Complex Regional Pain Syndromes (CRPS) – Stages

Clinical Stages

In an attempt to define treatment rationale, Drucker et al. in 1959 divided their observations on the natural history of causalgia into three clinical stages. Numerous authors have subsequently tried to define the natural progression of this disease, no one more scientific than the other. All agree, however, that there is a progressive nature to the phenomenon, and all arbitrarily divide it into three stages which are partially based on TIME, and partially based on SYMPTOM SEVERITY.

Stage I: Acute/Traumatic (0-3 months)
�In stage I, pain may start immediately or not until several weeks after injury. There may be an increase in dermal blood flow, hyperthermia, edema, increased growth of hair, increased longitudinal nail growth, hyperhidrosis, and wasting of the muscles with the onset of pain. The pain in the extremity is burning or aching in character and is constant. It is made worse by movement, emotion and auditory and visual stimuli. It is out of all proportion to the severity of the antecedent injury. The pain is usually lessened in a quiet environment, by sleep, and by narcosis. There may be no radiographic changes in the first or acute stage until four to eight weeks following the onset of pain.�

Stage II: Dystrophic (3-12 months)
�Stage II develops in about three months. In this dystrophic stage, the skin is cool, pale, gray, and frequently cyanotic. The edema changes from a soft to a brawny type with glazed overlying skin. The hair becomes scant. The nails become brittle and crack and have heavy grooves. The pain persists and motion is very restricted. X-ray films reveal diffuse rarefaction of bone, especially in the epiphyseal region.�

Stage III: Atrophic (>12 months)
�Stage III, or the atrophic stage, is generally considered irreversible with extensive structural changes. There are decreased dermal blood flow, atrophic skin, decreased fat pads in the fingers, and pointed fingertips. The growth of hair and nails and formation of sweat may again be normal. Bones may show marked decalcification with fixed articular rigidity. In this stage the pain has frequently spread proximally in the limb and has become intractable.�

Cardinal versus Secondary Symptoms

The literature often refers to �cardinal symptoms� and �secondary symptoms� in the clinical complex. The cardinal symptoms include: severe pain, swelling, stiffness, and discoloration. The secondary symptoms include: osteoporosis, sudomotor changes, temperature changes, trophic changes, vasomotor instability, and palmar fibrosis. Numerous authors indicate that the diagnosis is made on the presence of the four cardinal symptoms; the secondary symptoms are not always present. This system, of course, does not correspond to the 1994 Classification of Chronic Pain description of the definition of Complex Regional Pain Syndrome Type I. Nonetheless, the �cardinal� and �secondary� symptoms conceptually helps the physician when faced with the patient with a painful extremity, and this discrepancy in the literature again punctuates the void in our understanding of this disorder.

DIAGNOSIS

The first priority is to rule out other conditions that could produce a painful, swollen, stiff hand. These include: Dupuytren�s disease, hypertrophic arthritis, psoriatic arthritis, carpal tunnel syndrome, stenosing tenosynovitis.

The diagnostic criteria outlined in by Merskey and Bogduk are listed earlier. Again, the system of �cardinal� and �secondary� symptoms is used commonly for making the diagnosis clinically.

Diagnostic Studies

X-rays
Variable demineralization, most marked in the subchondral regions, with epiphyseal regions more affected than metaphysis.

Three Phase Technetium Bone scans
Controversial. No good studies that have been adequately controlled. Numerous conflicting reports in the literature. Questionable value beyond one�s own clinical suspicion, and if x-rays already show demineralization (indicative of increased blood flow).