The phenomenon of complex regional pain syndrome type I (CRPS-I), once known as reflex sympathetic dystrophy (RSD), is not well understood. Clinicians are left feeling helpless and frustrated by the lack of effective treatment modalities when patients present with unexpected pain and disability. If this occurs after surgery, the event often strains the surgeon-patient relationship. Even patients who receive the most meticulous and well-executed surgical treatment are at some risk. For the most severe cases, when all else fails, some physicians consider amputation of the extremity to be an option.
CRPS-I is diagnosed on the basis of nonspecific, subjective observations; lacks a consensus reference standard; and may prove to be an illness construction1 rather than an actual disease (think "whiplash"). The International Association for the Study of Pain (IASP) changed the label from RSD to CRPS-I because it was believed that the implication of sympathetic nervous system dysfunction was unsubstantiated and that treatments to suppress the sympathetic nervous system were overused2. Although CRPS-I would seem to be an appropriately descriptive term, it still carries the implication that a pathophysiological process (one that is as yet undiscovered) is known to be responsible. It therefore seems preferable to use a more neutral description such as "disproportionate pain and disability," which acknowledges the possibility that this illness has important psychological and sociological aspects that may prove predominant.
In their review of uncontrolled retrospective studies, Bodde et al. documented "recurrence" in nearly half of the patients with CRPS-I treated with amputation (including phantom-limb symptoms in fifteen patients), prosthetic use by fewer than half of the patients who had been fitted with a prosthesis after amputation, and return to employment in fewer than one-third of the patients. They maintain that amputation remains a "valid" treatment for resistant infection but encourage seeking other options before amputation is performed because of pain or dysfunction. The authors remark that they see no reason to alter the current guidelines put forth by Perez et al.3, which represent the efforts of a multidisciplinary task force of the Dutch Society of Rehabilitation Specialists and the Netherlands Society of Anaesthesiologists that had been charged with the creation of evidence-based guidelines for CRPS-I treatment. These guidelines state, "There is insufficient evidence that amputation positively contributes to the treatment of CRPS-I."
It is not clear that amputation for CRPS-I achieves better outcomes than the natural history of the disease, other medical or surgical interventions, or cognitive behavioral therapy and similar treatments.
Going forward, it seems that further research efforts concerning CRPS-I should concentrate on the following: (1) a reliable, objective reference standard for diagnosis; (2) consideration of all aspects of illness-related behavior (pathophysiology, psychology, and sociology); (3) collaboration with the experts in each of these areas; and (4) scientific experiments that can adequately account for biases, the natural course of the illness, and the placebo effect.