence that they differ in pathophysiology and even treatment.

EPIDEMIOLOGY

Determination of the true incidence of CRPS has been hindered by the absence of definitive diagnostic tests. Two retrospective studies using the 1994 International Association for the Study of Pain (IASP) criteria have demonstrated an incidence of 5 per 100,000 person-years for CRPS type I and 0.8 per 100,000 person-years for CRPS type II. However, the more recently established Budapest criteria have been shown to decrease the diagnostic incidence by 50%. Additionally, the National Order for Rare Disorders has distinguished CRPS as a “rare” disease, which reflects less than 200,000 cases in the United States. Population-based studies do demonstrate a higher preponderance in females with a 4:1 ratio compared to men and a peak incidence at 50 to 70 years of age. There appears to be an increased propensity for involvement of the upper limbs with a ratio of 3:2 compared to the lower limbs in adults. Risk factors for this condition include individuals with a history of migraine, osteoporosis, asthma, and angiotensin-converting enzyme (ACE) inhibitor therapy and individuals with an elevated intracast pressure or extreme extremity positions. Pediatric population reviews show ...

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